Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: EC:3.2.1.17 (lysozyme)
 21,489 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Retrospective analysis of 23 cases of chronic myelomonocytic leukemia (CMML) was presented. Clinical findings, peripheral blood and bone marrow indices, ultrastructural, cytochemical, biochemical (the level of serum and urine lysozyme), cytogenetic investigations as well as the type of leukemic cell growth in culture (monolayer) were considered. Proceeding from the above analysis, the authors found it appropriate to attribute CMML to myelodysplasia.
 ...
PMID:[Chronic myelomonocytic leukemia]. 323 62

We report 60 cases of chronic and subacute myelomonocytic leukaemias (CMML and SMML) in the adult, using the FAB group criteria. The M/F sex ratio was 3.3 and the mean age 67.5 years. Splenomegaly was found in 32% of cases, hyperleucocytosis in 52% of cases and mean blood monocytosis was 4.3 X 10(9)/l. Marrow smears showed an excess of blasts in 57% of patients, a moderate increase in monocytes in most cases and frequent myelodysplastic features. An increase in serum lysozyme and polyclonal hypergammaglobulinaemia were usual and clonal cytogenetic anomalies found in about half of the patients tested. Treatment was usually palliative and the median survival was 28 months, a blastic transformation being responsible for a third of the deaths. Prognostic factors at diagnosis were analysed retrospectively in the 46 patients who had sufficient follow up. Percentage of marrow blasts haemoglobin level and blood monocytosis at diagnosis, were subject to multivariate analysis, resulting in a discriminant 'score'. This allowed assignment of each patient into one of two prognostic subgroups (10.9% probability of error): a poor prognosis one, with a life expectancy of less than 1 year and a high risk of acute transformation (subgroup termed SMML) and a better prognosis subgroup (termed CMML), with some CMML patients surviving over 5 years.
 ...
PMID:Chronic and subacute myelomonocytic leukaemia in the adult: a report of 60 cases with special reference to prognostic factors. 346 95

A 15-year-old boy with lymphadenopathy and marked leukocytosis was evaluated. At 2 years old, he was diagnosed to have malignant testicular carcinoma for which radiotherapy and cytotoxic drugs were administered. On admission, the peripheral blood showed an increase of all stages of the granulocytic cell line and atypical monocytoid cells. Cervical lymph node biopsy showed infiltration by immature granulocytic cells. Eleven percent of the peripheral leukocytes was positive for combined staining by alpha-naphthyl butyrate esterase and naphthol-ASD-chloroacetate esterase activity. The leukocyte alkaline phosphatase score was low. Serum and urinary lysozyme levels were markedly elevated. Philadelphia chromosome was not detected. Although chronic myelomonocytic leukemia (CMML) has been described in elderly patients, the findings in this pediatric patient were most compatible with a diagnosis of CMML.
 ...
PMID:Chronic myelomonocytic leukemia in a 15-year-old boy. 347 43

Chronic myelomonocytic leukemia (CMML) is a rare leukemia, which is now included in myelodysplastic syndromes. In a small number of patients with CMML, problems in the diagnosis have been reported, especially when atypical morphological features in both monocytic and granulocytic cells due to dysmyelopoiesis are prominent, or when cytochemical characteristics are lost in the leukemic cells. The case history of a sixty-seven year-old male patient with CMML is described. The diagnosis of CMML in the patient was supported by the following evidence: chronic course of his disease; increased monocyte-like cells without other cause; normocytic anemia; immature granulocytic cells with hypogranular feature and giant platelets were observed in the peripheral blood. The bone marrow showed myeloid hyperplasia. Serum muramidase and vitamin B12 levels were increased, while neutrophil alkaline phosphatase score was low in the peripheral blood. Ph' chromosome was negative. The monocyte-like cells completely lacked nonspecific esterase. However the cells were confirmed as monocytic cells by flow cytometry using monoclonal antibodies to monocytes (OKM5).
 ...
PMID:Report of a case with chronic myelomonocytic leukemia: demonstration of leukemic monocytes lacking nonspecific esterase by flow cytometry using monoclonal antibodies. 350 52

A retrospective analysis of 30 patients with chronic myelomonocytic leukemia (CrMML) was performed to define the natural history of the disease and the risk of acute transformation. Our patients fulfilled the following criteria of diagnosis: blood monocytosis over 1 X 10(9)/l, blast cell percentage in bone marrow up to 30, and in peripheral blood less than 5. The most common presenting feature was anemia; seven patients had fever; three patients complained of purpura and bleeding. Anysopoikilocytosis and macrocytosis were frequent. Abnormal granulocyte morphology, defective granulation and abnormal leukocyte alkaline phosphatase were often observed. Blast cells in peripheral blood smears were found in 14 patients. Serum and urine lysozyme levels were increased in 82 per cent and 93 per cent, respectively. Dysplastic changes involving erythroid, granulocytic and megakaryocytic lineages were constant features in all cases. Agranulated blasts above 5 per cent of marrow nucleated cells were seen in 13 patients (43 per cent). Seven of the 20 patients showed non-specific chromosomal abnormalities at diagnosis. Median survival from diagnosis was 18 months (range, 3-112). Evolution into acute myeloid leukemia occurred in 11 patients. No difference in survival was found between patients who developed acute leukemia and patients who did not. A shorter survival has correlated to the following parameters: leukocytes greater than 10 X 10(9)/l, the presence of blasts in peripheral blood and agranulated blasts in the marrow above 5 per cent.
 ...
PMID:Chronic myelomonocytic leukemia: clinical features, cytogenetics, and prognosis in 30 consecutive cases. 386 Apr 66

Thirty-five patients who fulfilled the FAB diagnosis criteria of chronic myelomonocytic leukemia (CMML), i.e., myelodysplastic features, monocytosis over 10(9)/liter, bone marrow monocyte infiltration, blast cells less than 5% in the peripheral blood and less than 30% in the bone marrow, are analyzed. CMML appears as an entity distinct from myelodysplastic and myeloproliferative disorders. Splenomegaly, anemia, thrombocytopenia, leukocytosis with monocytes and granulocytic cells in all stages of development, increased blood and urine lysozyme levels without renal failure, and polyclonal hyperimmunoglobulinemia are its main clinical and biologic features. With conventional cytotoxic drugs (6-mercaptopurine, hydroxyurea), the prognosis of CMML appears poor (median survival 475 days). None of the clinical hematologic or biologic parameters tested had a significant effect on prognosis. As other chemotherapy trials seemed necessary, we recently administered small doses of cytosine-arabinoside (ARA-C) to six patients over several consecutive days and obtained a complete remission in four. These preliminary results must be confirmed by larger series using the diagnostic criteria proposed by the FAB cooperative group.
 ...
PMID:Chronic myelomonocytic leukemia according to FAB classification: analysis of 35 cases. 658 39

A four-year-old child with recurrent infections and increasing hepatosplenomegaly over a three-year period was evaluated. Increased numbers of myeloid precursors packed the bone marrow and infiltrated the peripheral blood. A diagnosis of chronic myelogenous leukemia (CML) was considered but could not be confirmed by laboratory studies appropriate for the types of CML usually observed in childhood. Examination of the patient's peripheral blood smears revealed many atypical monocytoid cells with unipolar hairy projections. Scanning electron microscopy showed these to be leukemic monoblasts with characteristic broad-based ruffles on the cell surface. A population of myeloid precursors possessing narrow ridge-like profiles was also observed. Progressive infiltration of the spleen caused hypersplenism which necessitated splenectomy. Subsequently, massive liver and bone marrow involvement led to the patient's death. Terminally, the proliferating blast cells were demonstrated to be leukemic monoblasts by analysis of cytochemical staining patterns, surface immunoglobulins, serum lysozyme levels, and monocyte-mediated antibody-dependent cellular cytotoxicity studies. The findings in this case are most compatible with a diagnosis of chronic myelomonocytic leukemia (CMML), a condition not previously described in childhood. Several myeloproliferative disorders with prolonged survival have been reported in children, but special studies were not performed to determine which cell lines were abnormally proliferating. The similarities between these children and our patient with CMML suggest that monocyte studies may be useful in the diagnosis of these unusual disorders, provide insights into their pathogenesis, and aid in the selection of appropriate therapy.
 ...
PMID:Chronic myelomonocytic leukemia in childhood. 694 Apr 41

The clinical and laboratory features of 37 patients with variants of acute monocytic leukemia are described. Three of these 37 patients who had extensive extramedullary leukemic tissue infiltration are examples of true histiocytic "lymphomas." Three additional patients with undifferentiated leukemias, one patient with refractory anemia with excess of blasts, one patient with chronic myelomonocytic leukemia, one patient with B-lymphocyte diffuse "histiocytic" lymphoma and one patient with "null" cell, terminal deoxynucleotidyl transferase-positive lymphoblastic lymphoma had bone marrow cells with monocytic features. Another patient had dual populations of lymphoid and monocytoid leukemic cells. The true monocytic leukemias, acute monocytic leukemia (AMOL) and acute myelomonocytic leukemia (AMMOL), are closely related to acute myelocytic leukemia (AML) morphologically and by their response to chemotherapy. like AML, the leukemic cells from the AMMOL and AMOL patients form leukemic clusters in semisolid media. Cytochemical staining of leukemic cells for nonspecific esterases, presence of Fc receptor on the cell surface, phagocytic ability, low TdT activity, presence of surface "ruffles" and "ridges" on scanning EM, elevations of serum lysozyme, and clinical manifestations of leukemic tissue infiltration are features which accompanied monocytic differentiation in these cases.
 ...
PMID:The acute monocytic leukemias: multidisciplinary studies in 45 patients. 700 98

A systematic morphological analysis of cutaneous infiltrates in acute myelogenous leukemia and myelodysplastic syndrome revealed that in many cases the infiltrating cells have a different phenotype from those in the bone marrow. This study sought to answer two questions: (a) How wide is the range of cytological features and immunoreactivity of the cutaneous infiltrates and what danger is there of misinterpretation? (b) What are the possible causes of the wide spectrum of differentiation of the cells infiltrating the skin? Skin biopsy specimens from 16 patients with myelogenous leukemia or myelodysplastic syndrome were investigated. The diagnosis was acute myelomonocytic leukemia (M4, according to the French-American-British/FAB system of classification of acute leukemias) in eight cases, acute monocytic leukemia (M5) in four cases, aleukemic leukemia cutis as a recurrence of M2 leukemia after treatment in one case, and myelodysplastic syndrome in three cases, including one case of myelodysplasia with an excess of bone marrow blasts (RAEB-T) and two cases of chronic myelomonocytic leukemia, one of which presented as aleukemic leukemia cutis. Reactivity with the macrophage-associated antibodies anti-CD68, Ki-M1p, and anti-lysozyme was the most consistent. However, the naphthol AS-D chloroacetate esterase reaction and staining with DAKO-M1, Ki-My2p, anti-neutrophil elastase, and anti-CD34 were found to be of little value for identifying the cutaneous infiltrate as myelogenous. Some antibodies (e.g., anti-S100 protein and MB2) even produced staining in a few cases that could have led to a mistaken diagnosis of histiocytic neoplasm or malignant lymphoma.(ABSTRACT TRUNCATED AT 250 WORDS)
 ...
PMID:Skin involvement in myelogenous leukemia: morphologic and immunophenotypic heterogeneity of skin infiltrates. 754 88

Myelodysplastic syndromes (MDS) are stem cell disorders of clonal origin in which infections and leukemic transformation are quite frequent. Neutrophils from 28 patients with MDS were analysed by flow cytometry for the expression of the two complement receptors CR1 and CR3, the antigenic reactivity of some granule constituents--myeloperoxidase, lysozyme, elastase, lactoferrin--and functional activities, such as locomotion, respiratory burst and cytotoxicity. The results were correlated with the FAB disease subtypes, grouped as low risk (RA) and high risk patients (RAEB, RAEB-t, CMML) and with 30 healthy subjects. A significant reduction in the percentage of neutrophil CR1, CR3 positivity and chemotaxis induced by endotoxin-activated serum was detected in the high risk group when compared with the low risk group and healthy controls. Furthermore, the high risk group also showed a low amount of myeloperoxidase, elastase, lysozyme and superoxide anion, but both low and high risk groups displayed reduced cellular cytotoxicity in comparison with the control. This work indicates that MDS patients belonging to the more advanced FAB categories frequently show multiple abnormalities in the expression of neutrophil complement receptors, and granular components (> 3), as well as in cell functions, suggesting the possibility of using these phenotypic abnormalities in the monitoring of disease progression.
 ...
PMID:Neutrophils from patients with myelodysplastic syndromes: relationship between impairment of granular contents, complement receptors, functional activities and disease status. 806 92


<< Previous 1 2 3 Next >>