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hairy cell leukemia (leukemic reticuloendotheliosis)

Named for appearance of neoplastic B-cells with hairy projections* seen on smear preparations. * described as atypical lymphoid cells with radial projections in [9] Pathology: 1) lymphoid cells with oval to bean shaped nuclei, less clumped chromatin & abundant pale cytoplasm with hairy projections seen on smear preparation 2) malignant B-cell in the bone marrow & spleen a) bone marrow is always involved b red pulp in spleen involved with white pulp atrophic 3) lymph node involvement uncommon 4) abnormal T-cell function Immunophenotype: B cell antigens (CD19, CD20, CD22, CD79a) +, sIg+ (M +/- D, G or A), CD11c+, CD25+, CD103+, CD5-, CD10-, CD23- Genetics: - Ig heavy & light chain genes rearranged - overexpression of RGS1 Clinical manifestations: 1) adults with splenomegaly without lymphadenopathy 2) indolent course, spontaneous remissions recorded 3) increased susceptibility to infection Laboratory: - complete blood count (CBC): - pancytopenia with relatively more profound monocytopenia [9] - peripheral blood smear - circulating hairy cells [4] - typical lymphocytes with radial projections [9] - bone marrow biopsy [7], often unsuccessful [4] - tartrate resistant acid phosphatase (TRAP) in blood/bone marrow present in most cases, but is not diagnostic - chemistry 14 panel [7] - serum lactate dehydrogenase [7] - hepatitis B serology Complications: - abnormal T-cell function increases risk of infection with: - intracellular pathogens - Mycobacterium tuberculosis - Listeria - Cryptococcus - Mycobacterium avium complex (MAC) Management: 1) indications for treatment initiation [7] - symptomatic disease with excessive fatigue - physical discomfort due to splenomegaly/hepatomegaly - unexplained weight loss (>10% within prior 6 months) - cytopenias (hemoglobin <11g/dl, platelets <100,000/uL, &/or absolute neutrophil count <1000/uL) - progressive lymphocytosis - lymphadenopathy 2) treatment a) splenectomy b) does not respond to conventional chemotherapy c) interferon-alpha 2b, 2'deoxycoformycin or cladribine (CDA) can induce long term remission d) cladribine (CDA) is drug of choice [4] - a single cycle with > 80% cure rate [4] - pentostatin is alternative [4] e) rituximab for resistant cases [4] f) moxetumomab, an anti-CD22 monoclonal Ab FDA-approved to treat refractory hairy cell leukemia 3) if CD4 count < 200/mm3. - acyclovir for herpes virus prophylaxis - trimethoprim-sulfamethoxazole for Pneumocystis jiroveci prophylaxis - prophylaxis for a minimum of 2 months & until CD4 count > 200/mm3

Interactions

disease interactions

Related

2-chlorodeoxyadenosine (2-CdA) [cladribine] (Leustatin) interferon pentostatin (Nipent, DCF, 2'deoxycoformycin) splenectomy

General

lymphoid leukemia peripheral B-cell lymphoid neoplasm

References

  1. Harris NL, Jaffe ES, Stein H, Banks PM, Chan JK, Cleary ML, Delsol G, De Wolf-Peeters C, Falini B, Gatter KC, et al. A revised European-American classification of lymphoid neoplasms: a proposal from the International Lymphoma Study Group. Blood. 1994 Sep 1;84(5):1361-92. Review. PMID: 8068936
  2. Harrison's Principles of Internal Medicine, 14th ed. Fauci et al (eds), McGraw-Hill Inc. NY, 1998, pg 539
  3. WHO Classification Tumours of Haematopoietic and Lymphoid Tissues. IARC Press 2001
  4. Medical Knowledge Self Assessment Program (MKSAP) 15, 16, 17, 18, 19. American College of Physicians, Philadelphia 2009, 2012, 2015, 2018, 2021. - Medical Knowledge Self Assessment Program (MKSAP) 19 Board Basics. An Enhancement to MKSAP19. American College of Physicians, Philadelphia 2022
  5. Allsup DJ, Cawley JC. The diagnosis and treatment of hairy-cell leukaemia. Blood Rev. 2002 Dec;16(4):255-62. PMID: 12350368
  6. Else M, Ruchlemer R, Osuji N et al Long remissions in hairy cell leukemia with purine analogs: a report of 219 patients with a median follow-up of 12.5 years. Cancer. 2005 Dec 1;104(11):2442-8. PMID: 16245328
  7. Anello J, Feinberg B, Lindsey R et al Hairy Cell Leukemia National Comprehensive Cancer Network Clinical Practice Guidelines, December 2017 Medscape. Dec 6, 2017 https://reference.medscape.com/viewarticle/889632_4 - Wierda WG, Byrd JC, Abramson JS, et al. Hairy Cell Leukemia, Version 2.2018, NCCN Clinical Practice Guidelines in Oncology. Natl Compr Canc Netw. 2017 Nov;15(11):1414-27. PMID: 29118233 http://www.jnccn.org/content/15/11/1414.long
  8. Getta BM, Park JH, Tallman MS. Hairy cell leukemia: Past, present and future. Best Pract Res Clin Haematol. 2015 Dec;28(4):269-72. Review. PMID: 26614906 Free PMC Article
  9. NEJM Knowledge+ Hematology - Troussard X, Cornet E. Hairy cell leukemia 2018: Update on diagnosis, risk-stratification, and treatment. Am J Hematol. 2017 Dec;92(12):1382-1390 PMID: 29110361 PMCID: PMC5698705 Free PMC article
  10. Hairy Cell Leukemia (PDQ): Treatment http://www.nci.nih.gov/cancertopics/pdq/treatment/hairy-cell-leukemia/HealthProfessional