Efficacy of splenectomy in patients with non-hodgkin's lymphomas associated with autoimmune hemolytic anemia and Evans — Fisher syndrome
Objective: to determine indications for surgical technique and outcomes of splenectomy in non-Hodgkin’s lymphomas (NHL) associated with autoimmune hemolytic anemia (AIHA) or Evans — Fisher syndrome (EFS) based on our 20-year experience. Subject and methods: ten splenectomies in NHL associated with...
Збережено в:
| Дата: | 2017 |
|---|---|
| Автори: | , , , , , |
| Формат: | Стаття |
| Мова: | English |
| Опубліковано: |
Інститут експериментальної патології, онкології і радіобіології ім. Р.Є. Кавецького НАН України
2017
|
| Назва видання: | Онкологія |
| Теми: | |
| Онлайн доступ: | http://dspace.nbuv.gov.ua/handle/123456789/145300 |
| Теги: |
Додати тег
Немає тегів, Будьте першим, хто поставить тег для цього запису!
|
| Назва журналу: | Digital Library of Periodicals of National Academy of Sciences of Ukraine |
| Цитувати: | Efficacy of splenectomy in patients with non-hodgkin's lymphomas associated with autoimmune hemolytic anemia and evans — fisher syndrome / Y.L. Yevstakhevych, Y.I. Vyhovska, I.Y. Yevstakhevych, M.M. Semerak, H.B. Lebed, V.E. Loginsky // Онкологія. — 2017. — Т. 19, № 2. — С. 103-109. — Бібліогр.: 23 назв. — англ. |
Репозитарії
Digital Library of Periodicals of National Academy of Sciences of Ukraine| id |
irk-123456789-145300 |
|---|---|
| record_format |
dspace |
| spelling |
irk-123456789-1453002019-01-21T01:24:20Z Efficacy of splenectomy in patients with non-hodgkin's lymphomas associated with autoimmune hemolytic anemia and Evans — Fisher syndrome Yevstakhevych, Y.L. Vyhovska, Y.I. Yevstakhevych, I.Y. Semerak, M.M. Lebed, H.B. Loginsky, V.E. Оригинальные исследования Objective: to determine indications for surgical technique and outcomes of splenectomy in non-Hodgkin’s lymphomas (NHL) associated with autoimmune hemolytic anemia (AIHA) or Evans — Fisher syndrome (EFS) based on our 20-year experience. Subject and methods: ten splenectomies in NHL associated with AIHA (group I) and 18 splenectomies in NHL associated with AIHA and immune thrombocytopenia — EFS (group II) have been performed. Outcomes were determined both post-operatively and over a long-term follow up observation during 3–201 months (median 68 months) and 1–290 months (median 195.5 months) in group I and group II, respectively. Results: splenectomy in NHL is indicated in those immune cytopenias refractory to steroids and cytotoxic therapy associated with massive splenomegaly, regional portal hypertension and hypersplenism. The surgical technique of splenectomy in large spleen is quite complicated and depends on both the spleen size and presence of adhesions. Postoperative mortality (progression of lymphoma, bleeding) reaches 7%. A favorable immediate outcome of surgery was observed in 93% of patients. Median value of postoperative event-free survival in NHL with AIHA reached 29.0 months (12.0–49.0 months), and median value of overall survival estimated 31.0 months (18.1–52.2 months), with a 3-year survival observed in 45% of patients and a 5-year survival in 22% of patients. Median event-free survival in patients with NHL and EFS reached 22 months (5.0–52.0 months), while median overall survival being 50 months (12.0–97.0 months), with a 3-year survival observed in as much as 62% of patients and a 5-year survival in 31% of patients. The lowest post-spenectomy life expectancy was stated in patients with aggressive lymphomas associated with immune cytopenias (diffuse large B-cell lymphoma, T-cell types). Conclusions: splenectomy has proved both an effective and quite safe treatment option and diagnostic tool in NHL complicated by immune cytopenias, particularly those associated with massive splenomegaly, regional portal hypertension and hypersplenism. Long-term results of splenectomy are mainly determined by a variant of NHL. Мета: визначити показання, хірургічну техніку і наслідки спленектомії при неходжкінських лімфомах (НХЛ), ускладнених аутоімунною гемолітичною анемією (АІГА) або синдромом Еванса — Фішера (СЕФ), на основі двадцятирічного досвіду виконання таких операцій. Об’єкт і методи: проведено 10 спленектомій при НХЛ, ускладненій АІГА (I група), та 18 спленектомій — при НХЛ, ускладненій АІГА та імунною тромбоцитопенією — СЕФ (II група). Результати оцінювали безпосередньо після операції та при тривалому спостереженні протягом 3–201 міс (медіана 68 міс) у І групі та 1–290 міс (медіана 195,5 міс) — у ІІ групі. Результати: показання до спленектомії при НХЛ — наявність імунних цитопеній, резистентних до кортикостероїдів і цитостатичної терапії, які супроводжуються масивною спленомегалією, регіонарною портальною гіпертензією та гіперспленізмом. Операція спленектомії при масивній селезінці технічно досить складна, залежить від розміру органа та наявності прилягання. Післяопераційна смертність (прогресування лімфоми, кровотеча) становить 7%. У 93% хворих спостерігали позитивний безпосередній результат хірургічного втручання. ПриНХЛ, ускладненій АІГА, медіана безподійної виживаності після операції становила 29 міс (12–49 міс), а загальної виживаності — 31,0 міс (18,1–52,2 міс) з 3-річною виживаністю 45% пацієнтів і 5-річною — 22%. У хворих на НХЛ ізСЕФ медіана безподійної виживаності сягала 22міс (5–52міс), амедіана загальної виживаності— 50міс (12–97міс), причому 3-річну виживаність відмічено у 62% хворих, а 5-річну— у 31%. Найменша тривалість життя після спленектомії зафіксована у хворих на агресивні лімфоми, ускладнені імунними цитопеніями (B-великоклітинна дифузна лімфома, T-клітинні лімфоми). Висновки: спленектомія залишається ефективним і доволі безпечним діагностично-лікувальним методом при НХЛ, ускладнених імунними цитопеніями, особливо занаявності масивної спленомегалії, регіонарної портальної гіпертензії та гіперспленізму. Віддалені результати спленектомії значною мірою залежать від варіанта НХЛ. 2017 Article Efficacy of splenectomy in patients with non-hodgkin's lymphomas associated with autoimmune hemolytic anemia and evans — fisher syndrome / Y.L. Yevstakhevych, Y.I. Vyhovska, I.Y. Yevstakhevych, M.M. Semerak, H.B. Lebed, V.E. Loginsky // Онкологія. — 2017. — Т. 19, № 2. — С. 103-109. — Бібліогр.: 23 назв. — англ. 1562-1774 http://dspace.nbuv.gov.ua/handle/123456789/145300 en Онкологія Інститут експериментальної патології, онкології і радіобіології ім. Р.Є. Кавецького НАН України |
| institution |
Digital Library of Periodicals of National Academy of Sciences of Ukraine |
| collection |
DSpace DC |
| language |
English |
| topic |
Оригинальные исследования Оригинальные исследования |
| spellingShingle |
Оригинальные исследования Оригинальные исследования Yevstakhevych, Y.L. Vyhovska, Y.I. Yevstakhevych, I.Y. Semerak, M.M. Lebed, H.B. Loginsky, V.E. Efficacy of splenectomy in patients with non-hodgkin's lymphomas associated with autoimmune hemolytic anemia and Evans — Fisher syndrome Онкологія |
| description |
Objective: to determine indications for surgical technique and outcomes of splenectomy in non-Hodgkin’s lymphomas (NHL) associated with autoimmune hemolytic anemia (AIHA) or Evans — Fisher syndrome (EFS) based on our 20-year
experience. Subject and methods: ten splenectomies in NHL associated with
AIHA (group I) and 18 splenectomies in NHL associated with AIHA and immune
thrombocytopenia — EFS (group II) have been performed. Outcomes were determined both post-operatively and over a long-term follow up observation during
3–201 months (median 68 months) and 1–290 months (median 195.5 months)
in group I and group II, respectively. Results: splenectomy in NHL is indicated
in those immune cytopenias refractory to steroids and cytotoxic therapy associated with massive splenomegaly, regional portal hypertension and hypersplenism.
The surgical technique of splenectomy in large spleen is quite complicated and depends on both the spleen size and presence of adhesions. Postoperative mortality (progression of lymphoma, bleeding) reaches 7%. A favorable immediate outcome of surgery was observed in 93% of patients. Median value of postoperative
event-free survival in NHL with AIHA reached 29.0 months (12.0–49.0 months),
and median value of overall survival estimated 31.0 months (18.1–52.2 months),
with a 3-year survival observed in 45% of patients and a 5-year survival in 22%
of patients. Median event-free survival in patients with NHL and EFS reached
22 months (5.0–52.0 months), while median overall survival being 50 months
(12.0–97.0 months), with a 3-year survival observed in as much as 62% of patients and a 5-year survival in 31% of patients. The lowest post-spenectomy life
expectancy was stated in patients with aggressive lymphomas associated with immune cytopenias (diffuse large B-cell lymphoma, T-cell types). Conclusions: splenectomy has proved both an effective and quite safe treatment option and diagnostic tool in NHL complicated by immune cytopenias, particularly those associated with massive splenomegaly, regional portal hypertension and hypersplenism.
Long-term results of splenectomy are mainly determined by a variant of NHL. |
| format |
Article |
| author |
Yevstakhevych, Y.L. Vyhovska, Y.I. Yevstakhevych, I.Y. Semerak, M.M. Lebed, H.B. Loginsky, V.E. |
| author_facet |
Yevstakhevych, Y.L. Vyhovska, Y.I. Yevstakhevych, I.Y. Semerak, M.M. Lebed, H.B. Loginsky, V.E. |
| author_sort |
Yevstakhevych, Y.L. |
| title |
Efficacy of splenectomy in patients with non-hodgkin's lymphomas associated with autoimmune hemolytic anemia and Evans — Fisher syndrome |
| title_short |
Efficacy of splenectomy in patients with non-hodgkin's lymphomas associated with autoimmune hemolytic anemia and Evans — Fisher syndrome |
| title_full |
Efficacy of splenectomy in patients with non-hodgkin's lymphomas associated with autoimmune hemolytic anemia and Evans — Fisher syndrome |
| title_fullStr |
Efficacy of splenectomy in patients with non-hodgkin's lymphomas associated with autoimmune hemolytic anemia and Evans — Fisher syndrome |
| title_full_unstemmed |
Efficacy of splenectomy in patients with non-hodgkin's lymphomas associated with autoimmune hemolytic anemia and Evans — Fisher syndrome |
| title_sort |
efficacy of splenectomy in patients with non-hodgkin's lymphomas associated with autoimmune hemolytic anemia and evans — fisher syndrome |
| publisher |
Інститут експериментальної патології, онкології і радіобіології ім. Р.Є. Кавецького НАН України |
| publishDate |
2017 |
| topic_facet |
Оригинальные исследования |
| url |
http://dspace.nbuv.gov.ua/handle/123456789/145300 |
| citation_txt |
Efficacy of splenectomy in patients with non-hodgkin's lymphomas associated with autoimmune hemolytic anemia and evans — fisher syndrome / Y.L. Yevstakhevych, Y.I. Vyhovska, I.Y. Yevstakhevych, M.M. Semerak, H.B. Lebed, V.E. Loginsky // Онкологія. — 2017. — Т. 19, № 2. — С. 103-109. — Бібліогр.: 23 назв. — англ. |
| series |
Онкологія |
| work_keys_str_mv |
AT yevstakhevychyl efficacyofsplenectomyinpatientswithnonhodgkinslymphomasassociatedwithautoimmunehemolyticanemiaandevansfishersyndrome AT vyhovskayi efficacyofsplenectomyinpatientswithnonhodgkinslymphomasassociatedwithautoimmunehemolyticanemiaandevansfishersyndrome AT yevstakhevychiy efficacyofsplenectomyinpatientswithnonhodgkinslymphomasassociatedwithautoimmunehemolyticanemiaandevansfishersyndrome AT semerakmm efficacyofsplenectomyinpatientswithnonhodgkinslymphomasassociatedwithautoimmunehemolyticanemiaandevansfishersyndrome AT lebedhb efficacyofsplenectomyinpatientswithnonhodgkinslymphomasassociatedwithautoimmunehemolyticanemiaandevansfishersyndrome AT loginskyve efficacyofsplenectomyinpatientswithnonhodgkinslymphomasassociatedwithautoimmunehemolyticanemiaandevansfishersyndrome |
| first_indexed |
2025-07-10T21:21:54Z |
| last_indexed |
2025-07-10T21:21:54Z |
| _version_ |
1837296558359445504 |
| fulltext |
ОРИГИНАЛЬНЫЕ ИССЛЕДОВАНИЯ
103ОНКОЛОГИЯ • Т. 19 • № 2 • 2017 103
INTRODUCTION
Non-Hodgkin’s lymphomas (NHL) as other lym-
phoproliferative disorders (LPD) are accompanied with
a high frequency of autoimmune processes among which
the autoimmune cytopenias prevail [1, 2]. In particu-
lar, the autoimmune hemolytic anemia (AIHA), mostly
due to warm antibodies (positive direct Coombs’ test),
accounts for 2.6–3.0% of all cases of NHL [3, 4]. It is
frequently combined with an immune thrombocytope-
nia (ITP) and immune leukopenia, the immune char-
acteristics of which are more difficult to confirm, since,
thrombocytopenia and leukopenia may be a manifes-
tation of hypersplenism in massive splenomegaly [5].
The pathogenesis of autoimmune processes in lymphoid
proliferations remains a matter of considerable dispute
and controversy, especially as a high frequency of LPD
is also observed in AIHA [6]. It is believed that increased
tendency to autoimmune antibodies formation in LPD
is caused by rearrangements of the immunoglobulin V-
region genes or T-cell receptor in the malignant B-/T-
cells. It triggers the generation of autoreactive lympho-
cyte clones. Failure to eliminate these clones or the break-
down in the mechanisms that prevent their expansion
may lead to the occurrence of autoimmune processes [4].
NHL with autoimmune cytopenias requires speci fic
treatment approach that is caused by a whole range of
conditions. It has been proved that the presence of AIHA
in patients with NHL is an adverse prognostic sign [4].
Autoimmune process of such patients shows a poor re-
sponse to corticosteroids and intravenous immunoglo-
bulin, and slightly better response to cytostatic therapy
of lymphoma [1, 3]. Finally, the massive splenomegaly
with all possible consequences (abdominal discomfort,
regional portal hypertension (RPH), hypersplenism) is
EFFICACY OF SPLENECTOMY
IN PATIENTS WITH
NON-HODGKIN’S LYMPHOMAS
ASSOCIATED WITH AUTOIMMUNE
HEMOLYTIC ANEMIA
AND EVANS — FISHER SYNDROME
Objective: to determine indications for surgical technique and outcomes of splen
ectomy in nonHodgkin’s lymphomas (NHL) associated with autoimmune hemo
lytic anemia (AIHA) or Evans — Fisher syndrome (EFS) based on our 20year
experience. Subject and methods: ten splenectomies in NHL associated with
AIHA (group I) and 18 splenectomies in NHL associated with AIHA and immune
thrombocytopenia — EFS (group II) have been performed. Outcomes were de
termined both postoperatively and over a longterm follow up observation during
3–201 months (median 68 months) and 1–290 months (median 195.5 months)
in group I and group II, respectively. Results: splenectomy in NHL is indicated
in those immune cytopenias refractory to steroids and cytotoxic therapy associa
ted with massive splenomegaly, regional portal hypertension and hypersplenism.
The surgical technique of splenectomy in large spleen is quite complicated and de
pends on both the spleen size and presence of adhesions. Postoperative mortali
ty (progression of lymphoma, bleeding) reaches 7%. A favorable immediate out
come of surgery was observed in 93% of patients. Median value of postoperative
eventfree survival in NHL with AIHA reached 29.0 months (12.0–49.0 months),
and median value of overall survival estimated 31.0 months (18.1–52.2 months),
with a 3year survival observed in 45% of patients and a 5year survival in 22%
of patients. Median eventfree survival in patients with NHL and EFS reached
22 months (5.0–52.0 months), while median overall survival being 50 months
(12.0–97.0 months), with a 3year survival observed in as much as 62% of pa
tients and a 5year survival in 31% of patients. The lowest postspenectomy life
expectancy was stated in patients with aggressive lymphomas associated with im
mune cytopenias (diffuse large Bcell lymphoma, Tcell types). Conclusions: splen
ectomy has proved both an effective and quite safe treatment option and diagnos
tic tool in NHL complicated by immune cytopenias, particularly those associat
ed with massive splenomegaly, regional portal hypertension and hypersplenism.
Longterm results of splenectomy are mainly determined by a variant of NHL.
Y.L. Yevstakhevych
Y.I. Vyhovska
I.Y. Yevstakhevych
M.M. Semerak
H.B. Lebed
V.E. Loginsky
State Institution «Institute
of Blood Pathology
and Transfusion Medicine»,
Lviv, Ukraine
Key Words: nonHodgkin’s
lymphoma, splenectomy,
autoimmune hemolytic anemia,
immune thrombocytopenia.
ОНКОЛОГИЯ • Т. 19 • № 2 • 2017
ОРИГИНАЛЬНЫЕ ИССЛЕДОВАНИЯ
104
often observed in these NHL [5, 7, 8]. In such cases, the
need for splenectomy arises having positive outcomes as
well as some risk for a patient [7, 9].
The aim of this study was to specify the indications
for surgical equipment and consequences of splenecto-
my in NHL associated with AIHA or Evans — Fisher
syndrome (EFS) as based on twenty years’ experience
of performance of the surgery in our clinic.
MATERIALS AND METHODS
During the period of 1986–2015 at the Department of
Surgery and Clinical Transfusion Medicine of the State
Institution «Institute of Blood Pathology and Transfu-
sion Medicine of National Academy of Medical Scien-
ces of Ukraine» (Lviv) and at the premises of the Depart-
ment of Surgery of Lviv City Communal Clinical Hos-
pital No. 5 among 107 splenectomies in patients with
NHL, 10 splenectomies were performed in patients with
NHL and AIHA (group 1) and 18 splenectomies were
performed in patients with NHL associated with AIHA
and ITP — EFS (group 2).
The diagnostics of NHL and its variant was per-
formed as based on the results of the clinical and instru-
mental examinations (Table 1), cytological examination
of periphe ral blood, bone marrow, aspirates and imprints
of the affected organs, histological examination of biop-
sy specimens of the removed lymph nodes and/or spleen,
immunophenotypic features of malignant cells of the in-
vestigated tissues in accordance with current classifica-
tions (WF, REAL with further specification pursuant to
WHO classification) [10] (Table 2). The extending (stage)
of the disease was estimated on grounds of clinical, X-ray,
ultrasonographic examinations and computed tomogra-
phy according to Ann Arbor staging system [11]. 15 pa-
tients were diagnosed with III-E or III-Es stage disease,
and 13 patients were diagnosed with IV stage disease.
Splenectomy was performed under intubation anesthe-
sia via the upper middle laparotomy. In patients with mas-
sive splenomegaly (spleen weight > 3 kg), marked perisple-
nitis and vascular anastomoses among organs resulting from
RPH the laparotomy incision was exten ded towards pubic
articulation or supplemented by a lateral incision on the left
2 cm above the umbilicus. The classical technique for sple-
nectomy was applied. The enlarged spleen removal was start-
ed with mobilization of the inferior pole that technically al-
lowed for a control of the splenic pedicle vessels, gradual
visualization of splenic ligaments and junctions, and its dis-
section while systema tically attaining hemostasis by virtue
of vascular ligation or electrocoagulation. After releasing the
spleen from liga ments and junctions and its exterio rization,
the vascular pedicle and other vessels were ligated. Since 2002
all patients were immunized with vaccine Pneumo-24 and
recently with Prevenar vaccine before splenectomy in order
to prevent post-splenectomy infection.
The results of splenectomy were assessed in the imme-
diate post-splenectomy period (on the fourteenth- sixteenth
day) pursuant to criteria described in the referen ces [12] and
during follow-up control. The follow-up period after the
surgery ranged between 3 and 201 months (median time
68 months) for group 1, and between 1 and 290 months (me-
dian time 195.5 months) for group 2.
The results were analyzed by means of descriptive statis-
tical methods. The event-free survival (event — recurrence of
immune cytopenia or when a patient died of lymphoma) and
overall patients’ survival were determined by the Kaplan —
Meier method and described as a median survival (lower-
Table 1
Clinical and hematologic characteristics and results of splenectomy
in patients with NHL associated with immune cytopenias
Criteria NHL + AIHA NHL + AIHA +
ITP
Number of patients 10 18
Sex: male/female 6/4 12/6
Age > 60 years 2 5
Disease duration before splenectomy
(months) 1–48 2–132
General condition according to ECOG ≥ 2 1 2
Clinical signs
Peripheral lymph nodes 4 5
Mediastinal lymph nodes 2 2
Intra-abdominal lymph nodes 10 14
Splenomegaly 10 18
Hepatomegaly 5 13
Bone marrow involvement ≥ 30%
of lymphoid cells
3 9
Stage of disease:
ІІІ
ІV
3
7
12
6
Indices of peripheral blood
Anemia < 100 g/l 8 10
Thrombocytopenia < 100•109/l − 18
Leukocytosis > 10•109/l 3 7
Lymphocytosis 5–10•109/l − 2
Lymphocytosis ≥ 10•109/l 2 6
Leukopenia ≤ 3.5•109/l 4 8
Reticulocytes > 20‰ 10 13
Erythrocyte sedimentation rate > 20 mm/h 7 14
М-gradient 1 2
Therapy before splenectomy 6 9
Intraoperative indices
Massive splenomegaly
(weight > 1500 g) 10 12
Perisplenitis 1 6
Regional portal hypertension 6 12
Ascite 3 3
Bleeding during surgery 3 10
Blood loss > 500 ml 3 6
Response to splenectomy
Remission > 12 months
Remission 2–12 months
Absence of remission (progression)
4
6
−
5
11
2
No need for treatment after splenectomy
Received treatment after splenectomy
9
1
12
6
Table 2
Morphological variants of NHL with immune cytopenias
Types of NHLs
NHL + AIHA
(number
of patients)
NHL + AIHA +
ITP (number
of patients)
Splenic lymphoma with villous lympho-
cytes (SLVL) − 5
Splenic marginal zone lymphoma (SMZL) 2 3
Nodal marginal zone lymphoma (NMZL) 1 –
Follicular lymphoma − 1
Mantle cell lymphoma (MCL) – 3
Diffuse large B-cell lymphoma (DLBCL) 6 5
В-cell NHL, unclassified 1 –
T-cell NHL, unclassified – 1
ОРИГИНАЛЬНЫЕ ИССЛЕДОВАНИЯ
105ОНКОЛОГИЯ • Т. 19 • № 2 • 2017 105
upper quartiles) and 3-year and 5-year survival. The surviv-
al curves were compared using the log-rank test.
RESULTS AND DISCUSSION
Splenectomy was performed in 10 patients with NHL
associated with warm-antibody AIHA. Among them there
were 6 men and 4 women 25–68 years old (median age —
51.5 years). All patients, except for one of them, were in sat-
isfactory clinical condition complaining of general weak-
ness, easy fatigability and weight loss (grade 1–2 according
to ECOG scale). The disease duration before splenecto-
my ranged from 1 to 48 months. Enlargement of peripheral
lymph nodes occurred in 4 patients, mediastinal — in 2 pa-
tients, and enlargement of intra-abdominal lymph nodes was
observed in all patients. Splenomegaly was found in all sub-
jects, thereby, in 4 patients the spleen occupied all the left
side of the abdomen, in 2 patients — it occupied the whole
abdomen and its inferior pole descended into the small pel-
vis. Hepatomegaly (the liver protruded below the edge of the
costal margin to 1–6 cm) was observed in 5 patients of the
group. Bone marrow involvement (> 30% of lymphoid cells)
was found in 3 patients with peri pheral blood lymphocyto-
sis > 10.0•109/l in 2 cases. M-gradient (see Table 1) appears
on proteinogram of 1 patient with NMZL. Classification of
patients according to NHL variants is presented in Table 2.
All patients showed signs of profound hemolysis: icter-
ic skin and mucosa, anemia (8 patients showed hemoglo-
bin level of 60–96 g/l), reticulocytosis 20–128‰ accom-
panied by increased total bilirubin due to indirect fraction,
and in particular cases, followed by increased transaminase
level, positive direct Coombs’ test and warm agglutinins.
Leukopenia (1.0–3.5•109/l) occurred in 4 patients. In one
patient, hemolysis was the first sign of disease, while in
other patients it occurred after NHL manifestations. Ab-
dominal manifestations (discomfort and pain in the left
hypochondriac region) were noticed in 5 patients. RPH
was sonographically diagnosed in 5 patients based on in-
creased diameter of the splenic and portal veins.
Six patients administered corticosteroids, under-
went chemotherapy, erythrocyte transfusions before sur-
gery, however, that has not resulted in hemolysis cessa-
tion. Thus, the indications for splenectomy in the pa-
tients with NHL associated with AIHA were as follows:
profound hemolysis which was refractory to drug thera-
py, leukopenia as a possible manifestation of hypersple-
nism, massive splenomegaly with abdominal symptoms,
and presence of RPH.
In 3 patients splenectomy was followed by blood loss
of more than 500 ml secondary to enlarged spleen, peri-
splenitis, excessive bleeding and RPH. In other cases
(7 patients), the surgery was performed without compli-
cations with blood loss of less than 500 ml. The ascitic
fluid was found in 3 patients. The weight of the removed
spleens ranged from 1.5 to 10.0 kg. Abdominal lymph-
adenopathy was confirmed in all patients of group 1; en-
larged lymph nodes only at splenic hilum were observed
in 3 patients, in other 7 patients the lymph node packages
were found along the lesser and greater curvatures of the
stomach, in the hepatoduodenal ligament, at the splenic
and renal hilums, along the upper edge of the pancreas,
and enlarged mesenteric lymph nodes were also found.
One patient had two accessory spleens removed, and an-
other patient simultaneously underwent cholecystecto-
my for chronic calculous cholecystitis.
The histologic and immunohistochemical exami-
nations of the removed spleens (including two accesso-
ry spleens) and abdominal lymph nodes confirmed the
initial diagnosis of NHL variant in 5 patients. In another
5 patients who had no affected peripheral lymph nodes
and/or leukemization available for morphological exami-
nation the variant of lymphoma was determined or speci-
fied that substantiates the diagnostic significance of sple-
nectomy for these patients.
Complications occurred in 2 (20%) patients in the
postoperative period. One patient developed a bilater-
al pneumonia that was resolved after proper antibacte-
rial therapy. Another patient developed a lower extrem-
ity superficial thrombophlebitis in varicose veins sec-
ondary to post-splenectomy hyperthrombocytosis; the
thrombectomy was performed (Table 3). The immediate
result of splenectomy in all of the patients (100%) with
NHL associated with AIHA was good: hemolysis ceased,
hemoglobin level increased, in patients with leukopenia
the leukocytes stabilized, bilirubin level reduced to the
normal value, M-gradient disappeared, and no mani-
festations of abdominal discomfort were observed. Only
one (10%) patient of group 1 required cytostatic thera-
py within the 1-year period after surgery.
Table 3
Complications after splenectomy in patients with NHL
with immune cytopenias
Type of complications NHL + AIHA NHL + AIHA +
ITP
Pneumonia 1 1
Acute postoperative hemorrhage − 1
Acute thrombophlebitis of the lower
extremity superficial veins 1 −
Postoperative pancreatitis − 2
Acute adrenal insufficiency − 1
While analyzing the long-term outcomes of splen ectomy
in patients with NHL associated with AIHA it has been de-
fined that the median event-free survival was 29 months
(12–49 months) (Fig. 1), and the median overall surviv-
al — 31 month (18.1–52.2 months) with the 3-year surviv-
al rate of 45% and 5-year survival rate of 22% (Fig. 2). The
patient with NMZL had the shor test survival time after the
surgery (3 months), and the best effect has been achieved in
patient with mature B-cell NHL (low-grade malignancy ac-
cording to WF). In this patient, NHL and massive spleno-
megaly has been associated with warm type AIHA (hemo-
globin level — 50 g/l) and leukopenia (1.4•109/l). Numer-
ous blood transfusions, chemotherapy and corticosteroids
therapy had no effect. The patient underwent splenectomy.
The removed spleen weight constituted 10 kg. Splenecto-
my resulted in termination of hemolysis, leukocytes stabili-
zation, and elimination of abdominal discomfort caused by
giant spleen. The patient has been living for 201 months af-
ter the splenectomy having achieved complete clinical re-
mission, without any additional treatment.
ОНКОЛОГИЯ • Т. 19 • № 2 • 2017
ОРИГИНАЛЬНЫЕ ИССЛЕДОВАНИЯ
106
Fig. 1. The Kaplan — Meier curves of overall survival in pa-
tients with NHL, associated with АІHА, and NHL, associa-
ted with АІHА and ІТP
Fig. 2. The Kaplan — Meier curves of event-free survival in pa-
tients with NHL, associated with АІHА, and NHL, associa-
ted with АІHА and ІТP
One patient with SMZL (who underwent surgery when
there was no possibility to perform prophylactic vacci-
nation) died 9 months after splenectomy due to untreat-
ed post-splenectomy infection (overwhelming postsple-
nectomy infection syndrome) that manifested as a sep-
tic shock. Antibiotic therapy and resuscitation at Intense
Care Unit proved ineffective, and the patient died. In pa-
tients with DLBCL (n=6), despite good short-term results
of splenectomy, the median overall survival was short —
26 months, wherein, five of them died of lymphoma pro-
gression, and one of them — died of myocardial infarc-
tion 64 months after the surgery.
In group 2, there were 18 patients with NHL associa-
ted with AIHA and ITP (EFS), 12 of them were men (25–
65 years old) and 6 of them were women (56–69 years
old), the median age — 53.5 months. General condition of
16 patients corresponded to grade 1–2 according to ECOG
scale, and condition severity of two patients corresponded
to grade 3. The disease duration before surgery ranged from
1 month to 11 years. Five patients were diagnosed with en-
largement of peripheral lymph nodes. All of the patients suf-
fered from a pronounced splenome galy: in 10 patients the
spleen occupied all the left side of the abdomen, in 4 pa-
tients it protruded beyond the median line and its inferi-
or pole descended into the small pelvis. Hepatomegaly was
observed in 13 cases. During instrumental examination of
the chest and abdominal cavity all patients were diagnosed
with splenomegaly, abdominal (14 patients) and mediasti-
nal lymphadenopathy (2 patients). Bone marrow involve-
ment was found in 9 patients, lymphoid cells count in the
bone marrow exceeded 30%. M-gradient in blood serum
was revealed in two subjects with lymphoma (see Table 1).
All patients showed signs of profound hemolysis, had
positive direct Coombs’ test and warm agglutinins. Reduced
hemoglobin level of less than 100 g/l was observed in 10 pa-
tients, the number of reticulocytes varied from 22 to 138‰.
Bilirubin concentration in blood increased due to indirect
fraction. At the same time these patients were diagnosed
with thrombocytopenia: number of thrombocytes ranged
from minimum values to 92.0•109/l. However, moderate
hemorrhages (skin petechiae, mucosal and abdominal he-
morrhages) occurred only in one patient with DLBCL when
thrombocytes number was less than 30•109/l. The number
of leukocytes remained within normal range only in 3 pa-
tients, 8 patients were diagnosed with leukopenia, and an-
other 7 were diagnosed with leukocytosis. Absolute lympho-
cytosis 5–10•109/l was noticed in 2 patients, and more than
10•109/l — was observed in 6 patients. In 5 patients with
EFS, the atypical morphology of peripheral blood lympho-
cytes which corresponded to villous lymphocytes morpho-
logy was determined that constituted a ground for diagnosing
SLVL [13, 14]. Marked manifestations of abdominal discom-
fort were noticed in 12 patients. 15 patients developed signs
of RPH: increase in diameter of splenic vein (15 patients), in-
crease in diameter of portal vein (9 patients), grade 1 esopha-
geal varices determined by fibrogastroduodenoscopy (3 pa-
tients). Classification of patients according to NHL variants
is presented in Table 2.
Nine patients of group 2 received treatment (chemo-
therapy, corticosteroids) before the surgery without possi-
ble effect on immune cytopenia. The indications for sple-
nectomy in patients with NHL associated with AIHA and
ITP were as follows: AIHA, thrombocytopenia, hemor-
rhagic syndrome, previous treatment failure, splenomegaly
with abdominal manifestations and hypersplenism, RPH.
The spleen removal in patients of group 2 was techni-
cally difficult. In 6 patients the surgery was associated with
marked perisplenitis in the form of adhesions of the spleen
to the diaphragm, abdominal wall, colon and left kidney.
9 patients developed the increased bleeding, apparent-
ly secondary to thrombocytopenia, due to which the dif-
ferent hemostatic methods were applied: pharmacologi-
cal method, electrocoagulation, tamping with hemostatic
sponge, and in particular cases — with gauze pads. In one
patient the upper middle laparotomy incision had to be
supplemented by oblique incision in the left hypochon-
driac region due to marked perisplenitis, massive spleno-
megaly and excessive bleeding.
ОРИГИНАЛЬНЫЕ ИССЛЕДОВАНИЯ
107ОНКОЛОГИЯ • Т. 19 • № 2 • 2017 107
The weight of the removed spleens ranged from 1.5 to
6.0 kg, where in 12 cases the spleen was particularly large-
sized (26–45 cm long). In one case the accessory spleens
located at the hilum of the main spleen were detected and
removed. The surgical blood loss constituted 200–1000 ml.
The enlarged abdominal lymph nodes (14 patients) were
detected mostly at the splenic hilum, along the greater cur-
vature of the stomach, along the upper edge of the pancre-
as, less frequently — in the hepatoduo denal ligament, as
well as enlarged paraaortic and mesenteric lymph nodes.
In 10 patients the RPH was manifested by splenic, abdom-
inal, diaphragmatic and omental varices; and the ascitic
fluid was found in 3 patients.
The morphological and immunological examinations
of the spleen and abdominal lymph nodes confirmed the
diagnosis of NHL variant in 11 patients, enabled to de-
termine or specify the lymphoma variant in 6 patients,
and in one case it constituted a ground for changing the
initial diagnosis of myelofibrosis for T-cell lymphoma
(high-grade according to WF). Thus, the splenectomy
was diagnostically significant for 7 patients.
The short-term results of splenectomy in 16 (89%)
patients of group 2 were classified as good: hemoly-
sis ceased, hemoglobin level has stabilized, number of
thrombocytes increased or stabilized, in patients with leu-
kopenia the leukocytes stabilized, in particular patients
with leukocytosis the number of leukocytes reduced, and
the abdominal discomfort syndrome was eliminated. The
M-gradient disappeared in one patient with a MCL.
The splenectomy proved ineffective in two (11%) pa-
tients of group 2. In one patient with DLBCL associated with
AIHA and ITP with severe hemorrhagic syndrome, leuko-
penia, massive (30×15 cm) spleen adjacent to the neighbor-
ing organs and abdominal wall, collateral varices in the ab-
dominal cavity, the splenectomy was accompanied by great
technical difficulties and excessive bleeding (blood loss —
1000 ml). The surgery has not resul ted in hemolysis termi-
nation and increasing thrombocyte count, the patient died
one month after splenectomy due to hemorrhagic syndrome.
Another patient was admitted with a provisional diagno-
sis of myelofibrosis, splenome galy, AIHA (hemoglobin —
51 g/l, positive Coombs’ test), ITP (thrombocytes count —
34.0•109/l), and leukopenia (3.9•109/l). Due to splenomeg-
aly, concomitant pancytopenia refractory to corticosteroids
and for diagnosis confirmation the patient was assigned to
undergo splenectomy. The surgery was accompanied by great
technical difficulties, bleeding (blood loss — approximate-
ly 1000 ml) as a consequence of perisplenitis, thrombocy-
topenia and RPH. A high-grade (according to WF) malig-
nant T-cell lymphoma was found in the removed spleen by
means of morphological examination. On the twelfth day
after splenectomy the peripheral blood indices improved
(hemoglobin — 98 g/l, leukocytes — 27.0•109/l, thrombo-
cytes — 589.0•109/l). However, the patient’s condition re-
mained unstable and he died one month after the surgery
due to rapid progression of the lymphoma.
Complications after splenectomy (Table 3) occurred in 3
(17%) patients of group 2. The patient with SMZL deve loped
pneumonia and postoperative pancreatitis after splenecto-
my. Both complications were successfully cured. The patient
with SMZL and M-gradient in spite of pre- and periopera-
tive bleeding prevention and unassociated with surgery de-
veloped intra-abdominal bleeding 2 hours after splenecto-
my, which was successfully suppressed with great difficul-
ties without relaparotomy after consecutive administration
of haemostatic agents, eptacog alfa, human prothrombin
complex, fresh frozen plasma transfusion and finally, local
administration of aprotinin. On the fifth day after the sur-
gery the same patient developed postoperative pancreatitis
that was timely diagnosed and treated. The acute adrenal in-
sufficiency which was manifested by a sudden drop in blood
pressure occurred in patient with MCL on the second day
after the surgery. The patient’s condition has stabilized af-
ter high corticosteroids dose administration. Six (33%) pa-
tients of group 2 underwent cytostatic therapy in the first
year after the surgery.
While analyzing the long-term outcomes of splen-
ectomy in patients with NHL associated with AIHA and ITP
(Fig. 1, 2) it has been defined that the median event-free sur-
vival after the surgery was 22 months (5–52 months), and
the median overall survival — 50 months (12–97 months)
with the 3-year survival rate of 62% and 5-year survival rate of
31%. Depending on lymphoma’s category the overall median
survival time after splenectomy in patients of this group with
SLVL constituted 39.8 months, with MCL — 159.7 months,
with DLBCL — 21.4 months. The median event-free survi-
val in patients with SLVL constituted 10.6 months, with
MCL — 6.3 months and with DLBCL — 21.4 months.
One patient with follicular lymphoma has lived 6 months
after splenectomy and died due to the disease progression;
another patient with SMZL has lived 143 months after the
surgery without chemotherapeutic agents’ administration.
Statistical comparison of overall and event-free sur-
vival after splenectomy showed no significant difference
between the 2 groups (long-rank test for overall survi-
val p = 0.97607, for event-free survival — p = 0.21350).
Thus, similar to other researchers [5, 8, 12, 15–19], we
determined that splenectomy remains an effective and rel-
atively safe method for treating NHL associated with im-
mune cytopenias. Indications for splenectomy in patients
of the represented groups were both diagnostic and thera-
peutic. Similar indications are discussed in the references [8,
16, 18, 20]. Most of patients showed more than one indica-
tion for the surgery that is mentioned in the references [16].
The splenectomy was diagnostically significant for 12
(43%) patients of both groups who suffered from isolat-
ed splenomegaly without involvement of accessible lymph
nodes and bone marrow that is closely related to the issue of
splenic lymphomas [20]. As a result of histological and im-
munophenotypic analysis of the affected splenic tissue, the
diagnosis of NHL variant was determined or specified in
11 patients, and T-cell lymphoma with the initial diagnosis
of myelofibrosis was found in one case.
An alternative to splenectomy in such patients (while
there are no any other indications for the surgery) may
be a punch biopsy of the spleen under ultrasound moni-
toring that, however, cannot be applied as a routine di-
agnostic method [20].
All patients of our group developed clear therapeutic
indications for splenectomy: immune cytopenia (AIHA or
ОНКОЛОГИЯ • Т. 19 • № 2 • 2017
ОРИГИНАЛЬНЫЕ ИССЛЕДОВАНИЯ
108
AIHA + ITP) which was refractory to previous therapy in 15
(54%) patients, and massive splenomegaly with abdominal
signs and portal hypertension (in 6 patients of group 1 and
in 14 patients of group 2, totally — 71% of patients). Some
researchers qualify signs of hypersplenism as indications for
the surgery in patients with NHL [5, 12, 19], which may in-
clude massive splenome galy, thrombocytopenia, leukocy-
topenia, the immune origin of which is difficult to confirm
in patients with NHL, and portal hypertension.
Splenectomy in case of massive splenomegaly is tech-
nically relatively difficult due to splenic adhesion to oth-
er organs of abdominal cavity and its vascularization [7,
8, 21]. We did not apply preliminary ligation of the sple-
nic artery trunk, injection of adrenaline into the spleen
for reducing its size, as it was recommended by some
researchers. In our opinion, thereby we reduce the risk
of ischemia of a pancreatic tail segment.
Different perioperative mortality rates in patients with
NHL undergoing splenectomy are presented in the refe-
rences — from 0% [16], 1% [8], 2.9% [17], 3.5% [18] to 3.8%
[19], and the postoperative mortality within one month is
reported in 9% of patients [12]. There were no incidences of
perioperative mortality in patients of the group represented
in the study (0%), however, 2 (7%) patients of group 2 died
of T-cell lymphoma progression and hemorrhagic syndrome
one month after the surgery. The postoperative complica-
tions (pneumonia, bleeding, thrombosis, pancreatitis) oc-
curred in 5 (18%) patients that coincides with the data re-
ported in the refe rences (17% 37% [17], 29% [5], 21% [12],
19.2% [19], 17% [16]). The complications after splenectomy
in patients with NHL can be divided into four types: those re-
sulting from the disease severity; complications arising from
technical difficulties during the surgery; suppurative septic
and thrombotic complications.
The immediate clinical hematologic effect after the sur-
gery achieved in 93% of patients: 100% in patients with NHL
associated with AIHA and 89% in those with NHL associ-
ated with AIHA + ITP. The investigators confirm the high
response rate to splenectomy in patients with NHL: 89% [5],
80% [16], 72% [17], 64% in patients with NHL associated
with AIHA and 89% in patients with NHL associated with
ITP [12] and report that 90–100% of patients became in-
dependent of red blood cell and platelet transfusions after
the surgery [8].
The overall survival rate of patients with NHL after sple-
nectomy was slightly better in patients of group 2, and the
event-free survival rate — in patients of group 1, notwith-
standing that the survival curves did not differ in both groups.
The similar survival rates of the patients with NHL after sple-
nectomy are reported by other researchers [5, 8, 12, 17]. The
obtained results prove the statement of some investigators
that operative treatment success depends mainly on NHL
variant rather than pre sence or absence of concomitant cy-
topenia [8]. Among patients participating in the study, the
shortest survi val time after splenectomy was in those with
aggressive lymphomas associated with immune cytopeni-
as (DLBCL, T-cell). The simultaneous anemia, thrombo-
cytopenia and leucopenia are the adverse prognostic signs
of the splenectomy efficacy in patients with NHL associat-
ed with AIHA and ITP: 5 such patients of group 2 died 1 to
12 months after the surgery. Some researchers found the
considerable survival time in patients with SMZL/SLVL [13,
14], how ever, in our group of patients it constituted on av-
erage only 39.8 months, only one patient with SMZL re-
mained alive for 143 months after splen ectomy. The partic-
ular splenic subtype of MCL with continuous remission af-
ter splen ectomy is described in the references [19, 22]. We
now follow one patient with MCL who had leukemic bone
marrow and blood patterns and underwent lifesaving sple-
nectomy due to massive splenome galy, AIHA, and throm-
bocytopenia. He has been living without signs of lympho-
ma and therapy for 290 months after the surgery.
In case of massive splenomegaly, the spleen represents
a great tumor mass. Five patients with a giant spleen un-
derwent chemotherapy before the surgery without con-
siderable effect: splenic size did not reduce, hemolysis
and thrombocytopenia were preserved. Only one patient
of group 1 and six patients of group 2 (25%) needed cy-
tostatic therapy after splenectomy. The removal of a sub-
stantial tumor mass with spleen leads to improvement of
the NHL course and response to treatment.
Those who had underwent splenectomy have 2 to 3 times
higher risk of infection development (pneumonia, meningi-
tis, sepsis), 2 times higher risk of deep vein thrombosis, in-
cluding, 4.5 higher risk of pulmonary embolism [9] and por-
tal venous thrombosis [8, 23]. We ascertain one (4%) case
of post-splenectomy infection with fatal outcome in patient
with SMZL + AIHA 9 months after the surgery. Smeltzer et
al. [8] while examining 60 patients who had undergone sple-
nectomy due to hematological pathology found the asymp-
tomatic portal and splenic vein thrombosis in 38.3% of pa-
tients, whereby, in 5% of cases it as led to death.
Thus, despite considerable achievements in the field
of modern hematology and surgery in treatment of NHL
associated with immune cytopenias, splenectomy re-
mains a serious challenge for specialists and requires
a thorough approach in every clinical case.
CONCLUSIONS
1. Splenectomy remains an effective and relatively safe
diagnostic and therapeutic method for treating NHL asso-
ciated with immune cytopenias, particularly, in the presence
of massive splenomegaly, hypersple nism and RPH.
2. Splenectomy in case of massive splenomegaly is
technically relatively difficult depending on splenic size,
adhesion to other organs of abdominal cavity and its vas-
cularization. The perioperative mortality may be avoid-
ed under condition of proper pre- and intraoperative pa-
tient care, and the postoperative mortality rate (lympho-
ma progression, bleeding) equals to 7%.
3. As a result of splenectomy, the final diagnosis of
NHL variant was specified or determined in 43% of pa-
tients, hemolysis relieved, hemoglobin and thrombo-
cytes level stabilized, abdominal discomfort was elim-
inated, and the need for chemotherapy was reduced in
93% of patients.
4. The long-term outcomes of splenectomy and surviv-
al of patients with NHL depend mostly on NHL variant
rather than presence or absence of concomitant cytopenia.
ОРИГИНАЛЬНЫЕ ИССЛЕДОВАНИЯ
109ОНКОЛОГИЯ • Т. 19 • № 2 • 2017 109
REFERENCES
1. Hauswirth AW, Skrabs C, Schutzinger C, et al. Autoimmune
hemolytic anemia, Evans’ syndromes, and pure red cell aplasia in
non-Hodgkin lymphomas. Leuk Lymphoma 2007; 48 (6): 1139–49.
2. Hauswirth AW, Skrabs C, Schutzinger C, et al. Autoimmune
thrombocytopenia in non-Hodgkin’s lymphomas. Haematologi-
ca 2008; 93 (3): 447–50.
3. Lechner K, Jager U. How I treat autoimmune hemolytic
anemias in adults. Blood 2010; 116 (11): 1831–8.
4. Sallah S, Sigounas G, Vos P, et al. Autoimmune hemolytic
anemia in patients with non-Hodgkin’s lymphoma: characteristics
and significance. Ann Oncol 2000; 11 (12): 1571–7.
5. Delpero JR, Houvenaeghel G, Gastaut JA, et al. Splenecto-
my for hypersplenism in chronic lymphocytic leukaemia and ma-
lignant non-Hodgkin’s lymphoma. Br J Surg 1990; 77 (8): 957.
6. Sallah S, Wan JY, Hanrahan LR. Future development of lym-
phoproliferative disorders in patients with autoimmune hemolytic
anemia. Clin Cancer Res 2001; 7 (4): 791–4.
7. Bickerstaff KI, Morris PJ. Splenectomy for massive spleno-
megaly. Br J Surg 1987; 74 (5): 346–9.
8. Smeltzer J, Habermann TM, Timucin T, et al. Long-term
clinical outcomes in patients with massive splenomegaly and non-
Hodgkin’s lymphoma treated with splenectomy. Blood 2012; 120:
2692.
9. Kristinsson SY, Gridley G, Hoover RN, et al. Long-term
risks after splenectomy among 8149 cancer-free American veter-
ans: a cohort study with up to 27 years follow-up. Haematologi-
ca 2014; 99 (2): 392–8.
10. Jaffe E, Harris N, Stein H, Vardiman J, eds. Pathology and
genetics of tumors of hematopoietic and lymphoid tissues. Lyon,
France: IARC Press; 2001. 351 p.
11. Moormeier JA, Williams SF, Golomb HM. The staging of
non-Hodgkin’s lymphomas. Semin Oncol 1990; 17 (1): 43–50.
12. Kehoe JE, Daly JM, Straus DJ, et al. Value of splenecto-
my in non-Hodgkin’s lymphoma. Cancer 1985; 55 (3): 1256–64.
13. Vyhovska YaI, Yevstakhevych IY, Lebed HB, et al. Splenec-
tomy in treatment of patients with marginal zone lymphoma. Hos-
pital Surgery 2004; 1: 74–9 (in Ukrainian).
14. Iannitto E, Ambrosetti A, Ammanuta E, et al. Splenic mar-
ginal zone lymphoma with or without villous lymphocytes: he-
matologic findings and outcomes in a series of 57 patients. Can-
cer 2004; 101 (9): 2050–7.
15. Akpek G, McAneny D, Weintraub L. Comparative response
to splenectomy in coombs-positive autoimmune hemolytic anemia
with or without associated disease. Am J Hematol 1999; 61: 98–102.
16. Brodsky J, Abkar A, Styler M. Splenectomy for non-Hodg-
kin’s lymphoma. Am J Clin Oncol 1996; 19 (6): 558–61.
17. Lehne G, Hannisdal E, Langholm R, et al. A 10-year expe-
rience with splenectomy in patients with malignant non-Hodg-
kin’s lymphoma at the Norwegian Radium Hospital. Cancer 1994;
74 (3): 993–9.
18. Xiros N, Economopoulos T, Christodoulidis C, et al. Sple-
nectomy in patients with malignant non-Hodgkin’s lymphoma.
Eur J Haematol 2000; 64 (3): 145–50.
19. Yoong Y, Kurtin PJ, Allmer C, et al. Efficacy of splenecto-
my for patients with mantle cell non-Hodgkin’s lymphoma. Leuk
Lymphoma 2001; 42 (6): 1235–41.
20. Iannito E, Tripodo C. How I diagnose and treat splenic
lymphomas. Blood 2011; 117 (9): 2585–95.
21. Silayev MA, Karapolyan SR, Bulanov AYu, et al. Splenec-
tomy for massive and giant splenomegaly. Hematology Transfusi-
ology 2011; 56 (1): 6–10 (in Russian).
22. Ruchlemer R, Wotherspoon AC, Thompson JN, et al. Sple-
nectomy in mantle cell lymphoma with leukaemia: a comparison
with chronic lymphocytic leukaemia. Br J Haematol 2002; 118
(4): 952–8.
23. Loring LA, Panicek DM, Rarpeh MS. Portal system throm-
bosis after splenectomy for neoplasm or chronic hematologic dis-
order: is routine surveillance imaging necessary. J Comp As To-
mography 1998; 22 (6): 856–60.
ЕФЕКТИВНІСТЬ СПЛЕНЕКТОМІЇ
ПРИ НЕхОДЖКІНСЬКИх ЛІМФОМАх,
УСКЛАДНЕНИх АУТОІМУННОЮ
ГЕМОЛІТИЧНОЮ АНЕМІЄЮ
ТА СИНДРОМОМ ЕВАНСА — ФІШЕРА
Ю.Л. Євстахевич, Я.І. Виговська, І.Й. Євстахевич,
М.М. Семерак, Г.Б. Лебедь, В.Є. Логінський
Резюме. Мета: визначити показання, хірургічну
техніку і наслідки спленектомії при неходжкінських
лімфомах (НХЛ), ускладнених аутоімунною гемо
літичною анемією (АІГА) або синдромом Еванса —
Фішера (СЕФ), на основі двадцятирічного досвіду ви
конання таких операцій. Об’єкт і методи: проведено
10 спленектомій при НХЛ, ускладненій АІГА (I гру
па), та 18 спленектомій — при НХЛ, ускладненій
АІГА та імунною тромбоцитопенією — СЕФ (II гру
па). Результати оцінювали безпосередньо після опе
рації та при тривалому спостереженні протягом
3–201 міс (медіана 68 міс) у І групі та 1–290 міс (ме
діана 195,5 міс) — у ІІ групі. Результати: показання
до спленектомії при НХЛ — наявність імунних цито
пеній, резистентних до кортикостероїдів і цитоста
тичної терапії, які супроводжуються масивною спле
номегалією, регіонарною портальною гіпертензією та
гіперспленізмом. Операція спленектомії при масивній
селезінці технічно досить складна, залежить від роз
міру органа та наявності прилягання. Післяоперацій
на смертність (прогресування лімфоми, кровотеча)
становить 7%. У 93% хворих спостерігали позитив
ний безпосередній результат хірургічного втручання.
При НХЛ, ускладненій АІГА, медіана безподійної вижи
ваності після операції становила 29 міс (12–49 міс),
а загальної виживаності — 31,0 міс (18,1–52,2 міс)
з 3річною виживаністю 45% пацієнтів і 5річною —
22%. У хворих на НХЛ із СЕФ медіана безподійної ви
живаності сягала 22 міс (5–52 міс), а медіана загальної
виживаності — 50 міс (12–97 міс), причому 3річну ви
живаність відмічено у 62% хворих, а 5річну — у 31%.
Найменша тривалість життя після спленектомії за
фіксована у хворих на агресивні лімфоми, ускладнені
імунними цитопеніями (Bвеликоклітинна дифузна
лімфома, Tклітинні лімфоми). Висновки: спленекто
мія залишається ефективним і доволі безпечним діа
гностичнолікувальним методом при НХЛ, ускладне
них імунними цитопеніями, особливо за наявності ма
сивної спленомегалії, регіонарної портальної гіпертензії
та гіперспленізму. Віддалені результати спленектомії
значною мірою залежать від варіанта НХЛ.
Ключові слова: неходжкінська лімфома,
спленектомія, аутоімунна гемолітична анемія,
імунна тромбоцитопенія.
Correspondence:
Yevstakhevych Yu.L.
45 General Chuprynka str., Lviv 79044, Ukraine
Institute of Blood Pathology and Transfusion
Medicine
E-mail: msemerak@ukr.net
Submitted: 20.03.2017
|