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 association between myelofibrosis (MF) and chronic granulocytic leukemia (CGL) has been recognized. MF is usually a sign of a poor prognosis but its relation to other important parameters of CGL is not known. We observed a 54-year-old, white male patient who was well until May 1983 when he began developing gradually increasing right hip and left shoulder pain. Clinical evaluation 3 months later revealed splenomegaly and a white blood count of 126,000 with 29 segmented neutrophils, 22 bands, 7 metamyelocytes, 11 myelocytes, 6 promyelocytes, 5 blasts, 2 eosinophils, 5 basophils, and 3 lymphocytes. Cytogenetic analysis by G-banding technique showed a male karyotype with all 20 bone marrow cells examined positive for the Philadelphia chromosome. The patient was placed on busulfan therapy with good symptomatic improvement, but later suffered severe thrombocytopenia. At the end of October 1983, he was admitted with blast crisis and thrombocytopenia and was initiated on vincristine and cytosine arabinoside therapy. His bone marrow was repeatedly inaspirable and the biopsy was characterized by diffuse fibrosis. Chromosome analysis of 16 spontaneously dividing cells in the blood at this time revealed that 86% of cells had a karyotype of 46,XY,t(9;22)(q34;q11),t(1;3)(p32;p21) with the rest of the cells having only the Ph chromosome. The patient died 4 months later of intracranial hemorrhage. Chromosome #3 involvement has been reported in acute MF and essential thrombocytosis, but no specific cytogenetic abnormalities have been found in MF associated with CGL. It is unclear whether t(1;3) in this case represents a cytogenetic marker of MF or blast transformation, but it is certainly associated with poor prognosis and short survival.
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PMID:Possible cytogenetic marker associated with myelofibrosis in chronic granulocytic leukemia and its prognostic significance. 347 Jan 22

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

The computed tomography appearance of adrenal hemorrhage secondary to adrenal vein thrombosis is illustrated. The lesion appeared radiolucent, with a small focal calcification, mimicking an adrenal adenoma. Hypercoagulopathy was an underlying factor in this patient with myelofibrosis and chronic myelogenous leukemia.
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PMID:Computed tomography appearance of adrenal vein thrombosis. 347 74

10 patients with CLL and 2 with CML were treated with gradually increasing doses of 1 alpha(OH)D3, up to 4 micrograms daily during 6 wk. 3 patients with preleukemia and 1 with myelofibrosis were treated with 2 micrograms daily of 1 alpha(OH)D3 for a prolonged period up to 17 wk. The treatment with 1 alpha (OH)D3 did not result in changes of disease parameters in any of the patients under study. Receptor studies for 1,25(OH)2D3 were performed in 8 CLL patients and revealed only 1 patient with increased specific receptor binding capacity. The maximum tolerable dose of 1 alpha(OH)D3 varied individually, but was in the range of 2-4 micrograms daily.
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PMID:1 alpha(OH)D3 (ETALPHA) treatment and receptor studies in 16 patients with chronic and myeloproliferative disorders. 347 70

A case of well-documented and -illustrated megakaryoblastic transformation is described in a patient with thrombocythemia passing through a stage of myelofibrosis without features of chronic granulocytic leukemia. Immunocytologic studies with the use of conventional and monoclonal antibodies against platelet membrane glycoproteins and electron microscopic investigations, demonstrating bull's-eye granules and platelet peroxidase positivity, proved the megakaryocytic differentiation of the blast cells. From the onset of the disease as well as during the megakaryoblastic transformation, the Philadelphia (Ph1) karyotype, 46XX t(9:22) (q34:q11), was found in peripheral blood and bone marrow cells as the only clonal abnormality. Southern blot analysis of DNA extracted from the blast cells revealed a rearrangement within the bcr on chromosome 22 similar to findings in chronic granulocytic leukemia. The presentation with excessive small and abnormal megakaryocytes in the initial and subsequent bone marrow and the rapid progressive myelofibrosis and splenomegaly differentiate the Ph1 chromosome-positive thrombocythemia from the chronic myeloproliferation of thrombocythemia in its primary form or associated with polycythemia vera.
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PMID:Philadelphia chromosome-positive thrombocythemia and megakaryoblast leukemia. 347 3

A phase II study of the oral agent methyl 6-[3-(2-chloroethyl)-3-nitrosoureido]-6-deoxy-alpha-D-glucopyranoside (MCNU tablet) for myeloproliferative disorders was performed. Fifty-two patients were treated with MCNU tablets and 43 patients were evaluated for clinical effects and 45 for adverse effects. The standard regimen was as follows; oral administration of 50mg (one tablet)/body/day every 4-6 days was considered as one course, and this was repeated at 6-8-week intervals if possible, with certain modifications according to dosage, period of administration and dose interval wherever necessary. Of 16 patients with chronic myelogenous leukemia (CML) in the chronic phase, 13 achieved complete remission (CR), and 3 achieved partial remission (PR). The overall response ratio was 100%. Rapid reduction of leucocytes was detected within two weeks. One patient with CML in blast crisis achieved PR (100%). Of 15 patients with polycythemia vera, 13 showed an excellent effect (87%), and 1 a moderate effect (6.7%), the overall response ratio being 93%. In essential thrombocythemia, an excellent effect (70%) was obtained in 7 of 10 patients. One patient with myelofibrosis showed an excellent effect (100%). Nausea & vomiting (33%) and anorexia (13%) were major adverse effects, but these symptoms were observed only transiently. Liver dysfunction was also seen in 8.9% of patients, but no patient showed severe manifestations. Our study supports the contention that MCNU tablet is a useful agent against myeloproliferative disorders.
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PMID:[Phase II study of a new nitrosourea derivative, MCNU, in tablet form. Takai Blood Cancer Study Group]. 356 4

N4-Palmitoyl-1-beta-D-arabinofuranosylcytosine (PL-AC) was administered p.o. to 199 patients with acute leukemia, myelodysplastic syndromes (MDS) and myeloproliferative disorders (MPD). Of 76 patients with AML, 11 achieved complete remission (CR) and 7 achieved partial remission (PR). Of 8 patients with ALL, 2 achieved CR and 1 achieved PR. Of 3 patients with blast crisis of MPD, 1 achieved CR. CR was reached with PL-AC at 100-900 mg/day after 5-98 (median 26) days. Of 50 patients with MDS, 2 achieved CR, 2 showed good response and 7 partial response. Response was reached with 100-400 mg/day after 13-122 (median 32) days. Improvement of polycythemia vera was observed in 6 of 13 patients, and reduction of thrombocytosis was observed in 20 of 23 patients with essential thrombocythemia and myelofibrosis. Of 18 patients with CML, 1 achieved CR. Major side effects were GI toxicities and myelosuppression. In spite of the disadvantages of the oral form of the drug, such as unpredictable absorption, PL-AC may be useful in the treatment of acute leukemia, especially that of the aged, a condition for which intensive chemotherapy is not always indicated, and MDS, which do not necessarily require admission to a hospital.
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PMID:[A phase II study of N4-palmitoyl-1-beta-D-arabinofuranosylcytosine (PL-AC) in patients with acute leukemia and myelodysplastic syndromes. Cooperative Study Group for PL-AC]. 361 59

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

N4-Palmitoyl-1-beta-D-arabinofuranosylcytosine (PLAC) was administered PO to 76 patients with acute leukemia, myelodysplastic syndromes (MDSs), and myeloproliferative disorders (MPDs). Of 20 patients with acute myelogenous leukemia, 2 achieved complete remission, and the only patient with acute lymphoblastic leukemia achieved partial remission. Remission was reached with PLAC 100-300 mg/day 25-66 days after the start of therapy. Among 22 patients with MDS, 1 patient achieved a good response and 8 achieved partial response. Responses were reached with PLAC 50-200 mg/day 7-153 days (median, 33 days) after the start of therapy. Improvement of polycythemia was observed in all 5 patients with polycythemia vera, and reduction of thrombocytosis was observed in 5 out of 6 patients with essential thrombocythemia and myelofibrosis. An antileukemia effect was noted in 1 of 5 with chronic myelogenous leukemia. Major side effects were gastrointestinal toxicities and myelosuppression. In spite of the disadvantages, such as unpredictable absorption and a lower response rate to acute leukemia compared with its parent compound, this antileukemia Ara-C analogue that is administrable PO will be useful in the treatment of MDSs and MPDs, which do not necessarily require admission to hospital, and in the treatment of acute leukemia of the aged, a condition for which intensive chemotherapy is not appropriate.
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PMID:Treatment of leukemia and myelodysplastic syndromes with orally administered N4-palmitoyl-1-beta-D-arabinofuranosylcytosine. 371 96

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


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