Outcomes of splenectomy in T-cell large granular lymphocyte leukemia with splenomegaly and cytopenia.
OBJECTIVE
T-cell large granular lymphocyte leukemia (T-LGL) is a chronic clonal lymphoproliferation of cytotoxic T cells often complicated by cytopenia. Because the outcomes of splenectomy in patients with T-LGL have been only reported sporadically, we objectively assessed the outcomes of splenectomy.


METHODS
When a cohort of 56 T-LGL patients was analyzed, patients with splenomegaly (n = 34) and had higher frequency of bi- and pancytopenia than patients with no splenomegaly (70% vs 27%; p = 0.001). We identified 15 patients who, in their clinical course, underwent splenectomy and studied their hematological and clinical outcomes.


RESULTS
Indications for splenectomy included symptomatic splenomegaly and/or severe refractory cytopenia. Median spleen weight was 1300 g, consistent with diagnosis of splenomegaly; T-cell receptor (TCR)-gamma rearrangement and typical T-LGL were detected by immunophenotype in all specimens. There was no surgery-related mortality, with the median follow-up and survival of 719 and 498 days, respectively. Two patients died due to causes possibly related to the splenectomized state and/or primary disease. All patients showed lineage-specific hematologic response and achieved transfusion independence; however, precise molecular analysis of TCR and variable chain Vbeta flow cytometry showed persistence of the LGL clones.


CONCLUSIONS
We conclude that splenectomy constitutes a viable and safe therapeutic option for patients with T-LGL, splenomegaly, and refractory cytopenia.
Subbiah
Vivek
V
Experimental Hematology and Hematopoiesis Section, Cleveland Clinic Foundation, Cleveland, Ohio, USA.
Viny
Aaron D
AD
Rosenblatt
Steven
S
Pohlman
Brad
B
Lichtin
Alan
A
Maciejewski
Jaroslaw P
JP
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001772:Blood Cell Count; D015331:Cohort Studies; D005260:Female; D005500:Follow-Up Studies; D015334:Gene Rearrangement, gamma-Chain T-Cell Antigen Receptor; D006801:Humans; D016130:Immunophenotyping; D054066:Leukemia, Large Granular Lymphocytic / Q000097:blood / Q000150:complications / Q000601:surgery*; D008297:Male; D008875:Middle Aged; D018365:Neoplasm, Residual; D010198:Pancytopenia / Q000209:etiology*; D012074:Remission Induction; D012189:Retrospective Studies; D013156:Splenectomy / Q000706:statistics & numerical data*; D013163:Splenomegaly / Q000209:etiology / Q000601:surgery*; D016019:Survival Analysis; D016896:Treatment Outcome