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Fatores Imunológicos/uso terapêutico , Púrpura Trombocitopênica Trombótica/tratamento farmacológico , Rituximab/uso terapêutico , Proteína ADAMTS13/imunologia , Adulto , Feminino , Humanos , Fatores Imunológicos/administração & dosagem , Masculino , Pessoa de Meia-Idade , Púrpura Trombocitopênica Trombótica/imunologia , Rituximab/administração & dosagem , Resultado do Tratamento , Adulto JovemRESUMO
INTRODUCTION: Spontaneous splenic rupture is a rare occurrence in primary cytomegalovirus infection. CASES: We report two cases of spontaneous rupture of the spleen associated with primary cytomegalovirus infection in young immunocompetent adults. One patient had iron deficiency anemia, and the other a pyruvate kinase deficiency. Nonoperative management was successful in both cases. DISCUSSION: Nine other cases identified by a search of the medical literature are also reviewed. These cases do not show evidence of any particular risk factor.
Assuntos
Infecções por Citomegalovirus/complicações , Esplenopatias/virologia , Adulto , Feminino , Humanos , Masculino , Ruptura Espontânea , Esplenomegalia/virologia , Tomografia Computadorizada por Raios X , Resultado do TratamentoRESUMO
Type I cryoglobulinemia vasculitis (CryoVas) is considered a life-threatening condition; however, data on the characteristics and outcome are scarce. To analyze the presentation, prognosis, and efficacy and safety of treatments of type I CryoVas, we conducted a French nationwide survey that included 64 patients with type I CryoVas between January 1995 and July 2010: 28 patients with monoclonal gammopathy of unknown significance (MGUS) and 36 with hematologic malignancy.Type I monoclonal CryoVas was characterized by severe cutaneous involvement (necrosis and ulcers) in almost half the patients and high serum cryoglobulin levels, contrasting with a lower frequency of glomerulonephritis than expected. The 1-, 3-, 5-, and 10-year survival rates were 97%, 94%, 94%, and 87%, respectively. Compared to MGUS, type I CryoVas related to hematologic malignancy tended to be associated with a poorer prognosis. Therapeutic regimens based on alkylating agents, rituximab, thalidomide or lenalinomide, and bortezomib showed similar efficacy on vasculitis manifestations, with clinical response rates from 80% to 86%.Data from the CryoVas survey show that the prognosis of type I CryoVas does not seem to be as poor as previously suggested. Besides alkylating agents, the use of regimens based on rituximab, thalidomide or lenalinomide, and bortezomib are interesting alternative options, although the exact role of each strategy remains to be defined.