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)

One thousand and five bone marrow biopsies were performed in patients with haematologic or oncologic disorders during a ten year period from 1976 to 1985 according to the method of Jamshidi and Swaim. Indications and method of biopsy are discussed in detail. Major side effects were not observed, however minor accidents (0.2%) as well as problems in yielding biopsy-material (1.6%) are reported. The rate of biopsy-failure, including biopsies with insufficient (crushed) material, was 5%. In our hands the predominant value of the Jamshidi-biopsy for diagnosis of hematologic disorders is given by the following reasons: Bone marrow histology gives a more detailed architectural picture than cytologic smears. Sampling of bone marrow for both methods (cytology and histology) through the same instrument is possible. The procedure is easily performed and gives the patient no more discomfort than a simple sternal puncture. Chronic myeloproliferative disorders (CMPD, 31%), malignant lymphomas (40%) and aplastic (hypoplastic) syndromes (4%) were the most frequent indications for bone marrow biopsy. Clinical and histological findings were compared in 235 patients with CMPD. The histological defined entity of chronic megacaryocytic-granulocytic myelosis could be differentiated easily from chronic granulocytic leukemia (CGL), however it was not always distinguishable from primary thrombocythemia by means of clinical and hematological criteria. Myelofibroses on the basis of CGL were separated from idiopathic or postpolycythemic fibroses by hematological findings. The diagnostic value of bone marrow biopsies was superior to cytology in all CMPD and proved to be an essential diagnostic method in cases with high platelet count. Marrow involvement was found in 59% of 218 previously untreated patients with non Hodgkin's lymphomas and in 9% of 123 patients with Hodgkin's disease. Jamshidi-biopsy proved to be a simple and indispensable procedure in staging of Hodgkin's and non-Hodgkin's lymphomas.
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PMID:[Jamshidi biopsy in clinical hematology. Method, indications and results of over 1,000 completed biopsies with special reference to chronic myeloproliferative diseases]. 347 Oct 9

We reviewed 2110 bone marrow aspirations from the same number of patients to establish the incidence and associations of peripheral and bone marrow basophilia. Of these, 125 cases of marrow basophilia (5.9% incidence) and 63 cases of peripheral basophilia (3.0% incidence) were identified. There were 33 patients with simultaneous marrow and peripheral basophilia, which was only significantly associated with chronic myelogenous leukemia (24 cases). Isolated peripheral basophilia was rarely seen (30 patients, 1.4% incidence) and it did not reflect any significant pathologic association. Marrow basophilia was significantly present in chronic myeloproliferative disorders, idiopathic myelodysplasia, certain erythrocyte disorders, such as iron deficiency anemia, and aplastic anemia. The incidence of marrow basophilia in patients with lymphoma, acute leukemia, or solid carcinoma was not significantly different from what it would be as a chance occurrence. Our findings suggest that marrow basophilia is a specific, but not sensitive, marker of myeloproliferative and dysmyelopoietic syndromes.
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PMID:Incidence and clinical significance of peripheral and bone marrow basophilia. 350 57

The total urinary hydroxyproline excretion was assessed in 47 patients with chronic myeloproliferative disorders. Urinary hydroxyproline excretion was normal in 16 patients with idiopathic myelofibrosis and in 5 out of 6 patients with acute myelofibrosis. In patients with osteomyelosclerosis (n = 8) values for urinary hydroxyproline excretion were higher (median 202, range 54-652) than those in idiopathic myelofibrosis (median 139, range 84-216). This difference was not significant (p greater than 0.1). Elevated values for urinary hydroxyproline excretion were found in 10 patients (1 AMF patient, 3 OMS patients and 6 patients with CML in the accelerated phase of the disease). All but 1 of these patients had been treated, or were being treated, with cytotoxic agents at the time of investigation. These findings are compatible with impaired degradation of bone marrow collagen which, together with enhanced collagen synthesis from bone marrow fibroblasts, accounts for progressive accumulation of connective tissue in the bone marrow. This process appears to be influenced by cytotoxic treatment as reflected in increased urinary hydroxyproline excretion in those patients receiving cytotoxic agents.
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PMID:Urinary hydroxyproline excretion in the myelofibrosis-osteomyelosclerosis syndrome and related diseases. 369 62

In acute myeloid leukemia (AML-46 patients) and various entities of chronic myeloproliferative diseases (CMPD-58 patients) an evaluation and comparison of clinical and postmortem findings has been performed. This study included also aspirates and core biopsies of the bone marrow which were initially taken on admission of those patients. Classification of CMPD was done following the concept of Georgii et al. (1984) into CGL -24-, CMGM-6-, E-MS-13- and MS/OMS-15 cases. There was a significant increase in blastic crisis in CGL compared with the other entities and in the latter a prolongation of the total course of disease due to a long period between symptoms--clinical diagnosis. As revealed by the autopsies causes of death were mostly infections (pneumonia, septicemia-50%) and lethal hemorrhages (gastrointestinal and cerebral--about 30%) in both AML and CMGM patients. Rare causes comprised fatal pulmonary embolism due to a peripheral thrombocytosis in CMPD, acute rupture of the spleen and extensive leukemic infiltrates of the myocard in AML. In addition to the well known giant enlargement of the spleen in MS/OMS, the relatively high frequency of a meningeal involvement (meningeosis leukemica) in AML (about 35%) and during an acute transformation in CMPD (up to 30%) was conspicuous. The examination of the bone marrow at various sites became feasible during the postmortem procedure and thus provided the opportunity to investigate the development and extent of a myelosclerosis evolving in CMPD. In contrast to the a- or hypoplasia and regeneration of the hematopoiesis following chemotherapy, the evolution of myelosclerotic lesions showed a very uniform pattern throughout the skeleton and obviously no reversal of a manifest MS/OMS after cytotoxic treatment.
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PMID:Autopsy and clinical findings in acute leukemia and chronic myeloproliferative diseases--an evaluation of 104 patients. 385 35

An examination was carried out of certain parameters of basophil production in semisolid agar cultures by granulomonocyte precursors (CFU-C) from normal subjects and from patients with chronic myeloproliferative disorders. Basophils were rare in the first four days of culture, after which their number increased gradually to reach a plateau on about the 12th day. Pure basophil colonies were frequent only in cultures from a patient with marked basophilia. In the other cases the colonies also included other cells of the granulomonocyte pathway. There was a linear correlation between basophilia in vivo and basophil production in vitro. The latter, however, was influenced by the type of colony-stimulating activity (CSA) used. Different CSA sources, inducing the production of a comparable number of colonies, did not stimulate basophilic differentiation in the same way. Serum from patients with chronic myelocytic leukemia and varying degrees of basophilia did not have a significant effect on autologous in vitro basophilopoiesis, nor did it increase that of normal CFU-C.
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PMID:An in vitro study of basophil production in chronic myeloproliferative disorders. 633 9

The wide clinical range of CMPD can be understood as leukaemia of pluripotent stem cells according to the pathogenic concepts reviewed above. Blastic metamorphoses of CMPD are regressions to a more primitive level of cellular differentiation. The predominant proliferative cell line characterizes the classical entities of PV, PT and CML, and their different prognoses. Pure erythrocytic and megakaryocytic proliferations are more compatible with sustained physiologic bone marrow functions than granulocytic proliferations. The combinations of granulocytic and megakaryocytic growth are especially prone to develop MF/OMS, in which participation of immune reactions, of granulocytic and of platelet factors is probable. An etiologic role for ineffective thrombocytopoiesis is supported by experimental as well as by histologic evidence. Myelofibrosis and osteomyelosclerosis may have similar causes, but develop independently. The prevalence of the female sex among thrombocythaemic patients was proven statistically also for the increase of giant type megakaryocytes in the form of clusters in the bone marrow, and for longer median survival of females in CMPD, especially when there is megakaryocytosis in the bone marrow. It is assumed that females may be better protected against the detrimentous effects of abnormal platelet production. An arbitrary classification according to haematologic and histologic criteria was applied to PV, PT and CML, and groups with typical and atypical haematologic and histologic signs were distinguished. The latter cannot be separated from each other by their various haematologic manifestations, but by histology and their different propensity to progress into more immature and/or fibrotic stages. Three major groups are characterized by histology: mixed granulocytic-megakaryocytic myelosis with giant megakaryocytic clusters, a similar variant with diffuse distribution of giant megakaryocytes, and immature and/or pleomorphic megakaryocytic myelosis. Transitions from each of these groups have been observed as well as transitions from each of the typical CMPD-entities into these less typical forms. CML, frequently accompanied by dwarf-megakaryocytes, often develops into pleomorphic megakaryocytic or blastic myelosis. Blastic dedifferentiation and myelofibrosis manifest themselves as closely related end stages, to which principally all groups proceed after a longer or shorter period of time, modified by the proliferating cell lines in each group. Clinical, experimental and histologic evidence of this natural history has been reviewed, with special emphasis on the re-evaluation of technically optimal bone marrow biopsies of untreated patients.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Chronic myeloproliferative disorders (CMPD). 639 25

The chronic myeloproliferative disorders (MPD) are well characterized clinical entities comprising polycythaemia vera (PV), idiopathic thrombocythaemia (IT) (also called megakaryocytic myelosis, mature type; MegM), chronic myeloid leukaemia (CML), and myelofibrosis/osteomyelosclerosis (MF/OMS). Three thousand one hundred and eight bone biopsies of 2629 patients with established MPD were examined to investigate the histologic features of MPD in a large material in order to identify criteria for the histologic classification and differential diagnosis of these disorders. Detailed histologic characteristics were defined for each of the disorders and the results showed that in the majority of cases MPD may be recognized and classified by the initial bone marrow histology. Utilizing the predominant proliferative cell(s) in the bone marrow, PV was categorized into 4 types: 1. the classic, tri-linear type; 2. a bi-linear type with hyperplasia of the erythroid and granulocytic lines; 3. a bi-linear type with hyperplasia of the erythroid and megakaryocytic cell lines and 4. a uni-linear type with isolated increased erythrocytic proliferation. CML showed 2 sub-divisions: 1. the granulocytic, uni-linear type and 2. the bi-linear type with proliferation of myeloid and megakaryocytic lines. The former had a tendency to evolve into blastic crisis, while the latter was prone to develop into MF/OMS. It was primarily uni-linear exhibiting increased megakaryocytes. In most cases, MF/OMS was shown (by means of follow-up biopsies) to arise out of those entities of MPD which had included megakaryocytic hyperplasia and to which the proliferation of fibroblasts was secondary. The conclusion is drawn that an initial bone marrow biopsy provides additional diagnostic and prognostic data in this group of haematologic malignancies.
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PMID:Histologic criteria for classification and differential diagnosis of chronic myeloproliferative disorders. 653 29

Serum erythropoietin (ESF) levels and the numbers of marrow and blood erythroid progenitors (CFU-Es) of patients with chronic myeloproliferative disorders (CMPD) were studied simultaneously. The numbers of marrow and blood CFU-Es per 1 x 10(5) cells were normal or greatly elevated. There was an inverse correlation between the hemoglobin concentration and the serum ESF level in patients with chronic myelogenous leukemia when the hemoglobin concentration ranged from 9.0 to 13.0 g/100 ml. The serum ESF level was closely related to the hemoglobin concentration in CMPD and it was suggested that the negative feedback mechanism might operate in anemic patients with CMPD.
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PMID:Serum erythropoietin (ESF) levels and erythroid progenitors (CFU-Es) of patients with chronic myeloproliferative disorders. 658 39

In order to determine the relationship between the anomalies affecting two types of blood cell in myeloproliferative disorders (MPD), a functional study was performed in individuals presenting with such diseases. Thus, platelet function was investigated by means of Ivy's method for bleeding time, platelet retention to glass beads, aggregation with epinephrine and density distribution on a discontinuous sucrose gradient. Simultaneously, three granulocyte functions, i.e. capillary tube random migration, particle ingestion activity and nitroblue tetrazolium dye reduction were studied. This investigation was carried out in 47 patients presenting with chronic myeloproliferative disorders (MPD): chronic granulocytic leukemia (18 cases), polycythemia vera (18 cases), myelofibrosis (6 cases) and essential thrombocythemia (5 cases). The results of the present study indicate that functional abnormalities are more frequent and more strongly marked in platelets than in phagocytes. The tests most affected were platelet density distribution and granulocytic random migration. Simultaneous assessment of platelet and phagocytic functions, though insufficient in itself to determine the type of MPD or to appraise the prognosis of the disease, could be useful in the diagnosis of some atypical cases of myeloproliferative disorders.
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PMID:Simultaneous study of platelets and phagocytes in myeloproliferative disorders. 659 27

Blood coagulation and fibrinolysis were studied in 65 patients with chronic myeloproliferative disorders (MPD). They consisted of 28 patients with chronic granulocytic leukemia (CGL) in chronic phase, 7 with CGL in blast crisis, 9 with polycythemia vera (PV), 13 with primary thrombocythemia (PTh) and 8 with primary myelofibrosis (MF). Hemorrhagic and thrombotic complications were observed in 19 and 8 patients, respectively. Activated partial thromboplastin time and prothrombin time were prolonged in many patients. Low factor II levels were observed in some CGL patients. Factor V was decreased in CGL patients in chronic phase and in PV patients. Fibrinogen was either normal or increased in most patients, but an elevation of fibrin/fibrinogen degradation products (FDP) was found in some patients. The VIIIR: Ag/VIII:C ratio was increased in CGL patients in blast crisis, in PV patients and in PTh patients. Antithrombin III and plasminogen were below normal in some patients. Most patients showed a decrease in alpha 2-plasmin inhibitor. These findings suggest that blood coagulation and fibrinolysis are involved in the pathogenesis of the thrombotic and hemorrhagic complications in these patients. Chronic low-grade intravascular coagulation might be present in some patients with MPD.
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PMID:Profile of blood coagulation and fibrinolysis in chronic myeloproliferative disorders. 695 82


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