The Di Guglielmo Syndrome: A Study of 17 Cases

Abstract

Based on a series of 17 patients with Di Guglielmo's syndrome, i.e., erythraemic myelosis and erythroleukaemia, a description is given of the clinical and haematological manifestations of this disease. Three cases were anerythraemic, i.e. no erythroblasts were seen in the blood smears, and three were aleukaemic, i.e. no myeloblasts and/or promyelocytes were found in the blood. The percentage of erythroblasts in the bone marrow smears varied from 34 to 96. In all cases, abnormalities (often megaloblastoid changes) of varying severity were observed in the erythroblasts. Proerythroblasts showing erythrophagocytosis, multinuclearity and vacuolation of the cytoplasm are features of great diagnostic importance. Five patients had myeloblasts or promyelocytes containing Auer rods, six had neutrophilic granulocytes showing pseudo-Pelger- Huët anomaly, and in most cases agranular polymorphonuclears were present. Brief comments are given on the PAS, nuclear arylsulphatase and naphtol-AS-D-chloroacetate-esterase staining methods. Four of five patients studied cytogenetically revealed abnormal stem lines. The survival times after the onset of the disease ranged from 17 days to 20 months. The disease is closely related to myeloblastic leukaemia. In the course of Di Guglielmo's syndrome, the leukaemic component often becomes more conspicuous, so that, in the terminal phase, the disease cannot be distinguished from typical myeloblastic leukaemia. As, in Di Guglielmo's “leukaemia”, the diagnostic problems are greater than and different from those in typical myeloblastic leukaemia, it is still reasonable to place this disease in a separate group under acute granulocytic leukaemia.