Pregnancy-Associated Atypical Hemolytic Uremic Syndrome: A Case Report with MCP Gene Mutation and Successful Eculizumab Treatment
We present a case of a 25-year-old primigravida who experienced severe preeclampsia and HELLP syndrome followed by the development of complicated P-aHUS during the early postpartum period. The patient exhibited severe clinical manifestations, including hypertensive emergency, central nervous system involvement, renal impairment, and microangiopathic hemolytic anemia. Timely initiation of eculizumab therapy resulted in successful disease remission. Further genetic analysis revealed a likely rare pathogenic MCP gene variant. [...] Thieme Medical Publishers, Inc. 333 Seventh Avenue, 18th Floor, New York, NY 10001, USAArticle ...
Source: American Journal of Perinatology Reports - February 21, 2024 Category: Perinatology & Neonatology Authors: Dom ínguez-Vargas, Alex Ari ño, Fanny Silva, Diana Gonz ález-Tórres, Henry J. Aroca-Martinez, Gustavo Egea, Eduardo Musso, Carlos G. Tags: Case Report Source Type: research

Clinical Evaluation and Management of Thrombotic Microangiopathy
Thrombotic microangiopathy (TMA) refers to a diverse group of diseases that share clinical and histopathologic features. TMA is clinically characterized by microangiopathic hemolytic anemia, consumptive thrombocytopenia, and organ injury that stems from endothelial damage and vascular occlusion. There are several disease states with distinct pathophysiological mechanisms that manifest as TMA. These conditions are associated with significant morbidity and mortality and require urgent recognition and treatment. Thrombotic thrombocytopenic purpura and hemolytic uremic syndrome are traditionally considered to be primary forms ...
Source: Arthritis and Rheumatology - December 1, 2023 Category: Rheumatology Authors: Joshua Leisring, Sergey V. Brodsky, Samir V. Parikh Tags: Review Source Type: research

HUS and TTP: traversing the disease and the age spectrum
Hemolytic uremic syndrome (HUS) and thrombotic thrombocytopenia purpura (TTP) are rare diseases sharing a common pathological feature, thrombotic microangiopathy (TMA). TMA is characterized by microvascular thrombosis with consequent thrombocytopenia, microangiopathic hemolytic anemia and/or multiorgan dysfunction. In the past, the distinction between HUS and TTP was predominantly based on clinical grounds. However, clinical presentation of the two syndromes often overlaps and, the differential diagnosis is broad. (Source: Seminars in Nephrology)
Source: Seminars in Nephrology - November 9, 2023 Category: Urology & Nephrology Authors: Roberta Donadelli, Aditi Sinha, Arvind Bagga, Marina Noris, Giuseppe Remuzzi Source Type: research

Clinical Features and Risk Factors of Relapse and Mortality in Thrombotic Thrombocytopenic Purpura Patients: A Seven-Year Experience
Conclusion: Based on the largest study of TTP patients ever in Iran, the demographic and clinical characteristics of Iranian TTP patients are similar to other existing reports. Knowledge of the risk factors for TTP relapse and mortality could be useful to alert hematologists for prompt therapeutic actions when necessary.PMID:37817970 | PMC:PMC10560647 | DOI:10.18502/ijhoscr.v17i3.13305 (Source: Cell Research)
Source: Cell Research - October 11, 2023 Category: Cytology Authors: Sahar Tavakoli Shiraji Mohammad Reza Rostami Hosein Kamranzadeh Foumani Seied Asadollah Mousavi Mohammad Vaezi Soroush Rad Davood Babakhani Maryam Barkhordar Tanaz Bahri Ghasem Janbabaei Ashraf Malekmohammadi Saeed Mohammadi Vahid Mansouri Source Type: research

The Clinical Evaluation and Management of Thrombotic Microangiopathy
This article is protected by copyright. All rights reserved. (Source: Arthritis and Rheumatology)
Source: Arthritis and Rheumatology - August 23, 2023 Category: Rheumatology Authors: Joshua Leisring, Sergey V Brodsky, Samir V Parikh Tags: Review Source Type: research