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Acquired Thrombotic Thrombocytopenic Purpura

A Disease due to Inhibitors of ADAMTS13

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Book cover ADAMTS13

Abstract

Conventionally, thrombotic thrombocytopenic purpura (TTP) has been defined as a syndrome of thrombocytopenia and microangiopathic hemolysis (MAHA) without another plausible cause. In some versions of the definition, neurologic deficits (triad), renal abnormalities, and fever (pentad) are also included in the diagnostic criteria. These definitions of TTP fail to distinguish among the various causes of thrombocytopenia and MAHA. The discovery of ADAMTS13 and its deficiency in a subset of conventionally defined “TTP” has broadened our knowledge and transformed our views on how to define TTP as a disease. With a mechanistic definition, it is now possible to distinguish TTP from other causes of MAHA, to make the diagnosis of TTP in patients without thrombocytopenia or MAHA, and to manage the disease more rationally.

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Abbreviations

ADAMTS13:

A disintegrin and metalloprotease with thrombospondin type 1 repeat, member 13

aHUS:

Atypical hemolytic-uremic syndrome

CUB:

Complement C1r/C1s, Uegf, Bmp1

Cys:

Cysteine-rich region

DIC:

Disseminated intravascular coagulopathy

Dis:

Disintegrin

ELISA:

Enzyme-linked immunosorbent assay

FRET:

Fluorescence resonance energy transfer

GPI:

Glycosylphosphatidylinositol

HELLP:

Hemolysis, elevated liver enzymes, and low platelet counts

IFN-γ:

Interferon-gamma

IL-4:

Interleukin-4

ITP:

Idiopathic thrombocytopenic purpura

MAHA:

Microangiopathic hemolytic anemia

MP:

Metalloprotease

SDS-PAGE:

Sodium dodecyl sulfate-polyacrylamide gel electrophoresis

Spa:

Spacer domain

TMA:

Thrombotic microangiopathy

TNF-α:

Tumor necrosis factor-alpha

TSR:

Thrombospondin type 1 repeat

TTP:

Thrombotic thrombocytopenic purpura

VWF:

von Willebrand factor

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Authors and Affiliations

  1. iMAH Hematology Associates, New Hyde Park, USA

    Han-Mou Tsai M.D.

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  1. Han-Mou Tsai M.D.
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Correspondence to Han-Mou Tsai M.D. .

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  1. Division of Hematology, University of Utah Health Sciences Center, Salt Lake City, Utah, USA

    George M. Rodgers

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Tsai, HM. (2015). Acquired Thrombotic Thrombocytopenic Purpura. In: Rodgers, G. (eds) ADAMTS13. Springer, Cham. https://doi.org/10.1007/978-3-319-08717-7_6

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