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Interesting Case

HPI: 29 year-old G6P4014 admitted for preeclampsia and thrombocytopenia. Induction on HD

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Interesting Case

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    1. Interesting Case October 18, 2005

    10. Night of 10/13/05: upper GI bleeding with coffee-ground emesis patient became hypotensive, then stabilized with NS volume resuscitation Hb dropped from 10.6 to 9 Patient suddenly became hypotensive and pulseless aggressive resuscitation unsuccessful Cause of death: GI bleed, possible cardiac tamponade 2o to TTP

    17. Thrombotic thrombocytopenic purpura

    20. Thrombotic thrombocytopenic purpura Demographics Incidence: 1:100,000 - 1:500,000 Male:female 1:2 Age Most common in 30-40 year olds 90% of patients = 60 years old No racial differences No seasonal difference

    21. Clinical Findings in TTP Microvascular thrombi: MAHA Normal coagulation Thrombocytopenia (< 20,000/µl) Anemia < 10g/dl Reticulocytosis £ LDH £ Indirect bilirbuin ¤Haptoglobin Pentad of symptoms

    22. Pentad of TTP

    23. Alternative diagnoses of patients who have clinically suspected TTP/HUS Apparent after the plasma exchange has begun Autoimmune disorders Systemic lupus erythematosus Scleroderma Anti-phospholipid antibody syndrome Sepsis Malignant hypertension Heparin-induced thrombocytopenia/thrombosis Disseminated malignancy

    24. Presentations of TTP/HUS Thrombotic microangiopathy Idiopathic No apparent etiology or associated condition Drug-induced Allergic: Quinine, ticlopidine Dose-related: Mitomycin, gemcitabine, cyclosporin Pregnancy/postpartum Diarrhea-associated Bone marrow transplantation Congenital

    25. TTP in pregnancy (1) GI complaints often initial sign (2) preeclampsia (hypertension) often present (3) most common at term with progression post-partum (4) severe neurologic abnormalities and renal failure (5) disseminated microvascular thrombosis in absence of plasma exchange

    26. TTP in Pregnancy risk increases near term and post-partum accounts for 10% of all TTP decreased ADAMTS-13 activity and increased plasma vWF in 2nd / 3rd trimesters difficult to distinguish TTP / HELLP syndrome

    29. vWF Multimers Made in megakaryocytes and endothelial cells Stored in platelet alpha granules and Weibel-Palade bodies of endothelial cells Ultralarge molecule cleaved to smaller subunits by protease ADAMTS-13 ULVWF : highly adhesive to platelets

    30. vWF, Proteolysis, and Platelet Adhesion

    31. von Willebrand Factor Multimers

    33. Treatment Plasma exchange removes antibody restores ADAMTS-13 improves mortality rate to 10 – 20% daily for 1-2 weeks Hemodialysis if kidney fails Plasma infusion if exchange not available Corticosteroids for non-responders

    34. Summary Lab diagnosis of TTP: Thrombocytopenia Microangiopathic anemia: Schistocytes Increased LDH due to tissue necrosis Rule out other causes of MAHA Treatment: Urgent plasma exchange

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