Relapsed thrombotic thrombocytopenic purpura presenting as an acute cerebrovascular accident.
Thrombotic thrombocytopenic purpura (TTP) is an uncommon but severe disorder that classically presents with microangiopathic hemolytic anemia (MAHA), thrombocytopenia, and fluctuating neurological changes. Previously, it was impossible to make a diagnosis of TTP in the absence of thrombocytopenia or microangiopathic hemolysis (MAHA). We describe two cases of relapsing TTP that presented with acute cerebrovascular accident (CVA) without concurrent thrombocytopenia or MAHA after initial classical presentation of TTP. In both cases, the diagnosis of TTP as the cause of the CVA was attributed to severe deficiency of the von Willebrand factor cleaving protease, ADAMTS13 in plasma (11 and 12%, normal 79-127%). Each patient had a dramatic clinical improvement in response to therapeutic plasma exchange. The experience in these two cases suggests that TTP should be considered as a potential cause among patients presenting with a CVA, particularly if the patients have a history of TTP.
Department of Pathology, Case Western Reserve University and University Hospitals of Cleveland, Cleveland, Ohio, USA.
Katharine A
KA
Downes
Roslyn
R
Yomtovian
H M
HM
Tsai
Bernard
B
Silver
Cynthia
C
Rutherford
Ravindra
R
Sarode
D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; D000328:Adult; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D008666:Metalloendopeptidases / Q000172:deficiency; D011697:Purpura, Thrombotic Thrombocytopenic / Q000175:diagnosis* / Q000473:pathology; D012008:Recurrence; D020521:Stroke / Q000175:diagnosis*; D013921:Thrombocytopenia / Q000175:diagnosis; D013997:Time Factors
D051722:ADAM Proteins; D008666:Metalloendopeptidases; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human