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Query: UMLS:C0023418 (leukemia)
 93,477 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The bone marrow biopsies and laboratory data of 248 patients with untreated chronic myeloproliferative disorders have been evaluated according to the Hannover Classification. 47.6 per cent of the cases were classed as primary or basic diseases, including chronic myeloid leukaemia common type, chronic myeloid leukaemia megakaryocytic type, chronic megakaryocytic-granulocytic myelosis, polycythaemia vera and essential thrombocythaemia. In 52.4 per cent of the biopsies the advanced stages of the primary diseases like increase of fibers and loss of differentiation were noted; the increase of fibers in myeloid leukaemia megakaryocytic type and in chronic megakaryocytic-granulocytic myelosis, and loss of differentiation in chronic myeloid leukaemia common type were frequently noted. In 14.1 per cent of the cases the advanced myelofibrosis and blast cell accumulations obscured the histological features of the primary disease, therefore these cases were placed in the unclassifiable group. The cases without increase of fibers and loss of differentiation accounted for only 4 per cent of the unclassifiable category. Leucocyte and platelet count as well as haematocrit values showed considerable overlapping and scattering and were generally lower in cases which developed myelofibrosis.
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PMID:[Diagnosis of chronic myeloproliferative diseases based on bone marrow biopsy]. 143 18

Studies with G6PD and molecular probes indicate that the myeloid leukemias and the chronic myeloproliferative disorders are clonal diseases. The G6PD data indicate that chronic myelogenous leukemia, polycythemia vera and essential thrombocythemia involve stem cells pluripotent for granulocytes, erythrocytes, megakaryocytes and lymphocytes. Agnogenic myeloid metaplasia is also a clonal disease that involves multipotent hematopoietic stem cells. However, myelofibrosis, the predominant clinical manifestation, occurs secondarily and is not a component of the abnormal clonal proliferation. Acute nonlymphocytic leukemia is a clonal disease, but G6PD studies suggest that there are at least two forms of this leukemia. In one type of ANL, the involved stem cells exhibit pluripotent differentiative expression. In another type of ANL, differentiative expression is largely restricted to the granulocytic pathway. The heterogeneity of ANL has both clinical and pathogenetic implications.
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PMID:Stem cell origin of human myeloid blood cell neoplasms. 170 32

Thirty-six patients with chronic myeloproliferative disorders (CMPD) with thrombocytosis (essential thrombocythaemia 19 patients, chronic megakaryocytic granulocytic myelosis five, polycythaemia vera six, chronic myelogenous leukaemia six) were treated with interferon alfa-2b to reduce the platelet count. The pre-treatment platelet count was in the range 450-700 x 10(9)/L in eight patients, 700-1000 x 10(9)/L in eight and above 1000 x 10(9)/L in 20. In the induction phase of treatment 22 patients were treated with interferon alfa-2b 3 million units (MU) daily subcutaneously for 2 months or until the platelet count returned to normal, if earlier. Fourteen patients received 5 MU interferon alfa-2b daily in the same way. In the maintenance phase the doses were reduced to 3 MU and 5 MU thrice weekly, respectively. Complete response (CR), defined as a reduction of platelet count to below 450 x 10(9)/L, was achieved in 78% of patients (within 2 months of induction in 64%). The platelet depleting effect was dose dependent: CR in 2 months in 52% on 3 MU interferon alfa-2b versus 75% on 5 MU. Reduction of interferon dose was followed by an increase in platelet count in 39% of patients. The white cell count fell by 50% in Philadelphia-negative CMPD. Side effects were common, though generally mild, but led to withdrawal of treatment in six patients. Three patients suffered cerebrovascular events during treatment and one shortly thereafter.
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PMID:Treatment of thrombocytosis in chronic myeloproliferative disorders with interferon alfa-2b. 179 82

Restriction fragment length polymorphisms of the X-chromosome genes phosphoglycerate kinase and hypoxanthine phosphoribosyl transferase were used to study clonality in peripheral blood leukocytes from 48 women with chronic myeloproliferative disorders (c-MPD). A total of 50% of patients were heterozygous for one or both of the polymorphic loci. These included 17 cases with polycythemia vera, four patients with essential thrombocythemia (ET), and three cases with idiopathic myelofibrosis (IMF). A clear-cut monoclonal X-inactivation pattern was observed in 17 of 24 cases including all IMF patients. Only one patient with PV exhibited a nonclonal composition of her leukocytes, while six cases demonstrated a predominantly clonal pattern in peripheral blood cells. Among the latter category reckoned three of four ET patients. Cell separation analyses were performed in one ET and three PV patients. In all four cases a monoclonal pattern of the granulocyte fraction could be established, while T lymphocytes of these patients were of nonclonal origin. These data suggest that the vast majority of c-MPDs arise from multipotent hematopoietic stem cells. Moreover, this type of clonal analysis might be of help in discriminating between primary MPD and reactive processes.
Leukemia 1990 Apr
PMID:Clonal analysis of chronic myeloproliferative disorders using X-linked DNA polymorphisms. 197 5

In 98 patients with chronic myeloproliferative disorders (45 chr. myeloid leukaemia (CML), 19 myelofibrosis primaria (MP), 28 polycythaemia vera (PV) and 6 idiopathic thrombocythaemia (IT)) the incidences of increased numbers of MPO-deficient polymorphonuclear (PMN) were 60% in CML, 32% in MP, 7% in PV and 0% in IT patients. The CML figure differed significantly from the others (p less than 0.001). This study confirms the finding of low NAP scores in CML compared to normal or high NAP scores in the other groups of the myeloproliferative syndrome. The incidences of increased numbers of MPO-deficient PMN in this study are comparable to those found in the primary myelodysplastic syndromes and in acute myeloid leukaemia. The finding supports the view that some of the CML cases and may be other cases of the chronic myeloproliferative disorders may be fundamentally the same disease as in primary myelodysplastic syndromes and in acute myeloid leukaemias.
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PMID:Myeloperoxidase-deficient polymorphonuclear leucocytes (VII): Incidence in untreated myeloproliferative disorders. 300 24

The iron status of 50 patients with Ph'-positive chronic granulocytic leukaemia (CGL) was evaluated at diagnosis by means of bone marrow and blood studies. A decreased or absent iron in semiquantitative estimation on bone marrow smears was observed in 92% of patients, and 88% had a low sideroblast score. In contrast, normal Hb and serum iron concentrations were found in the majority of cases, and only two out of the 50 patients displayed a decreased serum ferritin. To ascertain whether the bone marrow pattern of iron depletion could be due to an expansion of the red cell mass, the latter parameter was measured by isotopic methods in a subgroup of 11 patients. Normal or slightly increased values were obtained in all cases. We conclude that absent or decreased marrow iron is a common feature in the chronic phase of CGL, that generally does not reflect true iron deficiency. Since such a finding is also usual in polycythaemia vera and idiopathic myelofibrosis, it should be included among the features shared by the chronic myeloproliferative disorders.
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PMID:Decreased bone marrow iron in chronic granulocytic leukaemia: a consistent finding not reflecting iron deficiency. 346 68

The level and composition of glycosaminoglycans (GAG) were studied in leukocytes of healthy donors and patients with different forms of leukemia. GAG level was found to increase in chronic myeloproliferative disorders. This parameter was far below the norm in most cases of acute leukemia. An evaluation of GAG profile established a relationship between cell GAG level variation, the type of proliferating cells and leukemic transformation. Peculiarities of GAG profile in myeloid and lymphoid cells were identified. It was shown that a study of GAG in leukocytes of leukemic patients may contribute to identification of blast elements and differential diagnosis between leukemia types.
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PMID:[Leukocyte glycosaminoglycans in various forms of myelo- and lymphoproliferative blood diseases]. 346 89

Plasma fibronectin concentrations were measured in 49 patients with chronic myeloproliferative disorders and compared to sex- and age-matched controls. A significantly lower plasma fibronectin concentration was observed in patients with idiopathic myelofibrosis as compared with the control group (p less than 0.01). In addition, plasma fibronectin concentrations in myelofibrosis patients differed significantly, when compared with patients with polycythaemia vera (p less than 0.01), whereas no significant difference was found between myelofibrosis patients and those with a transitional myeloproliferative disorder or chronic myelogenous leukaemia (p greater than 0.05). An inverse relationship was demonstrated between plasma fibronectin and spleen size, the lowest plasma fibronectin levels being found in patients with large spleens. It is supposed that low plasma fibronectin concentrations in splenomegalic patients may be due to enhanced consumption of the opsonin in the expanded splenic mononuclear-macrophage system.
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PMID:Plasma fibronectin in idiopathic myelofibrosis and related chronic myeloproliferative disorders. 347 50

Myelofibrosis is characterized by excessive deposition of interstitial and basement membrane collagens in the bone marrow. In this study, specific radioimmunoassays for the aminoterminal propeptide of type III procollagen and for the 7S collagen domain of type IV (basement membrane) collagen were used to determine how this accumulation is reflected in serum. Of the 41 patients with chronic myeloproliferative disorders studied, the highest levels of both parameters were found in idiopathic myelofibrosis and in chronic myelogenous leukaemia associated with bone marrow fibrosis. Increasing degrees of bone marrow fibrosis were accompanied by increasing serum concentrations of both markers, except for osteomyelosclerosis, where notably low values were seen. Pathologically high values of one or both parameters were also found in a few patients with polycythaemia vera or a transitional myeloproliferative disorder. The antigens related to type III procollagen and type IV collagen correlated significantly with each other and with the leucocyte count. These parameters should provide noninvasive means for following the accumulation of interstitial and basement membrane collagens in the bone marrow.
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PMID:Serum markers for type IV collagen and type III procollagen in the myelofibrosis-osteomyelosclerosis syndrome and other chronic myeloproliferative disorders. 375 65

Using a radioimmunoassay the serum concentration of the N-terminal propeptide of type III procollagen (P-III-P) was measured in 35 patients with chronic myeloproliferative disorders, including idiopathic myelofibrosis (n = 10), osteomyelosclerosis (n = 4), transitional myeloproliferative disorder (n = 5), polycythaemia vera (n = 10) and chronic myelogenous leukaemia (n = 6). The normal range in 35 healthy controls was 4.9-11.7 ng/ml. The serum concentration of P-III-P increased with increasing degrees of bone marrow reticulin fibrosis. By contrast, almost normal levels were detected in osteomyelosclerosis with an indolent clinical course, in which an excessive deposition of mature collagen fibres was found, representing mainly type I collagen. These observations indicate that the serum P-III-P level is positively correlated to the degree of bone marrow reticulin fibrosis, whereas levels are near normal in patients with osteomyelosclerosis and stable disease. Measurement of serum P-III-P may be a useful indicator of disease activity in myelofibrosing conditions.
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PMID:Serum procollagen III peptide in chronic myeloproliferative disorders. 408 33


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