Splenectomy in relapsing and plasma-refractory acquired thrombotic thrombocytopenic purpura.
OBJECTIVE
Acquired thrombotic thrombocytopenic purpura (TTP) is often due to autoantibodies inhibiting ADAMTS-13 activity resulting in impaired processing of very large von Willebrand factor multimers. TTP usually presents with an acute onset and a fulminant, sometimes fatal course. With appropriate treatment including plasma exchange, and fresh frozen plasma replacement, often supplemented by immuno-suppressive therapy, the acute episode generally resolves within days to weeks.


METHODS
We describe the clinical course of 3 patients with acquired TTP. One was refractory to PE, the other 2 relapsed after this treatment. All three were treated with splenectomy. ADAMTS-13 activity and inhibitor levels were monitored.


RESULTS
ADAMTS-13 activity was initially < 5% in all 3 patients. After splenectomy the inhibitor against ADAMTS-13 disappeared rapidly in 2 patients and there was full recovery of ADAMTS-13 activity in all 3 patients.


CONCLUSIONS
Splenectomy, by eliminating a source of pathogenic autoantibody production, can be a successful treatment for patients with relapsing or plasma-refractory acquired TTP due to autoantibody-mediated ADAMTS-13 deficiency.
Kremer Hovinga
Johanna A
JA
Central Hematology Laboratory, Inselspital, University of Bern, Bern, Switzerland.
Studt
Jan-Dirk
JD
Demarmels Biasiutti
Franziska
F
Solenthaler
Max
M
Alberio
Lorenzo
L
Zwicky
Caroline
C
Fontana
Stefano
S
Taleghani
Behrouz Mansouri
BM
Tobler
Andreas
A
Lämmle
Bernhard
B
D000071120:ADAMTS13 Protein; D000293:Adolescent; D000328:Adult; D000368:Aged; D001323:Autoantibodies / Q000276:immunology; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D008666:Metalloendopeptidases / Q000037:antagonists & inhibitors / Q000276:immunology / Q000378:metabolism; D010951:Plasma Exchange; D011697:Purpura, Thrombotic Thrombocytopenic / Q000209:etiology / Q000276:immunology / Q000601:surgery*; D013156:Splenectomy
D001323:Autoantibodies; C502939:ADAMTS13 protein, mouse; D008666:Metalloendopeptidases; D000071120:ADAMTS13 Protein