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Medical consult: aHUS, TTP? How to distinguish and what to do.
Story, Charlotte M; Gerber, Gloria F; Chaturvedi, Shruti.
Affiliation
  • Story CM; Department of Medicine, Brigham and Women's Hospital, Harvard Medical School, Boston, MA.
  • Gerber GF; Division of Hematology, Department of Medicine, Johns Hopkins University School of Medicine, Baltimore, MD.
  • Chaturvedi S; Division of Hematology, Department of Medicine, Johns Hopkins University School of Medicine, Baltimore, MD.
Hematology Am Soc Hematol Educ Program ; 2023(1): 745-753, 2023 Dec 08.
Article in En | MEDLINE | ID: mdl-38066937
ABSTRACT
Immune thrombotic thrombocytopenic purpura (iTTP) caused by an autoantibody-mediated deficiency of ADAMTS13 and atypical hemolytic syndrome (aHUS) caused by alternative complement dysregulation are the most common primary thrombotic microangiopathies (TMAs). The evaluation of a patient with TMA is a medical emergency since it is critical to quickly distinguish iTTP and aHUS from other causes of TMA. Untreated iTTP is rapidly fatal, and delays in initiating complement inhibition in aHUS increase the risk of irreversible renal failure. An ADAMTS13 activity level of less than 10% is diagnostic of iTTP in the appropriate clinical setting. In settings where rapid-turnaround ADAMTS13 testing is not available, clinical features and clinical prediction tools are useful to identify patients who should receive emergent plasma exchange. We present an evidence-based approach to the initial (first 24 hours) diagnosis and management of iTTP and review the clinical and laboratory features that can be used to identify patients with aHUS who will benefit from early C5 blockade. We also discuss the potential use of complement blockade to improve outcomes in selected patients with secondary TMA.
Subject(s)

Full text: 1 Collection: 01-internacional Database: MEDLINE Main subject: Purpura, Thrombotic Thrombocytopenic / Purpura, Thrombocytopenic, Idiopathic / Thrombotic Microangiopathies Limits: Humans Language: En Journal: Hematology Am Soc Hematol Educ Program Journal subject: HEMATOLOGIA Year: 2023 Type: Article Affiliation country: Morocco

Full text: 1 Collection: 01-internacional Database: MEDLINE Main subject: Purpura, Thrombotic Thrombocytopenic / Purpura, Thrombocytopenic, Idiopathic / Thrombotic Microangiopathies Limits: Humans Language: En Journal: Hematology Am Soc Hematol Educ Program Journal subject: HEMATOLOGIA Year: 2023 Type: Article Affiliation country: Morocco