RESUMO
A 23-year-old man with chronic myelocytic leukemia (CML) in the first chronic phase underwent allogeneic peripheral blood stem cell transplantation (PBSCT) from his HLA-identical sibling. Pretransplant evaluations showed that he had a low risk of transplantation-related mortality and that the interval between the diagnosis of CML and PBSCT was only 6 months. However, he developed a variety of complications, including acute renal failure requiring hemodialysis, severe hepatic damage, hemorrhagic cystitis, and gastrointestinal hemorrhage leading to hypovolemic shock. Pathological examination of the colonic mucosa showed vascular endothelial damage and thrombotic lesions, leading to the diagnosis of thrombotic microangiopathy. Later, we found that he had the constitutional abnormality XYY. XYY syndrome is a frequent congenital abnormality, and mental disorders and congenital abnormalities of kidney and liver are common manifestations. Considering his clinical course, it was interesting that complications were severe in the organs which are frequently involved in cases of XYY syndrome. These organs may have poor function or poor reserves and may be more vulnerable to endothelial damage caused by high-dose cytotoxic chemotherapy. Patients with XYY syndrome might have a high risk of transplantation-related mortality.
Assuntos
Leucemia Mielogênica Crônica BCR-ABL Positiva/genética , Leucemia Mielogênica Crônica BCR-ABL Positiva/cirurgia , Transplante de Células-Tronco de Sangue Periférico/efeitos adversos , Cariótipo XYY , Adulto , Humanos , Masculino , Microcirculação , Trombose/etiologia , Transplante Homólogo , Cariótipo XYY/genéticaAssuntos
Serviço Hospitalar de Oncologia , Vigilância da População , Infecções por Vírus Respiratório Sincicial/transmissão , Infecção Hospitalar/transmissão , Infecção Hospitalar/virologia , Transmissão de Doença Infecciosa , Feminino , Neoplasias Hematológicas/complicações , Neoplasias Hematológicas/terapia , Humanos , Japão , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos , Vírus Sincicial Respiratório HumanoRESUMO
We have recently seen a patient who developed Pneumocystis carinii pneumonia (PCP) in the course of treatment for chronic lymphocytic leukemia (CLL). This case showed uncommon pathological findings with extensive formation of granulomatous lesions. Despite advanced CLL associated with poor B-cell function, she responded well to anti-PCP treatment. In contrast to B-cell function, the T-cell functions were well preserved in vitro, and the numbers of peripheral CD4- and CD8-positive cells were normal, and T-cell functions were normal. These findings suggest that the production of granulomatous lesions to PCP may have been associated with the patients' immune status, and that it may constitute a good indicator in PCP infection in patients with underlying hematological malignancy.
