Di Guglielmo's disease
This syndrome of unknown origin is characterised by enormous numbers of nucleated red cells appearing in the bone marrow and blood. The term is used to encompass a number of entities whose relationship is still uncertain. It is a variant of acute myeloblastic leukaemia developing in three basic phases: (1) erythremic myelosis, marked by characteristic blood changes with anaemia and bizarre red cell morphology; (2) erythroleukaemia, characterized by the prominence of the myeloblasts in the bone marrow, amegakaryocytic thrombocytopenia, and impairment of granulopoiesis, and (3) acute myelogenous leukaemia. with the replacement of marrow by leukaemic blast cells.
The disturbance was first described by Di Guglielmo in 1923. The eponymic designation applied by William Dameshek (1900-1969) and M. Baldini applies to the several variations of the condition, which ranges from the «pure» red cell proliferation to the «pure» myeloblastic form.
- G. Di Guglielmo:
Bollettino - Societa medico chirurgica, 1926, 1: 665-673.
- William Dameshek, in: Blood, New York, 1951, 6: 372.
- William Dameshek and M. Baldini, in: Blood, New York, 1958, 13: 192.
- E. S. Henderson:
Acute myelogenou leukemia.
In: W. J. Williams, E. Beutler, A. J. Erlev, M. A. Lichtman, editors: Hematology. 3rd edition, New York, McGraw-Hill, 1983; pp 239-253.