Acute pancreatitis-induced thrombotic thrombocytopenic purpura with recurrent acute pancreatitis

Fujino, Yasuhisa; Inoué, Yoshihiro; Onodera, Makoto; Kikuchi, Shinichi; Sato, Masayuki; Kojika, Masahiro; Sato, Hisaho; Suzuki, Keijiro; Matsumoto, Masanori

doi:10.1007/s12328-016-0632-0

Cited by 5 publications

(2 citation statements)

References 25 publications

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“…Acute pancreatitis (presumably resulting from ischemia) and bloody diarrhea (presumably resulting from bowel ischemia) have been described as initial manifestations. 19,20 The latter may easily be mistaken for Shiga toxin-associated HUS. Acute neurological symptoms, such as stroke or seizure, may precede the development of MAHA or thrombocytopenia.…”

Section: Epidemiology and Natural History Of Ttpmentioning

confidence: 99%

Clinical and laboratory diagnosis of TTP: an integrated approach

2018

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Thrombotic thrombocytopenia purpura (TTP) is a rare, life-threatening disease with an incidence of approximately 2 persons per million per year. It is characterized by severe deficiency of the von Willebrand cleaving protease, ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13), leading to formation of platelet-rich thrombi in the microvasculature. Prompt initiation of appropriate therapy, particularly plasma exchange, may be life-saving. Diagnosis of TTP is challenging because of its diverse clinical manifestations, overlap in clinical presentation with other thrombotic microangiopathies, and limited availability of ADAMTS13 testing. Clinical prediction scores have been developed to estimate the pretest probability of severe ADAMTS13 deficiency and may be used as an adjunct to clinical judgment to guide initial management decisions. An ADAMTS13 activity level of less than 10% supports the diagnosis of TTP in appropriate clinical contexts, but many centers do not offer testing in-house and must send out the test to a reference laboratory with a turnaround time of several days. In such instances, initial management decisions must be made without the benefit of laboratory testing. In patients with TTP, inhibitor tests may be useful for distinguishing immune-mediated from congenital TTP. In this article, we review the epidemiology, natural history, and clinical presentation of TTP and laboratory assays for TTP including ADAMTS13 activity and inhibitor assays. We also describe an evidence-based approach to the evaluation of a patient with suspected TTP that integrates clinical and laboratory assessment.

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Section: Epidemiology and Natural History Of Ttpmentioning

confidence: 99%

Clinical and laboratory diagnosis of TTP: an integrated approach

2018

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“…Pancreatitis is defined as typical abdominal pain associated with a lipase level above 3 N. Although there are no solid data on its prevalence, there are several articles reporting on cases of pancreatitis in TTP. Therefore, a lipase assay should be performed at the diagnosis of TTP to detect pancreatitis [60][61][62][63][64]. Some authors even hypothesize a role for acute pancreatitis and subsequent endothelial activation in the development of TTP [65].…”

Section: Pancreatitis and Gastrointestinal Disorder In Ttpmentioning

confidence: 99%

Severe Thrombotic Thrombocytopenic Purpura (TTP) with Organ Failure in Critically Ill Patients

Fodil

Zafrani

2022

JCM

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Thrombotic thrombocytopenic purpura (TTP) is a multiorgan disorder. Organ dysfunction occurs as a consequence of widespread microvascular thrombosis, especially in the heart, brain and kidney, causing transient or partial occlusion of vessels, resulting in organ ischemia. Intensive care unit (ICU) admission varies between 40% and 100% of patients with TTP, either because of severe organ failure or in order to initiate emergency plasma exchange (PEx). Severe neurologic manifestations and cardiac involvement have been associated with higher mortality. Acute kidney injury, although usually less severe than that in hemolytic and uremic syndrome, is common during TTP. Initial management in the ICU should always be considered in TTP patients. The current treatment of TTP in the acute phase is based on urgent PEx, combined with corticosteroid therapy, B-cell-targeted immunotherapy, rituximab and inhibition of the interaction between ultra-large Von Willebrand factor multimers and platelets, using caplacizumab, a monoclonal antibody. ICU management permits close monitoring and the rapid introduction of life-sustaining therapies. This review details the epidemiology of TTP in the ICU, organ failures of critically ill patients with TTP, and the initial management of TTP patients in the ICU.

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