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Transfusion and Apheresis Science :... Apr 2023
Review
Topics: Humans; Prognosis; Biomarkers; Purpura, Thrombotic Thrombocytopenic
PubMed: 36890096
DOI: 10.1016/j.transci.2023.103676 -
Pediatrics in Review Jul 2022
Topics: Eczema; Humans; Infant; Purpura; Sudden Infant Death
PubMed: 35773537
DOI: 10.1542/pir.2020-004900 -
Hematology. American Society of... Dec 2023Immune thrombotic thrombocytopenic purpura (iTTP) caused by an autoantibody-mediated deficiency of ADAMTS13 and atypical hemolytic syndrome (aHUS) caused by alternative...
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.
Topics: Humans; Purpura, Thrombotic Thrombocytopenic; Thrombotic Microangiopathies; Complement System Proteins; Syndrome; Autoantibodies; Purpura, Thrombocytopenic, Idiopathic; ADAMTS13 Protein
PubMed: 38066937
DOI: 10.1182/hematology.2023000501 -
JAAPA : Official Journal of the... Jul 2021
Topics: Humans; Purpura, Thrombotic Thrombocytopenic
PubMed: 34162809
DOI: 10.1097/01.JAA.0000750992.29972.11 -
Sultan Qaboos University Medical Journal Nov 2022
Topics: Humans; Purpura; Pigmentation Disorders; Exanthema; Eczema
PubMed: 36407701
DOI: 10.18295/squmj.1.2022.004 -
MMW Fortschritte Der Medizin Nov 2023
Topics: Humans; Leg; Purpura; Face
PubMed: 37919574
DOI: 10.1007/s15006-023-3126-2 -
Blood Jan 2023
Topics: Pregnancy; Female; Humans; Purpura, Thrombocytopenic, Idiopathic; Cohort Studies; Thrombocytopenia; Patients
PubMed: 36602822
DOI: 10.1182/blood.2022018082 -
British Journal of Haematology Jun 2022Thrombotic thrombocytopenic purpura (TTP) is a rare and potentially fatal condition, with >90% mortality if untreated; deficiency of ADAMTS13 leads to widespread... (Review)
Review
Thrombotic thrombocytopenic purpura (TTP) is a rare and potentially fatal condition, with >90% mortality if untreated; deficiency of ADAMTS13 leads to widespread microvascular thromboses and organ injury particularly affecting organs with high shear stress, including the brain. The acute neurological complications have historically been those most feared by clinicians and synonymous with a poor prognosis. TTP, however, is no longer perceived as two extremes of acute presentation and remission, rather once diagnosed a chronic condition with the potential for a long-term symptom burden. Optimal neuroimaging timing and modality lacks consensus and as we learn more about the changes seen during the acute and chronic stages of TTP, there is scope for neuroimaging to play a greater role in guiding management and the secondary prevention of vascular disease. Reduced ADAMTS13 activity levels have been associated with increased thrombotic risk and novel therapies including caplacizumab and recombinant ADAMTS13 may offer a neuroprotective role. Given the increasing evidence of the neurocognitive and psychological disease in TTP, the importance of screening and timely intervention should not be underestimated. As more patients are surviving their initial TTP presentation, it is crucial for us to develop a greater understanding of the longer-term morbidity affecting these patients.
Topics: ADAMTS13 Protein; Humans; Purpura, Thrombotic Thrombocytopenic
PubMed: 35277972
DOI: 10.1111/bjh.18127 -
Journal of the European Academy of... Feb 2022
Topics: Genitalia; Humans; Purpura; Suction
PubMed: 34487607
DOI: 10.1111/jdv.17644 -
Cutis May 2022
Topics: Humans; Leg; Levamisole; Purpura
PubMed: 35856753
DOI: 10.12788/cutis.0524