Myelosis involving the granulocytic and erythrocytic systems.

There are several syndromes in which an erythriblastic reaction, comparable with the granulocytic reaction of granulocytic leukaemia, is prominent. One is the "leukanaemia" of von Leube (1900) defined by Foy, Kondi, and Murray (1946) as " a haematological condition in which there co-exist a pernicious anaemia-like and a leukaemia-like blood picture with corresponding marrow disorder of the erythroid and myeloid issues "; they conclude that " as a disease sui generis [it] does not exist, being either atypical pernicious anaemia or atypical myeloid (usually myeloblastic) leukaemia." Garnier, Cordier, and Sigwalt (1941) described a variant unusual in its intensity, and many other variants may be found in the French and Italian literature. Anglo-Saxon writers rarely describe such cases, for they usually regard the erythroblastosis or the granulocytic reaction, as the case may be, as symptomatic epiphenomena of little importance, and " erythroleukaemia " and " leukanaemia " are dismissed each in 15 to 20 lines by Whitby and Britton (1950). However, Di Guglielmo (1945) haF brought forward evidence which suggests that the acute and chronic erythraemic myeloses which bear his name may well prove jo be disease entities he also describes an erythrolek7aeiiii&whTlch can de difficult to differentiate from erythraemic myelosis on the one hand and leukanaemia on the other. Acute erythraemic myelosis, always fatal within two months of onset (Di Guglielmo, 1923, 1926, 1945), is characterized by anaemia, fever, splenomegaly proportionally greater than hepatomegaly, and proliferation in marrow, spleen, lymph nodes, and liver of dysplastic erythroblasts which fail to mature. The nuclei of these cells are usually megaloblastoid or reticii1endothelioid.j The chronic form (Duesberg, 194l Hanlilee and Sch6ner, 1941 ; Di Guglielmo and Quattrin, 1942) has a more chronic course, up to two years, and the peripheral blood erythroblasts are orthochromatic. It is the only form so far described in England (Israels, 1939; Neumark, 1949 ; Emery, 1951). In erythroleukaemia hepatomegaly may be more marked than splenomegaly, and erythroblastosis, though it persists till death, is more intense at the beginning of the disease, giving place to the uncontrolled proliferation of primitive granulocytes which may have grossly atypical, histiocytic nuclei. Recently, evidence has been accumulating that erythraemic myelosis and erythroleukaemia may be unusual modes of onset of acute leukaemia (Bussi and Franzini, 1951; Rachet, Mallarme, and Bourel, 1951; Mallarme and Bourel, 1951). It appears that the longer the patient survives, the more likely is he to develop the classical picture of acute granulocytic leukaemia, and now that intensive transfusion is so commonly practised patients with leukaemia-like syndromes rarely die from anaemia in the early stages of the disease, so that the change from erythraemic myelosis to leukaemia is more commonly seen. Nevertheless, patients still die with the picture of erythraemic myelosis (Emery, 1951; Schwartz and Critchlow, 1952). It seems as yet uncertain whether erythroleukaemia will have to be separated from erythraemic myelosis, or whether these " diseases" can be grouped together as unusual variants of acute granulocytic leukaemia. The case recorded here appeared at first to be erythraemic myelosis, but ultimately had to be classed as erythroleukaemia, resembling closely the case of Verloop, Deenstra, and van der Hoeven (1952).