Path 1 Block 3 Pt 3

ptheodore's version from 2015-08-02 18:29


Question Answer
Skin & Renal biopsy: IgA, C3, fibrin deposits, Fibrinoid necrosisHenoch-Schönlein purpura
Following viral infection; Varicella zoster and EBV.Acute Immune Thrombocytopenic purpura (ITP)
Anti platelet antibodies (GpIIb/IIIa)Acute/Chronic Immune Thrombocytopenic purpura (ITP)
Lab findings: Platelets: ↓ (Thrombocytopenia), bleeding time: ↑, coagulation study: normal, PT/PTT: NormalAcute Immune Thrombocytopenic purpura (ITP)
Drug Induced: Aplastic anemia, Leukemia’s, bone marrow infiltration, Hypersplenism & SLESecondary Acute Immune Thrombocytopenic purpura (ITP)
Present in young to middle aged women. Findings in BM: slight increase in megakaryocytesChronic Immune Thrombocytopenic Purpura (ITP)
Subconjunctival retinal hemorrhagesChronic Immune Thrombocytopenic Purpura (ITP)
Lab investigations:Platelets:↓ (Thrombocytopenia) <20k. Bleeding time:↑. Coagulation study: normal. PT/PTT: Normal. BM: Slight increase in megakaryocytesChronic Immune Thrombocytopenic Purpura (ITP)
Subarachnoid hemorrhages, intracerebral hemorrhages (are rare) . Transplacental transfer of IgG may produce transient thrombocytopenia in infantsChronic Immune Thrombocytopenic Purpura (ITP)
Easy bruising, menorrhagia, melena (dark bloody feces), bleed from mucus membranesChronic Immune Thrombocytopenic Purpura (ITP)
ADAMTS 13 deficiencyThrombotic Thrombocytopenic Purpura (TTP)
Excess of vWF (as it cannot be cleared), resulting in thrombocytopeniaThrombotic Thrombocytopenic Purpura (TTP)
Lab Findings: WBC:↑ (Leukocytosis). Platelet count:↓. Schistocytes. Bleeding time:↑. Coagulation studies: normal (PTT is normal). Urine: hematuria, proteinuriaThrombotic Thrombocytopenic Purpura (TTP)
Clinical features (pentad): Female, Fever, Thrombocytopenia, Renal failure, Microangiopathic hemolytic anemiaThrombotic Thrombocytopenic Purpura (TTP)
Epidemic, summer months (camps, hikes), under cooked red meat, unpasteurized milk, contaminated waterHemolytic-Uremic syndrome (HUS)
E.coli strain 0157:H7, Shiga like toxinHemolytic-Uremic syndrome (HUS)
Acute renal failure, thrombocytopenia, Microangiopathic hemolytic anemia, Bloody diarrhea, Vomiting & FeverHemolytic-Uremic syndrome (HUS)
Lab Findings: Platelet count:↓(thrombocytopenia), Hemoglobulinemia: excessive amounts of free hemoglobin in the blood plasma. Haptoglobulin: reduced, there is some amount of intravascular hemolysis. Fibrin degradation product, and D-dimers: present. Peripheral blood smear: Schistocytes. Bleeding time:↑. Coagulation studies: PTT and PT are normal. Serum creatinine:↑. Urine analysis: proteinuriaHemolytic-Uremic syndrome (HUS)
Antibiotics & PlasmapheresisHemolytic-Uremic syndrome (HUS)
Consanguinity. Defective platelet adhesion to subendothelium. Due to a genetic GPIb deficiency. Failure to bind von Willebrand factor and thrombin.Bernard-Soulier syndrome
Patients must avoid anti-platelet treatment (aspirin)Bernard-Soulier syndrome
Total platelet count:↓(mild). Peripheral blood smear: Giant platelets. Bleeding time:↑. Coagulation studies: normal.Bernard-Soulier syndrome
Platelet aggregation: abnormal study, due to defective adhesion to collagen in vitro. Ristocetin test: abnormalBernard-Soulier syndrome
Consanguinity. Defective platelet aggregation due to genetic GPIIb/IIIa deficiency.Bleeding time:↑ Total platelet count: normalGlanzmann’s Thromboasthenia
Aspirin causes platelet aggregation defect. Inhibits COX pathway, which blocks synthesis of thrombaxane A2 (TXA2). Reduction in ADP induced platelet aggregationGlanzmann’s Thromboasthenia
Spontaneous bleed from mucous membrane: epistaxis. Bleed from trivial wounds, menorrhagia, dental procedures, minor surgeriesvon Willebrand Disease
Lab Findings: Platelet count: normal. Bleeding time:↑(this is due to a platelet adhesion defect). PT: normal (Factor 7). PTT:↑. vWF activity:↓. Factor VIII activity:↓. Ristocetin test (Ristocetin cofactor assay): abnormal.von Willebrand Disease
Ristocetin binds vWF and therefore leads to platelet aggregation (normal)von Willebrand Disease
Coagulation defect due to deficiency of factor VIII von Willebrand Disease & Hemophilia A
Mutation in Factor VIII. Affects malesHemophilia A
Spontaneous hemorrhages to knee joints (hemarthroses)Hemophilia A
Decrease in Factor IXHemophilia B
Lab Findings: Platelet count: normal. Bleeding time: normal. Prothrombin time: normal. Partial Thromboplastin Time:↑. Factor VIII levels:↓Hemophilia A
Secondary to: Obstetric complications (abruptio placentae, amniotic fluid embolism, abortion, intra uterine death. Infections (sepsis). Neoplasms (mucin secreting adenoca). Massive tissue injury. Snake bite (viper)Disseminated Intravascular Coagulation (DIC)
Utilization of the coagulation factors and platelets (consumption coagulopathy). Once triggered, there will be formation of thrombin, which converts fibrinogen to fibrin. Plasmin, which then cleaves fibrin causes all the problemsDisseminated Intravascular Coagulation (DIC)
Waterhouse-Friderichsen syndrome (Neisseria meningitidis sepsis) & Sheehan postpartum pituitary necrosisDisseminated Intravascular Coagulation (DIC)
Lab Findings: Platelet count:↓. PT/PTT:↑. Fibrinogen:↓. Schistiocytes, polychromatophils. Microangiopathic hemolytic anemia. Elevated fibrin split products, particularly D-dimer are presentDisseminated Intravascular Coagulation (DIC)
Elevated D-dimer is the best screening test forDisseminated Intravascular Coagulation (DIC)
Needed for factors II (prothrombin), VII, IX, X, and proteins C and S to workVitamin K (gamma carboxylates )
Deficiency occurs in newborns due to lack of GI colonization by bacteria that normally synthesize Vitamin K Deficiency

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