Cryoprecipitate poor plasma does not improve early response in primary adult thrombotic thrombocytopenic purpura (TTP)

Zeigler, Z.R.; Shadduck, R.K.; Gryn, Jeffrey; Rintels, Peter; George, James N.; Besa, Emmanuel C.; Bodensteiner, David; Silver, Bernard J.; Krämer, Robert

doi:10.1002/jca.1003.abs

Cited by 47 publications

(67 citation statements)

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“…Byrnes and colleagues reported on seven patients who did not respond to plasma exchange with FFP but responded to TPE with cryosupernatant of normal human plasma [77]. The advantage of cryosupernatant in the therapy of TTP was recently challenged by the results of the North American TTP Group [79] indicating similar efficacy of FFP and cryosupernatant. The use of cryosupernatant as replacement fluid appears to be a reasonable approach because the cryosupernatant not only contains about as much vWF-cp as FFP, but is deficient in the largest vWF multimers that are present in normal plasma.…”

Section: Inhibitor Presentmentioning

confidence: 99%

Deficient activity of von Willebrand factor-cleaving protease in thrombotic thrombocytopenic purpura

Furlan

2003

Expert Review of Cardiovascular Therapy

106

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Thrombotic thrombocytopenic purpura (TTP) is a dramatic intravascular platelet-clumping disorder characterized by microangiopathic hemolytic anemia, thrombocytopenia, neurologic abnormalities, renal insufficiency and fever. TTP is a rare disease but is almost always fatal if untreated. More than 80% of patients survive with plasma therapy. In healthy individuals, the proteolytic cleavage of ultralarge von Willebrand factor (vWF) multimers prevents spontaneous clumping of platelets in the microcirculation. Patients with TIP have either severe congenital deficiency of von Willebrand factor-cleaving protease (vWF-cp), or have autoantibodies that inhibit the protease. Determination of vWF-cp levels in patient plasma helps to distinguish between TTP and other thrombotic microangiopathies with similar clinical signs and symptoms. vWF-cp is a member of the ADAMTS family of metalloproteases and has been designated ADAMTS13.

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Section: Inhibitor Presentmentioning

confidence: 99%

Deficient activity of von Willebrand factor-cleaving protease in thrombotic thrombocytopenic purpura

Furlan

2003

Expert Review of Cardiovascular Therapy

106

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“…76,85,86 Severe ADAMTS13 deficiency is specific for idiopathic TTP and identifies a subgroup of patients with a high likelihood of response to plasma exchange. 98 Other prospective studies have not shown an apparent advantage to the use of CSP in TTP. Patients with normal ADAMTS13 activity have a much worse prognosis, although many factors probably contribute to this difference.…”

Section: Thrombotic Thrombocytopenic Purpuramentioning

confidence: 97%

Fresh Frozen Plasma and Related Products

Crookes¹,

Hillyer²

2007

Blood Banking and Transfusion Medicine

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“…Reports of ADAMTS13 activity in CPP show nearly equal to approximately 20 percent decreased levels compared to FFP 26,53 , 54 . A modest reduction of ADAMTS13 activity in CPP compared to FFP could explain trends in some studies showing a slightly slower response to treatment with CPP compared to FFP 42,52 and recent preliminary data showing approximately 20 percent lower ADAMTS13 activity levels after the first apheresis procedure in patients treated with CPP compared to FFP 55…”

Section: The Efficacy Of Cpp Versus Ffpmentioning

confidence: 98%

“…The only prospective, randomized, controlled trial comparing CPP to FFP as the sole replacement fluid in plasma exchange reported no significant differences between the two treatment groups in any outcome 52 . Survival rates were 77 and 79 percent in the FFP‐ and CPP‐treated groups, respectively, and the data showed trends toward slower treatment responses in the CPP‐treated group and a greater likelihood of relapse.…”

Section: The Efficacy Of Cpp Versus Ffpmentioning

confidence: 98%

The pathogenicity of von Willebrand factor in thrombotic thrombocytopenic purpura: reconsideration of treatment with cryopoor plasma

Raife

Friedman²,

Dwyre³

2005

Transfusion

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New developments in the understanding of thrombotic thrombocytopenic purpura (TTP) provide opportunities for improved patient care. A widely held historical model of TTP microvascular thrombosis implicated circulating ultra large von Willebrand factor (ULVWF) in causing spontaneous platelet (PLT) aggregation. From this pathogenic model, concerns about ULVWF in fresh-frozen plasma (FFP) used to treat patients led to widespread use of cryopoor plasma (CPP) as an alternative. There is scant evidence, however, that circulating ULVWF contributes to microvascular thrombosis in TTP. New evidence suggests that the formation of PLT aggregates in TTP may be mediated by VWF in the process of being released from endothelium. Moreover, clinical studies do not demonstrate superior efficacy of CPP compared to FFP in the treatment of TTP. Because CPP may have reduced concentrations of factors important in the treatment of TTP, including ADAMTS13 metalloprotease, a reappraisal of the use of CPP in the treatment of TTP is warranted.

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Cryoprecipitate poor plasma does not improve early response in primary adult thrombotic thrombocytopenic purpura (TTP)

Cited by 47 publications

References 0 publications

Deficient activity of von Willebrand factor-cleaving protease in thrombotic thrombocytopenic purpura

Deficient activity of von Willebrand factor-cleaving protease in thrombotic thrombocytopenic purpura

Fresh Frozen Plasma and Related Products

The pathogenicity of von Willebrand factor in thrombotic thrombocytopenic purpura: reconsideration of treatment with cryopoor plasma

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