Pancreatitis preceding acute episodes of thrombotic thrombocytopenic purpura-hemolytic uremic syndrome: report of five patients with a systematic review of published reports

Swisher, Karen K.; Doan, John; Vesely, Sara K.; Kwaan, Hau C.; Kim, Benjamin; Lämmle, Bernhard; Hovinga, Johanna A. Kremer; George, James N.

doi:10.3324/haematol.10963

Cited by 82 publications

(72 citation statements)

References 46 publications

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“…However, these patients were not specifically questioned about premorbid infections or recent antibiotic usage, compared with our patient group so the incidence of premorbid infection may have been underestimated. It is interesting that pancreatitis or hepatobiliary sepsis was also a common premorbid factor in our study [18] and pancreatitis has also been reported as a potential precipitant for TMA in a series of patients from the Oklahoma Registry [28]. One potential confounding factor may be misdiagnosis of DIC associated with acute sepsis as TMA.…”

Section: Discussionmentioning

confidence: 80%

Infection frequently triggers thrombotic microangiopathy in patients with preexisting risk factors: A single‐institution experience

Douglas

Pollock

Young

et al. 2010

J of Clinical Apheresis

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Thrombotic microangiopathies are rare conditions characterized by microangiopathic hemolytic anemia, microthrombi, and multiorgan insult. The disorders, which include hemolytic uremic syndrome and thrombotic thrombocytopenic purpura, are often acute and life threatening. We report a retrospective analysis of 65 patients presenting to our institution from 1997 to 2008 with all forms of thrombotic microangiopathy. Therapeutic plasma exchange was a requirement for analysis and 65 patients were referred to our institution; 66% of patients were female and median age at presentation was 52 years. Bacterial infection was the most commonly identified etiologic factor and in the multivariate model was the only significant variable associated with survival outcome (odds ratio 5.1, 95% confidence interval, 1.2-21.7). As infection can be considered a common trigger event for thrombotic microangiopathy, patients with hepatobiliary sepsis may benefit from elective cholecystectomy. We conclude that bacterial infection frequently triggers TTP and other thrombotic microangiopathies in patients with preexisting risk factors and propose a model for the development of these syndromes. J. Clin. Apheresis 25:47-53, 2010. V V C 2010 Wiley-Liss, Inc.

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Section: Discussionmentioning

confidence: 80%

Infection frequently triggers thrombotic microangiopathy in patients with preexisting risk factors: A single‐institution experience

Douglas

Pollock

Young

et al. 2010

J of Clinical Apheresis

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“…[2][3][4] Patients defined as idiopathic were also heterogeneous. Although these patients were not recognized to have any of the conditions defining the other established clinical categories, some had preceding or concurrent conditions, such as pancreatitis, 34 infections, 35 or surgery, 36,37 which may have triggered the onset of TTP, reflecting the current arbitrary definition of "idiopathic" TTP. Only 46 (47%) of the 98 patients defined as idiopathic had ADAMTS13 activity Ͻ 10%.…”

Section: Discussionmentioning

confidence: 99%

Survival and relapse in patients with thrombotic thrombocytopenic purpura

Hovinga

Vesely²,

Terrell³

et al. 2010

Blood

491

540

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Survival of patients with thrombotic thrombocytopenic purpura (TTP) improved dramatically with plasma exchange treatment, revealing risk for relapse. The Oklahoma TTP Registry is a population-based inception cohort of all 376 consecutive patients with an initial episode of clinically diagnosed TTP (defined as microangiopathic hemolytic anemia and thrombocytopenia with or without signs and symptoms of ischemic organ dysfunctions) for whom plasma exchange was requested, 1989 to 2008. Survival was not different between the first and second 10-year periods for all patients (68% and 69%, P ‫؍‬ .83) and for patients with idiopathic TTP (83% and 77%, P ‫؍‬ .33). ADAMTS13 activity was measured in 261 (93%) of 282 patients since 1995. Survival was not different between patients with ADAMTS13 activity < 10% (47 of 60, 78%) and patients with 10% or more (136 of 201, 68%, P ‫؍‬ .11). Among patients with ADAMTS13 activity < 10%, an inhibitor titer of 2 or more Bethesda units/mL was associated with lower survival (P ‫؍‬ .05). Relapse rate was greater among survivors with ADAMTS13 activity < 10% (16 of 47, 34%; estimated risk for relapse at 7.5 years, 41%) than among survivors with ADAMTS13 activity of 10% or more (5 of 136, 4%; P < .001). In 41 (93%) of 44 survivors, ADAMTS13 deficiency during remission was not clearly related to subsequent relapse. (Blood. 2010;115:1500-1511)

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“…She also had no history of prior clotting events or miscarriages as one might expect for someone suffering from a catastrophic antiphospholipid antibody syndrome presenting as TTP/HUS (13). Other potential causes of TTP/HUS, such as pancreatitis, disseminated malignancy, severe hypertension, or stem cell or bone marrow transplant, were not present (14)(15)(16)(17).…”

Section: Discussionmentioning

confidence: 99%

Attending Rounds

Clark

Hildebrand

2012

Clinical Journal of the American Society of Nephrology

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SummaryThe classification of thrombotic microangiopathy has evolved and expanded due to treatment and advances in understanding of the diseases associated with this clinical presentation. The three clinical forms of thrombotic microangiopathy-thrombotic thrombocytopenic purpura (TTP), hemolytic uremic syndrome (HUS), and disseminated intravascular coagulation-encompass a wide range of disorders that can be classified as either primary (idiopathic) or secondary to another identifiable disease or clinical context. Identification of an inhibitor to a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13 (ADAMTS13) in the idiopathic and acute forms of TTP, recognition of the absence of ADAMTS13 inhibition in diarrheal HUS, identification of complement abnormalities in atypical HUS, and a better understanding of the role of plasma therapy, rituximab, and eculizumab therapy have all had a major effect on current understanding of the thrombotic microangiopathies. In this Attending Rounds, a patient with a thrombotic microangiopathy is presented, along with discussion highlighting the difficulty of differentiating TTP from HUS and disseminated intravascular coagulation, the need for a prompt diagnosis, and the role for plasma therapy in appropriately selected patients. The discussion attempts to provide a simple clinical approach to the diagnosis, treatment options, and future course of adults and children suffering from a thrombotic microangiopathy.

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Pancreatitis preceding acute episodes of thrombotic thrombocytopenic purpura-hemolytic uremic syndrome: report of five patients with a systematic review of published reports

Cited by 82 publications

References 46 publications

Infection frequently triggers thrombotic microangiopathy in patients with preexisting risk factors: A single‐institution experience

Infection frequently triggers thrombotic microangiopathy in patients with preexisting risk factors: A single‐institution experience

Survival and relapse in patients with thrombotic thrombocytopenic purpura

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