[HTML][HTML] Hairy cell leukemia 2018: Update on diagnosis, risk‐stratification, and treatment
X Troussard, E Cornet - American Journal of Hematology, 2017 - ncbi.nlm.nih.gov
X Troussard, E Cornet
American Journal of Hematology, 2017•ncbi.nlm.nih.govDisease overview: Hairy cell leukemia (HCL) and HCL-like disorders, including HCL variant
(HCLV) and splenic diffuse red pulp lymphoma (SDRPL), are a very heterogeneous 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 …
(HCLV) and splenic diffuse red pulp lymphoma (SDRPL), are a very heterogeneous 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 …
Abstract
Disease overview: Hairy cell leukemia (HCL) and HCL-like disorders, including HCL variant (HCLV) and splenic diffuse red pulp lymphoma (SDRPL), are a very heterogeneous 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 medullary infiltration and the presence of BRAF V600E 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. VH4-34 positive HCL cases are associated with poor prognosis
Risk adapted therapy: Purine analogs (PNA) are indicated in symptomatic first line HCL patients. The use of PNA followed by rituximab represents an alternative option.
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