This article represents a critical attempt to survey the cumulative information of the rare syndrome ordinarily called Di Guglielmo’s disease. The historical background lies predominately in Europe, more particularly in Italy, where the recording of cases has been fairly extensive and where interpretation has been pioneered.

The present study does not presume to be definitive. It attempts to trace the thinking that has gone before, and to define the terms of reference so as not to compound nosologic confusion. It presents examples of cases in the literature acceptable diagnostically by definition. Criteria for the differential diagnosis of acute and chronic erythremic myelosis and erythroleukemia are offered. These three conditions are regarded as variants of reticulo-endotheliosis. This conclusion is based on what is known of the pathogenesis, and on what has been observed clinically and hematologically.

All evaluation is necessarily offered with the reservations that must be held regarding a rare disease of unknown etiology.

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