Outcomes of splenectomy in T-cell large granular lymphocyte leukemia with splenomegaly and cytopenia
Received 17 January 2008; received in revised form 1 April 2008; accepted 8 April 2008. published online 12 June 2008.
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.
Materials and 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)-γ 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 Vβ flow cytometry showed persistence of the LGL clones.
Conclusion
We conclude that splenectomy constitutes a viable and safe therapeutic option for patients with T-LGL, splenomegaly, and refractory cytopenia.
aExperimental Hematology and Hematopoiesis Section, Cleveland Clinic Foundation, Cleveland, Ohio, USA
bDepartment of Hematologic Oncology and Blood Disorders, Cleveland Clinic Taussig Cancer Institute, Cleveland Clinic Foundation, Cleveland, Ohio, USA
cDepartment of General Surgery, Cleveland Clinic Foundation, Cleveland, Ohio, USA
dCleveland Clinic Lerner College of Medicine of Case Western Reserve University, Cleveland, Ohio, USA
eDepartment of Internal Medicine/Pediatrics, Case Western Reserve University School of Medicine, Cleveland, Ohio, USA
Offprint requests to: Jaroslaw P. Maciejewski, M.D., Ph.D., Experimental Hematology and Hematopoiesis Section, Taussig Cancer Center R/40, Cleveland Clinic Foundation, 9500 Euclid Avenue, Cleveland, OH 44195
ABSTRACT