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)

Cell cycle phases of bone marrow cells from 8 patients with iron deficiency anemia (IDA), 8 aplastic anemia (AA), 30 myelodysplastic syndrome (MDS), 41 acute leukemia (AL) before treatment, 8 acute leukemia in relapse, 17 acute leukemia in complete remission (CR), 12 chronic myelogenous leukemia (CML) and 4 chronic lymphocytic leukemia (CLL) were analysed with flow cytometry. The proportions of phases of S. G2 M in patients with IDA, refractory anemia, and refractory anemia with ring sideroblast were similar to these in normal controls (P > 0.05). However, they were significantly lower in patients with AA, refractory anemia with excess of blast (RAEB) and transformed RAEB than those in normal controls (P < 0.01, respectively), and CML patients than in normal controls (P < 0.05). The S G2M% was apparently higher in patients with CML than that in CLL (P < 0.01). But, there was no difference between in ALL and ANLL (P > 0.05). It was higher in patients with AL in CR and in relapse than AL before treatment (both P < 0.01). It was still lower in the former than that in normal controls. (P < 0.05). The clinical significance of cell cycle status was also discussed in this paper.
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PMID:[Flow cytometric analysis of bone marrow cell cycles in patients with hematologic diseases]. 147 30

We report two patients with a myelodysplastic syndrome and the Philadelphia (Ph) chromosome. The first patient was a 73-year-old man who was diagnosed as having a chronic myelomonocytic leukemia in combination with features suggestive of a myeloproliferative syndrome. Chromosomal analysis showed a normal karyotype in the majority of cells, mixed with metaphases containing a standard Ph translocation, t(9;22)(q34;q11), as well as a translocation between chromosome 4 and 6: t(4;6)(p15;p12). Southern blot analysis showed breakpoint cluster region rearrangement as observed in classic chronic myeloid leukemia. The second patient was a 63-year-old man with a myelodysplastic syndrome, type refractory anemia. Cytogenetic study of bone marrow cells at the time of diagnosis revealed a normal karyotype: 46,XY. The initial myelodysplastic syndrome evolved to a myeloproliferative phase with progressive leukocytosis and thrombocytosis. During the terminal phase the Ph chromosome was discovered in 100% of the examined cells. We discuss the correlation between MDS and myeloproliferative diseases, the de novo acquisition of the Ph chromosome during the course of a myelodysplastic syndrome, and review the literature.
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PMID:Cytogenetic and molecular studies of the Philadelphia translocation in myelodysplastic syndromes. Report of two cases and review of the literature. 158 81

Translocation (6;9)(p23;q34) is a cytogenetic aberration that can be found in specific subtypes of both acute myeloid leukemia (AML) and myelodysplastic syndrome (MDS). This translocation is associated with an unfavourable prognosis. Recently, the genes involved in the t(6;9) were isolated and characterized. Breakpoints in both the dek gene on chromosome 6 and the can gene on chromosome 9 appear to occur in defined regions, which allows us to diagnose this type of leukemia at the molecular level. Moreover, because of the translocation a chimeric dek-can mRNA is formed which, as we show here, is an additional target for diagnosis via cDNA-preparation and the polymerase chain reaction (PCR). We studied 17 patients whose blood cells and/or bone marrow cells showed a t(6;9) with karyotypic analysis. Fourteen patients suffered from AML, one patient had a refractory anemia with excess of blasts in transformation (RAEBt), one patient had an acute myelofibrosis (AMF), and one patient a chronic myeloid leukemia (CML). In nine cases studies at the DNA and RNA levels were possible while in seven cases only the DNA could be analyzed. In one case only RNA was available. Conventional Southern blot analysis showed the presence of rearrangements of both the dek gene and the can gene. In both genes, breakpoints cluster in one intron in the patients investigated. The presence of a consistent chimeric dek-can product after cDNA preparation followed by the PCR was demonstrated. We conclude from our data that the t(6;9) is found in myeloproliferative disorders with typical clinical characteristics. This translocation results in highly consistent abnormalities at the molecular level.
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PMID:The translocation (6;9) (p23;q34) shows consistent rearrangement of two genes and defines a myeloproliferative disorder with specific clinical features. 158 43

Myelodysplastic syndrome (refractory anemia with excess of blasts; RAEB) with marked basophilia and eosinophilia is described. An 82-year-old male was admitted to our hospital because of severe normocytic normochromic anemia (Hb 5.6 g/dl). The white cell count was 9,200/microliters with marked basophilia (34.5%) and eosinophilia (19.5%). The bone marrow aspiration also revealed both basophilia and eosinophilia, with blast contents of 9%. Diagnosis of RAEB was established. Although the treatment with red cell transfusion and ubenimex (Bastatin) was started, anemia was not improved. A karyotype of the bone marrow cells from this patient showed 47, XY, +8, i (17q), which has been observed as additional chromosomal abnormalities in blastic crisis of chronic myelogenous leukemia. The diagnosis of CML was not compatible with this case, because Ph1 chromosome and bcr gene rearrangement were negative. It is concluded that eosinophilia and basophilia might be derived from clonal abnormalities associated with MDS.
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PMID:[Myelodysplastic syndrome associated with marked eosinophilia and basophilia]. 163 67

We have used recombinant human erythropoietin (rHuEPO) in a phase I/II clinical trial to evaluate its ability to reverse refractory anemia in hematologic disorders. rHuEPO was administered subcutaneously 5 days per week at escalating doses (50 to 150 U/kg per day). The aim of treatment was a hemoglobin (Hb) level greater than or equal to 10 g/dL without blood transfusion. Of 25 patients treated, 17 were evaluable, most of them with a regular need for transfusion. Eight of these had lymphoproliferative disorders (three cases of malignant lymphoma and five of monoclonal gammopathy) and were exposed to cytotoxic therapy. The other nine patients had hematopoietic stem cell disorders (four cases of myelodysplastic syndrome, three of idiopathic myelofibrosis, and two of chronic myelogenous leukemia). All patients with lymphoproliferative disorder had serum EPO levels inappropriately low for the degree of anemia, while patients with stem cell disorder showed variable values. Erythroid marrow activity was inadequate in all cases. Seven of eight patients with lymphoproliferative disorder responded to treatment maintaining Hb above 10 g/dL without transfusion. The median dose of rHuEPO required for correction of anemia was 75 U/kg. In four cases response was maintained with 50 U/kg, three times per week. There was no complete response among patients with hematopoietic stem cell disorder, although transfusion requirement was eliminated or reduced in four cases. Four patients developed functional iron deficiency during rHuEPO treatment and required iron supplementation to obtain response. Aggravation of splenomegaly was observed in two cases of myeloproliferative disorder. We conclude that: (1) subcutaneous administration of rHuEPO can be effective and safe in patients with lymphoproliferative disorder exposed to chemotherapy and showing inappropriate EPO response to anemia; (2) this is less likely in hematopoietic stem cell disorders, although favorable responses may be observed in occasional patients; and (3) functional iron deficiency as a cause of nonresponse to rHuEPO is frequent also in nonrenal anemia.
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PMID:Subcutaneous erythropoietin for treatment of refractory anemia in hematologic disorders. Results of a phase I/II clinical trial. 163 33

Twenty cases of myelodysplastic syndrome (MDS) were treated with ubenimex. Seventeen cases were treated with the drug over 90 d. Among these, 10 showed improvement of anemia, 12 an increase in platelet count which had decreased before treatment and 10 an increase in neutrophil count; however, 14 showed an increase in blast percentage in bone marrow aspirate. CD4/CD8 ratio was increased in 4 cases and shifted to a normal from an abnormal range in 6 cases. When the MDS cases were observed in refractory anemia (RA) and refractory anemia with excess blasts (RAEB), great improvement was seen, but in RAEB increase in blast percentage was also observed. CD4 increased mostly in RA and CD8 increased in RAEB. Ten cases of chronic myelocytic leukemia (CML) were first treated with ubenimex and cytostatics, then with ubenimex only. Six cases attained partial remission within 3 months, but one case showed a marked increase in white cell count and blast count and in another case a progression of splenomegaly associated with increase in white cell count. From these findings we conclude that ubenimex could be utilized in MDS or CML if the patient was at risk for strong chemotherapy.
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PMID:Effects of ubenimex, a biological response modifier, on myelodysplastic syndrome and chronic leukemia. 191 74

Chronic myelomonocytic leukemia (CMML) is a polymorphous malignant hematological stem cell disorder, characterized by abnormal hyperplasia of mature or immature cells of both monocytic and granulocytic series and with abnormal cellular morphology. It is an independent entity of chronic leukemia, as its prestage course is manifested by refractory anemia with monocytosis and the disease gradually evolves to CMML. In some cases, it finally becomes acute leukemia. In this study, the average white cell count of the patients was 29.3 x 10(9)/L.14 cases had leucocytosis, 7 leucopenia and 5 normal count. The absolute value of monocytes was 19 x 10(9)/L and the proportion of monocytes was 10-87%, with an average of 49%. In the leukopenic group with white cell count less than 4 x 10(9)/L, the absolute value of monocytes was less than 1 x 10(9)/L in 5 of the 7 cases. However, it was noticed that all the 5 cases had a proportion of monocytes greater than or equal to 10%. The authors would like to take this percentage as the diagnostic criteria for CMML, which is different from that adopted in FAB classification of 1982 as well as in the literatures. Statistics showed that P value of M/E, Mo/E, Mo/M were of apparent significance in the differentiation of CMML from normal controls and patients with other hematological diseases such as RA, RAEB, CML, CNL, M4 and M5.
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PMID:[An analysis of 26 cases of chronic myelomonocytic leukemia]. 226 33

We report a 56 year old patient with acute myelogenous leukemia (FAB classification: M2), in whom the number of mature myeloid cells similar to those seen in Ph-negative chronic myelogenous leukemia increased markedly 2 months after the diagnosis of refractory anemia with excess of blasts (RAEB). This is a rare case of leukemic evolution as a terminal event of RAEB.
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PMID:Transformation of refractory anemia with excess of blasts into acute myelogenous leukemia with Ph-negative chronic myelogenous leukemia-like characteristics. 229 65

We report on eight patients who were 35 to 77 years old with an isochromosome 17q as the sole structural chromosomal anomaly. Additional numerical chromosomal changes were a trisomy 8 or 17 in two cases each and a trisomy 19 in one case. Five patients had myelodysplastic syndrome (MDS) diagnosed according to the FAB nomenclature as chronic myelomonocytic leukemia (CMML) in two cases, refractory anemia with excess of blasts in transformation (RAEBt) in two cases, and refractory anemia with excess of blasts (RAEB) in one case. One patient suffered from a myeloproliferative disorder (MPS). All cases progressed to acute nonlymphocytic leukemia (ANLL) type M1, M2, or M4 in a period of 2 to 30 months after initial diagnosis, except one patient with RAEBt who died within 2 months. Two patients presented with ANLL-M2 at time of diagnosis. Treatment during the chronic phase of disease consisted of mild cytoreduction and/or substitution of platelets or red blood cells. One patient with CMML received an allogeneic bone marrow graft and relapsed after 33 months with ANLL-M1. Treatment results for overt leukemia were poor, and survival was short, lasting from 1 to 4 months. Overall survival was 1 to 37 months (median duration, 6.5 months). Molecular studies in two cases revealed neither a BCR rearrangement nor a translocation of the ABL protooncogene, as observed in Ph1-positive chronic myeloid leukemia (CML). Thus, an i(17q) anomaly seems to identify a distinct subgroup of mostly myelodysplastic and, less frequently, myeloproliferative disorders that progress rapidly to ANLL, respond poorly to chemotherapy, and are associated with short survival after transformation.
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PMID:Isochromosome 17q in Ph1-negative leukemia: a clinical, cytogenetic, and molecular study. 222 38

In the present study we have evaluated the efficacy and toxicity of a 6-day continuous constant rate intravenous infusion of anthracyclines added to the standard conditioning regimen for allogeneic bone marrow transplantation (BMT). In 22 consecutive recipients of a lymphocyte depleted bone marrow graft, either demethoxydaunomycin (n = 11) or daunorubicin (n = 11) were added to high-dose cyclophosphamide and total body irradiation. Five patients had acute non-lymphoblastic leukemia in first complete remission, six patients acute lymphoblastic leukemia in first or second complete remission, nine patients chronic myelogenous leukemia in chronic phase and two patients refractory anemia with excess of blasts. After a median observation period of 18 months, only one leukemic relapse has been observed. Six patients died in the post-transplant period. In 17 of the 22 patients a severe, transient mucositis developed. No cardiac toxicity, as assessed with radioisotope studies, was observed. We conclude that anthracyclines may be effectively and safely incorporated in conditioning regimens before BMT, provided that they are administered as long-term continuous infusions in order to avoid toxicity due to excessive plasma levels.
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PMID:Prevention of leukemic relapse after transplantation with lymphocyte depleted marrow by intensification of the conditioning regimen with a 6-day continuous infusion of anthracyclines. 265 Jul 87


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