Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0023418 (leukemia)
 93,477 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Alkylating agents and 32P have been widely employed in the treatment of patients with essential thrombocythemia (ET). During a four-month period, we observed 3 cases of ET that had transformed into leukemia. Two patients had been treated with uracil mustard: One developed acute myelogenous leukemia 79 months after institution of therapy, and the other patient developed chronic myelomonocytic leukemia 24 months after the start of therapy. The third patient had been treated with busulfan, and ET evolved into myelofibrosis and eventually into acute undifferentiated leukemia with myelofibrosis. The patient who developed acute myelogenous leukemia was asymptomatic at the time of diagnosis of ET but was treated because his platelet count was greater than 1,000,000/mm3. He died 1 month after leukemic transformation, during induction chemotherapy. The other 2 patients presented with symptoms referable to their thrombocythemia. Review of the English literature revealed 12 other definite or probable cases of ET with leukemic transformation, all but 1 having been treated with alkylating agents and/or 32P. We propose that the natural history of ET may be similar to that of polycythemia vera, with evolution into leukemia being an unusual occurrence except in the setting of previous chemotherapy. Therefore, the current practice of treating asymptomatic patients with ET may not be justified, since administration of alkylating agents or 32P may increase the risk of subsequent development of leukemia.
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PMID:Essential thrombocythemia and leukemic transformation. 346 86

Two patients who developed acute lymphoblastic leukaemia following polycythaemia rubra vera (PRV) are described. In both cases the diagnosis was made using cytochemistry and immunological markers. One patient's cells marked as T-cell acute lymphoblastic leukaemia (T-ALL), the other as unclassified (null) ALL. These cases support the concept that PRV is a stem cell disorder.
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PMID:Polycythaemia rubra vera transforming to acute lymphoblastic leukaemia. 347 36

Several platelet function abnormalities have been described in the myeloproliferative syndromes. We have measured the intraplatelet vWF:Ag and fibrinogen (FI) in the platelet lysates by Laurell technique in 11 patients with polycythemia vera (PV), 10 with essential thrombocythemia (ET), 14 with chronic myelocytic leukaemia (CML) and 3 with myelofibrosis (MF) and these results were correlated with platelet function abnormalities. Decreased intraplatelet levels of vWF:Ag and FI were found in all the patients with ET and MF, in 8 out of 11 PV and 3 out of 14 CML. A statistical significant correlation was observed between the intraplatelet levels of vWF:Ag and FI in the control group and in CML and PV, but no correlation was found in ET and MF. No correlation was observed between the plasmatic and the intraplatelet levels of vWF:Ag and FI in any group. Evidences of platelet activation (spontaneous platelet aggregation or circulating platelet aggregates) were observed in 40% of the cases with ET and PV, and all these cases had low intraplatelet levels of both antigens. None of the cases with MF had evidences of platelet activation and 2 out of 14 patients with CML had platelet activation. The deficiency of the dense bodies was less frequent than the depletion of the alpha granules (5 out of 11 PV, 4 out of 10 ET, 6 out of 14 CML and 2 out of 3 MF). The low intraplatelet contents of vWF: Ag and FI, more frequently observed in ET and PV, may be the result of platelet activation and in vivo release, but megakaryocyte dysfunction is more likely in myelofibrosis.
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PMID:Intraplatelet levels of vWF:Ag and fibrinogen in myeloproliferative disorders. 350 18

Clinical and cytogenetic details of 12 patients with polycythemia vera and complete or partial trisomy of the long arm of chromosome 1 are reported. All patients had trisomy for at least the segments 1q22 to 1qter. The 1q or material from 1q was translocated to another chromosome in eight patients. This was chromosome 9 in four patients, and those cases all had trisomy also for 9p. The trisomy 1q was found at the time of diagnosis in three patients, later during the polycythemic phase in five, and in four patients when they were first examined during a late stage of the disease. Acute leukemia or a myelodysplastic syndrome developed in eight of the 12 patients. Signs of advanced disease, eg, myeloid metaplasia or myelofibrosis, preceded the leukemia in four cases and was noted in one more patient. Trisomy 1q was the most frequent structural chromosome abnormality in patients with polycythemia vera. It is thus one of several nonrandom abnormalities that can appear at any stage of the disease. It seems to occur with higher frequency in patients with myelofibrosis and/or leukemia, but it is not a specific characteristic of these complications.
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PMID:Trisomy 1q in polycythemia vera and its relation to disease transition. 370 91

A large cohort of petroleum refinery workers with long duration of employment, long latency, and relatively young age at hire had its vital status updated through Dec. 31, 1980. The standardized mortality ratio (SMR) for all causes was 78. Each nonneoplastic cause had an SMR below 100, including SMRs of 63 for emphysema and for all diseases of the genitourinary system and of 73 for chronic nephritis. The SMR for all cancers was 87. SMRs for specific neoplasms included digestive system, 90; lung, 85; kidney, 68; brain, 89; leukemia, 101; multiple myeloma, 123; unspecified lymphoma, 112; polycythemia vera (four deaths), 455; myelofibrosis (three deaths), 201; and benign and unspecified brain neoplasms, 108. There were nine deaths from mesothelioma; all nine employees had more than 20 years of employment, with an SMR of 241.
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PMID:Update of a mortality study of workers in petroleum refineries. 373 21

One hundred four patients with a diagnosis of polycythemia vera and a variable period of follow-up had one or more cytogenetic investigations. Chromosome abnormalities were found in 13% of untreated patients, in 56% of cases treated with radioactive phosphorus (32P) or cytotoxic drugs, and in 85% of patients in which transformation of the disease had occurred. Nonrandom chromosome abnormalities found before treatment included +8, +9, 13q-, 20q-; their prognostic value is little, as they are often associated with longstanding, stable disease. In contrast, 5q- anomaly and the appearance of subclones in patients with an abnormal karyotype were found to be poor prognostic signs, as they are usually coincidental with evolution of the disease to myelofibrosis or leukemia. Chromosomally two patterns of acute leukemia were observed in polycythemia vera patients. The first type resembles de novo acute leukemia, in that the clinical and cytologic characteristics of the disorder are easily defined by FAB criteria and the chromosome changes compatible with the types usually found in those conditions. In the second type, assignment to a FAB morphologic subgroup was more difficult, myelodysplastic changes were often present, and the karyotype showed complex abnormalities frequently involving chromosomes #5 and #7. All these features suggest the occurrence of secondary leukemia.
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PMID:A chromosomal profile of polycythemia vera. 382 70

Based on the previous finding that erythrocytes from patients with chronic myelogenous leukemia stain with the fluorescent dye merocyanine 540, erythrocytes from patients with other myeloproliferative disorders were examined for their ability to bind the membrane probe. As assessed by both fluorescence staining and a quantitative dye removal assay, all samples of erythrocytes from patients with chronic myelogenous leukemia, polycythemia vera, myelofibrosis with myeloid metaplasia and essential thrombocythemia bound more dye than did erythrocytes from normal, healthy individuals. Erythrocytes from three of six patients with acute myelogenous leukemia also showed increased affinity for the dye. In contrast, erythrocytes from three patients with acute lymphocytic leukemia and one with unclassifiable leukemia bound only normal amounts of dye. The procedures described may be useful as a supplemental aid to diagnosis of myeloproliferative disorders or for investigation of hematological diseases where multilineage involvement is suspected.
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PMID:A fluorescent dye which recognizes mature peripheral erythrocytes of myeloproliferative disorders. 382 30

The authors describe 4 families whose members showed myeloproliferative diseases. In one of the families, polycythemia vera (PV) was seen in twin brother and sister, in the other one, chronic myeloleukemia (CML) afflicted both daughter and mother, and in the two remaining families PV and CML afflicted two brothers and mother and daughter, respectively. It was established that neutrophil phosphatase activity was lowered not only in the afflicted brother but also in healthy members of the third family. Based on the reported and their own data the authors arrive at the conclusion that familial myeloproliferative diseases occur in rare cases. In all the cases of familial myeloproliferative diseases, the transmission of the illness by heredity was discovered to be impossible. It was also ascertained that transmitted by heredity are only those cell deficiencies of the tissues that later on will be afflicted by leukemia or will develop immunodeficiency manifested by increased mutation of the myelopoietic cells (DNA repair deficiencies) or by inability to eliminate the leukemic cells.
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PMID:[Familial myeloproliferative syndrome (study of 4 families and review of the literature)]. 386 57

15 patients with myeloproliferative diseases and thrombocythaemia (7 Polycythaemia vera, 5 essential thrombocythaemia, 3 chronic myelogenous leukaemia) were assigned to a therapy with recombinant interferon (recombinant IFN-alpha-2C) in a prospective, controlled trial. Under therapy all patients showed a significant decrease in thrombocyte values. 51% of the patients revealed thrombocyte values within the normal range after 3 months of IFN therapy. Noted side effects of IFN therapy were dose-dependent and clinically well tolerated by the patients.
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PMID:[Therapy with interferon (recombinant IFN-alpha-2C) in myeloproliferative diseases with severe thrombocytoses]. 391 73

Thirty-six patients with polycythemia vera were treated with hydroxyurea for 12 to 67 months. Nineteen patients were previously treated with other drugs. In the vast majority of patients, an average dose of 1 g/day was sufficient to control hematocrit value and platelet count. Half of the patients experienced relief of pruritus, and two thirds experienced regression of splenomegaly. None of the patients had either thrombotic complications or leukemia. Four patients suffered from mild side effects, which included fever, hyperbilirubinemia, and stomatitis, and were relieved of their symptoms when treatment was stopped. However, two patients experienced renal failure, a possible major complication not described previously.
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PMID:Treatment of polycythemia vera with hydroxyurea. 394 10


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