Introduction:
Thrombotic microangiopathy (TMA) is characterised by the classic triad of thrombocytopenia, microangiopathic hemolytic anemia (MAHA), and organ damage. The classification of the TMAs is constantly evolving. Currently, TMAs are classified as primary, secondary and infection-associated. TMAs are associated with considerable mortality and morbidity, including ESRD, but the timely implementation of supportive and targeted therapy can improve outcomes. Without access to eculizumab; plasma therapy, immunosuppressants, and supportive treatment form the basis for the management of TMAs in India and most developing countries.
Here, we report a case series of patients with TMA of varied etiologies and clinical presentations. This discussion aims to explore the features, etiology, clinical presentation, and management of TMA through a case series.
Methods:
A retrospective analysis was carried out on individuals who had biological TMA syndrome at the time of admission or as it developed while they were in the hospital, from the year 2021 to 2023. The medical records of the patients were reviewed to confirm the diagnosis of TMA and determine the etiology. Following a hierarchical analysis in accordance with existing classification, patients were classified as primary, secondary, and infection-associated TMAs. Medical records were analysed to collect information on plasma exchange/infusion, immunosuppressant usage, and the need for transfusions or kidney replacement therapy. Outcomes such as mortality, hematological, and kidney recovery were also recorded.
Results:
A total of 15 patients with the full biological syndrome of TMA were recruited and analysed. Out of 15 patients, 11 (73.3%) were female and 4 (26.6%) were of paediatric age. Overall, our case series had favourable results. A total of 86% patients (13/15) had renal involvement, 38% (5/13) patients required renal replacement therapy and 76% (10/13) had complete renal recovery over a variable period. Overall mortality was 33% (5/15) in our study group: 50% (2/4) of patients died in the primary TMA group, 11% (1/9) died in the secondary TMA group, and both patients (100%) died in the infection-associated TMA group. A plasma exchange was required for 8/15(54%) individuals. In addition to plasma exchange, immunosuppressants were needed for 3/4 of the patients in the primary TMA group. In the drug-induced TMA group, CNI dosage reduction or CNI-to-mTOR conversion were used to treat all four patients and one patient required plasma exchange due to severe features. In the pregnancy-associated TMA group, two out of three patients needed plasma exchange since their severe symptoms persisted even after delivery and supportive measures had been taken. ADAMTS13 activity and complete complement function evaluation were only performed on selected individuals for a variety of reasons.
Conclusions:
Thrombotic microangiopathy is a complex disorder with diverse etiologies and clinical presentations. A case-series discussion serves to consolidate knowledge about diagnosis, treatment, and outcomes while also highlighting the variety in symptoms and clinical presentation, emphasizing the importance of a thorough assessment to identify TMA promptly.
I have no potential conflict of interest to disclose.
I did not use generative AI and AI-assisted technologies in the writing process.