《毛细胞白血病》由会员分享，可在线阅读，更多相关《毛细胞白血病（39页珍藏版）》请在金锄头文库上搜索。
1、Hairy cell leukemia,past,present,furture by Yehonghui,The Fifth Affiliated Hospital of Sun Yat-sen University,Introduce,extremely rare form of leukemiamiddle aged menpancytopenia and splenomegalylong life span,history of HCL(1923-1953),Edward in 1923 describedsplenomegaly without lymphadenopathypanc
2、ytopenia with lymphocytosis and monocytopeniaGosselin in 1944-1953 3 distinctive subtypebone lesioncutaneous manifestation,history of HCL(1958-1974),named as hairy cell leukemiamicroscopic signmedian mature lyphocytecytoplasm pseudopods protruding serrated borderlymphoproliferative disorder,ACP and
3、TRAPbiopsyelctron microscope,Bone marrow biopsy in HCL reticulin stain,predict clinic outcomesplenectomy 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 yearside-effect2-4-8ORR 70% CR 8%,in 90s,Aetiology HTLV EBV HPV-B+5 de
4、l(5q13)Origin of HC CD19+CD20+CD22+SIg+ CD10- PCA-1Scretion TNF-alfa IL-6,in 90s,purine nucleoside analogsPentostatin 4mg/m2/2W total 8 timesORR 79% CR 76%Cladribine 0.1mg/Kg/day for 7daysORR 97% CR 85%not identical therapy,Cladribine: recurrence rate 26%, median time 29 monthsSide effect: progressi
5、vely worse responsecumulative myelotoxic effectsecond tumor,In The New Era,Multi-colored Flow CytometryGene mutation BRAF-MEK-ERK pathwayImmunotherapy or targeted therapy,Expert consensus on diagnosis of B cell chronic lymphoproligerative disorders in China 2014,Rituximab,Expression of CD20 antigenA
6、s a single agentNew 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 melanoma100% harbored BRAF V600E mutation orign?Vemurafenib inducing hairy cellsapoptosis,Vemurafenib,p
7、hase 2 multicenter studyearly replase, refractory to PA,bone marrowhypoplasia at the time of relapse,severe sideeffect960 mg twice daily for a minimum of 8 weeks ORR 96-100% medium response time 8-12w,HCL Variant,10% of HCL casesSimilarity: age gender splenomegaly anemia etc.morphologyDissimilarity:
8、 higher white blood cell countlack of monocytopeniaabsent of Annex-1 CD25 BRAF V600Eless durable responses to PAmore aggressive,IgHV 4-34 rearrangement and othersMEK inhibitionClassified as a separate entity by WHO 2008,Future Direction,Optimizing therapy of relapsed patientsrole of MRD role of ongo
9、ing therapyBRAF-MEK-ERK pathway,Summary,rare cas classified as B-CLPD in WHO 2008Clinical manifestations:splenomegaly pancytopenia bone lesion skin lesionLaboratory examination: blood bone marrow biopsyMFC molecular biologyDifferential Diagnosis with other B-CLPDMyelofibrosis and Hypersplenism,Treatment strategies: purine nucleoside analogsImmunotherapy or targeted therapynormal life expectation,Timeline,Thank you for your attention,
