Objectives: Mature lymphoid neoplasms presenting with 'prominent splenomegaly without significant lymphadenopathy' are uncommon and pose unique diagnostic challenges as compared to those associated with lymphadenopathy. Their descriptions in the literature are largely limited to a few case series. We analyzed the spectrum of these lymphomas diagnosed by peripheral blood (PB) and/or bone marrow (BM) examination.
Methods: Over a period of 6 years, 75 patients were diagnosed with a lymphoma from PB/BM who had presented with predominant splenomegaly. Their clinical and laboratory records including PB and BM morphology; immunophenotyping using multi-parametric flow-cytometry and immunohistochemistry were reviewed. Wherever indicated, an extended panel of immunohistochemistry (IHC) was performed on BM biopsies for accurate sub-classification.
Results and discussion: The commonest lymphomas were hairy cell leukemia (HCL) (32%) and splenic marginal zone lymphoma (SMZL) (24%). Others included diffuse large B cell lymphoma (8%), chronic lymphocytic leukemia/small lymphocytic lymphoma (8%), mantle cell lymphoma (2.7%), and follicular lymphoma (1.3%), all of which usually presents with lymphadenopathy. SMZL was the commonest lymphoma among females and those with massive splenomegaly and lymphocytosis; while HCL was commonest in patients with pancytopenia. SMZL commonly presented with lymphocytosis; however, 22% of them also presented with pancytopenia.
Conclusion: The high diagnostic efficacy of PB and BM examination using flow-cytometry and immunohistochemistry in confirming and sub-classifying splenic lymphomas suggests that a thorough hematological evaluation should always precede a diagnostic splenectomy. Immunohistochemistry remains the best modality to identify sparse or intra-sinusoidal infiltration on BM biopsy and is particularly useful in patients with fibrotic marrows and pancytopenia.
Keywords: Immunophenotype; Mature lymphoid neoplasms; Splenic lymphoma; Splenomegaly.