关于血小板减少的二三事.pptx
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关于血小板减少的二三事 Thrombocytopenia 施焘 08-14 生理学小知识 1 造血干细胞巨核系祖细胞原始巨核细胞巨幼核细胞 成熟巨核细胞megakaryocyte血小板 (8-10d) 2 约2/3在外周循环血液中,1/3在脾脏 3 mm in diameter= petechiae; 3 mm = ecchymoses) platelet disorder: thrombocytopenia, defect in platelet function thromboemboli: DIC,TTP, cholesterol or fat emboli trauma or vascular fragility: amyloidosis, Ehlers-Danlos, scurvy Palpable (papular) vasculitis: leukocytoclastic, HSP, PAN, RMSF infectious emboli: meningococcemia, bacterial endocarditis Risk of Plt Thrombocytopenia and Risk of Bleeding Platelet count (cells/L)Risk 50,000-100,000Risk with major trauma; can proceed with general surgery 20,000-50,000Risk with minor trauma or surgery 20,000Risk of spontaneous bleeding (less so with ITP) 10,000Risk of severe, life-threatening bleeding ThrombocytopeniaPlt 100,000/L Etiologies Exclude pseudothrombocytopenia True thrombocytopenia production destruction Abnormal distribution or pooling Unknown Pseudothrombocytopenia Platelet clumping in EDTA 0.1% have EDTA- dependent agglutinins 凝集素 a naturally occurring platelet autoantibody directed against a normally concealed epitope on the platelet membrane glycoprotein (GP)IIb/IIIa,which becomes exposed by EDTA- induced dissociation of GPIIb/IIIa repeated usingheparin or sodium citrate as an anticoagulant Production Hypocellular bone marrow: aplastic anemia, rarely MDS, drugs (eg, thiazides噻嗪类, antibiotics), alcohol, cirrhosis Hypercellular bone marrow: MDS, leukemia, severe megaloblastic anemia Marrow replacement: myelofibrosis, hematologic and solid malignancies, granulomas Destruction immune-mediated (distinguish primary from secondary) Primary (idiopathic): immune thrombocytopenic purpura (ITP) Secondary: infxn (HIV, HCV,HSV单疱), collagen vascular diseases (SLE), APS, lymphoproliferative (CLL, lymphoma), drugs (many, including heparin, abciximab 阿昔单抗, quinidine 奎尼丁, sulfonamides, vancomycin), alloimmune (posttransfusion) nonimmune-mediated: MAHA (DIC, HUS,TTP), ticlopidine 噻氯匹定/clopidogrel 氯吡格雷, vasculitis, preeclampsia/HELLP syndrome, cardiopulmonary bypass, CVVH, IABP 主动脉内球囊泵, cavernous hemangioma 海绵状血管瘤 Abnormal distribution or pooling: splenic sequestration, dilutional, hypothermia Unknown: ehrlichiosis 埃里希体病/anaplasmosis 边虫病, babesiosis 巴贝西虫病, RMSF 落矶山斑点热 Diagnostic evaluation H can see inclusion bodies (anaplasma), parasites (babesia) r/o pseudothrombocytopenia due to platelet clumping (platelet count in nonEDTA-containing tube, eg, citrate-containing yellow top tube) Approach to thrombocytopenia Diagnostic evaluation Additional laboratory evaluations as indicated (eg. Viral filters, flow cytometry, ANA, APLA) if anemia:reticulocyte count, LDH, haptoglobin, bilirubin to detect hemolysis if hemolytic anemia:PT, PTT, fibrinogen, D-dimer, Coombs, ANA BM bx for unexplained thrombocytopenia, esp. if associated with splenomegaly 谢谢!展开阅读全文
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