Cladribine therapy in a patient with an aleukemic subvariant of mast cell leukemia
Charité Universitätsmedizin Berlin, Berlín, Berlin, GermanyAnnals of Hematology (Impact Factor: 2.63). 11/2005; 84(10):692-3. DOI: 10.1007/s00277-005-1057-x
Article: Rare Leukemias[Show abstract] [Hide abstract]
ABSTRACT: The leukemias included in this chapter are those seen infrequently even by physicians specializing in leukemia in cancer centers and tertiary referral centers. Some are rare primary leukemias (e.g. chronic neutrophilic leukemia [CNL], acute megakaryocytic leukemia, hairy cell leukemia [HCL]), while others are rare primary leukemic presentations of neoplasms that do not usually present as leukemias (e.g. mantle cell leukemia, plasma cell leukemia [PCL]). The prognosis of some rare leukemias has improved with the development of novel and targeted therapies.
Article: Treatment of Systemic Mastocytosis[Show abstract] [Hide abstract]
ABSTRACT: Systemic mastocytosis is a heterogeneous disorder characterized by diverse signs and symptoms. No curative therapy presently exists. Conventional management has relied on agents that antagonize mediators released by mast cells, inhibit mediator secretion, or modulate mast cell proliferation. Recent advances in our molecular understanding of the pathophysiology of systemic mastocytosis have provided new therapeutic considerations. Conventional management and promising novel agents are summarized in this review.
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ABSTRACT: Mastocytosis is a heterogeneous group of rare diseases characterized by the proliferation and accumulation of mast cells in one or more organs such as the skin, bone marrow, liver, spleen, and lymph nodes. According to the WHO classification, mastocytosis is divided into seven subvariants. The symptoms are associated with mediator release and impaired organ function due to infiltration by neoplastic mast cells. There is a higher risk of anaphylactic shock; therefore education of the patients is very important. Patients may be asymptomatic. Symptomatic treatment is used in cutaneous mastocytosis and in indolent systemic mastocytosis. More aggressive subvariants of mastocytosis are treated with chemotherapy, targeted therapy, and bone marrow transplantation.
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