Hairy cell leukemia: A clinicopathological study of 18 cases

  • Kazoomi Patel GCS medical college, hospital and Research centre ,Naroda road, opp.to DRM office, Ahmedabad, 380025 Gujarat. India
  • Rupal Shah GCS medical college, hospital and Research centre ,Naroda road, opp.to DRM office, Ahmedabad, 380025 Gujarat. India
  • Biren Parikh GCS medical college, hospital and Research centre ,Naroda road, opp.to DRM office, Ahmedabad, 380025 Gujarat. India
  • Jyoti Sawhney Pathology dept,Gujarat Cancer & Research Institute ,Asarva,Ahmedabad, Gujarat, India
  • Bina Brahmbhatt Pathology dept,Gujarat Cancer & Research Institute ,Asarva,Ahmedabad, Gujarat, India
Keywords: hairy cell leukemia, flow cytometry, cladribine, bone marrow aspiration

Abstract

INTRODUCTION: Hairy cell leukemia (HCL) is an uncommon chronic B-cell lymphoproliferative disorder. It affects primarily elderly men and presents with splenomegaly, pancytopenia and monocytopenia. It has to be differentiated from various chronic lymphoproliferative disorders (CLPDs) because of different protocol of treatment and clinical course.MATERIALS AND METHODS : This study included 18 cases of HCL out of 300 cases of CLPDs diagnosed over a period of 4 years (2009-2012) at tertiary cancer centre. All 18 cases were evaluated for clinical history, signs and symptoms, laboratory data, all initial and follow up peripheral blood smear (PBS) examinations, bone marrow aspirate (BMA) with imprint smear, bone marrow biopsy, immunophenotyping data, treatment details and response to treatment from medical records.RESULTS: This study included 18 cases, age group of 35-69 years, with a median age of 40 years and male predominance. M:F ratio was 6:1. HCL was 6% out of all CLPDs. Patients were presented with common complaints of weakness, fever and abdominal pain. On clinical examination, commonest findings were pallor, splenomegaly, hepatomegaly and abdominal lump. On laboratory investigations, 17 cases had anemia, 4 cases had leucocytosis, 11 cases had leucopenia and 11 cases had pancytopenia. On bone marrow biopsy, findings were  diffuse infiltration of the marrow by atypical lymphoid cells having abundant cytoplasm. For immunophenotyping all patients` blood samples were  CD19   gated  on lymphocytes. It showed co expression of CD103, CD11c and CD25. Thirteen patients were treated with cladribine based chemotherapy with excellent responses.  CONCLUSION:   HCL clinically presents with  pancytopenia and splenomegaly in middle aged male but few patient can  present with unusual clinical features like absence of a palpable spleen, generalized lymphadenopathy and leucocytosis .DOI:10.21276/APALM.1660

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Published
2018-03-24
Issue
Vol 5 No 3 (2018)
Section
Original Article

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