A case of concomitant connective tissue disorder and thrombotic thrombocytopenic purpura in an Indian middle aged female

Authors

  • Shubhank Narula Department of Family Medicine, Sir Ganga Ram Hospital, New Delhi, India
  • Atul Kakar Department of Medicine, Sir Ganga Ram Hospital, New Delhi, India
  • Atul Gogia Department of Medicine, Sir Ganga Ram Hospital, New Delhi, India
  • Tanvi Batra Department of Medicine, Sir Ganga Ram Hospital, New Delhi, India

DOI:

https://doi.org/10.18203/2320-6012.ijrms20233055

Keywords:

Connective tissue disorder, Seizures, Thrombotic thrombocytopenic purpura, Rituximab

Abstract

Thrombotic thrombocytopenia (TTP) is a rare disease which is rarely present in adults. Adults usually have an acquired version of disease, associated with some underlying autoimmune disease. There has been paucity of literature about reports which shows the coexistence of connective tissue disorder in patients of acquired TTP. This is a case report of a female who presented with vague symptoms of breathlessness, abdominal pain and petechial rashes and was diagnosed as TTP, developed neurological complications but was stabilized by timely management through plasma exchanges and steroids. 

 

References

Scully M, Cataland S, Coppo P, De la Rubia J, Friedman KD, Kremer Hovinga J, et al. International Working Group for Thrombotic Thrombocytopenic Purpura. Consensus on the standardization of terminology in thrombotic thrombocytopenic purpura and related thrombotic microangiopathies. J Thromb Haemost. 2017;15(2):312-22.

Coppo P, Cuker A, George JN. Thrombotic thrombocytopenic purpura: Toward targeted therapy and precision medicine. Res Pract Thromb Haemost. 2019;3(1):26- 37.

Van Dorland HA, Taleghani MM, Sakai K, Friedman KD, George JN, Hrachovinova I, et al. Hereditary TTP Registry. The International Hereditary Thrombotic Thrombocytopenic Purpura Registry: key findings at enrolment until 2017. Haematologica. 2019;104(10):2107-15.

Shelat SG, Ai J, Zheng XL. Molecular biology of ADAMTS13 and diagnostic utility of ADAMTS13 proteolytic activity and inhibitor assays. Semin Thromb Hemost. 2005;31(6):659-72.

Yarranton H, Machin SJ. An update on the pathogenesis and management of acquired thrombotic thrombocytopenic purpura. Curr Opin Neurol. 2003;16(3):367-73.

Vasoo S, Thumboo J, Fong KY. Thrombotic thrombocytopenic purpura in systemic lupus erythematosus: disease activity and the use of cytotoxic drugs. Lupus. 2002;11(7):443-50.

Griffin D, Al-Nouri ZL, Muthurajah D, Ross JR, Ballard RB, Terrell DR, et al. First symptoms in patients with thrombotic thrombocytopenic purpura: what are they and when do they occur? Transfusion. 2013;53(1):235-7.

Chiasakul T, Cuker A. Clinical and laboratory diagnosis of TTP: an integrated approach. Hematology Am Soc Hematol Educ Program. 2018;2018(1):530-8.

Oliveira DS, Lima TG, Benevides FLN, Barbosa SAT, Oliveira MA, Boris NP, et al. Plasmic score applicability for the diagnosis of thrombotic microangiopathy associated with ADAMTS13-acquired deficiency in a developing country. Hematol Transfus Cell Ther. 2019;41(2):119-24.

Zheng XL, Vesely SK, Cataland SR, Coppo P, Geldziler B, Iorio A, et al. ISTH guidelines for treatment of thrombotic thrombocytopenic purpura. J Thromb Haemost. 2020;18(10):2496-502.

Merayo-Chalico J, Demichelis-Gomez R, Rajme-Lopez S, Aparicio-Vera L, Barrera-Vargas A, Alcocer-Varela J, et al. Risk factors and clinical profile of thrombotic thrombocytopenic purpura in systemic lupus erythematosus patients. Is this a distinctive clinical entity in the thrombotic microangiopathy spectrum?: a case control study. Thromb Res. 2014;134(5):1020-7.

Terrell DR, Vesely SK, Kremer Hovinga JA, Lammle B, George JN. Different disparities of gender and race among the thrombotic thrombocytopenic purpura and hemolytic-uremic syndromes. Am J Hematol. 2010;85(11):844-7.

Shah AA, Higgins JP, Chakravarty EF. Thrombotic microangiopathic hemolytic anemia in a patient with SLE: diagnostic difficulties. Nat Clin Pract Rheumatol. 2007;3(6):357-62.

Manadan AM, Harris C, Schwartz MM, Block JA. The frequency of thrombotic thrombocytopenic purpura in patients with systemic lupus erythematosus undergoing kidney biopsy. J Rheumatol. 2003;30:1227-30.

Roriz M, Landais M, Desprez J, Barbet C, Azoulay E, Galicier L, Wynckel A, et al. Risk factors for autoimmune diseases development after thrombotic thrombocytopenic purpura. Medicine (Baltimore) 2015;94:e1598.

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Published

2023-09-29

How to Cite

Narula, S., Kakar, A., Gogia, A., & Batra, T. (2023). A case of concomitant connective tissue disorder and thrombotic thrombocytopenic purpura in an Indian middle aged female. International Journal of Research in Medical Sciences, 11(10), 3888–3891. https://doi.org/10.18203/2320-6012.ijrms20233055

Issue

Section

Case Reports