Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0023473 (chronic myeloid leukemia)
 18,916 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

An attempt has been made in an experimental group of 20 patients, with leukemia or one of the myeloproliferative disorders, to devise a safe method of splenectomy by timing the operation to coincide with optimal response to chemotherapy, preoperative bowel preparation with antibiotics to minimize infections, and availability of platelet concentrates, matched for HL-A antigens when possible, to control massive hemorrhage. The results in this experimental group were compared with a historical control group of 26 patients. Two-thirds of all the patients had chronic myelocytic leukemia, and the mean weight of the spleens removed was just over 1,800 grams. Operative mortality was 35% in the historical group and zero in the more recent group, six of whom were operated on while in blastic crisis. Splenectomy can be done safely in leukemic patients, and is not contraindicated. Future planned studies will determine its possible therapeutic role in reducing tumor burden.
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PMID:The problem of splenectomy in a leukemic patient population. 106 81

Chronic myelocytic leukemia and the myeloproliferative diseases may present as asymptomatic elevations of peripheral blood counts or with relatively nonspecific symptoms. Full diagnostic evaluation is necessary to eliminate the many other causes of blood count elevation. Coincidence of a myeloproliferative disease and pregnancy is unusual. The pregnancy has no adverse effect on the course of the mother's hematologic disease. However, the myeloproliferative diseases, especially if uncontrolled, result in increased fetal prematurity and mortality. Treatment of the pregnant patient should be conservative, and cytotoxic therapy should be avoided until at least after the first trimester whenever possible. Busulfan has been the drug most widely used during pregnancy, and it appears not to cause fetal malformations when used alone.
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PMID:Chronic myelocytic leukemia and the myeloproliferative diseases during the child-bearing years. 106 96

A case report of serial chromosome studies on a child presumed to have acute lymphoblastic leukemia (ALL) is presented. Hematologic remission was achieved after 3 weeks and maintained until death 63 weeks later. The classic Philadelphia chromosome translocation was found, both at diagnosis and throughout the course of the disease, in a proportion of cells from PHA-stimulated blood cultures. The finding is related to other reports of Philadelphia-positive clones in ALL, as well as those in chronic myeloid leukemia and its acute transformation, and other myeloproliferative disorders. The origin of the Philadelphia chromosome in this case is considered in the light of current stem cell theory, and its relevance to lymphocytic neoplasia is discussed. We believe that the finding of a Ph1-positive clone in a cell line morphologically indistinguishable from normal lymphocytes in a case of acute leukemia is unique.
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PMID:Philadelphia chromosome in acute leukemia: case report. 106 42

Patients with myeloproliferative disorders were prospectively studied by in vitro agar-gel marrow culture technics to evaluate factors involved in the evolution of abnormal granulopoiesis. Marrow granulocytic colony-forming capacity was determined in 78 patients with chronic myeloid leukemia, subacute myeloid leukemia, preleukemia, Di Guglielmo's syndrome, polycythemia vera or essential thrombocythemia. A wide range of marrow colony-forming capacity values was noted early in disease courses; however, in 26 of 33 patients decreased colony-forming capacity was associated with disease transformation into acute myeloid leukemia or other clinically aggressive stages. An increased proportion of abnormally light buoyant density (less than 1.062 g/cm3) colony-forming cells was present in the marrow and peripheral blood of 15 of 16 patients with chronic myeloid leukemia, subacute myeloid leukemia, preleukemia or essential thrombocythemia; in seven of eight patients with greater than 35 per cent abnormally light colony-forming cells their disease subsequently underwent transformation. Elevated levels of urinary colony-stimulating factor output were noted in 17 of 31 patients, and in 10 of 12 patients whose disease subsequently underwent acute transformation within 10 months of study. In six of seven patients who simultaneously had an increased urinary output of colony-stimulating factor and low colony-forming capacity in marrow, transformation occurred within 10 months. These findings indicate that progressive abnormalities of both marrow clonal growth patterns and levels of possible humoral regulatory substances develop during evolution of these diseases. In contrast, patients with idiopathic sideroblastic ineffective erythropoiesis had normal values for marrow colony-forming capacity, proportion of light density colony-forming cells and urinary colony-stimulating factor output, and in none has their disease transformed into acute myeloid leukemia. These in vitro studies appear useful for clinical staging, evaluating prognosis and categorizing patients with myeloproliferative disorders.
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PMID:The myeloproliferative disorders. Correlation between clinical evolution and alterations of granulopoiesis. 108 34

Abnormalities in platelet dense granules, small intracellular organelles containing ATP, ADP, calcium, serotonin, and pyrophosphate, have frequently been reported in patients with leukemia and myeloproliferative disorders, particularly acute and chronic myelogenous leukemia. Recent studies of a family which includes several members with an autosomal dominant dense granule deficiency condition show an association between the presence of this form of dense granule deficiency and the development of acute myelogenous leukemia. Studies in two additional patients, one with the Monosomy 7 syndrome and the second with a myelodysplastic syndrome, revealed a defect in platelet dense granules. This defect appears to be due to an abnormality in the formation of these granules rather than the presence of empty vesicular structures or decreased contents due to activation associated secretion. The results suggest that the defect in platelet dense granules associated with leukemia or myelodysplastic syndromes may result from a chromosome alteration in the megakaryocyte cell line leading to decreased formation of dense granules. Studies in the family with an inherited bleeding disorder suggest that a gene coding for a protein important for the formation of dense granules is located adjacent to a gene which, when abnormal, may predispose to the development of leukemia.
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PMID:Platelet storage pool deficiency, leukemia, and myelodysplastic syndromes. 129 Sep 57

As compared to values recorded in 10 healthy normal-weight normolipidemic control subjects, serum cholesterol and apoprotein B levels as well as serum cholinesterase activity were found to be obviously decreased in the 28 patients with acute leukemia, the lowest levels being associated with the worst prognosis. The values of the above-mentioned biochemical variables in the 21 patients with chronic disorders (13 with chronic myeloproliferative disease and 8 with chronic lymphocytic leukemia) were not as low as in patients with acute leukemia. It should however be mentioned that in patients with chronic myelogenous leukemia, the lowest levels of serum cholesterol were correlated with a large tumor burden as assessed by a score taking into account for clinical and hematologic parameters. It is concluded that hypocholesterolemia could be regarded as a factor of adverse prognosis in hematologic malignancies, being probably the result of both enhanced catabolism of low density lipoproteins and impaired hepatic lipoprotein synthesis.
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PMID:Serum cholesterol and apoprotein B levels and serum cholinesterase activity in selected hematologic malignancies. 129 17

Irradiated mice reconstituted with bone marrow cells infected with a retrovirus carrying the bcr-abl oncogene of human chronic myeloid leukemia are subject to a range of neoplastic hematopoietic diseases, both myeloid and lymphoid. Comparison of DBA/2 and C57BL/6 mice has revealed a marked strain difference in susceptibility to the various tumor types. The present study, performed with BALB/c mice, indicates that the kinetics and nature of the induced disease can be modulated by the infection procedure, as well as the genetic background, and that retroviral regulatory sequences may influence the outcome. A distinctive clonal myeloproliferative disorder, somewhat akin to chronic myeloid leukemia but with prominent erythroid and mast cell components, as well as granulocytic excess, was characterized.
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PMID:Hematologic disease induced in BALB/c mice by a bcr-abl retrovirus is influenced by the infection conditions. 131 70

We describe here a patient with accelerated phase Philadelphia chromosome (Ph1) negative chronic myelogenous leukemia without BCR gene rearrangement, who received an allogeneic bone marrow transplant following a conditioning regimen consisting of busulfan (BU) and cyclophosphamide (CY). Hematopoiesis was restored following splenectomy performed 1 month post-transplant. There were no distinguishing cytogenetic differences between donor and host. Five years post-transplant the patient relapsed with the original disease. Restriction fragment length polymorphism (RFLP) studies performed at that time exhibited host specific DNA markers suggesting recurrent leukemia of host origin. RFLP analysis of the cells cryopreserved immediately post-transplant also revealed all cells to be of host origin. This patient experienced 5 years of remission with autologous hematopoietic recovery from an aggressive myeloproliferative disorder after high dose BU and CY without engraftment of donor hematopoietic cells.
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PMID:Prolonged remission of accelerated phase Philadelphia chromosome negative chronic myeloid leukemia following autologous recovery of normal hematopoietic elements after busulfan/cyclophosphamide and allogeneic marrow transplantation. 134 49

To study megakaryocyte activation, the argyrophilic staining method of nucleolar organizer regions (AgNOR) has been applied to decalcified bone marrow biopsies of 16 individuals with no haematopoietic disorders and 59 patients with chronic myeloproliferative disease. Of the 59 patients, 18 had chronic myeloid leukaemia (CML), 21 chronic megakaryocytic granulocytic myelosis (CMGM), 13 polycythaemia vera (PV) and 7 essential thrombocythaemia (ET). The AgNOR number of megakaryocytes in CML was significantly lower, and in CMGM, PV and ET significantly higher than in healthy individuals. The high number and the clusters of fine-grained AgNORs of megakaryocytes in CMGM, PV and ET are suggestive of active, proliferating cells. The AgNOR number of megakaryocytes and the platelet counts of the patients did not show a convincing correlation. In CMGM, PV and ET the pyknotic, heterochromatinized megakaryocytes with narrow rims of cytoplasm called bare (nude) nuclei, possessed few, large AgNOR granules. The AgNOR staining of bare nuclei and the roughly identical number of granules found in CMGM, PV and ET indicate a common, active mechanism of apoptosis.
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PMID:Nucleolar organizer regions of megakaryocytes in chronic myeloproliferative disorders. 137 98

Symptoms of autoimmune disease were evaluated in 125 patients with chronic myelogenous leukemia (CML) and in 12 patients with essential thrombocythemia undergoing treatment with recombinant interferon (IFN)-alpha-2b plus/minus low-dose recombinant IFN-gamma. Twenty-seven of 137 patients (20%) developed rheumatoid symptoms. Furthermore, the incidence of antinuclear antibody (ANA) formation was studied. Elevated ANA titers were found in 5/19 (26%) of CML patients at the time of diagnosis and in 3/18 (17%) of patients treated with hydroxyurea or busulfan. During IFN treatment, 18 of 25 tested patients (72%) had elevated ANA titers. In 15 of these ANA-positive patients, clinical signs of autoimmune disease appeared. All these patients were under long-term IFN treatment and were in remission of disease. In three patients criteria for systemic lupus erythematosus were fulfilled. Severity of side effects had led to the discontinuation of IFN treatment in these patients. The data indicate that IFN-alpha and IFN-gamma can induce ANA associated with autoimmune disease in patients with myeloproliferative disorders.
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PMID:Lupus-like autoimmune disease induced by interferon therapy for myeloproliferative disorders. 138 10


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