Acute erythremic myelosis (DiGuglielmo) following atypical XXXX .

@entity1 @entity417 @entity882 @entity548 @entity2150 @entity1307 @entity4446 @entity3028 @entity3800 @entity1779 @entity16748
A @entity1 presented with acute erythromyelosis (DiGuglielmo) which was developed after 3 yr of @entity2150 . @entity2150 differed from the classical form, since erythroid cells and megakaryocytes were relatively preserved in the bone marrow. Treatment with @entity3800 induced the increase of hematocrit and reticulocyte as well as general improvement. The sudden appearance of @entity548 due to @entity882 was associated with aggravation of @entity417 and @entity3028 . In the bone marrow, giant multinuclear proerythroblasts with bizarre nuclear morphology and PAS positivity with coarse granules was found. Serum @entity1307 (Ep) level was high. Bone marrow cells culture in vitro revealed two types of erythroid colonies: typical and giant multinuclear cells, both @entity16748 -positive. The number of colonies was irrespective to the Ep dose. "Autonomous" Ep independent growth of these colonies was also demonstrated. The number of colonies was more than 3 times higher per number of cells seeded when compared to normals, which indicated malignant proliferation and Ep independent growth. Treatment with @entity4446 and transfusions was without effect and the @entity1 died after 15 days with signs of @entity1779 .
