Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0032463 (polycythemia vera)
 3,374 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Cytologic and cytogenetic results obtained from patients fulfilling the FAB criteria for the diagnosis of acute nonlymphocytic leukemia (ANLL) of megakaryocytic lineage (ANLL-M7) are reported. Eleven cases were de novo ANLL-M7, of whom three presented with acute myelofibrosis. Four cases were megakaryoblastic transformations of chronic myelogenous leukemia (two cases), refractory anemia with excess of blasts (one case), and polycythemia vera (one case). Four patients showed a minority of granular blasts, with occasional Auer rods in one. Positive myeloperoxidase and/or sudan black-B stainings and CD13 positivity in these cases were consistent with the presence of a myeloid involvement. Morphologic evidence of associated myelodysplastic features was detected in all evaluable patients with de novo ANLL-M7. These cytologic findings indicate that ANLL-M7 may frequently represent a multilineage proliferation. Cytogenetic studies revealed -7/7q- and +8, alone or in combination with additional aberrations, in three cases each. Rearrangements involving bands 3q21 or 3q26 were seen in two patients and +21, as an additional aberration, in one. Other structural rearrangements all observed in a single patient were inv(16)(p13q22) at megakaryoblastic relapse with bone marrow eosinophilia, t(13;20)(q13 or 14;q11), del(20)(q11), and der(7)t(7;17)(p14;q22). Most breakpoints of these aberrations are located at bands frequently rearranged in malignant myeloid stem cell disorders. A review of 31 cases of the literature showed a frequent occurrence of -7/7q- and -5/5q- in ANLL-M7. Many of the chromosome aberrations so far described in ANLL-M7 appear to be shared by a spectrum of myeloid neoplasias and may be related to mechanisms conferring proliferative advantage to undifferentiated stem cells.
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PMID:Multipotent stem cell involvement in megakaryoblastic leukemia: cytologic and cytogenetic evidence in 15 patients. 279 Feb 2

The aim of the present study was to better understand the possibility of utilizing growth factors of the myelomonocytic line in acute leukemias. The study is an examination of morphological changes and marker behavior in peripheral and bone marrow cells in AML and APL during treatment both with all-transretinoic acid (ATRA) alone and in association with chemotherapy and G-CSF. The same treatment was carried out in a patient who had been diagnosed with Vaquez's disease 15 years earlier and currently presented a bone marrow and peripheral picture of AML (80% myeloblasts) with thrombocytopenia. We observed that treatment with ATRA, alone or in association with chemotherapy, was followed by a remission of AML and especially of APL, with amelioration of the general condition of the patients. The addition of G-CSF to ATRA at the end of chemotherapy, during consequent pancytopenia, produced a rapid increase in mature peripheral granulocytes and an apparent medullary complete remission, which was more prolonged in APL than in AML; there was no increase in peripheral blasts. Discontinuation of G-CSF was followed by a relapse in the patient with AML. A patient with Vaquez's disease, in remission for 15 years and presenting a progressive increase in bone marrow and peripheral myeloblasts, did not have a positive response to the administration of ATRA; however, the association of G-CSF to ATRA was followed by a complete remission. The morphological changes observed in bone marrow and peripheral granulocytes (with changes in the main cellular markers: CD11b, CD13, CD14, CD15, CD34) seemed to express progressive modification of the single elements towards differentiation, with progressive bone marrow reduction and peripheral disappearance of blasts. The data agree with the changes observed in in vitro blasts cultured in the presence of ATRA and G-CSF.
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PMID:Peripheral blood and bone marrow changes after treatment with ATRA and G-CSF in AML, APL and blast crisis following Vaquez's disease. 876 33

Acute erythroid leukemia in children is very rare. Here is a case of erythroleukemia in a child of age 1.5 years, which was diagnosed on peripheral smear, bone marrow examination, cytochemistry but was confirmed on immunophenotyping. CD45 versus side scatter demonstrated blast population (29%) expressing CD45 of variable intensity (dim to negative). The myeloid nature of blast population showed bright expression of cytoplasmic myeloperoxidase (MPO), heterogenous positivity of CD117 and dim expression of CD13, CD33. These blasts also showed bright positivity for CD71 which showed erythroid nature of blasts. Flow cytometry can be comprehensive enough to completely subtype cases of leukemias/myelodysplastic syndromes, polycythemia rubra vera, non-neoplastic conditions like reactive erythroid hyperplasia following immunosuppressive therapy or viral infections or nutritional deficiencies, unlyzed RBCs or thrombocytosis which may mimic acute erythroid leukemia on flow cytometry.
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PMID:Childhood acute erythroleukemia diagnosis by flow cytometry. 2139 10