Hematopoietic Disorders and TPE, Therapeutic plasma exchange, Clinical Pathology - Full Vignette wit

Описание: A 50-year-old woman with recent onset bruising, headache, and dark urine arrives with severe thrombocytopenia, anemia, and scattered petechiae. Her labs reveal hemolysis, schistocytes, and no clinical evidence of infection. How should you approach the diagnostic workup for a suspected thrombotic microangiopathy in this case? What clues in her history, physical, and labs can help distinguish between overlapping syndromes?

VIDEO INFO
Category: Hematopoietic Disorders and TPE, Therapeutic plasma exchange, Clinical Pathology
Difficulty: Easy - Basic level - Suitable for medical students
Question Type: Differential Testing
Case Type: Typical Presentation

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QUESTION
A 50-year-old woman who lives with her spouse in an assisted-living apartment presents with 24 hours of easy bruising, mild headache, and dark urine. She has a history of congestive heart failure with preserved ejection fraction, aortic regurgitation followed by cardiology, cerebral palsy with mild motor impairment, prior giardiasis, silicosis from remote occupational exposure, and Budd-Chiari syndrome resolved years ago....

OPTIONS
A. ADAMTS13 activity with inhibitor testing (severe deficiency less than 10% activity with an inhibitor or anti-ADAMTS13 IgG), drawn before plasma exchange and processed by a reference lab.
B. Peripheral blood smear to quantify schistocytes at a core lab to document microangiopathic hemolysis for ongoing monitoring.
C. Plasma viscosity measurement to evaluate for hyperviscosity from an IgM paraprotein as the cause of thrombocytopenia and anemia.
D. Direct antiglobulin (Coombs) test to identify autoimmune hemolysis as the mechanism of microangiopathy before any plasma treatment.

CORRECT ANSWER
A. ADAMTS13 activity with inhibitor testing (severe deficiency less than 10% activity with an inhibitor or anti-ADAMTS13 IgG), drawn before plasma exchange and processed by a reference lab.

EXPLANATION
This patient has severe thrombocytopenia with microangiopathic hemolytic anemia (schistocytes, elevated LDH, indirect hyperbilirubinemia, undetectable haptoglobin), normal PT/aPTT, and neurologic symptoms-a high-probability immune TTP picture. While therapeutic plasma exchange should be started immediately based on clinical probability, the single most specific confirmatory test to support immune TTP and distinguish it from other thrombotic microangiopathies is ADAMTS13 testing. The correct test is ADAMTS13 activity with inhibitor assessment (or anti-ADAMTS13 IgG), drawn before the first plasma exchange and sent to a reference laboratory. Severe deficiency ( less than 10% activity) plus an inhibitor strongly supports acquired (immune) TTP.

The distractors are either supportive but nonspecific or target different diseases. Repeating a smear to quantify schistocytes confirms microangiopathic hemolysis yet does not separate immune TTP from other TMAs such as atypical HUS. Plasma viscosity is a test for IgM-related hyperviscosity syndromes (for example, Waldenstro m macroglobulinemia) and is not relevant to this patient s pattern....


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