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Query: UMLS:C0023473 (chronic myeloid leukemia)
 18,916 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

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

Morphometric evaluation was performed on semi-thin sections of core biopsies of the bone marrow and included 20 cases of each group of diseases besides control specimens. (i) Hyperergic myelitis of rheumatic origin. (ii) Chronic granulocytic leukaemia (CGL). (iii) Polycythaemia vera (P. vera). (iv) Chronic megakaryocytic-granulocytic myelosis (CMGM). (v) Myelofibrosis or osteomyelosclerosis (MF/OMS). The following classification of megakaryopoiesis was applied: normal megakaryocytes; giant forms; microforms; intussusceptions; cytoplasmic fragments; naked nuclei. The density distribution shows an increase of megakaryocyte number in those 5 different marrow disorders, ranging from about 13/mm2 in the normal sample up to 65 cells/mm2 in MF/OMS. Microforms are most frequently encountered in CGL, whereas giant megakaryocytes, intussusceptions and many cytoplasmic fragments characterize P. vera, CMGM and MF/OMS. Our measurements suggests 3 distinct categories of bone marrow lesions with corresponding alterations of the megakaryopoiesis: (i) myelitis and CGL; (ii) P. vera; (iii) CMGM and MF/OMS.
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PMID:Density distribution and size of megakaryocytes in inflammatory reactions of the bone marrow (myelitis) and chronic myeloproliferative diseases. 657 91

Myeloid committed stem cells belong to a subpopulation of small nucleated cells, which are defined by their capacity to form colonies of mature myeloid cells in agar-medium. They are termed "Colony forming Unit, CFUC", and such cells are detectable in bone marrow and peripheral blood. Bone marrow cells from 15 control patients with regular myelopoiesis contained 86 +/- 46 CFUC/10(5) bone marrow cells and 23 +/- 14 CFUC/ml blood. In 10 patients with aplastic anemia, only 0-10, 5 CFUC/10(5) BM-cells were found and no CFUC were detectable in the peripheral blood. 17 patients with chronic myeloid leukaemia showed a moderate elevation of bone marrow CFUC (X = 105), while the circulating CFUC were markedly elevated (105-42.000/ml). The circulating CFUC were closely correlated with the number of leukocytes (p less than 0,001). In 12 patients with primary osteomyelofibrosis, the number of circulating CFUC was also (raised (325-22.199/ml) and again, a correlation with the number of leukocytes was observed (p less than 0,05). As, on the other hand, there was no difference in the leukocyte count between the control group and patients with osteomyelosclerosis, the simultaneous assessment of circulating leukocytes and CFUC proves a diagnostic tool. Pancytopenia with a hypercellular bone marrow results from either neoplastic or metabolic alterations of haemopoiesis; in pancytopenia with neoplastic infiltration or transformation, the number of CFUC was lowered, whereas it was slightly elevated in pancytopenia due to metabolic alterations. In patients with acute leukaemia, only a minority of cells was capable of proliferation in vitro. The growth of leukaemic cells in culture, their prolonged survival along with the expression of functional properties may be clinically used for a more subtle classification of blast populations. The data on patients with acute leukaemia indicate, that basic mechanisms of normal blood cell regulation operate in leukaemic haemopoiesis as well.
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PMID:[The determination of myeloid-committed stem cells in systemic disorders of myelopoiesis: implications for physiopathology, diagnosis and prognosis]. 694 36

The chronic myeloproliferative disorders (MPD) comprise polycythemia vera (PV), idiopathic thrombocythemia (IT), chronic myeloid leukemia (CML) and myelofibrosis/osteomyelosclerosis (MF/OMS). Bone marrow biopsies of 3500 patients with known or suspected MPD were studied, and the clinical and morphologic variables registered were utilized for multivariate data analysis by selected BMD computer programs. The histologic criteria and the histologic subdivisions, as well as the evolution and prognosis of disease are outlined for each of the clinical entities. The results show that a bone marrow biopsy provides independent diagnostic and prognostic data in this group of hematologic malignancies and therefore constitutes an additional parameter in the diagnostic investigation of patients with suspected or established MPD.
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PMID:Potential of bone marrow biopsy in chronic myeloproliferative disorders (MPD). 843 14

The chronic myeloproliferative syndromes are bone marrow stem cell disorders. An increase of cell counts of one or rather all three blood cell types is characteristic for these disorders. The most important diseases in this group are: chronic myelogenous/granulocytic leukemia, polycythemia rubra vera, osteomyelosclerosis or agnogenic myeloid metaplasia and essential thrombocythemia. The cells are normally differentiating in these diseases, while the control of cell dividing is abnormal and therefore the cells are produced and accumulated in excess. Splenomegaly is a common and characteristic clinical finding. The fibrotic or sclerotic transformation of the bone marrow can take place in all forms of the syndrome. Extramedullary haematopoiesis can occur in all of the above diseases, but it is most common in myelofibrosis/agnogenic myeloid metaplasia. In the last phase of the disease a terminal blastic crisis may terminate the course of chronic myeloproliferative diseases. The myeloproliferative disorders can be transformed in each other--the most common transformation is that of the polycythemia rubra vera into myelofibrosis. The greatest progress in the therapy of chronic myeloproliferative diseases is achieved in chronic myelogenous leukemia: bone marrow transplantation and interferon treatment (the latter also in essential thrombocythemia and polycythemia rubra vera) are routine modalities worldwide. A new drug, anagrelide is effective in the treatment of myeloproliferative thrombocytosis and thrombocythemia.
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PMID:[Chronic myeloproliferative diseases]. 971 44

The aim of the present research was to verify the prognostic value of some histologic bone marrow parameters in chronic myeloproliferative disorders. Diagnostic bone marrow biopsies were revised in 38 patients with chronic myeloid leukaemia, 30 with polycythemia vera, 14 with essential thrombocythemia and 16 with idiopathic myelofibrosis-myeloid metaplasia. An unfavourable clinical evolution was associated to "granulocytic" histotype in chronic myeloid leukaemia, to "erythrocytic/granulocytic and/or megacaryocytic" histotype in polycythemia vera, to "cluster" distribution of megacaryocytes in essential thrombocythemia, to classical myelofibrosis without osteomyelosclerosis in myelofibrosis-myeloid metaplasia. Bone marrow biopsy in chronic myeloproliferative disorders provides independent diagnostic and prognostic data; we support its routine execution in this group of hematologic malignancies.
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PMID:[Prognostic value of bone marrow biopsy in chronic myeloproliferative disorders]. 1286 37


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