Title: Hairy Cell Leukemia – Variant (HCL-V):  A Separate Entity

Authors: A.K. Kapoor, Jas Kamal, Akanksha Singh, Pratibha Singh

 DOI:  https://dx.doi.org/10.18535/jmscr/v5i9.32

Abstract

A female, aged 65 years had splenomegaly and atypical lymphocytes in blood smear.  Atypical cells had large round nuclei. Flow cytometric (FCM ) findings revealed positivity of atypical cells for CD19, CD103 and CD11c antigens.  She was diagnosed as a case of hairy cell leukemia – variant (HCL-V) subtype.  Satisfactory treatment protocol is not available for HCL-V.  However, she was advised treatment with monoclonal anti CD 20 antibody (Rituximab 600 mg ivi weekly × 4 doses).             

Keywords: Hairy cell leukemia – variant.

References

  1. Wang X, Spielberger R, Huang Q, Hairy cell leukemia variant, a new entity of the WHO 2008. J of Clin Oncol 2011; 29(36): 864-866.
  2. Pande P, Yeliskar BR, Mahesh U. A hairy cell leukemia variant – A rare case report.  J Clin and Diagnos Res 2013; 7(2); 358-360.
  3. Kao Hsiao-Wen, Dunn Po,Kuo Ming-Chung, Shih Lee-Yung, Lin Tung-Liang, Wu Jin-Hou, Tang Tzung-Chih, Chang Hung, Wu Hong-Cheng, Hung Yu-Shin.  Classical hairy cell leukemia and its variant : A 17-year retrospective survey in Taiwan Chinese.  Acta haematologica 2011; 126: 186-193.
  4. Gaman AM, Dobrea Camelia-Marioara, Gaman Mihnea Alexandru. A case of hairy cell leukemia variant.  Rom J Morphol Embryol 2015, 56(2): 553-556.
  5. Busemann C, Schuller F, Kruger W, Kiefer T, Wuppermann M, Androshchuck M, Dolken G. Late extramedullary relapse after allogeneic transplantation in a case of variant hairy cell leukemia.  Bone marrow transplant 2010; 45: 1117-1118.
  6. Quach H, Januszewicz H, Westerman D. Complete remission of hairy cell leukemia (HCL-V) complicated by red cell aplasia post treatment with rituximab.   Haematologica 2005; 90(7) : 72-73.
  7. Matutes E, Wotherspoon A, Catavsky D. The variant form of hairy-cell leukaemia.  Best pract Res Clin Haematol 2003; 16:41-56.
  8. Ruco LP, Procopio A, Maccallini V, Calogero A, Uccini S, Annino L, Mandelli F, Baroni CD. Severe deficiency of natural killer activity in the peripheral blood of patients with hairy cell leukemia.  Blood 1983; 61:1132-1137.
  9. Mackowiak PA, Demian SE, Sutker WL, Murphy FK, Smith JW, Tomsett R, Sheehan WW, Luby JP. Infections of hairy cell leukemia : clinical evidene of a pronounced defect in cell mediated immunity.  Am J Med 1980; 68: 718-724.
  10. Xi L, Arons E, Navarro W, Calvo KR, Steller-Stevenson M, Raffeld M, Kreitman RJ. Both variant and TGH4-34 expressing hairy cell leukemia lack BRAF1/600E mutation 2012; 119: 3330-3332.
  11. Arons E, Zhou H, Edelman DC, Gomez A, Steinberg SM, Petersen D, Wong Y, Meltzer PS, Kreitman RJ. Impact of telomere length on survival in classic and variant hairy cell leukemia.  Leuk Res 2015; 39 (12) : 1360-1366.

Corresponding Author

Ashok Kumar Kapoor

RML Mehrotra Pathology Pvt Ltd, B-171, Nirala Nagar,

Lucknow-226020, Uttar Pradesh, INDIA

Email: This email address is being protected from spambots. You need JavaScript enabled to view it.