Cytomegalovirus induced refractory TTP in an immunocompetent individual: a case report

Abstract

Background Thrombotic 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 presentation A 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 conclusions This 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.

True vs. false immune-mediated thrombotic thrombocytopenic purpura exacerbations: a clinical case in the caplacizumab era

“…Many agents can be used and in the case of resistant iTTP, they can be combined to achieve a deeper immunosuppression. This condition may expose the patient to infectious complications as CMV infection/reactivation, which needs to be considered, first of all, not only as a possible iTTP trigger [11] but also as a complication because of the deep immunosuppression. CMV may cause refractoriness at iTTP treatment, which may potentially aggravate CMV infection.…”

Immune‐mediated thrombotic thrombocytopenic purpura in a Jehovah’s Witness – Effectiveness of incorporating extracorporeal immunoadsorption to caplacizumab, steroids and rituximab

“…9 Further cases of i-TTP treated with caplacizumab, glucocorticoids, and rituximab without the use of TPE have been reported, 31,32 also in the context of anaphylaxis to TTP. 33 While the majority of i-TTP cases are of unknown ethology, secondary i-TTP can be associated to certain autoimmune disorders like systemic lupus erythematosus, malignancies, medications, as well as viral infections like HIV, 36 CMV, 37 and SARS-CoV-2. SARS-CoV2 has been reported as a possible trigger for autoimmune and inflammatory dysregulation 38 including i-TTP, which has been described as a further dramatic life-threatening clinical presentation of SARS-Cov-2 infection.…”

Immune Thrombotic Thrombocytopenic Purpura: Pathophysiology, Diagnosis and Open Issues.

“… 21 , 29 – 33 Cytomegalovirus (CMV) infection can also be considered a possible trigger of TTP and may cause refractoriness at iTTP treatment. 34 COVID-19 infection has been associated with endotheliopathy, and it is also associated with TTP. Recently, de novo and relapsed iTTP have been reported during SARS-Cov-2 infection.…”