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,*,单击此处编辑母版标题样式,单击此处编辑母版文本样式,第二级,单击此处编辑母版标题样式,单击此处编辑母版文本样式,第二级,*,Hairy cell leukemia,past,present,furture by Yehonghui,1,Hairy cell leukemiapast,presen,Introduce,extremely rare form of leukemia,middle aged men,pancytopenia and splenomegaly,long life span,2,Introduceextremely rare form o,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,3,history of HCL(1923-1953)Edwar,4,4,5,5,history of HCL(1958-1974),named as hairy cell leukemia,m,icroscopic sign,median mature lyphocyte,cytoplasm pseudopods protruding,serrated border,lymphoproliferative disorder,6,history of HCL(1958-1974)named,7,7,ACP and TRAP,biopsy,elctron microscope,8,ACP and TRAP8,Bone marrow biopsy in HCL reticulin stain,9,Bone marrow biopsy in HCL reti,10,10,predict clinic outcome,splenectomy,67%remained HCR after 6 month,5 years OS 61%,chlorambucil,11,predict clinic outcome11,in 80s,interferon-alfa,300m u/m,2,3time per week and lasted for one year,side-effect,2-4-8,ORR 70%CR 8%,12,in 80sinterferon-alfa 12,in 90s,Aetiology HTLV EBV HPV-B,+5 del(5q13),Origin of HC CD19,+,CD20,+,CD22,+,SIg,+,CD10,-,PCA-1,Scretion TNF-alfa IL-6,13,in 90s13,in 90s,purine nucleoside analogs,Pentostatin 4mg/m,2,/2W total 8 times,ORR 79%CR 76%,Cladribine 0.1mg/Kg/day for 7days,ORR 97%CR 85%,not identical therapy,14,in 90spurine nucleoside analog,15,15,Cladribine:recurrence rate 26%,median time 29 months,Side effect:progressively worse response,cumulative myelotoxic effect,second tumor,16,Cladribine:recurrence rate,In The New Era,Multi-colored Flow Cytometry,Gene mutation BRAF-MEK-ERK pathway,Immunotherapy or targeted therapy,17,In The New EraMulti-colored Fl,18,18,Expert consensus on diagnosis of B cell chroniclymphoproligerative disorders in China,2014,19,Expert consensus on diagnosis,20,20,Rituximab,Expression of CD20 antigen,As a single agent,New 375mg/m,2,weekly,4-8,CR 64%,Replase,375mg/m,2,weekly,4-8 CR 53%,As a,combination,New,375mg/m,2,weekly,4-8,CR 100%,21,RituximabExpression of CD20 an,22,22,Treatment algorithm,23,Treatment algorithm23,BRAF mutation,Tiacci in 2009 fist described in melanoma,100%harbored BRAF V600E mutation orign?,Vemurafenib,inducing hairy cells,apoptosis,24,BRAF mutationTiacci in 2009 f,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,25,Vemurafenibphase 2 multicente,26,26,27,27,28,28,29,29,30,30,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,31,HCL Variant10%of HCL cases31,32,32,33,33,IgHV 4-34 rearrangement and others,MEK inhibition,Classified as a separate entity by WHO 2008,34,IgHV 4-34 rearrangement and ot,Future Direction,Optimizing therapy of relapsed patients,role of MRD,role of ongoing therapy,BRAF-MEK-ERK pathway,35,Future DirectionOptimizing th,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,36,Summaryrare cas classified as,Treatment strategies,:,purine nucleoside analogs,Immunotherapy or targeted therapy,normal life expectation,37,Treatment strategies:purine n,Timeline,38,Timeline38,Thank you for your attention,39,Thank you for your attention39,
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