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Hairy cell leukemia — Clinical Guidelines

Overview

Hairy cell leukemia (HCL) is a rare, indolent B-cell lymphoproliferative disorder characterized by the proliferation of small B lymphocytes with distinctive hairy projections. 1 21

Diagnosis

Key Diagnostic Criteria: Presence of hairy cells in peripheral blood or bone marrow, often with splenomegaly.

Recommended Tests: Blood film examination, bone marrow biopsy, immunohistochemical staining for CD11c, CD103, tartrate-resistant acid phosphatase (TRAP), and cyclin D3.

Immunophenotyping: Use of antibodies like CD11c, CD25, CD103, and HC2 for minimal residual disease (MRD) assessment. 1 8 9 10

Management

First-Line Treatment: Cladribine is a primary therapy, often administered intravenously or orally. 4

Alternative Treatments: 2-Chlorodeoxyadenosine (2-CdA) for patients intolerant to cladribine. 10

Historic Approaches: Splenectomy and interferon therapy may be considered in relapsed or refractory cases. 7

Monitoring MRD: Post-treatment assessment of MRD using immunohistochemical techniques can predict relapse risk. 9 10

Special Populations

Pregnancy: Treatment decisions may necessitate termination of pregnancy due to therapeutic considerations. 11

Comorbidities: No specific guidelines provided in abstracts; management should consider individual patient comorbidities and treatment tolerance.

Key Recommendations

Use cladribine as first-line therapy for hairy cell leukemia (Evidence: Strong 4).

Assess minimal residual disease post-treatment to predict relapse risk (Evidence: Moderate 9 10).

Consider splenectomy or interferon therapy for patients with relapsed or refractory disease (Evidence: Expert opinion 7).

References

1 Ito M, Harada T, Lang L, Saga T, Kanagawa M, Matsuda R et al.. Hairy Cell Leukemia-Japanese Variant: Report of a Patient and Literature Review. International journal of surgical pathology 2022. link 2 Velazquez-Kennedy K, Crowe C, Craven B, Walsh J, Prendergast C, Krawczyk J. Audit of compliance with the British Committee for Standards in Haematology (BCSH) revised guidelines for the diagnosis and assessment of treatment response of hairy cell leukemia in University Hospital Galway. Irish journal of medical science 2017. link 3 Robak T, Smolewski P. New mutation in hairy cell leukemia. Blood 2015. link 4 Grever MR. Hairy cell: young living longer but not cured. Blood 2014. link 5 Dick JE. Tumor archaeology: tracking leukemic evolution to its origins. Science translational medicine 2014. link 6 Cawley JC. Hairy cell leukemia and the microenvironment. Leukemia & lymphoma 2011. link 7 Habermann TM, Rai K. Historical treatments of in hairy cell leukemia, splenectomy and interferon: past and current uses. Leukemia & lymphoma 2011. link 8 Hoyer JD, Li CY, Yam LT, Hanson CA, Kurtin PJ. Immunohistochemical demonstration of acid phosphatase isoenzyme 5 (tartrate-resistant) in paraffin sections of hairy cell leukemia and other hematologic disorders. American journal of clinical pathology 1997. link 9 Matutes E, Meeus P, McLennan K, Catovsky D. The significance of minimal residual disease in hairy cell leukaemia treated with deoxycoformycin: a long-term follow-up study. British journal of haematology 1997. link 10 Wheaton S, Tallman MS, Hakimian D, Peterson L. Minimal residual disease may predict bone marrow relapse in patients with hairy cell leukemia treated with 2-chlorodeoxyadenosine. Blood 1996. link 11 Patsner B, Penney RW, Walsh CM. Recurrent hairy cell leukemia during pregnancy: a case report. American journal of obstetrics and gynecology 1994. link70163-6) 12 Singh B, Verma R, Singh T, Prakash S, Gaiha M, Garg KC. Value of splenic aspirate in the diagnosis of hairy cell leukaemia. The Journal of the Association of Physicians of India 1990. link 13 Diez Martin JL, Li CY, Banks PM. Blastic variant of hairy-cell leukemia. American journal of clinical pathology 1987. link 14 Gualtieri RJ, Wheby MS. Hairy cell leukemia: a case of cryptococcosis appearing as hairy cell meningitis. Southern medical journal 1984. link 15 den Ottolander GJ, Jansen J. The effect of anticoagulant on the cell size of hairy cells and other malignant hematologic cells. A study with the Hemalog D. American journal of clinical pathology 1984. link 16 Tubbs RR, Savage RA, Sebek BA, Fishleder A, Weick JK. Antigenic phenotype of splenic hairy cells. The American journal of medicine 1984. link90774-5) 17 Arkel YS, Lake-Lewin D, Savopoulos AA, Berman E. Bone lesions in hairy cell leukemia. A case report and response of bone pains to steroids. Cancer 1984. link53:11<2401::aid-cncr2820531106>3.0.co;2-r) 18 Waxman JH. Hairy cell sarcoma. Postgraduate medical journal 1983. link 19 Kanakura Y, Machii T, Kurata Y, Tamaki T, Yonezawa T, Tarui S et al.. A human B lymphocyte antigen (P-76) shared by B-cell chronic lymphocytic leukemia cells and hairy cell leukemia cells. Blut 1983. link 20 Myers TJ, Granville NB, Witter BA. Hairy cell leukemia and sarcoid. Cancer 1979. link43:5<1777::aid-cncr2820430530>3.0.co;2-8) 21 Loukas DF. Leukemic reticuloendotheliosis: a medical technologist's diagnosis. The American journal of medical technology 1976. link

Hairy cell leukemia