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

There is no author summary for this article yet. Authors can add summaries to their articles on ScienceOpen to make them more accessible to a non-specialist audience.

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.

Related collections

Most cited references 16

Record: found
Abstract: found
Article: not found

Improved survival in thrombotic thrombocytopenic purpura-hemolytic uremic syndrome. Clinical experience in 108 patients.

Jared Ness, W. H. Bell, H G Braine … (1991)

Thrombotic thrombocytopenic purpura-hemolytic uremic syndrome (TTP-HUS) is characterized by microangiopathic hemolytic anemia, thrombocytopenia, fever, central nervous system abnormalities, and renal dysfunction. In early reports the mortality approached 100 percent. A treatment protocol was introduced in 1979 for patients admitted to Johns Hopkins Hospital with the diagnosis of TTP-HUS. Treatment regimens included 200 mg of prednisone a day, for patients with minimal symptoms and no central nervous system symptoms, and prednisone plus plasma exchange, for patients with rapid clinical deterioration who did not improve after 48 hours of prednisone alone and for patients presenting with central nervous system symptoms and rapidly declining hematocrit values and platelet counts. A total of 108 patients were treated, and 91 percent survived. Prednisone alone was judged to be effective in 30 patients with mild TTP-HUS (two relapses and two deaths). Plasma exchange plus prednisone was given to 78 patients with complicated TTP-HUS, resulting in 67 relapses and 8 deaths. Relapses occurred in 22 of 36 patients given maintenance plasma infusions. Neither splenectomy nor treatment with aspirin and dipyridamole was effective in those with a poor response to plasma exchange. None of the 71 patients tested had positive cultures for O157:H7 Escherichia coli. Nine percent of the patients were pregnant, and none gave birth to infants with TTP-HUS. Effective treatment with 91 percent survival is available for patients with TTP-HUS.

0 comments Cited 103 times – based on 0 reviews      Review now

Bookmark

Record: found
Abstract: found
Article: not found

Severe cytomegalovirus infection in immunocompetent patients.

Zachary S. Peacock, M Juniper, M Eddleston … (1996)

Severe cytomegalovirus (CMV) infection is rare in previously healthy immunocompetent individuals; to our knowledge, only thirty-four such cases have been reported in the worldwide literature. Multiorgan involvement was associated with a high mortality rate among these patients. Disease that clinically involves only the liver or lungs could be fatal; in contrast, none of the patients with isolated central nervous system infection died. Although few patients were treated with specific antiviral therapy, five of six patients with severe infection recovered after receiving therapy with ganciclovir or foscarnet. The rarity of severe CMV disease in immunocompetent patients probably precludes the performance of a clinical trial to evaluate the efficacy of specific antiviral therapy. However, the historically poor prognosis in the absence of such therapy suggests that rapid diagnosis of CMV disease and early instigation of specific treatment may be important.

0 comments Cited 49 times – based on 0 reviews      Review now

Bookmark

Record: found
Abstract: found
Article: not found

Viral-associated thrombotic microangiopathies.

Rodrigo Lopes da Silva (2011)

Thrombotic microangiopathies encompass a group of disorders characterized by microangiopathic hemolytic anemia, thrombocytopenia associated with hyaline thrombi (comprised primarily of platelet aggregates in the microcirculation), and varying degrees of end-organ failure. Many primary (genetic) and secondary etiological predisposing factors have been described-namely pregnancy, autoimmune disorders, cancer, drugs and antineoplastic therapy, bone marrow transplantation/solid organ transplantation, and infections. In the setting of infectious diseases, the association with Shiga or Shiga-like exotoxin of Escherichia coli 0157:h7 or Shigella dysenteriae type 1-induced typical hemolytic uremic syndrome is well known. Recently however, an increasing body of evidence suggests that viruses may also play an important role as trigger factors in the pathogenesis of thrombotic microangiopathies. This is a comprehensive review focusing on the current understanding of viral associated/induced endothelial stimulation and damage that ultimately leads to the development of this life-threatening multisystemic disorder.

0 comments Cited 45 times – based on 0 reviews      Review now

Bookmark

All references

Author and article information

Contributors

Medhini Boteju: medini.boteju@yahoo.com

Praveen Weeratunga:

ORCID: http://orcid.org/0000-0003-2306-0885

0094773521928 , prav782@yahoo.com

Ahalyaa Sivashangar: nallathambisivashankar@yahoo.com

Thashi Chang: thashichang@gmail.com

Journal

Journal ID (nlm-ta): BMC Infect Dis

Journal ID (iso-abbrev): BMC Infect. Dis

Title: BMC Infectious Diseases

Publisher: BioMed Central (London )

ISSN (Electronic): 1471-2334

Publication date (Electronic): 8 May 2019

Publication date PMC-release: 8 May 2019

Publication date Collection: 2019

Volume: 19

Electronic Location Identifier: 394

Affiliations

[1 ]ISNI 0000 0004 0556 2133, GRID grid.415398.2, University Medical Unit, National Hospital of Sri Lanka, ; Colombo, Sri Lanka

[2 ]ISNI 0000000121828067, GRID grid.8065.b, Department of Clinical Medicine, Faculty of Medicine, , University of Colombo, ; 25, Kynsey Road, Colombo, 00800 Sri Lanka

Author information

Praveen Weeratunga http://orcid.org/0000-0003-2306-0885

Article

Publisher ID: 4037

DOI: 10.1186/s12879-019-4037-9

PMC ID: 6507177

PubMed ID: 31068128

SO-VID: 8bd535a6-14ac-4ddd-a7f2-bfa723e610d0

License:

Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License ( http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver ( http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.