11/13. Effective stimulation of neutropoiesis with rh G-CSF in dyskeratosis congenita: a case report.dyskeratosis congenita (DC) is a rare congenital X-linked disorder. The major clinical manifestations are abnormal skin pigmentation, nail dystrophy, and leukoplakia of mucosal membranes. About 50% of the patients develop bone marrow failure, which is partly responsible for the poor prognosis. Recombinant human granulocyte-macrophage colony-stimulating factor (rhGM-CSF) has been administered to some neutropenic patients with DC, but only a moderate stimulation of neutropoiesis has been observed. We report on a patient with DC treated with recombinant human granulocyte-colony-stimulating factor (rhG-CSF). This treatment resulted in a substantial dose-dependent increase in the neutrophil count.- - - - - - - - - - ranking = 1keywords = leukoplakia (Clic here for more details about this article) |
12/13. Treatment of the hematological manifestations of dyskeratosis congenita.dyskeratosis congenita is a congenital multisystem disorder, characterized by skin pigmentation, dystrophic nails, and leukoplakia. Hematologic abnormalities progressing to severe pancytopenia play a significant role in the poor prognosis of afflicted patients. We report on a patient with dyskeratosis congenita and severe aplastic anemia, complicated by life threatening infection. The patient was treated with recombinant granulocyte-macrophage colony-stimulating factor. This therapy resulted in a moderate and transient improvement in absolute neutrophil counts. Current concepts regarding the pathogenesis and etiology of dyskeratosis congenita are discussed, while reviewing the available therapeutic options.- - - - - - - - - - ranking = 1keywords = leukoplakia (Clic here for more details about this article) |
13/13. Case report: gastric carcinoma as a complication of dyskeratosis congenita in an adolescent boy.dyskeratosis congenita (DC), or the Zinsser-Engman-Cole syndrome, is a rare X-linked heritable disorder, affecting primarily the ectodermal tissues, with hyperpigmentation of the skin, leukoplakia of the buccal and anal mucosa, and nail dystrophy (1, 2). Aplastic anemia (3) and a variety of neoplasms (4, 5) are some of the extraectodermal manifestation of this disorder, which although X-linked recessive, has also been described in a few females (6, 7). Mental retardation, diarrhea, and gastrointestinal bleeding have been considered to be less frequent features (8). We report an adolescent Indian male who presented with all the ectodermal manifestations, as well as mental retardation, bone marrow aplasia, and gastrointestinal hemorrhage secondary to adenocarcinoma of the stomach.- - - - - - - - - - ranking = 1keywords = leukoplakia (Clic here for more details about this article) |
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