A case report of thrombotic thrombocytopenic purpura with positive ANA activity

Neha Goel; Aman Chawla; Prashant Prabhakar

doi:10.18203/2349-3291.ijcp20250775

Authors

Neha Goel Department of Pediatrics, VMMC & Safdarjung Hospital, New Delhi, India https://orcid.org/0000-0001-9575-0805
Aman Chawla Department of Pediatrics, VMMC & Safdarjung Hospital, New Delhi, India https://orcid.org/0009-0003-7634-3296
Prashant Prabhakar Department of Pediatrics, VMMC & Safdarjung Hospital, New Delhi, India https://orcid.org/0000-0002-4707-6374

DOI:

https://doi.org/10.18203/2349-3291.ijcp20250775

Keywords:

ANA, ADAMTS13, Plasmapheresis, Thrombotic thrombocytopenic purpura

Abstract

Thrombotic thrombocytopenic purpura (TTP) is a life-threatening disorder, characterized by: consumptive thrombocytopenia, microangiopathic hemolytic anemia with the presence of schistocytes, renal impairment, neurological dysfunction and fever. A 12-years-old girl presented with TTP and had ADAMTS13 activity deficiency along with positive ANA activity. The child was planned for plasmapheresis but developed refractory seizures and pulmonary bleeding and succumbed to death. TTP with ANA positivity has a very fulminant course. ⁠Pulmonary involvement can be asymptomatic but has a poor prognosis with rapid clinical deterioration. A high index of suspicion is of utmost importance in low to middle-income countries settings as the facility of plasmapheresis is available at very few centers

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References

Sadler JE. Von Willebrand factor, ADAMTS13 and thrombotic thrombocytopenic purpura. Blood. 2008;112:11–8. DOI: https://doi.org/10.1182/blood-2008-02-078170

Ridolfi RL, Bell WR. Thrombotic thrombocytopenic purpura: report of 25 cases and review of the literature. Medicine. 1981;60(6):413-28. DOI: https://doi.org/10.1097/00005792-198111000-00003

Moake JL. Thrombotic microangiopathies. N Engl J Med. 2002;347:589-600. DOI: https://doi.org/10.1056/NEJMra020528

Musa MO, Nounou R, Sahovic E, Seth P, Qadi A, Alijurf M. Fulminant thrombotic thrombocytopenic purpura in two patients with systemic lupus erythematosus and phospholipid autoantibodies. European J of Haematology. 2000;64(6):67. DOI: https://doi.org/10.1034/j.1600-0609.2000.9l125.x

Thomas L, Shi Q, Patel N. Diffuse Alveolar Hemorrhage: A Life-Threatening Condition in Thrombotic Thrombocytopenic Purpura (TTP). Blood. 2013;21:4762. DOI: https://doi.org/10.1182/blood.V122.21.4762.4762

Wu H, Kiel M, Shi Q. Pulmonary manifestations in patients with Thrombotic Thrombocytopenic Purpura at a community hospital setting. Chest. 2013;144:365. DOI: https://doi.org/10.1378/chest.1703532

Levy GG, Nichols WC, Lian EC. Mutations in a member of the ADAMTS gene family cause thrombotic thrombocytopenic purpura. Nature. 2001;413(6855):488-94. DOI: https://doi.org/10.1038/35097008

Nguyen TC, Liu A, Liu L, Ball C, Choi H, May WS. Acquired ADAMTS-13 deficiency in pediatric patients with severe sepsis. Haematologica. 2007;92(1):121-4. DOI: https://doi.org/10.3324/haematol.10262

Pagnoux C, Korach JM, Guillevin L. Indications for plasma exchange in systemic lupus erythematosus in 2005. Lupus. 2005;14(11):871-7. DOI: https://doi.org/10.1191/0961203305lu2174rr

Coppo P, Wynckel A, Clabault K. Idiopathic Thrombotic Microangiopathies: Antinuclear Antibodies, Platelet Count and Creatinin Level Can Predict a Severe ADAMTS13 Deficiency. Blood. 2007;110(1):3923. DOI: https://doi.org/10.1182/blood.V110.11.3923.3923