毛细胞白血病PPT课件.ppt

Hairy cell leukemia past,present,furture by Yehonghui The Fifth Affiliated Hospital of Sun Yat-sen University Introduce extremely rare form of leukemia middle aged men pancytopenia and splenomegaly long life span history of HCL(1923-1953) Edward in 1923 described splenomegaly without lymphadenopathy pancytopenia with lymphocytosis and monocytopenia Gosselin in 1944-1953 3 distinctive subtype bone lesion cutaneous manifestation ACP and TRAP biopsy elctron microscope Bone marrow biopsy in HCL reticulin stain predict clinic outcome splenectomy 67% remained HCR after 6 month 5 years OS 61% chlorambucil in 80s interferon-alfa 300m u/m2 3time per week and lasted for one year side-effect 2-4-8 ORR 70% CR 8% in 90s Aetiology HTLV EBV HPV-B +5 del(5q13) Origin of HC CD19+CD20+CD22+SIg+ CD10- PCA-1 Scretion TNF-alfa IL-6 in 90s purine nucleoside analogs Pentostatin 4mg/m2/2W total 8 times ORR 79% CR 76% Cladribine 0.1mg/Kg/day for 7days ORR 97% CR 85% not identical therapy Cladribine: recurrence rate 26%, median time 29 months Side effect: progressively worse response cumulative myelotoxic effect second tumor In The New Era Multi-colored Flow Cytometry Gene mutation BRAF-MEK-ERK pathway Immunotherapy or targeted therapy Expert consensus on diagnosis of B cell chroniclymphoproligerative disorders in China 2014 Rituximab Expression of CD20 antigen As a single agent New 375mg/m2 weekly ×4-8 CR 64% Replase 375mg/m2 weekly ×4-8 CR 53% As a combination New 375mg/m2 weekly ×4-8 CR 100% Treatment algorithm BRAF mutation Tiacci in 2009 fist described in melanoma 100% harbored BRAF V600E mu