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)

We investigated 20 patients with hematologic disorders who had a clone of cells with a deletion of most of a chromosome #20 long arm (20q-) in the bone marrow. Three patients had polycythemia vera (PV), 6 had acute nonlymphocytic leukemia (ANLL), 8 had preleukemia (PL), and 3 had other myeloproliferative disorders. In our laboratory, a 20q- chromosome is found in 6% of patients with PV, 3% of patients with ANLL, and 1% of patients with PL. Among the 6 patients with ANLL and a 20q- abnormality, 3 had erythroleukemia. There were no apparent clinical differences among our patients with 20q- chromosomes compared with other patients with similar disorders who did not have a 20q- chromosome. The breakpoint of the 20q- anomaly, in each instance, was in band 20q11, but it occurred near the centromere at 20q1101 in 16 patients and at the distal part of this band at 20q1109 in 4 patients. Three of the 4 patients with a breakpoint at 20q1109 had PL.
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PMID:Hematologic manifestations associated with deletions of the long arm of chromosome 20. 671 60

Management of hematologic disorders in older patients must often be weighed in a setting of decreased physiological reserves and concurrent illnesses. Anemia in the elderly should never be attributed to old age. Even a mild anemia in collusion with multiple physical and mental problems may tip the balance for those previously able to cope with their disabilities. Iron deficiency anemia and the anemia of chronic disease are the most common types of anemia in the elderly. Nutritional anemias due to folate or vitamin B(12) deficiency are treatable and should not be overlooked. Newer chemotherapy regimens for acute nonlymphocytic leukemia have been effective in many older patients. Decisions to treat are sometimes difficult, often depending on the aggregate of coexistent physical and mental disorders. The most prevalent type of leukemia in the elderly is chronic lymphocytic leukemia. A benign asymptomatic course requires no therapy, but aggressive disease requires treatment. Multiple myeloma should be suspected in an elderly person who has both unexplained anemia and bone pain. After definitive diagnosis, phlebotomy therapy should be considered for both polycythemia vera and secondary erythrocytosis to reduce blood viscosity and increase cerebral blood flow.
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PMID:Hematologic disorders in the elderly. 680 66

Chromosomal deletions occurring in myeloid malignancies have sometimes been reported either with no breakpoints or as terminal deletions. It is of importance to deduce whether these deletions are actually terminal or interstitial because this has implications for their biologic consequences and the mechanism of their development. Chromosomal deletions have been observed in 38 patients with myeloid malignancies. Two or more deletions occurred in six cases, and in seven cases this was part of a complex abnormality. In all, 45 deletions were observed. In all cases analyzed, the deletions consistently were interstitial. Of the 38 cases, 16 were myelodysplastic syndromes (MDS) [refractory anemia (RA), three; RA with ringed sideroblasts (RARS) three; RA with excess of blasts (RAEB) eight; RAEB in transformation (RAEB-t) one; and unclassified, one], 11 cases were acute nonlymphocytic leukemia (ANLL), and 11 were other myeloproliferative disorders [polycythemia rubra vera (PRV) seven; essential thrombocytopenia (ET), three; unclassified, one]. In general, no uniformity of breakpoints could be identified other than del(9)(q13q22.2) most of which occurred with t(8;21) and del(20)(q11.2q13.3 or 13.1). The breakpoints corresponded to or were adjacent to fragile sites in 49% (proximal 64%, distal 33%). These data emphasize that chromosomal deletions in myeloid malignancies are interstitial. The uniformity of breakpoints in del 9q and del 20q supports the concept that in some instances the exact breakpoints may be important through juxtaposition of genes rather than loss of critical regions. The data also suggest that there may be different mechanisms for the development of proximal and distal breakpoints.
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PMID:Consistent interstitial chromosomal deletions in myeloid malignancies and their correlation with fragile sites. 843 16

HHT, one of the alkaloids from a Chinese natural plant, Cephalotaxus, has shown its potential in leukemia treatment. This compound demonstrated strong growth-inhibiting activities in vitro and in animal experiments, and obtained encouraging results in some clonal proliferative disease such as in chronic myeloid leukemia (CML) and in polycythemia vera. Evidences also confirmed HHT as an apoptosis inducer in tumor cell lines and fresh cells from cancer patients. The CR rate reported with HHT-based regimen in acute nonlymphocytic leukemia showed no statistic differences from that with DNR-based regimen, although the case number was limited. While used in clinical trial, the drug often cause noticeably cardiovascular disturbances if be given rapidly by intravenous infusion. Myelosuppression is the common complication in HHT-based chemotherapy. Although with the anti-growth activity in vitro and praisable achievement in acute and chronic myeloid leukemia treatment, the drug shows no beneficial effect in lymphocytic leukemia and solid tumors. The underlying mechanism for the discrepancy of efficacy keeps unknown. This review will present with the preclinical research data including the action mechanism, pharmacokinetics and drug resistance of HHT as well as the result from the clinical trial with HHT in China and the United States.
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PMID:Homoharringtonine: a new treatment option for myeloid leukemia. 1562 33

The aim of this study was to analyse the clinical characteristics and laboratory data, treatment and prognosis of polycythemia vera (PV). A retrospective study was performed for 71 PV patients treated in our hospital during January 2001 to July 2011 including analysis of clinical characteristics, laboratory data, myelogram chromosome karyotypes, BCR/ABL and JAK2V617F genes, as well as lactate dehydrogenase (LDH) and neuron-specific enolase (NSE) levels in serum and so on. The results showed that 71 patients (37 males and 34 females with a average age of 57.8 years) were diagnosed. Thrombosis and embolism occurred in 34 patients (47.89%), hemorrhage in 10 patients (14.08%), splenomegaly occurred in 44 patients. The onset of the disease was insidious, 13 patients (18.31%) were found to have PV during the treatments for other diseases. The average hemoglobin at diagnosis was 206.31 (171 - 242) g/L. JAK2V617F mutation was detected in 31 (81.58%) of 38 patients studied. The average levels of serum LDH and NSE were higher than normal and both positively correlated with hemoglobin (P = 0.007, P = 0.005). The disease outcomes were myelofibrosis for 3 patients, death from cerebral hemorrhage for 1 patient, and death from ineffective chemotherapy in 1 patient with ANLL-M2. It is concluded that PV is a chronic myeloproliferative disorder characterized predominantly by thrombosis and hemorrhage. The serum LDH and NSE levels are higher than the normal values. It is inferred that the serum LDH and NSE levels can reflect the degree of malignant proliferation of bone marrow hematopoietic cells and also can be used as an indicator to judge the therapeutic effect of PV.
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PMID:Analysis on 71 patients with polycythemia vera. 2273 79


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