Results: 8

Dyskeratosis congenita

Autops. Case Rep; 10 (3), 2020
Dyskeratosis congenita (DC) is a genetic syndrome with progressive multisystem involvement classically characterized by the clinical triad of oral leukoplakia, nail dystrophy, and reticular hyperpigmentation. Frequent complications are bone marrow failure, increased rate of malignancy, lung and liver dis...

Regression of extramedullary hematopoiesis with hydroxyurea therapy in ß-thalassemia intermedia

Excessive ineffective erythropoiesis in thalassemia intermedia may cause extramedullary hematopoiesis (EMH), resulting in spleen and liver enlargement or masses in several tissues, mainly paravertebrally. Other less frequent locations of diffuse compensatory EMH are kidneys, adrenal glands, breasts, spin...

Spinal cord compression in beta-thalassemia: follow-up after radiotherapy

Säo Paulo med. j; 116 (6), 1998
Context: Spinal cord compression due to extramedullary hematopoiesis is a well-described but rare syndrome encountered in several clinical hematologic disorders, including beta-thalassemia. Case Report: We report the case of a patient with intermediate beta-thalassemia and crural paraparesis due to spina...