Clinicopathological Spectrum and Treatment Outcome of Thrombotic Microangiopathy

Background: Thrombotic microangiopathy (TMA) is a rare but life-threatening disorder predominantly involving the small vessels. It is a pathological condition which consists of microangiopathic hemolytic anemia, thrombocytopenia, and microvascular occlusion resulting in end-organ damage predominantl...

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Main Authors: Sarang Vijayan, Swarnalatha Guditi, Raja Karthik, Megha Uppin, Gangadhar Taduri
Format: Article
Language:English
Published: Wolters Kluwer Medknow Publications 2023-04-01
Series:Indian Journal of Kidney Diseases
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Online Access:https://journals.lww.com/10.4103/ijkd.ijkd_12_23
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Summary:Background: Thrombotic microangiopathy (TMA) is a rare but life-threatening disorder predominantly involving the small vessels. It is a pathological condition which consists of microangiopathic hemolytic anemia, thrombocytopenia, and microvascular occlusion resulting in end-organ damage predominantly involving kidneys and brain. Methods: This was a single-center retrospective cohort study of all patients diagnosed with kidney biopsy proven TMA at Nizams Institute of Medical Sciences. Results: During the study period of 3 years from 2018 to 2020, native kidney biopsies were done in 1014 patients. Out of these, 19 patients were diagnosed to have biopsy proven TMA. The incidence of TMA in our study was 1.87%. The most common age group was 21–40 years (79%). The most common presentation of TMA was acute kidney injury (89.4%), which includes 15.8% of pregnancy-related acute kidney injury. Malignant hypertension was the most common underlying etiology among the study subjects (31.6%). Systemic features of TMA like hemolysis were present in 47.4% of total patients. The most common autoimmune disease associated was systemic lupus erythematosus. Complement-mediated TMA was present in 21.1% of total cases. Progression to end-stage renal disease was more common in malignant hypertension-related TMA. Autoimmune and complement-mediated TMA were treated with plasma exchange and intravenous immunoglobulin Conclusion: TMA is a pathological entity associated with heterogeneous group of diseases with different presentations and clinical outcomes. Depending on the underlying etiology, the clinical outcomes differ.
ISSN:2950-0761