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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.
Specific Therapy for Leukemias
Published in Tariq I Mughal, John M Goldman, Sabena T Mughal, Understanding Leukemias, Lymphomas, and Myelomas, 2017
Tariq I Mughal, John M Goldman, Sabena T Mughal
Most patients with hairy cell leukemia require treatment. In less than 10% of patients, who are elderly and have an impalpable spleen, the course is indolent and no treatment may be required. In the past splenectomy was the initial choice and almost all patients benefited for at least a few months, largely owing to fewer infections and lesser blood transfusion requirements.
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].