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)

We investigated the significance of p210 and p190 molecular abnormalities in 32 adults with Philadelphia chromosome (Ph)-positive acute leukemia. p210 was detected in 15 patients (47%), p190 in 16 (50%), and both in one (3%). p210 was noted in 11 of 24 patients (46%) with acute lymphocytic leukemia, and in four of eight patients (50%) with acute myelogenous or undifferentiated leukemia. Among 29 patients with untreated disease (p210, 14 patients; p190, 15 patients), no significant differences in the two molecularly distinct groups were observed by pretreatment characteristics including age, degree of organomegaly, anemia, leukocytosis, thrombocytopenia, occurrence of karyotypic abnormalities in addition to Ph, or residual diploid metaphases. Complete response (CR) rates were also similar. Although the remission duration tended to be longer with p190 (P = .08), the differences were minor (median duration 29 v 20 weeks) and not paralleled by differences in survival rate. In 10 patients studied by karyotypic analysis in remission, two of four patients with p190 and two of six patients with p210 showed 100% normal metaphases. One of the seven patients (14%) with p210 who achieved CR manifested a morphologic picture of second chronic-phase chronic myelogenous leukemia lasting for 1 month. We conclude that the molecular studies in Ph-positive acute leukemia are not associated with significantly different clinico-laboratory, karyotypic, or prognostic implications.
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PMID:Significance of the P210 versus P190 molecular abnormalities in adults with Philadelphia chromosome-positive acute leukemia. 193 53

A 47-year-old female developed autoimmune hemolytic anemia, autoimmune neutropenia, and autoimmune thrombocytopenia 19 months following allogeneic bone marrow transplantation for chronic myelogenous leukemia. Treatment with high-dose corticosteroids resulted in marked improvement in all three cell lines.
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PMID:Autoimmune pancytopenia following allogeneic bone marrow transplantation. 196 93

The prognostic importance of patient pretreatment clinical and laboratory features were investigated in a group of 50 patients with Philadelphia chromosome-positive benign phase chronic myelogenous leukemia. The overall median survival time was 30 months. The patient characteristic associated with shortened survival was the male sex. Anemia, thrombocytosis or thrombocytopenia tened to have adverse effect on survival. A multivariate regression analysis demonstrated only sex difference to be of prognostic importance. Evaluation of the effect of therapy showed that intensive chemotherapy was not superior to single agent chemotherapy.
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PMID:Multivariate analysis of prognostic factors in chronic myelogenous leukemia. 208 13

Blood findings in 61 cases of generalized mastocytosis (GM) were evaluated. The cases were divided into two major variants: Systemic mastocytosis (SM; n = 34) with urticaria pigmentosa-like skin lesions, and malignant mastocytosis (MM; n = 27), without skin involvement. The following results were obtained: (1) Significant differences between MM and SM were found in the main haematological parameters (erythrocyte, platelet and leucocyte counts and haemoglobin level); normal values were found in 16 of the SM cases, but never in MM. (2) The main pathological findings were: in SM, anaemia (9/34) and leucocytosis (5/34); and in MM, leucocytosis (19/27), monocytosis (14/27), eosinophilia (12/27), bicytopenia (12/27, mostly anaemia with thrombocytopenia), basophilia (10/27) and isolated anaemia (7/27). (3) The major finding was a significant difference between MM and SM in the incidence of myeloproliferative disorders (MPD), myelodysplasia and mast cell leukaemia (MCL): these disorders occurred in 23 (92%) MM patients, but only in two (6%) SM patients (P less than 0.001). The four instances of MCL and two of myelodysplasia all occurred with MM. Of the 19 cases of MPD, six (SM, 1; MM, 5) were acute variants (acute myeloid and myelomonocytic leukaemias) and 13 (SM, 1; MM, 12) were chronic variants. No case of malignant lymphoma was noted. (4) The blood picture in 10 of 13 chronic MPD cases represented an atypical chronic myeloid leukaemia for which the preliminary descriptive term 'mastocytosis-associated MPD' is proposed. (5) A survey of 103 published cases (SM, 77; MM, 26) yielded similar findings, including a high incidence of MPD and MCL in MM. These findings add further weight to the argument for recognizing SM and MM as two separate entities.
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PMID:Blood findings in generalized mastocytosis: evidence of frequent simultaneous occurrence of myeloproliferative disorders. 201 71

Platelet function and the clinical course of the disease were prospectively investigated in 29 patients with myeloproliferative disorders. Serial determinations (median: 5 investigations per patient within 17 months) of platelet aggregation, plasma and intraplatelet concentrations of beta-thromboglobulin (beta TG) and platelet factor 4 (PF4), and of fibrinopeptide A (FPA) plasma levels were carried out. In the chronic phase of polycythaemia vera, patients with thrombohaemorrhagic complications during the study period had higher platelet count, more severe platelet aggregation defects, and increased plasma levels of beta TG and FPA compared to patients without complications. However, thrombohaemorrhagic complications were not predicted by changes in these parameters in the individual patient during the chronic disease phase. When patients with chronic myelogenous leukaemia entered blast crisis, bleeding complications were related to thrombocytopenia, impaired platelet function and low intraplatelet concentrations of beta TG and PF4. Cytoreduction by chemotherapy in the chronic phase of CML did not alter beta TG and PF4 plasma levels, whereas treatment of polycythaemia rubra vera by venesection favourably influenced platelet alpha-granule secretion and increased intraplatelet concentrations of beta TG and PF4.
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PMID:A prospective study of haemostatic parameters in relation to the clinical course of myeloproliferative disorders. 214 44

Failure to obtain bone marrow on attempted marrow aspiration, "dry tap," has commonly been ascribed to faulty technique. All reports of simultaneous marrow aspirations and biopsies performed at the University of Virginia between January 1, 1983, and July 1, 1989, were reviewed to determine the frequency of dry taps, the diagnoses and pathologic findings in these cases, and the associated laboratory findings. Among 2,235 simultaneous bone marrow aspirations and biopsies, 87 were dry taps (3.9%). Of these 87 dry taps, only six (6.9%) showed normal marrow biopsies, whereas the majority showed significant marrow pathology, usually associated with fibrosis, or hypercellularity, or both. These conditions most likely account for the inability to aspirate marrow. The most frequent diagnoses were metastatic carcinoma (17.2%), chronic myelogenous leukemia (14.9%), idiopathic myelofibrosis (13.8%), and hairy cell leukemia (10.3%). The presence of peripheral blood nucleated red blood cells, thrombocytopenia, and elevation of the serum lactate dehydrogenase were frequent findings in patients who experienced dry taps. Methods to obtain sufficient marrow for rapid diagnosis in these cases are discussed.
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PMID:Dry tap bone marrow aspiration: clinical significance. 195 91

Leukocytosis, mild anemia, thrombocytosis, and panhyperplasia in the marrow characterize the early stages of most of the CMPD, whereas extramedullary hematopoiesis (such as in the spleen or liver), peripheral cytopenias (anemia, leukopenia, or thrombocytopenia), and myelofibrosis, with or without osteosclerosis, reflect the changes seen in the later stages. Transitions among the different CMPD and termination in acute leukemia or marrow failure also are common. CML often is characterized by leukocytosis and the presence of the entire spectrum of granulocytes (mature and immature) in the blood and marrow, reduced LAP, hypercellularity with prominent granulocytic hyperplasia in the marrow, Ph chromosome, and bcr-abl gene rearrangement. Typical features of AMM include leukoerythroblastosis, teardrop poikilocytosis, anemia, increased or normal LAP, prominent megakaryocytic hyperplasia in the marrow, dyshematopoiesis, and hyperplastic or fibrotic/sclerotic marrow.
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PMID:Myeloproliferative disorders. Classification and diagnostic features with special emphasis on chronic myelogenous leukemia and agnogenic myeloid metaplasia. 227 76

We report 3 patients with chronic myeloproliferative syndromes and active tuberculous infection. The first patient had chronic myelocytic leukemia. In this patient, primary tuberculosis was localized in the lung. In spite of tuberculostatic treatment, he developed laterocervical lymphadenopathy and a tuberculous abscess in right lower limb. The second patient had agnogenic myeloid metaplasia. In this patient splenic tuberculosis (TBC) with subsequent pulmonary involvement were diagnosed. The third patient had idiopathic thrombocytopenia with pulmonary tuberculous infection. In two patients, tuberculosis was diagnosed some time after the diagnosis of hematologic disease, while in the third one both conditions were simultaneously identified. One of the patients died of complications of alkylating therapy, and the other two are still alive. In none of the three patients there has been evidence of active tuberculous infection after therapy, although their course has been slow and two have required associated surgery to eradicate the infection. We review the hematologic conditions which can develop during tuberculosis, the incidence of this infection during chronic myeloproliferative syndromes, the possible pathogenetic relation between both conditions, and the clinical presentation of tuberculosis in these patients, which usually is disseminated in character.
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PMID:[Chronic myeloproliferative syndromes and tuberculosis: apropos of 3 cases]. 235 80

The major initial clinical, hematological and cytogenetical features of a series of 80 patients with blastic crisis (BC) in chronic myelocytic leukemia with positive Philadelphia chromosome (Ph) were evaluated, and also were their outcome and response to therapy. Mean age of patients was 45 years (SD: 14.3). Ten patients fulfilled the criteria for initial BC, and 14 had extramedullary blastic infiltration. In one third there was an acceleration phase before the development of BC. The mean leukocyte count was 69 (SD 75) X 10(9)/l. In 40% there was anemia with hemoglobin less than 90 g/l, and 37.5% had thrombopenia with less than 100 X 10(9) cells/l. In most patients, serum lactic dehydrogenase activity was increased, and in one fourth the index of granulocyte alkaline phosphatase was high. In 9 patients, blast cells had a lymphoid phenotype and in 47 (59%) cytogenetic abnormalities in addition to Ph chromosome were found, usually consisting of 8 trisomy, duplication of Ph chromosome, and the presence of a 17q isochromosome. The median survival of the series was 4.8 months. When analyzed as a time-dependent variable, the achievement of a favorable therapeutic response (found in 26% of patients) was associated with a longer survival.
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PMID:[Blast crisis of chronic myeloid leukemia with positive Philadelphia chromosome: course and clinico-hematologic profile in 80 patients]. 238 91

A 32-year-old male patient with chronic myelocytic leukemia in accelerated phase received a bone marrow allograft from his 42-year-old HLA/MLC-identical sister. He recovered from acute graft-versus-host disease (GVHD) grade III-IV of skin, liver and gut, but chronic GVHD of progressive onset developed. On day 556 post-graft severe thrombocytopenia was resistant to prednisolone, cyclophosphamide and high dose immunoglobulin. Splenectomy was followed by a normalization of platelet counts. The subsequent clinical course was characterized by progressive muscular atrophy and weight loss. Dysphagia, dysarthria, cachexia and ultimately recurrent pneumonic episodes ensued. The cachectic patient developed a highly abnormal breathing pattern with hypoventilation and intermittent apnea requiring mechanical ventilation. Auditory evoked potentials revealed a considerable dysfunction of the brainstem. The patient died on day 1120 post-graft from pneumonia, aggravated by thoracic muscular insufficiency. Postmortem examination revealed diffuse predominantly lymphoid perivascular infiltration in meninges and CNS tissue; proliferation of activated microglial cells expressing the HLA-DR antigen was prominent in the brainstem. These histologic changes are similar to those observed in the CNS in experimental GVHD. We suggest that this case represents the first documentation of CNS involvement in chronic GVHD.
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PMID:Fatal encephalitis in a patient with chronic graft-versus-host disease. 239 Jun 33


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