内科学-培训课件PPT
内科学-培训课件PPT,内科学,培训,课件,PPT
特发性血小板减少性紫癜特发性血小板减少性紫癜(idiopathic thrombocytopenic purpura,ITP)Huang Zhen-qian,ProfessorDept.of Hematology The First Affiliated Hospital,Guangzhou Medical U1IntroductionPathophysiologyManifestationDiagnosisTreatmentPrognosisContent2 1.Frequency(per year)50-100/million in adults 50/million in children New refractory cases:10/million ITP.Introduction32.Mortality/MorbidityThe mortality from hemorrhage:1%in children 5%in adults In patients with severe Plt,5-year mortality from bleeding 60 yrs:47.8%40 yrs:2.2%Spontaneous remission:80%in children uncommon in adults.Introduction43.Sex:In chronic ITP(adults),females 72%In acute ITP(children),males:52%females:48%4.Age:Adults:peak age 20-50 years.Children:peak age 2-4 years,40%of patients 10 years old.ITP/Introduction5 Three important elements:Antigen:Plt membrane glycoproteins(GP):GPb,b,a,a,a Antibody Plt destruction caused by macrophagesITP.Pathophysiology6AgFC-ROrgans producing Ab:Spleen Bone marrow(BM)Other lymphatic tissue Where are Plts destructed?Spleen Liver BM7 1.Bleeding Purpura Menorrhagia Epistaxis Gingival bleeding GI bleeding Hematuria Retinal hemorrhages Intracranial hemorrhage 2.Nonpalpable spleen:97%in adults 88%in childrenSpontaneous bleeding when Plt 20 x109/LITP/manifestation.Manifestation8 30%asymptomaticHeterogeneous presentation9 v血小板计数:血小板计数:急性型急性型 多多 202010109 9/L/L 慢性型慢性型 303080 80 10109 9/L/L 血小板形态:血小板形态:平均体积偏大,易见大型血小板平均体积偏大,易见大型血小板 出血时间延长,血块收缩不良;出血时间延长,血块收缩不良;血小板功能一般正常。血小板功能一般正常。Investigation of Lab10v骨髓像:骨髓像:急性型:巨核细胞数量轻度增加或正常急性型:巨核细胞数量轻度增加或正常 慢性型:巨核细胞显著增加慢性型:巨核细胞显著增加巨核细胞发育成熟障碍,急性型表现为巨核细胞发育成熟障碍,急性型表现为 幼稚型幼稚型巨核细胞增加;慢性型巨核细胞增加;慢性型颗粒型颗粒型巨巨 核细胞显著增加核细胞显著增加产板型产板型的巨核细胞显著减少(的巨核细胞显著减少(30%30%)红系及粒、单核系正常。红系及粒、单核系正常。11vPAIgPAIg及及PAC3PAC3:80%80%的患者的患者PAIgPAIg及及PAC3+PAC3+主要为主要为IgG,IgG,亦可为亦可为IgM,IgM,偶偶然有两种以上的抗体同时出现。然有两种以上的抗体同时出现。v其它:其它:90%90%以上患者血小板生存时间明显缩短;可有程度不等以上患者血小板生存时间明显缩短;可有程度不等的正常红细胞或小细胞低色素贫血,少数可发现溶血的正常红细胞或小细胞低色素贫血,少数可发现溶血证据(证据(Evans Evans 综合征)。综合征)。12 1.Pltwith or without bleeding2.No or mild splenomegaly3.Megakaryocytesor normal4.No other diseases causing Plt5.Good response to steroid6.Good response to splenectomy ITP/diagnosis.Diagnosis13 BM megakaryocyte number,relation between the number and prednison response(70 cases )megakaryocyte cases(%)prednison response increased 38(54.3)better normal 24(34.3)decreased 8 (11.4)ITP/diagnosis14 Acute ITPChronic ITPAge 26ys2024yssexNo differenceMore common in female,ratio of man/woman:1:3Exciting causeHistory of infection before1-3 weeksNo obviouslyonsetSuddenly,chilly,feverslowingbleedingSeverity,mucous membrane and internal organs bleedingPetechiae,menorrhagiaplatelet80%spontaneous remissionRecurrent attacks,no spontaneous remissionDifferential diagnosis of acute and chronic ITP15 Other Problems to be Considered:Other hematological disorders Autoimmune Infection(AIDS,ect.)Liver disease,Malignancy Drug-induced immune Plt (alcohol,heparin,quinidine,sulfonamides)Pregnancy-associated Plt TTP,DIC,Transfusion Pseudo-Plt(Plt clumping in EDTA)ITP/diagnosis16皮肤粘膜出血皮肤粘膜出血实验室室检查1.血小板血小板计数减少数减少2.脾脾脏正常或稍大正常或稍大3.3.骨骨髓髓巨巨核核细胞胞增增多多或或正正常常,成熟障碍成熟障碍4.有以下其中一有以下其中一项的的:1)泼尼松治尼松治疗有效;有效;2)切脾治切脾治疗有效;有效;3)PAIgG增多;增多;4)PAC3增多;增多;5)血小板寿命血小板寿命缩短短排除排除继发性性ITPSLE 抗磷脂抗磷脂综合征合征确确诊询问病史病史查体体1出血症状出血症状2疫苗接种情况疫苗接种情况3近近期期药物物应用用史史4HIV感感染染的的高高危危因素因素5家族史家族史vSafty value of platelet number dental check:10109/L tooth extraction or filling:30109/L minor surgery:50109/L major operation:80109/L spontaneous delivery:50109/L caesarean section:80109/L18 Basic regimen Efficacy Time of Plt Glucocorticoids 80%2-5 d Splenectomy 80%1-2 dIVIg 2-3 d.Treatment19 Guidelines for special therapy Children AdultsNot Plt 30 x109/L:Plt50 x109/L treatment asymptomatic or Plt 30-50 x109/L:only minor purpura only minor purpura Treatment Plt 30 x109/L Plt50 x109/L with significant mucous bleeding Plt 10-20 x109/L Plt20-30 x109/L with minor purpura with risk factors for bleeding Risk factors for bleeding Hypertension,Peptic ulcer Vigorous lifestyleITP/treatment20Primary ITP in adults:therapySymptomatic Bleeding PLT 2030 x 109/LVariables Age/sex Occupation/activities Comorbidities/treatments Pregnancy OtherGoals Prevention of bleeding CureWhen?Which patients?Which therapy?211.Glucocorticoids(the first option)Dose:1-2 mg/kg/d Qd or Bid orally is better than Qod Response after days 2-5 Obvious efficacy on day 5-14 Pregnancy:Usually safe but benefits must outweigh the risks ITP/treatment22 Withdraw of steroids:when Plt to normal level over 3 weeks can not tolerance to steroids dose dropping:5mg/week Maintenance:5-10mg/day for 6-12 monthsITP/treatment232.Splenectomy Indication:6 months course at least dependent or not sensitive to steroids obvious side effects of steroids Contraindication:6 months pregnancy can not receive surgical therapy children:why?The rate of natural remission and infectious rate after operation in children is higher than in adultEmergent splenectomy:the patients with life-threatening bleeding and medical therapy fails ITP/treatment24 Efficacy of steroids and splenectomy Responsive rate short period long-term Steroids 80%10-15%Splenectomy 80%45-60%After splenectomy:Plt increase within 24-48hrs,may reach to 1000 x109/L within 10 daysUse anti-Plt durgs if Plt 800 x109/LITP/treatment253.Intravenous immune globulin(IVIg)Indication:Severe,life-threatening bleeding,Children with Plt 20 x109/L with purpuraDose:Adult 0.4g/kg for 4-5 d Children 1g/kg once Contraindications:Hypersensitivity IgA deficiency Pregnancy:Safety has not been established Precautions:Check serum IgA before IVIgITP/treatment26 Side effects:Thromboembolic events Urticaria,pruritus Petechiae(2-30 d postinfusion)Aseptic meningitis(10%)Renal tubular necrosis in elderly patients and patients with diabetes 6-fold increase in ESR for 2-3 wk HyponatremiaITP/treatment274.Plt transfusionOnly for severe hemorrhageDosage6-8 U or 1 U/10 kg1 U can increase count:5x109/L for 70kg adult 20 x109/L for 18kg childITP/treatmentPlt survival is increased if the plt is transfused immediately after IVIg infusion28 5.Other therapies CTX Rituximab Danazol Vinca alkaloids Dapsone Accessory splenectomy IFN alfa Splenic radiation 6-MP Cases are too small to show a significantreduction in bleeding or mortality rateITP/treatment29 6.Treatment of pregnancy with ITP In early:dont need special therapy:Plt 50 x109/L and no bleeding pregnance should be stopped:if steroids is necessary because of its malformation efficacy In late:take steroids to Pltor hemorrhage stopped if Plt 50 x109/L IVIg can be used if emergency ITP/treatment30 7.For refractory Cases,according to recent studies,a combination seems promising:weekly VCR weekly methylprednisolone,both until Plt reached 50 x109/L,and Cyclosporine orally twice daily until the Plt is normal for 3-6 monthsITP/treatment31 .Prognosis Children AdultsSpontaneous 83%2%remissionEventually 89%64%recover Die 1-2%5%ITP/prognosis32复习思考题复习思考题1 1、儿童、儿童ITPITP与成人与成人ITPITP临床特点和治疗有何不同?临床特点和治疗有何不同?2 2、ITPITP与继发性血小板减少症如何鉴别?与继发性血小板减少症如何鉴别?33 34
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