How I treat patients with thrombotic thrombocytopenic purpura: 2010.

@article{George2010HowIT,
  title={How I treat patients with thrombotic thrombocytopenic purpura: 2010.},
  author={James N George},
  journal={Blood},
  year={2010},
  volume={116 20},
  pages={
          4060-9
        }
}
Thrombotic thrombocytopenic purpura (TTP) is the common name for adults with microangiopathic hemolytic anemia, thrombocytopenia, with or without neurologic or renal abnormalities, and without another etiology; children without renal failure are also described as TTP. The diagnosis of TTP is an indication for plasma exchange treatment, but beginning treatment requires sufficient confidence in the diagnosis to justify the risk of plasma exchange complications. Documentation of a severe… Expand
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How I treat patients with thrombotic thrombocytopenic purpura-hemolytic uremic syndrome.
TLDR
Although the increased survival rate is dramatic and recent advances in understanding the pathogenesis of these syndromes are remarkable, clinical decisions remain empirical, and the management decisions for patients with suspected TTP-HUS rely on individual experience and opinion, resulting in many different practice patterns. Expand
Survival and relapse in patients with thrombotic thrombocytopenic purpura.
TLDR
The Oklahoma TTP Registry is a population-based inception cohort of all 376 consecutive patients with an initial episode of clinically diagnosed TTP, and ADAMTS13 deficiency during remission was not clearly related to subsequent relapse. Expand
ADAMTS13 activity in thrombotic thrombocytopenic purpura-hemolytic uremic syndrome: relation to presenting features and clinical outcomes in a prospective cohort of 142 patients.
TLDR
Severe ADAMTS13 deficiency does not detect all patients who may be appropriately diagnosed with TTP-HUS and who may respond to plasma exchange treatment, and many patients in all ADAMts13 activity categories apparently responded to Plasma exchange treatment. Expand
Occult systemic malignancy masquerading as thrombotic thrombocytopenic purpura- hemolytic uremic syndrome
TLDR
Hematologists/ oncologists must be aware that patients with clinically diagnosed TTP-HUS may have an occult systemic malignant disorder, and patients may be treated with plasma exchange, a procedure with substantial risk. Expand
Disseminated malignancy misdiagnosed as thrombotic thrombocytopenic purpura: A report of 10 patients and a systematic review of published cases.
TLDR
A search for systemic malignancy, including a bone marrow biopsy, is appropriate when patients with TTP have atypical clinical features or fail to respond to plasma exchange, and Occult disseminated malignancies may mimic TTP. Expand
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TLDR
The data suggest that severe ADAMTS-13 deficiency does not detect all patients who may be appropriately diagnosed with TTP-HUS and who may respond to plasma exchange treatment. Expand
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TLDR
Comparison with the other 245 patients who had an allogeneic BMT during the same period demonstrated that patients with a TTP-like syndrome more frequently had unrelated and/or HLA-mismatched donors, and had also experienced more serious complications: grade III–IV acute GVHD and systemic bacterial, fungal, and viral infections. Expand
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The incomplete penetrance of mutations in either ADAMTS13 or complement regulatory genes suggests that precipitating events or triggers may be required to cause thrombotic microangiopathy in many patients. Expand
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TLDR
This review systematically reviewed all published reports describing patients who had been diagnosed with both TTP and SLE and identified 87 patients, limiting the discussion of TTP to the subset of patients who have an acquired severe deficiency of ADAMTS13 (A disintegrin and metalloprotease with thrombospondin-1-like repeats), a von Willebrand factor-cleaving protease. Expand
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TLDR
The substantial risks associated with PE treatment for TTP-HUS are confirmed, and the continuing experience during the subsequent 3 years, 2002 to 2005, is described and confirmed. Expand
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