Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0029713 (immaturity)
 4,335 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Basophilia is found in association with many diseases and is commonly seen in patients with chronic myelocytic leukemia (CML). Substantial basophilia development during the course of CML has been considered to be a poor prognostic sign. Prominent basophilia occasionally has been seen in patients at the time of the original diagnosis. These cases have been classified as "basophilic leukemia." A patient with a basophil count of 24,080/cu mm and myeloid immaturity in his peripheral blood had typical Ph1 chromosome in his bone marrow. G-banding studies showed typical 22q-;t(9q+;22q-) of CML. Since seven of the nine basophilic leukemias so far studied cytogenetically have Ph1 chromosomes and since Ph1 chromosomes are absent in the majority of eosinophilic leukemias, we believe it more appropriate to call basophilic leukemia basophilic CML.
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PMID:Cytogenetic studies in basophilic chronic myelocytic leukemia. 28 15

The incorporation of 3H-leucine as an indicator of protein synthesis has been studied of blast cells in acute leukaemia and of proliferative normal bone marrow cells. The results demonstrate a significantly higher 3H-leucine incorporation into normal immature cells as compared to leukaemic myeloblasts, also 3H-leucine labelling of lymphoblasts was significantly lower than both the values found in acute leukaemia and normals. Higher protein synthesis was found correlated to the degree of cell immaturity. No significant correlation between 3H-leucine labelled and 3H-thymidine labelled leukaemic blast cells was achieved. A marked decrease in 3H-leucine incorporation by puromycin was demonstrated in three patients with acute myeloid leukaemia.
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PMID:Leucine incorporation into leukaemic blast cells. 29 96

Moderately high levels of activity of the enzyme terminal deoxyribonucleotidyl transferase (TdT) were found in the leukemic cells of a patient with acute lymphyocytic leukemia. The proliferating cells were B lymphocytes bearing IgG antibody, and the disease was associated with an IgG monoclonal spike and a mediastinal mass. The observations in this case suggest that TdT is related more to the immaturity and proliferation of certain lymphoid stem cells than to their progress toward B- or T-cell differentiation.
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PMID:Terminal deoxyribonucleotidyl transferase activity in B-cell acute lymphocytic leukemia. 30 65

Natural or experimental feline immunodeficiency virus (FIV) infection in cats is often associated with hematologic abnormalities which are similar to those observed in human immunodeficiency virus (HIV) infected patients. To determine if cells in bone marrow are infected with FIV and whether severity of hematopoietic disorder is correlated with the level of viral infection, bone marrow tissues from ten experimentally and two naturally FIV infected cats were examined by in situ hybridization for presence of FIV RNA. Seven of the 12 FIV infected cats were also naturally or experimentally coinfected with feline leukemia virus (FeLV). FIV RNA was detected mainly in megakaryocytes and unidentified mononuclear cells in the bone marrow of cats that were sick and had marrow hypercellularity and immaturity. These included all cats in the acute phase of FIV infection and two of seven long term FIV infected cats. One long term FIV infected cat with lymphosarcoma was also positive for FIV RNA in bone marrow cells. The other four long term FIV infected cats were relatively healthy, with normal bone marrow morphology, and were negative for FIV infected cells. Bone marrow from three non-infected and two cats infected with FeLV alone were also negative for FIV RNA by in situ hybridization. We concluded that megakaryocytes and mononuclear cells were targets of the viral infection and that the presence of FIV RNA in cells of the bone marrow correlated with marrow hypercellularity and immaturity, and severity of illness.
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PMID:Detection of feline immunodeficiency virus infection in bone marrow of cats. 133 1

A case of non-secretory multiple myeloma presenting as primary plasma cell leukaemia in a 65 year old woman is presented. Bone pain was the initial clinical manifestation. Laboratory analysis showed 20% of circulating immature plasma cells. Despite the presence of osteolytic lesions, no M-component could be demonstrated in serum protein electrophoresis, and serum and urine immunoelectrophoresis. Bone marrow aspirate demonstrated an 83% infiltration of plasma cells showing various degrees of immaturity. Immunofluorescence with monoclonal antisera demonstrated intracytoplasmic kappa light chains in a high percentage of plasma cells. Immature plasma cells without cellular capacity to synthesize and excrete complete immunoglobulins could be more aggressive, leading to an initial leukaemic process. Previous work regarding possible pathogenetic mechanisms, clinical and laboratory features, and response to treatment of this extremely rare association are reviewed.
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PMID:Non-secretory multiple myeloma presenting as primary plasma cell leukaemia. 143 33

During the diagnostic investigation of 750 acute leukemias, nine cases were morphologically, cytochemically, and phenotypically undifferentiated. In seven of these cases the blasts were class II+, CD34+ and TdT+, in one were class II+, TdT+, CD7+ while in the remaining leukemia blasts expressed class II only. Cytoplasmic and membrane CD22, CD3, CD13, and Ig as well as membrane CD19, CD10, CD37, CD2, CD33, CD14, glycophorin C, and CD61 were absent. The further characterization of these rare leukemias yielded the following results. The TCR-beta, -gamma and -delta genes were in germline configuration in seven cases studied while IgH genes were rearranged on both alleles in two cases and germline in the other five. By ultrastructural analysis peroxidase activity was detected on unfixed cells in a minority of blasts from four of seven cases. In two of the peroxidase-positive cases a small proportion of blasts also reacted with an anti-myeloperoxidase monoclonal antibody. In one of the peroxidase-negative cases, 7% of blasts were labeled by the antibody, suggesting the presence of peroxidase in its proenzyme form. Importantly, the two cases with Ig gene rearrangements did not have cytochemically or immunologically detectable peroxidase. Three of the nine patients were treated as ALL while six received AML chemotherapy. In five patients complete remission was achieved while the other four died from infections during remission induction. Four patients are still in remission 7, 12, 24, and 30 months after diagnosis while one patient relapsed after 12 months. In conclusion, we have characterized the genotypic and ultrastructural features of subtype of acute leukemia in which blasts expressed immaturity markers and lacked lineage associated antigens. In contrast to previously reported "unclassifiable" cases, the leukemias were phenotypically homogeneous and showed a good response to chemotherapy.
Leukemia 1990 Sep
PMID:Phenotypic, genotypic, cytochemical, and ultrastructural characterization of acute undifferentiated leukemia. 239 82

Lymphocyte subpopulations in human cord blood have been examined using monoclonal antibodies, visualized with immunogold. The proportions of T11, T4, T8, and B1 cells in cord blood are very similar to values in adult peripheral blood. Some evidence of lymphocyte immaturity in cord blood is suggested by the presence of 12% CALLA-positive cells and the sum of T4 and T8 cells significantly exceeding the number of T11 cells; however, there were no TdT-positive cells. The presence of CALLA-positive lymphocytes in normal cord blood should be borne in mind when investigating blood smears from neonates for congenital leukemia.
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PMID:Characterization of lymphocyte subpopulations in human cord blood using the immunogold staining technique. 244 85

The syndrome of cholestatic jaundice in association with urinary tract infection with normal or slightly elevated liver enzymes has been reported mainly in newborns and infants below 2 months of age. The relative immaturity of the infant's liver and its sensitivity to bacterial endotoxins may explain the occurrence of this syndrome in this age group. A similar syndrome has been reported in adults with severe non-hepatic bacterial infection, including some with urinary tract infection. However, only three case reports in older children could be found. In this report, the case of a 4-year-old girl with acute lymphoblastic leukaemia, who presented with cholestatic jaundice with normal liver enzymes and urinary tract infection, is described. Treatment with antibiotics resulted in resolution of the jaundice and potentially hepatotoxic drugs were used for the treatment of leukaemia with no problems. Two adult patients with acute leukaemia, non-hepatic bacterial infection and cholestatic jaundice have been reported; both died shortly after diagnosis.
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PMID:Cholestatic jaundice with urinary tract infection in a child with acute lymphoblastic leukaemia. 246 Nov 52

To focus attention on the problem of infant mortality in Lebanon, data were compiled on infant mortality from 1978 to 1986 at the American University of Beirut Medical Center. Causes of death are analyzed for 602 males and 398 females. 54.9% deaths occurred at 1 month of age and 77.4% died within the 1st year. Autopsies were performed on .7%. 37.7% of all neonatal deaths were due to neonatal diseases such as hyaline membrane disease, asphyxia neonatorum, immaturity, necrotizing enterocolitis, hemorrhage, hemolysis, meconium aspiration, and kernicterus. Better prenatal care would reduce this group, or the administration of corticosteroids to the mother 24-48 hours prior to delivery, as well as rapid resuscitation at birth and prevention of the 5 curses: hypoxemia, hypoglycemia, hypothermia, hypotension, and acidosis. Although unavailable in Lebanon, administration of surfactants through an endotracheal tube would also help. Infections constitute 25.1% of deaths; many are preventable through adequate public health measures and strict personal hygiene, i.e., diseases such as sepsis, pneumonia, meningitis, gastroenteritis, hepatitis, encephalitis, and 1-2 cases of the following: diphtheria, measles, peritonitis, tetanus, tuberculosis, cytomegalis inclusion, herpes, parathyphoid, pertussis, poliomyelitis, and shigellosis. Congenital diseases were 21.6%. In utero diagnosis could prevent some diseases and in utero treatment is possible for hydrocephalus and hydronephrosis. Screening programs postnatally could lead to treatment. 5.9% were malignancies such as leukemia, lymphoma, brain tumors, histocytosis, Wilm's tumor, Ewing sarcoma, and Hodgkin's disease. Early diagnosis is critical if mortality is to be reduced in this group, but medical advances are still needed. 2.9% are miscellaneous diseases such as poisoning, rheumatic diseases, marasmus, Reye's syndrome, nephrosis, rickets, and epilepsy. Most of these diseases are preventable, except for rheumatic inflammation of the heart. Recommended necessary steps to reduce infant mortality are: prenatal care, diagnosis and screening, intrauterine surgery; resuscitation and intensive care centers with modern equipment and trained personnel; national vaccination and screening programs; adequate public health measures and hygiene; parental education; and well-equipped hospitals to serve all regardless of income level.
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PMID:Pediatric mortality: an avoidable tragedy. 251 28

Dipeptidyl peptidase IV (DPIV), a T cell enzyme, has been implicated in the regulation of lymphocyte proliferation in response to lectins and allogeneic cells. A sensitive fluorimetric assay has been established for the enzyme and used to investigate DPIV activity, kinetics and the subcellular localization in lymphocytes from control subjects, cord blood and patients with common variable hypogammaglobulinaemia (CVH) and chronic lymphatic leukaemia (CLL). Using sucrose density gradient centrifugation and organelle marker enzyme assays, in conjunction with digitonin as a selective plasma membrane perturbant and diazotized sulphanilic acid as a non-permeant enzyme inhibitor, DPIV was shown to be a plasma membrane ecto-enzyme. A significant decrease in lymphocyte DPIV activity was observed in cord blood and in patients with CVH and CLL compared to controls. Kinetic analysis showed a marked decrease in the Vmax of lymphocyte DPIV from cord blood and patients with CVH and CLL compared to controls. The apparent Km for the substrate was unaffected in cord blood and patients with CLL. However, in patients with CVH the Km was significantly reduced. Various enzyme inhibitors showed no differences between control subjects and CVH lymphocyte activities. The decreased Km for DPIV provides further evidence for a stem cell defect rather than cell immaturity in CVH.
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PMID:Dipeptidyl peptidase IV--subcellular localization, activity and kinetics in lymphocytes from control subjects, immunodeficient patients and cord blood. 290 80


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