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Haematological malignancy
Published in Peter Hoskin, Peter Ostler, Clinical Oncology, 2020
Hairy cell leukaemia usually has a long indolent course. It responds to treatment with interferon with which it may remain in remission for many years before it becomes resistant and bone marrow failure develops. The new purine analogue drugs pentostatin and cladribine are also highly active in this disease and can be used instead of interferon.
Lymphocyte and plasma cell malignancies
Published in Gabriel Virella, Medical Immunology, 2019
Juan Carlos Varela, Gabriel Virella
Hairy cell leukemia is a rare lymphoid B-cell malignancy, predominantly affecting middle-age males (median age of diagnosis: 55 years). The clinical presentation is nonspecific and includes malaise, fatigue, and frequent infectious episodes. The physical examination usually shows splenomegaly and, sometimes, generalized lymphadenopathy. Laboratory data show pancytopenia, anemia being most frequent, followed by thrombocytopenia, and leukopenia. The diagnosis is based on the finding of atypical lymphocytes with numerous finger-like (or hairy) projections in the peripheral blood (the name of the disease derives from the morphological characteristics of the abnormal lymphocytes). The abnormal cells express light-chain membrane immunoglobulins, often of several isotypes (IgG3 often predominating), and also express classical B-cell markers (CD19, CD20, CD22).
Pathology of the Spleen
Published in Harold R. Schumacher, William A. Rock, Sanford A. Stass, Handbook of Hematologic Pathology, 2019
Hairy cell leukemia is a B-cell malignancy that manifests characteristics splenic and peripheral blood morphology, cytochemistry, and phenotype. Splenomegaly occurs frequently and demonstrates a leukemic infiltrate involving the red pulp. The cut surface is beefy red, and on microscopic examination reveals infiltration with hairy cells and blood lakes lined by hairy cells. Hairy cells demonstrate strong positivity for acid phosphatase even after treatment with tartrate (tartaric acid-resistant acid phosphatase granules) (TRAP). These cells express monotypic immunoglobulin light chains and coexpress CD11c (myelomonocytic marker) and CD20 (B-cell marker) antigens.
Deciphering the genotype and phenotype of hairy cell leukemia: clues for diagnosis and treatment
Published in Expert Review of Clinical Immunology, 2019
Margot C.E. Polderdijk, Michiel Heron, Saskia Kuipers, Ger T. Rijkers
Hairy cell leukemia is a B-cell malignancy with typical hairy extensions of the cell membrane. It is a relatively rare disease, accounting for 2–3% of all non-Hodgkin lymphomas. The leukemia in general responds very well to treatment with either cytostatic drugs or specific BRAF inhibitors (vemurafenib, dabrafenib). Monoclonal antibodies directed against pan-B cell antigens such as CD20 (rituximab) and CD22 (moxetumomab) are also effective. Despite the fact that HCL is a rare disease, at the foundation of its pathogenesis lies a very common oncogenic mutation in the BRAF gene. Aside from its obvious effect on the RAS/RAF/ERK signaling pathway, the case of hairy cell leukemia can provide information on its interaction with passenger mutations. HCL is rare, but they can be (like rare immunodeficiency diseases) invaluable sources of information when it comes to unearthing pathogenic mechanisms.
For Massachusetts Eye and Ear Special Issue: Updates on Therapies for Multiple Sclerosis for the Ophthalmologist
Published in Seminars in Ophthalmology, 2019
Tatiana Bakaeva, Sashank Prasad
Cladribine is a synthetic purine nucleoside analog, which is a prodrug whose active metabolite, cladribine triphosphate, causes disruption of cellular metabolism, DNA damage, and subsequent apoptosis. It was originally developed for the treatment of hairy-cell leukemia. Cladribine produces rapid and sustained reductions in CD4+ and CD8+ cells and rapid, though more transient, effects on CD19+ B cells.47 After the initial encouraging results with short treatment courses of parenteral cladribine in patients with MS in 1990s, an oral formulation was developed.
Treatment of infections in cancer patients: an update from the neutropenia, infection and myelosuppression study group of the Multinational Association for Supportive Care in Cancer (MASCC)
Published in Expert Review of Clinical Pharmacology, 2021
Bernardo L. Rapoport, Tim Cooksley, Douglas B. Johnson, Ronald Anderson, Vickie R. Shannon
Hairy cell leukemia is an uncommon malignancy, predominantly affecting older males, resembling CLL because it is associated with the proliferation of mature, monoclonal B cells found predominantly in the bone marrow, blood and spleen [23]. Important frequent presenting features with respect to immune dysfunction include neutropenia, monocytopenia, and thrombocytopenia that may predispose to repeated infections [23].