Pearls from Dr. Andrew Eisenberger

Thrombotic thrombocytopenic purpura in a pregnant woman with aphasia and unilateral weakness

2016-05-16

  • Typical, sporadic form of TTP: auto-antibodies against ADAMTS-13 neutralize this vWF multimer protease --> multimer accumulation --> abnormal platelet adhesion and activation --> systemic microvascular aggregation of platelets --> CNS and other organ ischemia

    Posted 05/29/16 12:24:46 PM by Matthew Cummings

  • Microangiopathic hemolytic anemia (schistocytes + ↑LDH/indirect bili) AND thrombocytopenia without an alternative apparent cause? Strongly consider TTP

    Posted 05/29/16 12:25:44 PM by Matthew Cummings

  • Low threshold for plasma exchange if unexplained MAHA + thrombocytopenia (even if diagnosis not definitive), continue until platelets >150,000

    Posted 05/29/16 12:26:53 PM by Matthew Cummings

  • Severe/refractory TTP? Consider adjunctive therapies: glucocorticoids, rituximab

    Posted 05/29/16 12:27:45 PM by Matthew Cummings

  • For further reading...

    TTP: Review Article (NEJM) 
    Comparison of Plasma Exchange with Plasma Infusion in the Treatment of Thrombotic Thrombocytopenic Purpura


    Posted 05/29/16 12:30:29 PM by Matthew Cummings

  • 52yo F w/ SLE on long standing immunosuppressants who p/w pancytopenia of unclear etiology

    2017-05-23

  • Prednisone for ITP with decrease production of anti-platelet antibodies.  However, half life of IgG is 3 weeks, so immediate response to Prednisone is not this mechanism.  Prednisone also decreases expression of FcGamma receptor, which leads to fast response seen with steroids.  IVIG saturates the FcGamma receptor, which prevents opsonization of platelets coated with antibodies.   

    Posted 05/24/17 09:43:43 AM by Emily Miller

  • Hematologic Complications of Bariatric Surgery: B12 deficiency, Iron deficiency, Copper deficiency - causes dorsal column neuropathy, anemia and neutropenia.  

    Posted 05/24/17 09:45:40 AM by Emily Miller

  • A shortened PTT can be a risk factor for thrombosis.  Can see with conditions that cause increased fibrinogen, factor VIII levels or von willebrand factor.  

    Posted 05/24/17 09:47:16 AM by Emily Miller

  • 95yo F with lupus anticoagulant and prolonged aPTT

    2017-08-22

    40yo undomiciled M p/w CP, SOB and Hb 2, found to have severe B12 deficiency

    2017-11-06

    38yoF G1P0 26w w/ TTP requiring plasma exchange

    2018-02-07

    58yoM with large ICH in setting of PV and extreme thrombocytosis

    2018-05-01

    32 yo man p/w hemolytic anemia 2/2 TTP found to have new diagnosis of HIV

    2019-03-05


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