毛细胞白血病--培训课件.pptVIP

HCL Variant 10% of HCL cases Similarity: age gender splenomegaly anemia etc. morphology Dissimilarity: higher white blood cell count lack of monocytopenia absent of Annex-1 CD25 BRAF V600E less durable responses to PA more aggressive IgHV 4-34 rearrangement and others MEK inhibition Classified as a separate entity by WHO 2008 Future Direction Optimizing therapy of relapsed patients role of MRD role of ongoing therapy BRAF-MEK-ERK pathway Summary rare cas classified as B-CLPD in WHO 2008 Clinical manifestations:splenomegaly pancytopenia bone lesion skin lesion Laboratory examination: blood bone marrow biopsy MFC molecular biology Differential Diagnosis with other B-CLPD Myelofibrosis and Hypersplenism Treatment strategies: purine nucleoside analogs Immunotherapy or targeted therapy normal life expectation Timeline 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). Purin