Florid splenic gamma/delta T-cell proliferation in patients with splenomegaly and cytopenias: A "high stakes" diagnostic challenge

Publication date: Available online 22 February 2017
Source:Human Pathology
Author(s): Shanxiang Zhang, Michael G. Bayerl
ObjectivesSplenic gamma/delta T-cell proliferation is rare and correct diagnosis is critical for adequate clinical management.MethodsTwo splenectomy cases from patients with splenomegaly and cytopenias were studied by morphological evaluation, extensive immunophenotyping, FISH and molecular studies. The clinicopathologic findings were compared with splenic T gamma/delta neoplasia, notably hepatosplenic T-cell lymphoma (HSTL) and T-cell large granular lymphocytic leukemia (TLGL) of the variety T gamma/delta.ResultsThe enlarged spleens showed expanded red pulp with markedly increased gamma/delta T cells, which share significant to complete overlapping morphology and immunophenotype with the neoplastic gamma/delta T cells in HSTL and gamma/delta TLGL. However, they were polyclonal by molecular study and showed no evidence of isochromosome 7q. Splenectomy alone led to long-term clinical remission in both patients.ConclusionsTwo florid reactive splenic gamma/delta T-cell proliferations mimicking gamma/delta T-cell neoplasia were reported for the first time in English literature. Recognition of this exceedingly rare phenomenon is critical in prevention of misdiagnosis with potentially catastrophic consequences.



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