PubMed Health⌕ Search

SEARCH · PubMed Health

Results for “ERYTHREMIC MYELOSIS”

Explore indexed PubMed citations for clinical trials, systematic reviews and public health research. Read source abstracts and follow each citation to its original PubMed record.

Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.

At least 19 recordsLinked to original sources
PubMed1975

Ultrastructural localization of histones in chronic erythremic myelosis.

The localization of histones was studied ultrastructurally with the use of the ammoniacal silver stain in erythroid precursors from 6 patients with chronic erythremic myelosis (Di Guglielmo syndrome), 3 patients with severe untreated pernicious anemia, 2 patients with untreated autoimmune hemolytic anemia, and 4 presumedly normal individuals. Silver deposits, indicative of sites of arginine-rich histone within nuclei, were not observed in proerythroblasts from any of the individuals. Small deposits of silver were seen in the heterochromatin in very early intermediate normoblasts. Differences in the amount and distribution of the silver deposits in the various types of erythroid precursors first became recognizable in the intermediate normoblast stage. In all of the individuals, the silver deposits were localized predominantly in the heterochromatic regions of the nucleus. In 6 patients with chronic erythremic myelosis, unusually, dense deposits of silver were observed in erythroid precursors having amounts of heterochromatin comparable to that found in intermediate normoblasts of intermediate megaloblasts in other conditions. In these chronic erythremic myelosis erythroid precursors, the silver deposits were larger and, in many instances, appeared to be more aggregated than in erythroid precursors obtained from 3 patients with untreated pernicious anemia, 2 patients with autoimmune hemolytic anemia, and 4 presumedly normal persons. It is possible that the findings described in this study are involved in the pathogenesis of megaloblastoid erythropoiesis as found in chronic erythremic myelosis.

Anemia, Hemolytic, Autoimmune↗
PubMed1975

Chronic erythremic myelosis profiling a refractory anemia.

Chronic erythremic myelosis is a myeloproliferative disorder of unknown etiology that is characterized by refractory macrocytic anemia and marked megaloblastoid erythroid hyperplasia of the bone marrow. Ferrokinetic and cytologic evidence indicates that the disorder demonstrates ineffective erythropoiesis. Certain cytochemical tests may help both to substantiate the diagnosis and to provide insight into some of the biochemical abnormalities in the erythroid precursors. Therapeutic trials of androgen or pyridoxine (vitamin B6) or both may be of value in some cases but ineffective in others, and some patients may require administration of blood transfusions. Why an erythroid disorder characterized by a uniquetype of megaloblastoid erythropoiesis should be the forerunner of acute leukemia is one of the many unanswered questions in this often therapeutically frustrating disorder.

Adult↗
PubMed1975

Rapid detection of ringed sideroblasts in erythremic myelosis.

When normoblasts from patients with chronic erythremic myelosis were stained with the anthraquinone dye, alizarine red S, a purple perinuclear halo appeared. This halo corresponded in location to siderotic granules stained with a conventional iron stain. The rapidity of the alizarine red S stain makes it potentially useful for the detection of pathological sideroblasts in certain refractory anemias.

Bone Marrow↗
PubMed1975

Cytochemical abnormalities of atypical erythroblasts in acute erythremic myelosis.

Cytochemical properties of atypical erythroblasts and 'histioid' cells from the bone marrows of two patients with acute erythremic myelosis (Di Guglielmo's disease) were studied. In addition to strong PAS and strong nonspecific esterase positivity, intense specific esterase positivity was also detected in the majority of the erythroid precursors. Since specific esterase activity is considered to be a feature of granulocytic cells, its presence in erythroid precursors raises the possibility that the atypical erythroblasts of acute erythremic myelosis may share close metabolic relationships with granulocytic cells.

Acute Disease↗
PubMed1978

Arginine methyltransferase activity in chronic erythremic myelosis (Diguglielmo syndrome).

Activity of S-adenosylmethionine-dependent arginine methyltransferase was substantially higher in sonicated bone marrow samples from 6 patients with chronic erythremic myelosis than in bone marrow from 3 patients with untreated pernicious anemia, 2 patients with autoimmune hemolytic anemia, and 4 normal persons. Increased activity of this enzyme may be one of the factors contributing to the pathogenesis fo methylated arginines in histones of erythroblasts from patients with chronic erythremic myelosis.

Arginine↗
PubMed1995

Anerythremic form of acute erythremic myelosis (Di Guglielmo's syndrome) causing hepatosplenomegaly due to the infiltration of hemoglobin-bearing blast cells: an autopsy case.

An autopsy case of a 42 year old man with the anerythremic form of acute erythremic myelosis (Di Guglielmo's syndrome) is reported. The patient was admitted because of a 1 month history of fatigue and fever. Physical examination showed hepatosplenomegaly. Laboratory data showed leukopenia, mild normocytic anemia, and high levels of serum lactate dehydrogenase and vitamin B12. Bone marrow aspirate revealed an elevated number of erythroblasts, with dyserythropoiesis (E/M = 3.7). After admission, thrombocytopenia progressed rapidly, but blast cells were not seen in the peripheral blood throughout the clinical course. On the 56th hospital day, the patient died of pneumonia. At autopsy, the spleen weighed 550 g and the liver 1800 g. Histologically, the white and red pulps of the spleen and the portal region and sinusoid of the liver were diffusely infiltrated by blast cells that were positive for anti-hemoglobin (Hb) antibody on immunoperoxidase staining. The bone marrow, the lymph nodes, the adrenal glands, the pancreas, and the heart were also infiltrated by the blast cells. This was thus considered to be a rare case of the anerythremic form of acute erythremic myelosis (Di Guglielmo's syndrome), the findings showing that Hb immunoperoxidase staining is useful for the diagnosis of this condition.

Adult↗
PubMed1977

Cytochemical detection of homocysteine in pernicious anemia and in chronic erythremic myelosis.

Utilizing a unique ability of homocysteine to form a yellow-brown precipitate with nickel chloride, a cytochemical test was developed in an effort to identify this amino acid. Among a variety of types of anemias studied, bright yellow-colored erythrocytes and erythroid precursors were found only in marrows from patients who had untreated pernicious anemia and chronic erythremic myelosis. The results of the study support the "methyltetrahydrofolate-trap" hypothesis in vitamin B12 deficiency, in which decreased activity of the methylocobalamin-dependent methyltransferase enzyme is believed to lead to accumulation of methyltetrahydrofolate and homocysteine in the deficient cells. The findings also raise the possibility that similar intracellular accumulations of homocysteine may occur in chronic erythremic myelosis, perhaps as a result of a defect in the methyltransferase enzyme.

5-Methyltetrahydrofolate-Homocysteine S-Methyltran↗
PubMed2001

Acute erythremic myelosis (true erythroleukaemia): a variant of AML FAB-M6.

AIMS: Classic erythroleukaemia (acute myeloid leukaemia M6, or M6 AML) is defined as an excess of myeloblasts in an erythroid predominant background. Leukaemia variants in which the primitive blast cells are demonstrably erythroid are extremely rare and poorly characterised. Variably referred to as "true erythroleukaemia" or "acute erythremic myelosis", they are often included within the M6 AML category even though they do not meet strict criteria for this type of AML. METHODS: Two cases of acute erythroid neoplasia are presented with clinical, morphological, immunophenotypic, and cytogenetic analysis. RESULTS: Both patients presented with profound anaemia, one in a setting of long standing myelodysplasia. Bone marrow examination revealed a predominant population of highly dysplastic erythroid cells in both cases. In one case, the liver was infiltrated by neoplastic erythroid cells. Both patients died within four months of diagnosis. CONCLUSIONS: This report illustrates that cases of acute leukaemia occur in which the dominant neoplastic cell is a primitive erythroid cell without an accompanying increase in myeloblasts. This does not preclude the neoplastic clone originating in a multipotent haemopoietic stem cell, as suggested by cases arising in patients with myelodysplasia. Acute erythremic myelosis should be recognised as a distinct variant of M6 AML.

Adult↗
PubMed1975

[Acute erythremic myelosis as a terminal phase of polycythemia rubra vera].

A male, 66, developed Acute Erythremic Myelosis in the course of Polycythemia Vera. The time of onset of Polycythemia Vera could not be determined, his first symptoms being vascular complications. He received treatment with Phlebotomies and Myleran. Five years later he became ill with malaise, fever, splenomegaly; in the peripheral blood profound pancytopoenia with immature, bizzare erythro-and megaloblasts have been found. Bone marrow was full of atypical megaloblasts, some of them having two or more nuclei. The number of mitoses was increased. Chromosomal abnormalities consisted of ane-uploid cells, chromatid breakes and translocations (G to A-1). The therapy with B12, Cytosin-Arabinoside, Oncovin and Blood transfusions was unsuccessful. He died 21 days after being admitted to the Hospital.

Acute Disease↗
PubMed1975

Phosphorylase activity in chronic erythremic myelosis.

Phosphorylase activity was detected in the cytoplasm of erythroid precursors of 6 of 7 patients with chronic erythremic myelosis (Di Guglielmo syndrome), in proerythroblasts and megaloblasts from 3 patients with pernicious anemia and in 2 patients with severe folate deficiency in neoplastic lymphocytes from 2 patients with acute lymphoblastic leukemia, and in 1 patient with leukemic lymphosarcoma. In all of these patients, most of the erythroid precursors and/or neoplastic lymphocytes contained increased amounts of glycogen when stained with the PAS reagent. Phosphorylase activity was not detected in erythroid precursors obtained from 6 presumed normal individuals or from 3 of 7 patients with a variety of other types of anemia in which the erythroid precursors were PAS-negative. Similarly, phosphorylase activity was absent in lymphocytes obtained from presumed normal individuals. Although the mechanisms responsible for the pathogenesis of PAS positivity are unclear, it is possible that the increased phosphorylase activity found in cells that are PAS-positive may reflect a disorder in the biosynthetic pathway of glycogen.

Anemia, Hemolytic↗
PubMed1977

Nonspecific esterase activity in pernicious anemia and chronic erythremic myelosis: a cytochemical and electrophoretic study.

Cytochemically, nonspecific esterase activity was detected in megaloblasts from three patients with severe untreated pernicious anemia, in megaloblastoid erythroblasts from five patients with chronic erythremic myelosis (DiGuglielmo syndrome), and in normoblasts from a patient with severe untreated iron-deficiency anemia. Nonspecific esterase activity in all of these erythroblasts was inhibited by sodium fluoride. Enzymatic activity could not be detected in normoblasts from normal marrows. Electrophoretically, three bands of nonspecific esterase activity could be visualized in marrow sonicates from the anemic patients and normal persons. All of these bands were inhibited by sodium fluoride. The results demonstrate that electrophoretically and in terms of fluoride inhibition, nonspecific esterases obtained primarily from erythroid precursors in various types of anemias are similar to nonspecific esterases found in normal marrows presumably containing a more heterogeneous population of cells.

Anemia, Pernicious↗
PubMed2000

Erythremic myelosis (AML6er) in a cat.

A 4-year-old male European domestic cat was presented with dysorexia, weakness and depression. Normocytic normochromic non-regenerative anaemia, leucopaenia and thrombocytopenia were detected. Rubriblasts were detected both in the blood and in the bone marrow. Tests of blood chemistry revealed no alterations of renal and hepatic function and a positive reaction to FeLV antigen was detected in the cat's serum. Neoplastic cells did not show positive to cytochemical reactions against granulocytes, monocytes and lymphocytes. According to haematological and bone marrow cytological findings, a diagnosis of erythremic myelosis (AML6er) was made. Histopathology showed extramedullary haematopoiesis in the liver, spleen, kidney and lymph nodes and chronic nephropathy and degenerative signs in the liver.

Animals↗