The Di Guglielmo Syndrome: A study of 17 Cases

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Abstract
In 17 patients with Di Guglielmo's syndrome the PAS-stained erythroblasts in bone marrow smears have been assessed by a scoring system. Most of the PAS scores were either moderately high or very high--much higher than those normally seen in disorders with benign erythropoietic hyperplasia. On the other hand, very low scores were obtained in a few cases. Whereas a high PAS score always lends strong support to a diagnosis of Di Guglielmo's syndrome, a low score, or even a negative reaction, does not exclude this diagnosis. The highest scores were obtained in cases with many pathological erythroblasts. Staining after diastase digestion confirmed that the positive PAS reaction is due to glycogen. In this test, experience showed that a period of at least 2 hours is required for complete digestion of the glycogen.

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