1、血小板低下症的区分及血小板低下症的区分及血小板输注合理评估血小板输注合理评估 张志升张志升 台湾台大医院输血医学科台湾台大医院输血医学科 2016/6/26 Content 血小板低下症的原因 新生儿血小板低下症 药物诱导血小板低下症 血栓性血小板低下症 特发性血小板减少紫癜 肝素诱导血小板低下症 血小板低下症的原因血小板低下症的原因 骨髓制造血小板的功能减少 血小板制造可因放射线、化学治疗或病毒感染等原因受抑制，而骨髓本身疾病，如再生不良性贫血、白血病，骨髓分化不良等皆可引起制造减少。血小板在周边血液被破坏增加 常见的有经由产生自体免疫抗体来破坏，如全身性红斑性狼疮、特发性血小板减少性紫斑症
2、、某些药物等，而病毒感染也常引起血小板低下，如登革热病毒、人类免疫缺乏病毒等。此外血小板低下也常见于脾脏肿大病人，如肝硬化病人因门脉高压引起脾脏肿大，造成血小板破坏增加。Evaluation of a child with thrombocytopenia Platelet count3 mouths CBC,blood smear evaluation Anemia+thrombocytopenia pancytopenia Platelet clumps present pseudothrombocytopenia Ill appearing?No Congenital anomalies
3、?Yes No PMN hypersegmentation RBC macroovalocytosis?B12 or RBC folate B12 or folate deficiency Medications Immunizations Irradiation Toxins?No Yes Macrothrombocytes Drug-induces Live immunization Irradiation Toxins Yes No Other morphologic platelet changes No other platelet change Bone marrow Cyanot
4、ic heart disease Fanconi anemia Dyskeratosis congenita Trisomy 13 or 18 Syndromes:Kasabach-Merritt TAR Alport variants Syndromes:May-hegglin Hermansky-Pudiak Gray platelet ITP Hereditary thrombocytopenia Bernard-Soulier NI megakaryocytes megakaryocytes Leukemia Aplastic anemia Drug-induced Amegakary
5、ocytic thrombocytopenia Myelodysplasia ITP is a diagnosis of exclusion Response to therapy,if needed(corticosteroid,IVIG,anti-D antibody),confirms the diagnosis Yes PTT,PT,TT prolonged DIC R/O sepsis See Consumptional coagulopathy Normal Spleen Signs of portal hypertension platelet 50,000+/-pancytop
6、enia Male Eczema Recurrent infection Small platelets Lymphadenopathy Hepatosplenomegaly Superior vena cava syndrome Abdominal mass Chronically ill appearing Acute,fibrile illness WBC enzyme assays Ultrasonography Thick smear Biopsy of lymph node,mass or bone marrow consider tumor lysis and superior
7、vena cava syndromes HIV assay ANA U/A Renal function Blood culture?antibiotics Malaria Gaucher disease Portal hypertension Hepatic schistosomiasis Cavernous transformation of the portal vein Wiskott-Aldrich syndrome Lymphoma:Hodgkin Non-Hodkin Neuroblastoma leukemia Myelodysplasia HIV Autoimmune or
8、connective tissue disease HUS/TTP+other microangiopathies Prosthetic cardiac valve R/O ADMAT-13 DAT Auto/allo anti-platelet antibody Sepsis Varicella EBV CMV Denque hemorrhagic fever HIV HUS Hantavirus Parvovirus Other viruses TTP Auto/all anti-platelet antibodies study Heparin-induced thrombocytope
9、nia Check PF4 Thrombocytopenia in the ill neonate Any etiology of thrombocytopenia that occurs in the well child History,examination,CBC,blood smear evaluation See Thrombocytopenia in the well neonate Platelets 100,000149,000/uL Platelets 100,000/uL If platelets 150,000/uL no further evaluation 100,
10、000149,000 continue to fellow PTT,PT,TT High Hb Severe jaundice and low Hb Prolonged PTT,PT and/or TT+/-microangiopathic hemolytic anemia:Consider D-dimer of FSP,and/or fibrinogen+/-factors II,V and VIII Polycythemia Cyanotic congenital heart disease Erythroblastosis fetalis Exchange transfusion p p
11、hototherapy DIC Etologies Acute infection Asphyxia RDS Meconium aspiration Obstetrical complications Shock Thrombosis Severe hemolytic disease of the newborn Severe hepatic disease TP usually mild enough not to require transfusion except in DIC due to erythroblastosis fetalis Treat underlying diseas
12、e Maintain platelets 50,000 with transfusions Maintain fibrinogen 1.0g/L and PT WNL with FFP+/-cyrorecipitate Normal PTT,PT,TT RDS Pulmonary hypertansion Meconium aspiration Mechanical ventilation Infection Viral Bacterial Fungal Perinatal asphyxia No other specific etiology identified Unknown etiol
13、ogy Ongoing re-evaluation if platelets 20,000 in stable full term neonates,50,000 with hemorrhage,surgery,or more extremely preterm infants Observe for DIC 免疫性血小板低下免疫性血小板低下 Neonatal alloimmune thrombocytopenia(NAIT)Platelet transfusion refractoriness(PTR)Post-transfusion thrombocytopenic purpura(PTP
14、)Passive alloimmune thrombocytopenia(PAT)Transplantation-associated alloimmune thrombocytopenia(TAATP)Antigen NAIT PTR PTP PAIT TAATP HPA(+)(+)(+)(+)(+)ABH(+)(+)(-)(?)(?)Class I HLA (+)?(+)(-)(?)(?)CD36(+)(+)(+)?(?)(?)Alloantigens implicated in allo immune thrombocytopenia Dr.N.H.Tsuno presented in
15、24th regional congress of ISBT 血小板相关抗体疾病或异常血小板相关抗体疾病或异常 新生儿免疫性血小板减少症NAITP 输血后紫斑症PTP 血小板输血无效症PTR 免疫性血小板低下紫瘢症ITP 血栓性血小板低下紫癜TTP 肝素刺激血小板低下症HIT 药物抗体血小板低下症DIT 严重输血相关呼吸窘迫症候群TRALI 即输血小板两次以上无法达到预期血小板的增加数称为 “血小板输注无疗效”成因:异体免疫,自体抗体,ABO血型不符,病人因素(如急性出血或组织移植排斥.等),药物治疗(如抗生素vancomycin.)所引起 血小板输血后的品质评估:1小时后其“校正血小板增加数
16、”7000,此方法亦为侦测有无“异体免疫”的间接方式 CCI:(输血后血小板数-输血前血小板数)X BSA/输注血小板量。血小板输注无效症血小板输注无效症 与血小板抗体有关之疾病：与血小板抗体有关之疾病：Neonatal alloimmune thrombocytopenia 白种人中此病之报告较多，母体之抗血小板抗体进入胎儿内，造成新生儿之血小板异常低下，此病可以生于第一胎。西方人报告中以HPA-1a 抗体为主(又名anti-plA1)，多发生于HPA-1a阴性且HLA-DR3*0101之妇人。日本则曾报告过HPA-4b抗体引起之新生儿血小板减少症 Post-transfusion purpura(PTP)输血后紫斑症，病人输血后发生血小 板异常降低，引起反应的血品包括血小板，红血球等。部份输血病人体内可以验出血小板抗体，文献报告中最多的也是HPA-1a抗体(anti-plA1)Content 血小板低下症的原因 新生儿血小板低下症 药物诱导血小板低下症 血栓性血小板低下症 特发性血小板减少紫癜 肝素诱导血小板低下症 Neonatal Alloimmune Thrombocytope