Acquired thrombotic thrombocytopenic purpura due to antibody-mediated ADAMTS13 deficiency precipitated by a localized Castleman's disease: a case report.
Acquired ADAMTS13 inhibitor causing thrombotic thrombocytopenic purpura (TTP) may be precipitated by some infections, inflammatory diseases or neoplasia. We reported a case of refractory TTP precipitated by a newly diagnosed localized Castleman's disease (CD). TTP was initially treated with plasma exchange and immunosuppressive therapy with corticosteroids; however the treatment failed to promote sustained response. During hospitalization, an abdominal tumor was diagnosed and resected; the histological analysis revealed a CD of hyaline-vascular variant rich stroma. After tumor removal, the patient achieved a long-lasting clinical remission and normalized ADAMTS13 activity. This clinical case describes a novel association of acquired ADAMTS13 inhibitor and CD. The antibody to ADAMTS13 developed along with the systemic manifestation of CD and promptly disappeared after the resection of the tumor. There are reports of neoplasia-associated thrombotic microangiopathy however direct evidence of CD-dependent ADAMTS13 inhibitor had not yet been reported.
Hematology and Hemotherapy Center, University of Campinas, Hemocentro-Unicamp, Instituto Nacional de Ciência e Tecnologia do Sangue , Campinas , Brazil .
Thais Celi Lopes
TC
Benevides
Fernanda Andrade
FA
Orsi
Marina Pereira
MP
Colella
Priscila de Oliveira
Pde O
Percout
Muriel Silva
MS
Moura
Maria Almeida
MA
Dias
Betina Diniz
BD
Lins
Erich Vinicius de
EV
Paula
Jose
J
Vassallo
Joyce
J
Annichino-Bizzachi
D051722:ADAM Proteins / Q000172:deficiency* / Q000276:immunology*; D000071120:ADAMTS13 Protein; D000328:Adult; D000906:Antibodies / Q000276:immunology*; D001706:Biopsy; D005871:Castleman Disease / Q000150:complications* / Q000175:diagnosis / Q000276:immunology*; D018858:Germinal Center / Q000378:metabolism / Q000473:pathology; D006801:Humans; D007150:Immunohistochemistry; D008297:Male; D010976:Platelet Count; D011697:Purpura, Thrombotic Thrombocytopenic / Q000175:diagnosis / Q000209:etiology*; D014057:Tomography, X-Ray Computed
D000906:Antibodies; D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human
ADAMTS13; antibody; castleman’s disease; lymphoproliferative disorder; thrombotic microangiopathy; thrombotic thrombocytopenic purpura