RESUMO
Splenic rupture (SR) is a rare adverse event observed in patients treated with G-CSF as a peripheral hematopoietic stem cell (PHSC) mobilizing agent, mostly in myeloma multiple and amiloidosis; to date, to our knowledge, it has not been previously described in plasma-cell leukemia (PCL). We report a case of a woman with PCL, who presented a SR after PHSC mobilization with Cyclophosphamide+G-CSF. The spleen removed showed hematopoietic foci and amiloid material. In the course of a second mobilization, 2 months after, the patient died from sepsis. We considered it important to report this case, in order to keep in mind the possibility of SR in patients with malignant gammopathy.
Assuntos
Fator Estimulador de Colônias de Granulócitos/efeitos adversos , Leucemia Plasmocitária/complicações , Ruptura Esplênica/etiologia , Evolução Fatal , Feminino , Mobilização de Células-Tronco Hematopoéticas/métodos , Transplante de Células-Tronco Hematopoéticas/métodos , Humanos , Pessoa de Meia-Idade , Esplenomegalia/induzido quimicamente , Transplante AutólogoRESUMO
Despite improvement in the treatment of advanced classical Hodgkin lymphoma, approximately 30% of patients relapse or die as result of the disease. Current predictive systems, determined by clinical and analytical parameters, fail to identify these high-risk patients accurately. We took a multistep approach to design a quantitative reverse-transcription polymerase chain reaction assay to be applied to routine formalin-fixed paraffin-embedded samples, integrating genes expressed by the tumor cells and their microenvironment. The significance of 30 genes chosen on the basis of previously published data was evaluated in 282 samples (divided into estimation and validation sets) to build a molecular risk score to predict failure. Adequate reverse-transcription polymerase chain reaction profiles were obtained from 262 of 282 cases (92.9%). Best predictor genes were integrated into an 11-gene model, including 4 functional pathways (cell cycle, apoptosis, macrophage activation, and interferon regulatory factor 4) able to identify low- and high-risk patients with different rates of 5-year failure-free survival: 74% versus 44.1% in the estimation set (P < .001) and 67.5% versus 45.0% in the validation set (P = .022). This model can be combined with stage IV into a final predictive model able to identify a group of patients with very bad outcome (5-year failure-free survival probability, 25.2%).
Assuntos
Biomarcadores Tumorais/genética , Doença de Hodgkin/diagnóstico , Doença de Hodgkin/genética , Recidiva Local de Neoplasia/diagnóstico , Recidiva Local de Neoplasia/genética , Transdução de Sinais/efeitos dos fármacos , Algoritmos , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Biomarcadores Tumorais/metabolismo , Feminino , Perfilação da Expressão Gênica , Doença de Hodgkin/tratamento farmacológico , Humanos , Masculino , Pessoa de Meia-Idade , Modelos Estatísticos , Recidiva Local de Neoplasia/tratamento farmacológico , Neoplasia Residual/diagnóstico , Neoplasia Residual/tratamento farmacológico , Neoplasia Residual/genética , Análise de Sequência com Séries de Oligonucleotídeos , Inclusão em Parafina , RNA Mensageiro/genética , Indução de Remissão , Reação em Cadeia da Polimerase Via Transcriptase Reversa , Medição de Risco , Taxa de Sobrevida , Resultado do TratamentoRESUMO
We report the first case of hemophagocytic lymphohistiocytosis (HLH) induced by the monoclonal expansion of Epstein-Barr virus (EBV)-negative NK cells. Consanguinity of the patient's parents made it necessary to discard familial HLH in the patient and her sister with identical HLA markers and demonstrate that no cause other than the expansion of NK cells, which secrete high levels of gamma interferon, was inducing HLH in this patient.
Assuntos
Herpesvirus Humano 4/isolamento & purificação , Interferon gama/metabolismo , Células Matadoras Naturais/química , Células Matadoras Naturais/virologia , Linfo-Histiocitose Hemofagocítica/patologia , Receptores KIR2DL1/análise , Feminino , Humanos , Lactente , Células Matadoras Naturais/imunologia , Linfo-Histiocitose Hemofagocítica/imunologia , PaisRESUMO
Nonamyloidotic light-chain cardiomyopathy is a paraproteinemia-induced disorder. Unlike amyloidosis, light chain deposition may be reversible with appropriate treatment. We report a case of fatal light chain deposition disease manifested as cardiomyopathy, which was complicated by arterial embolism despite the maintenance of sinus rhythm.