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Case Discussion

Case Discussion. Huang Honghui Department of Hematology Ren Ji Hospital. Case Summary. 26 year-old , female patient persistent nasal bleeding for one day, have an upper respi- ratory infecti on 2 weeks previously. No fever, chills, nausea , vomiting, abdominal pain, or joint pain .

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Case Discussion

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  1. Case Discussion Huang Honghui Department of Hematology Ren Ji Hospital

  2. Case Summary • 26 year-old,female patient • persistent nasal bleeding for one day, have an upper respi- ratory infection 2weeks previously. No fever,chills, nausea, vomiting,abdominal pain,or joint pain. • PE: multiple l-mm reddish spots on her lower extremities. No lymphadenopathy orhepatosplenomegaly. • No excessive bleeding with menses,childbirth,priorepistaxis,easy bruisability,or bleeding into her joints. • No family history of abnormal bleeding. • Not take any medication.

  3. Objects of Analysis • Learn the clinical approach to bleeding disorders,specificly platelets disorders versus coagulation disorders. • Learn about the differentialdiagnosis ofthrombocytopenia,specifically thrombocytopenic purpura versus other platelet disorders,such asthrombotic thrombocytopenic purpura(TTP), hemolytic uremic syndrome (HUS),or disseminated intravascular coagulation (DIC). • Learn about thetreatment of ITP.

  4. Consideration • superficial petechiae and mucosal bleeding disordered primarymary hemostasis • laboratory testing • complete blood count • prothrombin time (PT) screening • partial thromboplastin time (PTT) • Bone marrow smear

  5. APPROACH TO SUSPECTED THROMBOCYTOPENIA

  6. HISTORY • Liver disease,uremia,malignancy,systemic lupus erythematous • Medications: over-the-counter products(aspirin) • Family history of abnormalbleeding • History of epistaxis,menorrhagia,excessive prolonged bleeding from minor cuts,bruising,prolonged or profuse bleeding after dental extraction,excessive bleeding after major surgery or obstetric delivery, trauma followed by bleeding considered excessive relative to the injury

  7. Timing of Bleeding • If bleeding following dental extraction is immediate and lasts for longer than 24 hours,a problem with primaryhemostatic plug formation may be present. Therefore,this may suggest a platelet disorder. • If initialhemostasis seemed normalbut prolonged bleeding developed 2-3days later,a problem in the coagulation phase is suspected.

  8. Type of Bleeding • Spontaneous mucus membrane bleeding,such as gum bleeding,nose bleeding,and petechiae are suggestive ofa vascular disorder,thrombocytopenia,or abnormal platelet function. • Hemarthrosis,deep hematoma,and retroperitoneal bleeding are more likely to reflect a severe coagulation abnormality, • such as hemophilia,ifproblems have been lifelong • spontaneous inhibitor offactor VIII,ifproblems appear later in iife.

  9. Vascular Disorders • Vascular purpura • present with bleeding from mucus membranes and the appearance of petechiae • but usually the platelet count and the coagulation profile (PT and PTT)are normal • Hereditary hemorrhagic telangiectasias • inherited as an autosomal trait of high penetrance. • the most common hereditary vascular disorder • The physical exam will show the presence of telangiectasias.

  10. Causes of thrombocytopenia • decreased platelet production • decreased platelet survival • sequestration (hypersplenism) • dilutional

  11. Spurious Thrombocytopenia • Automated cell counters reports spurious thrombocytopenia in approximately 0.l%ofpatients. • This is generally a result of platelet dumping after drawing blood into the anticoagulant ethylene diamine tetra acetate (EDTA) . • Confirmation • Identifying platelet aggregates on peripheral blood smear • Using citrate or heparin as an anticoagulant

  12. Impaired platelet production • Infiltration caused by malignancy or myelofibrosis • Marrow hypoplasia • Chemicals • Drugs • Radiation • Viruses

  13. Decrease platelet survival • immune thrombocytopenia purpua (caused by IgG antibody against the platelets), • drug-induced thrombocytopenic purpura • secondary immunologic purpura(as in lymphoma, lupus,infection with human immunodeficiency virus type 1) • posttransfusion purpura. • Disseminated intravascular coagulation • hemolytic uremic syndrome • cavernous hemangioma • acute infections

  14. ITP • Acute ITP • early childhood • antecedent upper respiratory infection • self-limiting, usually resolves spontaneously within 3-6months. • Chronic ITP • in adults, most likely to occur in women ages20-40years • an insidious or subacute present. • persist for months to Years, with uncommon spontaneous remission.

  15. Several immunologic disorders may mimic true ITP • drug induced thrombocytopenic purpura • Discontinuation of the medication should lead to improvement in the platelet count within a time frame consistent with the drug’s metabolism. • Many drugs are blown to cause thrombocytopenic purpura,such as quinidine andquinine,Sulfonamide,heparin,and gold compounds. • SLE • Lymphoma • Lymphadenopathy • splenomegaly • HIV-1 infection • Posttransfusion purpura

  16. Nonimmunologic disorders may mimic true ITP • DIC • TTP

  17. DIC • ETIOLOGY • Secondary to some other process: sepsis,trauma, metastatic malignancy,obstetric causes • CLINICAL COURSE • can be relativelymild indolent course,or severe llfe-threatening process; • ongoing coagulation and fibrinolysis; • can cause thrombosis or hemorrhage; • consumption of coagulation factors is seen as prolonged PT and PTT

  18. DIC • TREATMENT • Treatment aimed at underlyingcause. • No proven specific treatment for the coagulation problem. • Ifclotting,consider anticoagulate with heparin. • If bleeding,replace factors andfibrinogen with fresh frozen plasma or cryoprecipitate.

  19. TTP • ETIOLOGY • Multiple causes,many seeminglytrivial drugs/infection lead toendothelial injury and release ofvon Willebrand factor,triggeringformation of mlcrovascularthrombi. • CLINICAL COURSE • fever • altered mental states • thrombocytopenia • Microangiopathichemolytic anemia • Renal failure

  20. TTP • TREATMENT • Plasmapheresis (removalof theexcess/abnormal vWF), mostpatients recover • corticosteroids

  21. ITP • ETIOLOGY • Antiplatelet antibody leading toplatelet destruction • CLINICAL COURSE • Children:following a Viral illness withresolution. • Adults:a more indolentcourse with progression and rarelys spontaneous resolution. • Isolatedthrombocytopenia, normal PT,PTT. • Increased megakaryocytes on bonemarrow aspiration.

  22. ITP • TREATMENT • Oral Corticosterolds, • Splenectomy if resistant to steroids, • Immunosuppressants • Intravenous Immmoglobulin

  23. Answers • Most likely diagnosis: • Immune thrombocytopenic purpura • Best initial treatment: • Oral corticosteroids

  24. Comprehension Questions • A50-year-old man has been treated for rheumatoid arthritis for many years.He is currently taking corticosteroids for the disease.On examination,he has stigmata of rheumatoid arthritis and some fullness on his left upper abdomen.His platelet count is slightly low at 56,000/mm3.HisWBC count is3,100/mm3 and Hgb9.Og/dL. Which ofthe following is themost likely etiology ofthe thrombocytopenia? • A.Steroid induced • B.Sequestration • C.Rheumatoid arthritis autoimmune induced • D.Prior gold therapy

  25. Comprehension Questions • A30-year-old woman with ITP has been taking maximum corticosteroid doses and still has a platelet count of 20,000/mm3and frequent bleeding episodes.Which of the following should she receive before her splenectomy? • A.Washed leukocyte transfusion • B.Intravenous interferon therapy • C.pneumococcal vaccine • D.Bone marrow radiotherapy

  26. CLINICAL PEARLS • Bleeding abnormalities can be divided into primary hemostatic problems (platelet plug at time of injury)and secondary hemostasis (creation ofa stable fibrin clot). • Disorders of primary hemostasis(thrombocytopenia or von Willebrand)are characterized by mucosal bleeding and the appearance ofpetechiae or superficial ecchymoses.

  27. CLINICAL PEARLS • Disorders of secondary hemostasis (coagulation factor deficiencies such as hemophilia)are usually characterized by the development of superficial ecchymoses,as well as deep hematomas and hemarthroses. • ITP is a diagnosis of exclusion.Patients have isolated thrombocytopenia(i.e.,no red or white blood cell abnormalities),no apparent secondary causes such as systemic lupus erythematosus,HIV or medication-induced thrombocytopenia,and normal to increased numbers ofmegakaryocytes in the bone marrow.

  28. CLINICAL PEARLS • Treatment of ITP • Corticosteroids are the initial treatment of ITP. • Patients with moresevere disease may be treated with intravenous immunogiobulin (IVIG); • chronic refractory cases are treated with splenectomy.

  29. Thanks!

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