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PubMed1984

Cytochemical detection of erythroblastic enzymes in acquired dyserythropoiesis.

We have studied the amount and intracellular distribution of erythroblastic alkaline and acid phosphatase, nonspecific esterase, and N-acetyl-beta-glucosaminidase in 50 patients with acquired dyserythropoiesis. 19 morphologically normal bone marrow smears served as controls. Alkaline phosphatase was found in all controls (mean 9.4% of erythroblasts). The percentage of positive erythroblasts in pathological conditions varied greatly from absence to over 70%. Acid phosphatase and N-acetyl-beta-glucosaminidase were positive in pernicious anaemia and in acute erythremic myelosis. Nonspecific esterase was only detected in a case of erythremic myelosis. This case also showed a faint metachromasia with the dye azure A.

Acetylglucosaminidase↗
PubMed1983

An immunohistochemical study of hemoglobin A, hemoglobin F, muramidase, and transferrin in erythroid hyperplasia and neoplasia.

The bone marrow biopsy specimens of 35 patients with benign and malignant erythroid hyperplasias were examined for the presence of hemoglobin A, hemoglobin F, muramidase (lysozyme), and transferrin, using an indirect immunoperoxidase method (PAP) on Zenker's-fixed paraffin-embedded bone marrow biopsy specimens and particles. Five cases of each of the following entities were studied: erythroleukemia and erythremic myelosis, acute granulocytic leukemia with maturation (FAB M2), polycythemia rubra vera, myeloproliferative syndrome in childhood, megaloblastic anemia (B12 and folate deficiency), erythroid hyperplasia (regenerating bone marrow and hemolytic anemia), and Ph' chromosome positive chronic granulocytic leukemia. Hemoglobin A was present in both the early and late erythroid precursors in all conditions. Hemoglobin F was the predominant hemoglobin in early erythroblasts of pernicious anemia and in both early and late erythroid elements in erythroleukemia and erythremic myelosis. Small quantities of hemoglobin F were present in a few isolated clusters in other conditions. Staining for hemoglobin F may be useful in identifying immature erythroid precursors and in distinguishing some cases of dysplastic erythroid hyperplasia from neoplasia. Additionally, these findings suggest that the maturational switch in hemoglobin synthesis operates with distinct pathways under different conditions.

Bone Marrow↗
PubMed1976

Detection of methionine in pernicious anemia megaloblasts and other types of erythroid precursors.

Utilizing a bacterial-agar overlay technic incorporating the methionine-requiring bacterium Leukonostoc mesenteroides, little or no bacterial growth was seen surrounding the megaloblasts and proerythroblasts of eight patients who had severe untreated pernicious anemia. Similarly, scant bacterial growth was observed in five cases of chronic erythremic myelosis (DiGuglielmo syndrome). Heavy bacterial growth, indicating ample amounts of methionine, was seen in two cases of autoimmune hemolytic anemia, and in two cases of severe untreated folate-deficiency anemia. The results are consistent with the "methyltetrahydrofolate trap" hypothesis in pernicious anemia, in which a defect in the methylcobalamin-dependent methyltransferase leads to reduced amounts of methionine. These studies also suggest that a similar methyltransferase defect does not occur in folate deficiency or autoimmune hemolytic anemia. The generation of methionine, as estimated by the present technic, may also be defective in chronic erythremic myelosis.

5-Methyltetrahydrofolate-Homocysteine S-Methyltran↗
PubMed1988

Erythremic [corrected] myelosis in chronic lymphocytic leukemia.

Two patients who had both B-cell chronic lymphocytic leukemia (CLL) and dyserythropoiesis are described. One patient (Case 2) had both CLL and dyserythropoiesis. Erythrodysplasia developed in the other patient (Case 1) after treatment for CLL with alkylating agents and 2'-deoxycoformycin. The management of these patients and the possible mechanisms responsible for the development of dyserythropoiesis in CLL are discussed.

Blood Cell Count↗
PubMed1982

Erythroleukemia: a study of 15 cases and literature review.

Out of a total of 185 cases of acute leukemia at our institution from 1967 to 1978, fifteen cases (8.1%) were identified as erythroleukemia or erythremic myelosis. The symptoms at presentation were often related to anemia (10/15 cases); the presenting hemoglobin value was lower than 10.0 gm/100 ml. Nucleated red cells were present in the peripheral blood and reticulocyte response was inappropriate to the degree of anemia. Marrow biopsy showed erythroid hyperplasia with megaloblastic and dyserythropoietic features, increase in myeloblasts greater than 5% (10/15 cases), positive PAS staining of erythroid precursors (12/12 cases), and erythrophagocytosis by abnormal erythroid precursors (6/15 cases). Abnormalities were noted in monocytic and megakaryocytic cell lines, and it was concluded that erythroleukemia is probably a stem cell disorder. Response to chemotherapy was poor with median survival of four months from initial diagnosis. Intracranial hemorrhage and bacterial or fungal infection were the most frequent cause of death.

Adult↗