Relapsed thrombotic thrombocytopenic purpura presenting as an acute cerebrovascular accident

Abstract

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.

Thrombotic Thrombocytopenic Purpura: A Thrombotic Disorder Caused by ADAMTS13 Deficiency

“…Others may present with isolated thrombocytopenia and are mistaken to have ITP. Still others present with acute neurological deficits without thrombocytopenia or microangiopathic hemolysis [64]. Definitive diagnosis is difficult in atypical or complex cases without the aid of ADAMTS13 and VWF multimer analysis.…”

“…The course evolves variably, ranging from rapid deterioration within a few days to smoldering over weeks or months. Occasionally, a relapse may present with focal neurological deficits such as dizziness, hemiparesis, slurred speech or aphasia [64]. Such neurological complications may pose a diagnostic challenge when they are not accompanied by thrombocytopenia or microangiopathic hemolysis.…”

Atypical presentations of thrombotic thrombocytopenic purpura in middle-aged women with recurrent cerebral macrovascular thrombosis: a case report

“…The demonstration of large vessel thrombosis in TTP has been only rarely shown, ten similar cases of atypical TTP recurrence with only slight laboratory abnormalities have been reported by other authors [8][9][10][11][12][13], and (Table 2) .In atypical cases with a known diagnosis of TTP, symptoms mainly guide treatment at the time of recurrence.…”

“…Downes [11] reported two cases of relapsed atypical TTP with stroke, increased LDH serum levels, only slight decrease in haemoglobin levels and rare schistocytes, and one occurred in a Caucasian 42 years old woman and the other in a 40 years old African American woman. At the time of recurrence, ADAMTS13 activity was > 10% and inhibitors were not detectable.…”

Recurrent Disease in 25 Patients with Acquired Thrombotic Thrombocytopenic Purpura and Hemolytic Uremic Syndrome during 6.5 Years Follow-Up

“…This could be explained by timely diagnose and treatment. Although almost recurrent episodes of TTP are timely recognizable by both patient and physician, there is potential for diagnostic difficulty pertains to the occasional patients who present with neurological deficit and other signs of tissue ischemia before developing overt thrombotic microangiopathy [22]. In our patient n 0 7, the third disease episode was indistinguishable from the initial presenting one as well as the first relapse.…”