毛细胞白血病.ppt

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 mutation orign? Vemurafenib inducing hairy cells apoptosis Vemurafenib phase 2 multicenter study early replase, refractory to PA,bone marrow hypoplasia at the time of relapse,severe side effect 960 mg twice daily for a minimum of 8 weeks ORR 96-100% medium response time 8-12w 2% of leukemia and about 600-1000 new cases a year in US 4-5:1 male: female 50months * * 坏疽性脓皮病 红皮病 皮肤瘤 * lymphocproliferative disorder * fried eggs * dry tap ribosome-lamellae complexes * infection and massive splenomegaly temporary improvement with post-surgery and progressive patients * uncleared machanism regimen after one year? 2-4 weeks flu-syndrome will dismiss HC disappear in 2 weeks anemia rectified in 4 weeks plalete restored in 8weeks 1992 splenectomy vs interferon-alfa * CLL * MEK mitogen-activated protein-ERK kinanse ERK extracellular signal-regulated kinase * Mechanism of action of currently used therapies and potential therapeutic targets in hairy cell leukemia (HCL). Intracellular signaling pathways in HCL B cells are shown. B-cell receptor (BCR) and Raf-MEK/ERK signaling are the dominant pathways with major therapeutic implications in HCL. The pink lightning bolt symbol indicates therapeutic targets. (a) Purine analogues (shown in dark green). Purine analogues are the most commonly used chemotherapeutic agents in HCL. Cladribine and pentostatin inhibit DNA polymerase and adenosine deaminase, prevent DNA repair, and promote DNA damage. (b) Monoclonal antibodies (mAbs)