title: Role of blood and bone marrow examination in the diagnosis of mature lymphoid neoplasms in patients presenting with isolated splenomegaly.
abstract: OBJECTIVE
  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.
  
  
  CONCLUSIONS
  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.
  
  
  CONCLUSIONS
  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.
authors.forename: Sreejesh
authors.initials: S
authors.lastname: Sreedharanunni
authors.forename: Man Updesh Singh
authors.initials: MU
authors.lastname: Sachdeva
authors.forename: Pankaj
authors.initials: P
authors.lastname: Malhotra
authors.forename: Jasmina
authors.initials: J
authors.lastname: Ahluwalia
authors.forename: Shano
authors.initials: S
authors.lastname: Naseem
authors.forename: Gaurav
authors.initials: G
authors.lastname: Prakash
authors.forename: Alka
authors.initials: A
authors.lastname: Khadwal
authors.forename: Prashant
authors.initials: P
authors.lastname: Sharma
authors.forename: Narender
authors.initials: N
authors.lastname: Kumar
authors.forename: Neelam
authors.initials: N
authors.lastname: Varma
authors.forename: Subhash
authors.initials: S
authors.lastname: Varma
authors.forename: Reena
authors.initials: R
authors.lastname: Das
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D001853:Bone Marrow / Q000473:pathology*; D003937:Diagnosis, Differential; D005260:Female; D005434:Flow Cytometry; D006801:Humans; D007150:Immunohistochemistry; D016130:Immunophenotyping; D007943:Leukemia, Hairy Cell / Q000175:diagnosis / Q000473:pathology; D015451:Leukemia, Lymphocytic, Chronic, B-Cell / Q000175:diagnosis / Q000473:pathology; D007963:Leukocytes, Mononuclear / Q000473:pathology*; D008206:Lymphatic Diseases / Q000175:diagnosis* / Q000473:pathology; D008218:Lymphocytosis / Q000175:diagnosis / Q000473:pathology; D018442:Lymphoma, B-Cell, Marginal Zone / Q000175:diagnosis / Q000473:pathology; D008224:Lymphoma, Follicular / Q000175:diagnosis / Q000473:pathology; D016403:Lymphoma, Large B-Cell, Diffuse / Q000175:diagnosis / Q000473:pathology; D020522:Lymphoma, Mantle-Cell / Q000175:diagnosis / Q000473:pathology; D008297:Male; D008875:Middle Aged; D010198:Pancytopenia / Q000175:diagnosis / Q000473:pathology; D012189:Retrospective Studies; D013160:Splenic Neoplasms / Q000175:diagnosis* / Q000473:pathology; D013163:Splenomegaly / Q000175:diagnosis* / Q000473:pathology
keywords: Immunophenotype; Mature lymphoid neoplasms; Splenic lymphoma; Splenomegaly