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)

We have studied the clinical courses of 69 patients with blastic crises of Philadelphia chromosome positive CML to identify parameters that were associated with an increased response rate or survival. Cytogenetic analysis at the time of blastic transformation revealed additional chromosome changes in 70% of the patients tested. Bone marrow fibrosis was detected in 58% of evaluable patients. Lymphoblastic transformation was seen in 28% of the patients tested with cell surface marker analysis. The value of 5'-nucleotidase as a marker for distinguishing lymphoid from non-lymphoid blast crisis was confirmed. Of 57 evaluable patients, 23 (40%) responded to therapy (CR/PR longer than 14 days). Median survival was 75 days. Longer survival was related to the following factors: Ph1-chromosome as the only detectable cytogenetic abnormality; lymphoblastic transformation; no bone marrow fibrosis; high percentage of blasts and promyelocytes in the bone marrow, and response to therapy. No prognostic significance was associated with age, sex, Tdt, LDH, spleen size, duration of the chronic phase of the disease, white blood cell count, Hb, platelet count and percentages of basophils, eosinophils, erythroblasts and blasts and promyelocytes in the peripheral blood. These data confirm the poor prognosis of patients with blastic crisis of CML treated by conventional chemotherapy.
 ...
PMID:Blast crisis of Philadelphia chromosome-positive chronic myelocytic leukemia (CML). Treatment results of 69 patients. 316 89

An IgG1 monoclonal antibody, 31D8, that recognizes normal neutrophil (PMN) membranes, was used to study PMN from patients with chronic myelogenous leukemia (CML). Nineteen patients with Philadelphia chromosome positive CML were followed over a ten-month period and compared with 23 normals, six patients with leukemoid reactions, and eight patients with phagocytic cell defects. The percentage of PMN binding of 31D8 among normal subjects was variable about a normal distribution with an average of 95 +/- 2% of cells binding 31D8. In contrast, there were two groups of CML patients: in 14 patients 88 +/- 3% PMN bound 31D8 while in the remaining five patients only 6 +/- 6% PMN bound 31D8. PMN 31D8 binding was normal in the control patient groups. Control antibodies 7C3 (binds to PMN precursors) and OKM1 (binds to the CR3 (iC3b) receptor) bound normally to CML neutrophils. Functionally, CML cells had normal chemotaxis to several stimuli and normal superoxide generation to phorbol myristate acetate. However, superoxide production in response to fmet-leu-phe was significantly less in 31D8 negative CML PMN than both 31D8 positive CML PMN and normal PMN which contained 85% 31D8 positive and 15% 31D8 negative PMN. Clinically, 2 of 14 CML patients with 31D8 positive PMN were in blast crisis (one extramedullary) at the time of study and the other 12 patients remained clinically stable in the chronic phase during the ten months of study. In contrast, one of five patients with 31D8 negative PMN was in blast crisis at the time of study and all four of the remaining patients progressed to either the accelerated phase or blast crisis. Three of these patients died of their disease eight to ten months after their initial study. Thus, failure of CML cells to bind 31D8 may be useful for predicting which patients are likely to progress to the accelerated phase or blast crisis.
 ...
PMID:A neutrophil membrane marker reveals two groups of chronic myelogenous leukemia and its absence may be a marker of disease progression. 346 Jun 45

A 27-yr-old man developed blastic crisis after the chronic phase of Philadelphia chromosome positive chronic myeloid leukemia (CML). The blast cells expressed terminal deoxynucleotidyl transferase (TdT)+/common acute lymphoblastic leukemia antigen (CALLA)+ phenotypes, corresponding to common ALL type. A vincristine plus prednisolone regimen initially suppressed the blastic proliferation, but the blasts soon reappeared as lymphoblasts, and 65% of them possessed basophil-like granules. Immunologic markers were not altered. The blasts were negative for myeloperoxidase, Sudan black B and periodic acid-Schiff reactions, but were positive for toluidine blue (TB) stain and supravital peroxidase (PO) stain using diaminobenzidine (DAB). These blasts were considered to have immature basophil granules. The supravital staining, for TB or PO in combination with fluorescinated-CALLA staining, directly revealed that single blasts expressed both basophil and lymphoid markers. This biphenotypic blast population was found to be a distinct clone from the initial crisis clone by cytogenetic examination. These findings suggest that the CML clone is derived from a multipotent stem cell common to lymphoid and myeloid lineages, or that dual markers may be expressed on transformed lymphoid or basophil clone as the result of differentiation infidelity probably determined by the genetic derangement in acute crisis.
 ...
PMID:Dual expression of lymphoid/basophil markers on single blast cells transformed from chronic myeloid leukemia. 346 36

The Philadelphia chromosome with the classic translocation t(9;22) was detected in all metaphases of a patient with essential thrombocythemia who presented without hematologic signs of chronic myeloid leukemia (CML). After 12 months of treatment with hydroxyurea followed by 6 months of busulfan, the disease transformed into lymphoid blast crisis with the immunophenotype of pre-pre-B cells frequently seen in lymphoid blast crisis of CML. This progression was not associated with karyotypic evolution because lymphoblasts contained only the Philadelphia chromosome. Further clinical course in this patient confirmed the development of a Philadelphia chromosome positive stem cell with predominantly megakaryocytic committment through partially megakaryocytic/lymphoid differentiation to purely lymphoid committment, a previously unreported transformation.
 ...
PMID:Philadelphia chromosome positive essential thrombocythemia evolving into lymphoid blast crisis. 347 Jan 13

Twenty-eight adult patients with Philadelphia chromosome positive (Ph+) acute leukemia were studied to determine if additional chromosomal changes were related to specific morphologic and clinical features. Twenty patients had chronic myeloid leukemia in blast crisis (CML-BC), three had Ph+ de novo acute nonlymphocytic leukemia (ANLL), and five had de novo acute lymphoblastic leukemia (ALL). Chromosomal abnormalities in addition to a single Ph were noted in 90% of patients with CML-BC and included a second Ph (five patients), +8 or duplication of part of 8q (five patients), dicentric isochromosome 17 (two patients), and +19 (two patients). Octaploidy with 4 Ph was seen in one patient with megakaryoblastic transformation. One of two patients with a progranulocytic blast crisis had a t(15;17) abnormality. Hypodiploidy was noted in 4 of 20 patients with CML-BC. Each of the four patients had prominent extramedullary manifestations of blast crisis. All had received intensive chemotherapy prior to the detection of hypodiploidy, and the cytogenetic findings were similar to those often seen in patients with therapy-related leukemia. An inv(3)(q21q26) was noted in two patients (one CML-BC, one de novo Ph+ ANLL), one of whom had hypolobulated micromegakaryocytes. Additional cytogenetic abnormalities in de novo Ph+ ANLL (especially +19) were similar to those in CML-BC. In contrast, the additional karyotypic changes in de novo Ph+ ALL (eg, +4, -7, -20, markers) were those commonly seen in ALL without a Ph and were generally different from those seen in CML-BC.
 ...
PMID:Philadelphia chromosome-positive acute leukemia: morphologic and clinical correlations. 347 91

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.
 ...
PMID:Flow cytometric analysis of platelet surface glycoproteins: phenotypically distinct subpopulations of platelets in children with chronic myeloid leukemia. 347 97

A 20-year-old male with chronic myelogenous leukemia (CML) and severe bone marrow fibrosis underwent splenectomy, then ablative chemotherapy, followed by bone marrow transplantation from a histocompatible sister. The myelofibrosis completely resolved. Prompt marrow engraftment and disappearance of Philadelphia chromosome positive cells were documented. Therefore, the presence of marrow fibrosis which frequently accompanies CML is rapidly reversible following high dose chemotherapy and is not indicative of a hostile microenvironment which could preclude marrow transplantation for patients with CML and myelofibrosis.
 ...
PMID:The reversal of myelofibrosis associated with chronic myelogenous leukemia after allogeneic bone marrow transplantation. 635 32

The Philadelphia chromosome is rarely observed in acute myelogenous and acute lymphoblastic leukemias. There are only a few case reports about pediatric patients, the prognosis of whom seems to be extremely poor. Reviewing the case of a girl aged 18 months with Philadelphia chromosome positive acute lymphoblastic leukemia, the differences between this entity and blast crisis of chronic myelogenous leukemia are described. This subgroup of Philadelphia chromosome positive acute lymphoblastic leukemia may represent a new risk group.
 ...
PMID:[Philadelphia chromosome-positive acute lymphoblastic leukemia in childhood: a special risk group?]. 657 79

Of 597 cases of acute leukaemia in adults (greater than 16 years) seen at St. Bartholomew's Hospital, London, between May 1973 and January 1982, 412 were diagnosed as AML, 103 as ALL and 58 as Philadelphia chromosome positive blast crisis of CML (13 presenting as acute leukaemia and 45 having a prior chronic phase). The remaining 24 cases were considered to be acute undifferentiated leukaemia. Twenty-one of the latter were investigated using a panel of immunological markers at diagnosis and/or retrospectively using frozen cell suspensions. Eighteen out of 21 were shown to have a predominantly 'lymphoid' phenotype which comprised 12 cases of common ALL (two of whom were Ph1 positive), three cases of null-ALL, one case with a probable early thymic phenotype, and two cases with a monoclonal B lymphoblast phenotype. One 'common ALL' and one 'null-ALL' had a significant proportion of pre-B (cytoplasmic mu chain+) cells. One other case reacted with anti-myeloid sera. Leukaemic blasts from two patients were unreactive with all markers tested. No cases of glycophorin positive erythroleukaemia or anti-platelet (glycoprotein I) positive leukaemia were detected. These observations suggest that the overwhelming majority of acute leukaemias have an identifiable affiliation to the lymphoid or myeloid lineages and that patients diagnosed haematologically as 'AUL' might benefit by therapy appropriate for their leukaemic cell type.
 ...
PMID:ALL masquerading as AUL. 658 40

A patient with Philadelphia chromosome positive CML was studied to characterize the blast transformation. Peripheral blood and bone marrow at the onset of the blast crisis were evaluated with cytochemistry, chromosome analysis, cell surface markers, terminal transferase assay, and electron microscopy. Although light microscopic examination and cytochemistry suggested lymphoblastic morphology, ultrastructural cytochemistry demonstrated the myelomonocytic features of the transformation. This study suggests that electron microscopic cytochemistry is useful in evaluating the heterogeneous nature of the blast phase of CML.
 ...
PMID:Electron microscope cytochemical analysis of chronic myelocytic leukemia: a case report. 693 71


<< Previous 1 2 3 4 5 6 7 8 9 10 Next >>