[A woman suffering from systemic lupus erythematosus and acquired thrombotic thrombocytopenic purpura].
A 41-year-old woman with known systemic lupus erythematosus (SLE) was diagnosed with thrombotic thrombocytopaenic purpura (TTP). At the time of admission she was suffering from petechia, purpura and had neurological symptoms. At first a relapse of the SLE was suspected. Additional laboratory findings demonstrated haemolytic anaemia, thrombocytopaenia and high levels of fragmentocytes. After multiple plasmapheresis treatments and immunosuppressive therapy she recovered. TTP can be differentiated from other thrombotic microangiopathic syndromes by its normal levels of prothrombin time, partially activated thromboplastin time (APTT), fibrinogen and direct Coombs-test. Further investigation is needed to confirm the diagnosis by determination of the activity of Von Willebrand factor cleaving protease ADAMTS-13. In this patient, the ADAMTS-13 activity returned after plasmapheresis. This case demonstrates the importance of fast and appropriate laboratory testing in order to diagnose TTP quickly.
Verheijen
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FM
Afd. Klinisch Laboratorium, Ziekenhuisgroep Twente, locatie Twenteborg Ziekenhuis, Postbus 7600, 7600 SZ Almelo. f.verheijen@zgt.nl
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Fijnheer
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D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; D000328:Adult; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008180:Lupus Erythematosus, Systemic / Q000150:complications*; D008666:Metalloendopeptidases / Q000378:metabolism*; D010314:Partial Thromboplastin Time; D010956:Plasmapheresis; D011697:Purpura, Thrombotic Thrombocytopenic / Q000150:complications / Q000175:diagnosis* / Q000201:enzymology* / Q000628:therapy; D013997:Time Factors; D016896:Treatment Outcome; D014841:von Willebrand Factor / Q000378:metabolism*
D007166:Immunosuppressive Agents; D014841:von Willebrand Factor; D051722:ADAM Proteins; D008666:Metalloendopeptidases; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human