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Prolonged inhibition of von Willebrand factor‐cleaving protease after splenectomy in a 22‐year‐old patient with acute and plasma refractory thrombotic thrombocytopenic purpura
Author(s) -
Langer Florian,
Bergmann Frauke,
Budde Ulrich,
HegewischBecker Susanna,
Hossfeld Dieter Kurt
Publication year - 2002
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1046/j.1365-2141.2002.03566.x
Subject(s) - splenectomy , thrombotic thrombocytopenic purpura , medicine , refractory (planetary science) , platelet , von willebrand factor , gastroenterology , protease , immunology , thrombocytopenic purpura , spleen , chemistry , biology , enzyme , biochemistry , astrobiology
Summary. We report a 22‐year‐old woman with acute, plasma refractory thrombotic thrombocytopenic purpura (TTP) in whom splenectomy led to consistent stabilization of platelet counts, but who showed complete inhibition of vonWillebrand factor‐cleaving protease (VWF‐cp) after 6 months of follow up. Persistent protease deficiency and resolved clinical and haematological TTP symptoms resulted in the transient appearance of unusually large VWF multimers in the patient plasma. As low but significant protease activity (10%) was first detectable as late as 9 months after splenectomy, we conclude tentatively that, at least in a subgroup of patients with acquired TTP, the beneficial effect of splenectomy is not exclusively due to the removal of splenic B lymphocytes producing an inhibitor of VWF‐cp.