Clinical Profile and Outcomes of Thrombotic Microangiopathy - A Prospective Study From a Tertiary Centre in India

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Abstract

Background Thrombotic microangiopathies (TMAs) are rare but life-threatening syndromes characterized by microangiopathic hemolytic anemia, thrombocytopenia, and organ dysfunction. While thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS) dominate Western cohorts, secondary TMAs due to snake envenomation and sepsis are increasingly recognized in tropical regions. We conducted a prospective study to evaluate the clinical spectrum, laboratory features, risk stratification, treatment, and outcomes of TMA in an Indian tertiary care setting. Methods Consecutive patients with newly diagnosed TMA were enrolled between March 2024 and April 2025. Patients were categorized as TTP, HUS, or secondary TMA. Demographic, clinical, and laboratory data were recorded. PLASMIC scores were calculated, and ADAMTS13 activity was assessed where feasible. Treatment strategies, including plasma exchange, dialysis, antisnake venom, and supportive care, were documented. The primary outcome was discharge status (recovered vs. died). Results Sixty-six patients were included: TTP (n = 16), HUS (n = 34), and secondary TMA (n = 16). Patients with TTP were older and more likely to present with neurologic symptoms, whereas HUS demonstrated the highest renal burden and dialysis requirement. Secondary TMAs were predominantly due to snakebite (n = 20) and sepsis (n = 17). Hemoglobin was lowest in secondary TMA, creatinine was highest in HUS, and LDH was elevated across all subtypes. The PLASMIC score effectively stratified TTP, with severe ADAMTS13 deficiency confirmed in most tested cases. Plasma exchange was performed in 14 patients (mainly TTP), while all snakebite cases received antisnake venom, 4 patients received plasma exchange. Overall survival was 71.2%: HUS 82.4%, TTP 62.5%, and secondary TMA 56.2%. Mortality was highest in sepsis-associated TMA. Conclusion In this prospective Indian cohort, TMA presented with distinct subtype-specific patterns. The PLASMIC score enabled early recognition of TTP, while snakebite- and sepsis-associated TMAs contributed substantially to disease burden and outcomes. These findings highlight the need for context-specific strategies for TMA management in tropical settings.

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