Anerythraemic Di Guglielmo Syndrome

Petersen, Helena Søndergaard

doi:10.1111/j.0954-6820.1973.tb10593.x

Journal of Internal Medicine

1973

DOI: 10.1111/j.0954-6820.1973.tb10593.x

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Anerythraemic Di Guglielmo Syndrome

Helena Søndergaard Petersen

Abstract: As there may, perhaps, be a tendency to disregard a diagnosis of acute erythraemic myelosis when there are no erythroblasts in the peripheral blood, such a case observed in a 69‐year‐old man with pancytopenia and hypercellular bone marrow is reported. Of the bone marrow cells 85% were erythroblasts, most of which were basophilic. Many of the cells were megaloblastoid, often multilobular or multinuclear with a strongly positive PAS reaction. No erythroblasts were observed in the peripheral blood. The case must … Show more

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The Di Guglielmo Syndrome: A Study of 17 Cases

Søndergaard‐Petersen

1975

Journal of Internal Medicine

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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.

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The Di Guglielmo Syndrome: A Study of 17 Cases

Søndergaard‐Petersen

1975

Journal of Internal Medicine

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Aneryhremic form of acute erythremic myelosis (Di Guglielmo's syndrome) causing hepatosplenomegaly due to the infiltration of hemoglobin‐bearing blast cells: An autopsy case

Tsuji

Tamai

Terada

et al. 1995

Pathology International

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An autopsy case of a 42 year old man with the anerythremic form of acute erythremic myelosis (Di Guglielmo's syndrome) is reported. The patient was admitted because of a 1 month history of fatigue and fever. Physical examination showed hepatosplenomegaly. Laboratory data showed leukopenia, mild normocytic anemia, and high levels of serum lactate dehydrogenase and vitamin B12. Bone marrow aspirate revealed an elevated number of erythroblasts, with dyserythropoiesis (E/M = 3.7). After admission, thrombocytopenia progressed rapidly, but blast cells were not seen in the peripheral blood throughout the clinical course. On the 56th hospital day, the patient died of pneumonia. At autopsy, the spleen weighed 550 g and the liver 1800 g. Histologically, the white and red pulps of the spleen and the portal region and sinusoid of the liver were diffusely infiltrated by blast cells that were positive for anti-hemoglobin (Hb) antibody on immunoperoxidase staining. The bone marrow, the lymph nodes, the adrenal glands, the pancreas, and the heart were also infiltrated by the blast cells. This was thus considered to be a rare case of the anerythremic form of acute erythremic myelosis (Di Guglielmo's syndrome), the findings showing that Hb immunoperoxidase staining is useful for the diagnosis of this condition.

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Erythrophagocytosis by Pathological Erythroblasts in the Di Guglielmo Syndrome A Study of 18 Cases

Søndergaard‐Petersen

1974

Scandinavian Journal of Haematology

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Anerythraemic Di Guglielmo Syndrome

Cited by 3 publications

References 2 publications

The Di Guglielmo Syndrome: A Study of 17 Cases

The Di Guglielmo Syndrome: A Study of 17 Cases

Aneryhremic form of acute erythremic myelosis (Di Guglielmo's syndrome) causing hepatosplenomegaly due to the infiltration of hemoglobin‐bearing blast cells: An autopsy case

Erythrophagocytosis by Pathological Erythroblasts in the Di Guglielmo Syndrome A Study of 18 Cases

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