Abstract
BackgroundThrombotic thrombocytopenic purpura (TTP) is a rare, potentially fatal disease with multisystem involvement. Cytomegalovirus (CMV) infection as a cause of refractory TTP, has been reported only in immunocompromised individuals. We report a case of CMV-induced refractory TTP in an immunocompetent individual.Case presentationA 35-year-old, previously healthy Sri Lankan man, presented with fever for 3 days with gum bleeding and progressive drowsiness. His Glasgow coma scale score was 10/15. He did not have papilloedema or neck stiffness. Laboratory evaluation showed a severe thrombocytopenia with microangiopathic haemolytic anaemia. There was marginal renal impairment and normal coagulation profile. Non-contrast CT scan of brain was normal. A diagnosis of thrombotic thrombocytopenic purpura was made. Despite daily plasma exchanges and high-dose steroids, he failed to achieve the expected therapeutic response, thus demonstrating refractory TTP. On exploring for possible causes of refractoriness to treatment, a clinically significant PCR titre of CMV was detected. Treatment of CMV infection lead to complete recovery of TTP. His disease course was further complicated with spontaneous spinal haemorrhage leading to neurological sequelae.Discussion and conclusionsThis is the first report of CMV induced refractory TTP in an immunocompetent adult. It is also the first report of clinically significant spontaneous spinal haematoma in TTP. These two rare occurrences should be considered when patients with refractory TTP do not improve as expected.
Highlights
Thrombotic thrombocytopenic purpura (TTP) is a rare, potentially fatal disease with multisystem involvement
TTP is characterized by a pentad of features including thrombocytopenia, microangiopathic haemolytic anaemia, neurological abnormalities, renal failure and fever [1]
At three months’ review, he had normal haematological and biochemical parameters and a negative Polymerase chain reaction (PCR) quantification of CMV. He had regained ability to walk with support and had normal sphincter function. This is the first report of CMV induced refractory TTP in an immunocompetent individual
Summary
This is the first report of CMV induced refractory TTP in an immunocompetent adult. It is the first report of clinically significant spontaneous spinal haematoma in TTP. These two rare occurrences should be considered when patients with refractory TTP do not improve as expected
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