The Di Guglielmo Syndrome: A Study of 17 Cases

Abstract

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.

Study of clinical, haematological and cytogenetic profile of patients with acute erythroid leukaemia

“…One surprising feature was the occurrence of jaundice in about 31.25% of all AEL patients, but in all of the four patients of pEL. This is rarely though reported in other studies [24, 25]. Pancytopenia was seen in 81.25% of patients in this study which was higher than that in other studies.…”

Erythroleukaemia and daunorubicin‐induced cardiotoxicity in a young boy

“…Erythroleukaemia, a subtype of AML (M‐6), is uncommon in patients below 15 years of age, with a reported frequency of three out of 46 cases 3 and three out of 20 cases 4 . Other reports also indicate that the disease is uncommon in children 5,6 …”

“…Our patient was of interest because of two coexisting unusual features, 3–7,9 namely early age of onset of erythroleukaemia and daunorubicin‐induced CHF following the administration of relatively low doses. This case demonstrates, once again, that proper monitoring of cardiotoxicity is important when a cumulative dose of more than 300 mg/m 2 of anthracycline antineoplastic antibiotics is given, especially to children 9 …”