JOURNAL ARTICLE
REVIEW

[Thrombotic-thrombocytopenic purpura]

M Hellmann, M Hallek, I Scharrer
Der Internist 2010, 51 (9): 1136, 1138-44
20689903
Thrombotic-thrombocytopenic purpura (TTP) is a microangiopathic disorder characterized by multiple von Willebrand-Factor (vWF) rich microthrombi affecting the arterioles and capillary vessels of several organs. Ultra large von Willebrand multimers cause the blood clotting process by linking to platelets due to a lack of a plasma metalloprotease named ADAMTS13. Deficiency of this vWF-cleaving enzyme is caused by an inborn mutation in the gene coding or, more often, by acquired autoantibodies that inhibit ADAMTS13. TTP is a life-threatening disease which requires urgent admission to a hematological centre. Plasmapheresis therapy should be started immediately when diagnosis of primary TTP is likely. Patients typically present with schistozytes, hemolysis, thrombocytopenia and neurological abnormalities such as headache, focal deficits or coma. The monoclonal CD20 antibody rituximab targets ADAMTS13 antibody production and has the potential to be an effective therapy for relapsed TTP or initial treatment to shorten duration of plasma exchange.

Full Text Links

Find Full Text Links for this Article

Discussion

You are not logged in. Sign Up or Log In to join the discussion.

Related Papers

Remove bar
Read by QxMD icon Read
20689903
×

Save your favorite articles in one place with a free QxMD account.

×

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"