Warning: file_get_contents(https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&id=25784681
&cmd=llinks): Failed to open stream: HTTP request failed! HTTP/1.1 429 Too Many Requests
in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 215
Deprecated: Implicit conversion from float 211.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534
Deprecated: Implicit conversion from float 211.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534
Warning: imagejpeg(C:\Inetpub\vhosts\kidney.de\httpdocs\phplern\25784681
.jpg): Failed to open stream: No such file or directory in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 117 Blood
2015 ; 125
(25
): 3860-7
Nephropedia Template TP
gab.com Text
Twit Text FOAVip
Twit Text #
English Wikipedia
How I treat refractory thrombotic thrombocytopenic purpura
#MMPMID25784681
Sayani FA
; Abrams CS
Blood
2015[Jun]; 125
(25
): 3860-7
PMID25784681
show ga
Acquired thrombotic thrombocytopenic purpura (TTP) is characterized by
thrombocytopenia and microangiopathic hemolytic anemia (MAHA) without an obvious
cause, and may include fever, mild renal failure, and neurologic deficits. It is
characterized by a deficiency of the von Willebrand factor (VWF) cleaving enzyme,
ADAMTS13 (a disintegrin and metalloproteinase, with a thrombospondin type 1
motif, member 13), resulting in formation of microthrombi in the high sheer
environment of the microvasculature. This causes microvascular occlusion, MAHA,
and organ ischemia. Diagnosis is based on the presence of clinical symptoms,
laboratory aberrations consistent with MAHA, decreased ADAMTS13 activity, and
possibly presence of anti-ADAMTS13 autoantibodies. Upfront treatment of acute TTP
includes plasma exchange and corticosteroids. A significant number of patients
are refractory to this treatment and will require further interventions. There
are limited data and consensus on the management of the refractory TTP patient.
Management involves simultaneously ruling out other causes of thrombocytopenia
and MAHA, while also considering other treatments. In this article, we describe
our management of the patient with refractory TTP, and discuss use of rituximab,
increased plasma exchange, splenectomy, and immunosuppressive options, including
cyclophosphamide, vincristine, and cyclosporine. We also review recent evidence
for the potential roles of bortezomib and N-acetylcysteine, and explore new
therapeutic approaches, including recombinant ADAMTS13 and anti-VWF therapy.
free
free
free
