Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0023473 (chronic myeloid leukemia)
18,916 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

[Case 1] A 44-year-old female was referred to our hospital because of leukocytosis. The WBC count was 26400/microliters and NAP score 21. As Ph1 chromosome was detected, she was diagnosed as CML and treated with busulfan. Because of the rapid decrease of WBC, we stopped busulfan. Progressive pancytopenia and an increase of myeloblasts and promyeloblasts in the bone marrow was observed. We started vincristine and prednisolone therapy. Ph1 chromosome was not detectable and southern blot analysis did not show rearranged bands of M-bcr three years after the last therapy. [Case 2] A 74-year-old female was referred to our hospital by reason of leukocytosis and thrombocytosis. The WBC count was 22,500/microliters, the platelet 907,000/microliters, NAP score 53, and Ph1 chromosome was found. The diagnosis of CML was made, and she was treated with busulfan. The WBC rapidly fell to 1,900/microliters, when chromosome analysis revealed the presence of Ph1 negative clones (4/20). She was admitted due to thrombocytopenia and leukocytosis with the additional chromosome change of i (17q). Her peripheral blood and bone marrow pictures were consistent with blast crisis, and she died of cardiac tamponade. These two cases show the heterogeneity of CML patients, and also suggest the possibility that keeping the WBC count low may lead to a decrease of Ph1 positive clones.
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PMID:[Partial and complete disappearance of Ph1 chromosome in two patients with chronic myelogenous leukemia after conventional chemotherapy]. 836 76

A readable and reproducible 5-nucleotidase (5N) cytochemical reaction was developed for blood smear preparation, after modification of the technique of Wachstein and Meisel. The reaction was applied to normal polymorphonuclear neutrophils (NPN) and to neutrophils from patients with chronic myelogenous leukemia (CML), myelofibrosis with myeloid metaplasia (MMM), and polycythemia vera (PV). The following observations were made: (a) 5N was present in NPN, with a mean score of 83.2+/-15.7. (b) In patients with MMM and PV an increased 5N score was observed (mean score 111+/-63.8 and 178.3+/-83.3, respectively). (c) In CML the mean score was 4.9+/-2.2. (c) A statistical comparison of neutrophil 5-nucleotidase (N5N) between CML and MMM and PV patients demonstrated a highly significant difference (p<0.0001). In the present study, we showed that the N5N activity parallels that of NAP in chronic myeloproliferative disorders such as CML, MMM, and PV. It appears that, apart from the already known activity of NAP in myeloproliferative disorders, other enzymes (e.g., N5N) can present a similar behavior with increased or decreased activity.
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PMID:Neutrophil 5-nucleotidase reaction in chronic myelogenous leukemia, myelofibrosis with myeloid metaplasia, and polycythemia vera. 948 17

A 44-year-old man with CML in chronic phase was admitted for BMT from an HLA-identical sibling. Ph positive cells were undetectable at 3 and 7 months after BMT but became detectable by cytogenetic analysis of bone marrow aspirates at 12 months after BMT. He was treated with IFN-alpha (6 million units/day, 3 times a week) without apparent effect. Donor leukocyte transfusion (DLT) was performed four times between 20 months and 23 months after BMT, transfusing 3.4 x 10(8) mononuclear cells/kg. However, leukocytosis appeared and the NAP score declined at 25 months after BMT. FISH analysis revealed an increase in bcr-abl positive cells. IFN-alpha was restarted using the same schedule at 26 months after BMT. Three months after restarting IFN-alpha, the leukocyte count fell to the normal range, NAP score increased to a normal level, and bcr-abl positive cells decreased markedly. He has remained in hematological and cytogenetic remission for 20 months, and bcr-abl chimeric mRNA remained undetectable by PCR. These results suggest that CML which does not respond to DLT may be cured by subsequent IFN-alpha therapy, possibly by inducing anti-leukemia immune responses.
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PMID:[Successful treatment with donor leukocyte transfusion followed by interferon-alpha in a patient with relapsed chronic myelogenous leukemia after allogeneic bone marrow transplantation]. 969 73


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