Publications by authors named "E Maitre"
Hairy cell proliferations represent very different entities. They include hairy cell leukemia in its classic form (HCL), a well-defined entity, but also the variant form of HCL (LT-V ou HCL-V), whose presentation is far from HCL and whose prognosis is poorer. Other hairy cell proliferations include splenic red pulp lymphoma (SDRPL) and splenic marginal zone lymphomas (SMZL) with circulating villous cells.
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- Hairy-cell leukemia (HCL) is a rare blood cancer that has improved prognosis due to treatments like purine nucleoside analogs, but some patients experience relapses and chemotherapy resistance.
- Recent advances in understanding HCL's genetic and cellular mechanisms have led to the development of targeted therapies, including BRAF and MEK inhibitors.
- Updated guidelines emphasize the importance of BRAF mutation for diagnosis, the relevance of certain immunoglobulin mutations for prognosis, and recommend strategies for managing both HCL and similar disorders.
Disease Overview: Hairy cell leukemia (HCL) and HCL-like disorders, including HCL variant (HCL-V) and splenic diffuse red pulp lymphoma (SDRPL), are a very heterogenous group of mature lymphoid B-cell disorders characterized by the identification of hairy cells, a specific genetic profile, a different clinical course and the need for appropriate treatment.
Diagnosis: Diagnosis of HCL is based on morphological evidence of hairy cells, an HCL immunologic score of 3 or 4 based on the CD11c, CD103, CD123, and CD25 expression, the trephine biopsy which makes it possible to specify the degree of tumoral bone marrow infiltration and the presence of BRAF somatic mutation.
Risk Stratification: Progression of patients with HCL is based on a large splenomegaly, leukocytosis, a high number of hairy cells in the peripheral blood, and the immunoglobulin heavy chain variable region gene mutational status.
Article Synopsis
- A 33-year-old man had headaches and vision problems, and doctors found a mass in his brain that was linked to HCL.
- After treatment with special medicines, he got better quickly and is doing well a year later; other similar cases show that the same treatments can work for more patients with HCL in the brain.