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.

[Hyperandrogenic syndrome in a postmenopausal woman: a case report]. / Síndrome hiperandrogênica em mulher na pós-menopausa: relato de caso.

Hyperandrogenic syndromes include diseases that manifest through an increased biological activity of androgens and that can originate from neoplastic or functional diseases. Androgen-secreting ovarian tumors represent about 1% of ovarian neoplasias. Steroid cell tumors are among the more rare types which account for less than 0.1% of all ovarian tumors. They are usually benign, of small dimensions and unilateral. We report here a rare case of a unilateral steroid cell tumor. A 60-year-old woman was seen after four months of evolution of hirsutism, clitoris hypertrophy and elevation of serum estradiol levels. Her total testosterone and 17-OH-progesterone levels were also increased.

Síndrome hiperandrogênica em mulher na pós-menopausa: relato de caso / Hyperandrogenic syndrome in a postmenopausal woman: a case report

RESUMO As síndromes hiperandrogênicas englobam doenças que se manifestam através de um aumento da atividade biológica dos androgênios e podem ter origem em patologias neoplásicas ou funcionais. Os tumores ovarianos secretores de androgênios constituem cerca de 1 por cento das neoplasias do ovário. O tumor de células esteroides é um dos tipos mais raros, sendo responsáveis por menos de 0,1 por cento de todos os tumores ovarianos. São habitualmente benignos, de pequenas dimensões e unilaterais. Neste relato, apresenta-se o caso raro de um tumor unilateral de células esteroides. Paciente do sexo feminino, 60 anos, por hirsutismo, hipertorfia do clitóris e elevação dos níveis séricos de estradiol, com quatro meses de evolução. Apresentava níveis elevados de testosterona total e de 17-OH-Progesterona.

ABSTRACT Hyperandrogenic syndromes include diseases that manifest through an increased biological activity of androgens and that can originate from neoplastic or functional diseases. Androgen-secreting ovarian tumors represent about 1 percent of ovarian neoplasias. Steroid cell tumors are among the more rare types which account for less than 0.1 percent of all ovarian tumors. They are usually benign, of small dimensions and unilateral. We report here a rare case of a unilateral steroid cell tumor. A 60-year-old woman was seen after four months of evolution of hirsutism, clitoris hypertrophy and elevation of serum estradiol levels. Her total testosterone and 17-OH-progesterone levels were also increased.