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)

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

Antibody responses were measured in colostrum-fed (CF) and colostrum-deprived (CD) calves immunised at ages varying from birth to four months using a variety of antigens with and without adjuvant. In addition immunoglobulin production in CD calves over the same period was compared with that of CF calves. A marked unresponsiveness to antigens injected at birth was observed if maternal antibody specific for the antigen used was present in the circulation. However, significant responses to all antigens tested occurred in CD calves and also in CF calves immunised with an antigen (egg albumin) to which there was no maternal antibody. Since these responses were not as great as those in older calves immunised similarly it is clear that age was influencing the response. It was also found that subsequent responsiveness was not significantly enhanced or impaired by neonatal exposure to antigen. With respect to immunoglobulin production the results conclusively demonstrated that endogenous production occurred much earlier in CD calves than CF calves and even after 128 days the serum concentrations of IgG1 and IgA in CD calves exceeded those for CF calves. Thus it appears that the most important consideration in immunological responsiveness of neonates is not so much immaturity of the lymphoid system as the effects of maternal antibody on the cells in that system.
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PMID:Antibody responses to neonatal immunisation in calves. 80 67

Among the malignant lymphomas of the diffuse, poorly differentiated lymphocytic type, a cytologically distinctive form can be recognized. It is composed of immature lymphoid cells that are indistinguishable from the cells of acute lymphoblastic leukemia (ALL). Although these neoplasms usually have been classified as malignant lymphoma, lymphoblastic type, they contain, in addition to lymphoblasts, prolymphocytes in varying proportions. On the basis of the nuclear morphology, malignant lymphoma of the lymphoblastic type, (MLLB) can be further divided into those with and those without convoluted nuclei. In our series both groups had the following clinical features in common: 1) frequent occurrence in children and adolescents; 2) clinical presentation with mediastinal masses in 50% of cases; 3) a high incidence of bone marrow and perpheral blood involvement during the course of the disease; and 4) rapid progression of the disease with a median survival of 8 months. Our observations indicate that nuclear convolutions are helpful but not essential for the recognition of a clinicopathologic entity which is histologically and cytologically characterized by 1) the immaturity of the lymphoid cells indistinguishable from the lymphoblasts and prolymphocytes of ALL and 2) a high mitotic index. Because of the frequency with which MLLB progresses into ALL, systemic therapy may be indicated even before this progression is hematologically evident. This indicates the need for morphologic recognition of this malignant lymphoma regardless of the presence of nuclear convolution, age of the patient, and site of presentation.
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PMID:Malignant lymphoma, lymphoblastic. 106 94

Bone marrow transplant recipients are known to be immunodeficient in cellular and humoral immune responses for months to years. Mechanisms underlying the post-transplant immunodeficiency are mostly unknown and therefore it has been difficult to design therapeutic strategies to address this problem. Here, we review studies of post-transplant B cell (humoral) immunity including B cell blood counts, phenotype, function and origin, and the morphology of lymphoid organs. We postulate that the aetiology of post-transplant humoral immunodeficiency is multifactorial, involving: B cell immaturity due to recapitulation of their ontogenesis, and, in some patients, insufficient T cell help and/or exaggerated CD8+ T cell/NK cell suppression. Implications for clinical practice are outlined, e.g. hypersensitivity reactions and autoimmune diseases as part of the differential diagnosis of post-transplant disorders, questionable diagnostic benefit of serologic studies, and usefulness of prophylactic intravenous immunoglobulin.
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PMID:Reconstitution of B cell immunity following bone marrow transplantation. 162 22

It has been shown that human umbilical cord blood contains stem/progenitor cells comparable in number to that of adult bone marrow. We report here the first successful cases of transplantation of umbilical cord blood cells. The patients were suffering from Fanconi's anemia, complicated by severe aplastic anemia. During pregnancy, it was shown that the mother was carrying a sibling unaffected by the disease and with HLA identical to the patient. Cord blood was collected and frozen in liquid nitrogen at birth. After conditioning with low-dose cyclophosphamide (20 mg/kg) and thoraco-abdominal irradiation (5 grays), the patients received a cord blood transplant of thawed cells. Three patients have been transplanted without any immediate side-effect. One has not enough follow-up, but two patients are alive and well with complete donor hematologic reconstitution and no chronic graft versus host disease. Potential developments of this technique are an extension of applicability with regard to other diseases that might be transplanted and whether such transplants can be performed in adults. The relative immaturity of the lymphoid system at birth may be advantageous in decreasing the graft versus host reaction if these cells are used in a mismatched transplantation. Cord blood cell banks may be useful for transplants in patients lacking an HLA-identical donor.
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PMID:Transplantation of umbilical cord blood in Fanconi's anemia. 198 24

Two-colour immunofluorescence staining for intracellular J chain and IgA (or J chain and IgG) was performed on tissue sections of normal human ileal mucosa (eight adult kidney donors), mesenteric lymph nodes (MLN), peripheral lymph nodes, and palatine tonsils. The most prominent J chain positivity was seen for IgA (97.3%) and IgG (81.7%) immunocytes in the ileal lamina propria (LP). Moreover, the proportion of J chain-expressing extrafollicular immunocytes was significantly higher (P less than 0.05) in MLN than in peripheral lymph nodes for the IgA class (58.5% versus 25.6%); the same proportion for the IgG class was 45.9% versus 30.4%. In clinically normal palatine tonsils of adults, extrafollicular J chain expression was much lower than in peripheral lymph nodes; 14.2% for IgA cells and 5.5% for IgG cells. When related to subclass production, J chain expression was found to be higher for IgA2 than for IgA1 cells in all tissues examined (palatine tonsils excluded because of a small number of IgA2 cells), the difference being significant in MLN and ileal LP (P less than 0.05). The J chain positivity tended to be higher for all IgG subclasses in MLN than in peripheral lymph nodes; this difference was significant (P less than 0.05) for IgG2-producing immunocytes. Taking J chain expression as a marker of clonal immaturity, our results may reflect to some extent distribution of newly generated memory B cell clones from gut-associated lymphoid tissue to MLN, peripheral lymph nodes, and palatine tonsils in a strikingly decreasing order.
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PMID:Terminally differentiated human intestinal B cells. J chain expression of IgA and IgG subclass-producing immunocytes in the distal ileum compared with mesenteric and peripheral lymph nodes. 212 37

A panel of monoclonal antibodies directed against various lymphoid and non-lymphoid cell subsets was used to study the lymph nodes of human fetuses of 16-40 weeks. B cells were of intermediate size and were present at all ages in primitive follicles and in the outer cortex. The fetal B-cell immunophenotype is indicative of an intermediate stage of development, just preceding the differentiation to mature B cell. Forty to sixty per cent Leu1+ B cells were observed in the follicles until the end of the second trimester. At all stages, T cells showed an immunophenotype similar to type III thymocytes, different from adult peripheral T cells, with a marked predominance of CD4+ T cells. Leu7+ NK cells were generally absent. OKIa+ interdigitating reticulum cells were present in T-cell areas. Some axillary lymph nodes showed strongly CD1+ dendritic cells, probably Langerhans' cells. Macrophages and granulocytes were present in varying numbers. Altogether, our results indicate that fetal lymph nodes are quite well differentiated at an early fetal age, although T and B cells do not (yet) show adult immunophenotypes. The expression of the CD38 antigen may be a main marker related to the immaturity of fetal T and B cells.
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PMID:Immunohistological analysis of human fetal lymph nodes. 264 98

Three Hodgkin-derived cell lines (L428, L540, and CO) were studied for rearrangements and expression of immunoglobulin and T-cell receptor genes, and their genotype was compared to the phenotype. As far as the genotype is concerned, all 3 cell lines have characteristics of lymphoid cells; L428 of B, and L540 and CO of T-cell origin. L428 cells have one Ig heavy chain allele rearranged to C gamma and transcribed into RNA, while the second is deleted. Furthermore, L428 cells show an unusual immunoglobulin kappa light chain gene rearrangement involving deletion of the kappa constant gene in one allele, while the remaining kappa and lambda loci are in germline configuration. L540 and CO have, in contrast to L428 cells, the immunoglobulin genes in germline and T-cell receptor genes rearranged. The T-cell receptor beta and gamma genes are rearranged in both L540 and CO, whereas a rearrangement in the alpha locus was detected in L540 cells only. RNA of the size of functional beta chain transcripts was found in CO cells and of the size of functional alpha chain transcripts in L540 cells. All 3 cell lines are classified as immature lymphoid cells with respect to the limited expression of B- and T-cell antigens, respectively, and to the incomplete expression of their antigen receptor. The immaturity of lymphoid differentiation contrasts with the expression of activation antigens, i.e. Ki-1, Ki-24, HLA-DR, and IL-2 receptor. The immaturity of the cells excludes the possibility that the cells were activated along the physiological pathway, i.e. by interaction of the cell with antigen. The results obtained on the cell lines are in accordance with in vivo studies and suggest that Hodgkin and Sternberg-Reed cells are immature lymphoid cells which are activated by a still unknown mechanism.
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PMID:Phenotype versus immunoglobulin and T-cell receptor genotype of Hodgkin-derived cell lines: activation of immature lymphoid cells in Hodgkin's disease. 311 32

In non-specifically immunized rats, bred under conventional conditions, the first 'spontaneous' germinal centres were observed by 21 days after birth. Deliberate antigenic stimulation led to an earlier appearance of germinal centres in neonatal spleen: immunization with sheep red blood cells as early as 7 days after birth resulted in germinal centre formation in the spleen as observed 7 days later. By that time the first primary follicles could also be observed, in both immunized and non-immunized rats. Although 3-day-old rats upon antigenic stimulation failed to generate germinal centres in their spleen, transfer experiments of 3-day-old spleen cells to lethally X-irradiated syngeneic adult recipients indicated that 3-day-old spleens at least contained all the essential lymphoid elements (B and T cells) needed for germinal centre formation. These results strongly suggest that the failure to induce germinal centres in 3-day-old rats is most probably due to an immaturity of their splenic microenvironment. Immunohistochemical staining of frozen sections of neonatal rat spleen using mAb ED 5 and MRC OX-2 showed that follicular dendritic cells (FDC) were found as soon as primary follicles were found (i.e. by 14 days after birth). The appearance of FDC in neonatal spleens was not influenced by deliberate antigenic stimulation nor by the administration of adult spleen cells. We postulate that, during the development of FDC, a splenic microenvironment is created that allows primary follicle formation and the generation of germinal centres.
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PMID:The ontogeny of germinal centre forming capacity of neonatal rat spleen. 329 74

Forty-two bone marrow aspirates and biopsies during follow-up examinations from patients with multiple myeloma were reviewed to determine whether the results correlate with the clinical state of the patient at the time of examination. The percentage of plasma cells on biopsy and aspiration, cytological immaturity, patterns of plasma cell infiltration, and the presence or absence of multiple lymphoid nodules and marked fibrosis were cross-tabulated with clinical parameters (hemoglobin levels, osteolytic lesions, and renal function). Hemoglobin levels less than 10 g/dl were more frequent in those with greater than 70% plasma cells on either aspiration or biopsy (P less than 0.05). A nodular histological pattern on biopsy, however, had a higher correlation with hemoglobin levels less than 10 g/dl, and serum creatinine levels greater than 2 mg/dl, than did plasma cell number. The presence of lymphoid nodules correlated with less lytic bone lesions. The degree of fibrosis and plasma cell immaturity did not correlate with any of the clinical parameters. Our findings suggest that reports on bone biopsies should include in addition to the number of plasma cells, the pattern of plasma cell infiltration and the presence or absence of multiple lymphoid nodules.
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PMID:Bone marrow biopsy in multiple myeloma: a clinical pathological study. 340 26


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