EFFICACY OF SPLENECTOMY IN PATIENTS WITH NON-HODGKIN’S LYMPHOMAS ASSOCIATED WITH AUTOIMMUNE HEMOLYTIC ANEMIA AND EVANS — FISHER SYNDROME
Keywords:
nonHodgkin’s lymphoma, splenectomy, autoimmune hemolytic anemia, immune thrombocytopenia.Abstract
Objective: to determine indications for, surgical technique and outcomes of splen
ectomy in nonHodgkin’s lymphomas (NHL) associated with autoimmune he
molytic 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 de
termined 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 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
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 3year survival observed in 45% of patients and a 5year 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 3year survival observed in as much as 62% of pa
tients and a 5year survival in 31% of patients. The lowest post spenectomy 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.
Long term results of splenectomy are mainly determined by a variant of NHL.
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