[Transitory myelofibrosis in a case of diaphyseal dysplasia (Camurati-Engelmann's disease) (author's transl)].
A Greek child aged 2 with diaphyseal dysplasia developed an anaemia which required transfusion. The liver, spleen and lymph nodes were enlarged, there was pancytopenia and polyclonal hypergammaglobulinaemia. Biopsy provided evidence for myelofibrosis with extramedullary erythropoiesis. He was treated with small doses of corticosteroids and during his third year there was complete regression of the disease without recurrence when treatment was stopped. Disturbances of haematopoiesis in diaphyseal dysplasia has not been reported previously. These unusual findings are of clinical interest and also affect the clinical diagnosis.
D001848:Bone Diseases, Developmental / Q000150:complications*; D003966:Camurati-Engelmann Syndrome / Q000150:complications* / Q000175:diagnosis; D002675:Child, Preschool; D006801:Humans; D008297:Male; D011239:Prednisolone / Q000627:therapeutic use; D055728:Primary Myelofibrosis / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology*; D013997:Time Factors
D011239:Prednisolone
Transitorisches Myelofibrose-Syndrom bei diaphysärer Dysplasie (M. Camurati-Engelmann).