RESUMO
Although bone marrow failure in patients with dyskeratosis congenita (DKC) can be successfully treated with allogeneic hematopoietic cell transplantation (allo-HCT) using a reduced intensity conditioning (RIC) regimen, the outcome of nonhematological disorders in patients with DKC treated with allo-HCT using RIC has not been fully elucidated. Here, we describe the clinical course of nonhematological disorders after allo-HCT with RIC in 3 consecutive patients with DKC. Allo-HCT with RIC was feasible in all cases; however, patient 1 developed lethal pulmonary disease and patient 2 experienced progression of hepatic fibrosis. Careful follow-up of patient-specific complications is required after allo-HCT in patients with DKC.
Assuntos
Disceratose Congênita/terapia , Transplante de Células-Tronco Hematopoéticas/efeitos adversos , Adolescente , Aloenxertos , Pré-Escolar , Progressão da Doença , Disceratose Congênita/complicações , Humanos , Cirrose Hepática/etiologia , Pneumopatias/etiologia , Masculino , Condicionamento Pré-TransplanteRESUMO
We experienced a case of 10-year-old girl who developed hypersensitivity reactions after eating enokitake. The patient had food allergy to egg until 5 years old. When she was 4 years old, she ate enokitake with a hot-pot dish. Later, she felt itching in her mouth. Therefore, she never ate enokitake since that time. At the age of 10, she drank only the soup of enokitake with school lunch. After that she felt discomfort and itching in her oral cavity. The result of enokitake and other mushrooms (siitake, simeji, and eringi) skin prick to prick test were all positive. We performed Western blotting with enokitake extracts and the patient's serum. Enokitake protein's band (75kDa) reacted specifically with the patient's IgE. At the same time Western blotting was performed with the patient's serum of previously reported enokitake anaphylaxis, but a 75kDa band showing specific reaction in this case was not observed. This band we identified was a novel enokitake allergen.