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 report a single center experience of 222 patients (pts) less than 18 years old transplanted from 1973 to 1987. The median age was 11 years (1-18). The donor was a monozygotic twin (9 pts), an HLA-id sibling (193 pts), an HLA-id, parent (9 pts), a mismatched related donor (9 pts) and a matched unrelated donor (1 pt). Ninety-six pts were transplanted for SAA. Conditioning varied with time but the majority (59 pts) received CY 150 mg/kg and 6 Gy TAI. The long term actuarial survival is 66% with a median follow-up of 3 years. The group who received CY 200 mg/kg and MTX had a 33% long term survival (LTS). GVH was the main complication with 40% acute and 37% chronic GVHD. Chronic GVHD tended to improve with time after 2 to 4 years of evolution. Ninety pts were transplanted for leukemia (35 AML, 45 ALL and 11 CGL), 20 pts were in relapse. Pts in CR had a LTS of 40%, in pts in relapse, it was 12%. The main causes of death were: interstitial pneumonitis (30%), relapse (27%), GVH (15%). Thirty-five pts were transplanted for constitutional disease: Fanconi anemia (FA) (26 pts), Dyskeratosis congenita (2 pts), Blackfan-Diamond erythroblastopenia (2 pts), Glanzmann thrombasthenia (1 pt), osteopetrosis (1 pt) and Gaucher's disease (1 pt). In FA, the LTS is 70% with a CY 20 mg/kg, 5 Gy TAI regimen. In all disease categories, we did not find any influence of donor's sex on GVH and survival.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Pediatric bone marrow transplantation for leukemia and aplastic anemia. Report of 222 cases transplanted in a single center. 267 24

To determine whether distinct subpopulations of platelets exist in individual patients, platelets were incubated with monoclonal antibodies to glycoprotein Ib and the glycoprotein IIb-IIIa complex, and analyzed by flow cytometry. Normal donors (n = 15) had single glycoprotein Ib-positive and glycoprotein IIb-IIIa complex-positive populations of platelets, with no subpopulations. In normal donors there was a direct relationship between platelet size and the number of surface glycoprotein Ib and glycoprotein IIb-IIIa complex antigens per platelet. In patients with Bernard-Soulier syndrome and Glanzmann's thrombasthenia, all platelets were equally negative with regard to the glycoprotein Ib and glycoprotein IIb-IIIa complex phenotype, respectively. In contrast, each of six children with chronic myeloid leukemia (four of whom were Philadelphia chromosome negative and two of whom were Philadelphia chromosome positive) had two phenotypically distinct subpopulations of platelets: one glycoprotein Ib negative, the other glycoprotein Ib positive. In each of these six children with chronic myeloid leukemia, phenotypically distinct subpopulations of glycoprotein IIb-IIia complex-negative and glycoprotein IIb-IIIa complex-positive platelets were also detected. Polyclonal antiplatelet antibody bound to both the glycoprotein Ib-negative and glycoprotein IIb-IIIa complex-negative subpopulations. Age-matched controls (n = 3) and adults with Philadelphia chromosome-positive chronic myeloid leukemia (n = 3) showed single glycoprotein Ib-positive and glycoprotein IIb-IIIa complex-positive populations. In conclusion, flow cytometry detected distinct subpopulations of platelets in children with chronic myeloid leukemia. Flow cytometry is an important new method of identification and investigation of subpopulations of platelets in individual patients.
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PMID:Flow cytometric analysis of platelet surface glycoproteins: phenotypically distinct subpopulations of platelets in children with chronic myeloid leukemia. 347 97

A patient with an unusually prolonged course of Ph' positive chronic myeloid leukemia is presented. His disease was marked by cyclic leukocytosis, various chromosomal aberrations and secondary thrombasthenia. In vitro culture studies and granulocyte-macrophage colony stimulating factor (GM-CSF) production were consistent with responsiveness of the leukemic clone to GM-CSF. The possible relationship between the long survival and the feedback regulation of leukopoiesis is raised.
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PMID:Cyclic leukocytosis and long survival in chronic myeloid leukemia. 640 19

Several platelet abnormalities have been described in myeloproliferative diseases. The present study deals with 81 patients with polycythaemia vera, chronic myelogenous leukemia, essential thrombocythaemia and idiopathic myelofibrosis, and reports the analysis of the findings in platelet-induced aggregation. Platelet abnormalities induced by ADP, adrenaline and collagen were found in 41.9% of the patients. A defect of primary aggregation was documented in 13 cases and one of them showed an aggregation pattern similar to that of Glanzmann's disease. Fifteen patients had an impairment of secondary aggregation, and in one case of this group the platelet malondialdehyde and serotonin findings were consistent with a defect resembling that of typical congenital storage pool deficiency. A disturbance of the arachidonic acid pathways associated with a storage pool deficiency was found in a third patient belonging to a group with abnormalities of primary and secondary aggregation. In conclusion, platelets in myeloproliferative diseases have several defects and in a few cases their combination is similar to those of congenital diseases.
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PMID:Spectrum of platelet aggregation abnormalities in myeloproliferative diseases. 722 3

Chronic Myelogenous Leukemia (CML) is a myeloproliferative neoplasm characterized by proliferation of Philadelphia positive clonal pluripotent hematopoietic cells. Bleeding is a rare presentation of CML that can occur due to platelet dysfunction. Both pre-treatment and post-treatment platelet function abnormalities in CML have been described in the literature. We describe a rare case of childhood CML who presented with mucocutateous bleeding manifestations. On laboratory workup, a Glanzmann Thrombasthenia (GT) like platelet phenotype was demonstrated along with confirmation of diagnosis of CML in chronic phase. The acquired nature of platelet function defect was confirmed by demonstrating recovery of platelet antigens glycoprotein IIb/IIIa after achieving complete hematological response with Imatinib. Due to presenting complaint of bleeding diathesis and absence of hepatosplenomegaly, the case was undiagnosed for CML until the patient reported to us. Careful evaluation of complete blood counts, peripheral blood picture and detailed laboratory workup was the window to proper diagnosis and treatment in this case.
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PMID:Reversal of Glanzmann thrombasthenia platelet phenotype after imatinib treatment in a pediatric chronic myeloid leukemia patient. 2918 19

Chronic myelogenous leukemia (CML) is a clonal myeloproliferative neoplasm (MPN) characterized by dysregulated and uncontrolled proliferation of mature and maturing granulocytes with normal differentiation. A genetic hallmark of CML is the presence of the fusion gene product BCR-ABL. Bleeding diathesis in CML patients is rare (<10%) and primarily caused by acquired platelet dysfunction. We report a rare case of an adult CML chronic phase patient who presented with spontaneous muscle hematoma due to acquired Glanzmann's thrombasthenia (GT). On laboratory workup, a GT was confirmed along with the diagnosis of CML in chronic phase. The muscle hematoma was completely resolved following imatinib therapy. The present case demonstrates that bleeding is a complication of MPNs and highlights the importance of both acquired GT diagnosis to determine the cause of bleeding in CML and of prompt treatment with imatinib to reverse this condition.
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PMID:An Unusual Cause of Bleeding in a Patient with Chronic Myeloid Leukemia Chronic Phase. 3296 49