Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0019214 (hepatosplenomegaly)
 4,408 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

In schistosomiasis mansoni, the pathogenesis of hepatosplenic disease has been shown to be due primarily to immune mechanisms. The present study was designed to examine the relationship between the development of schistosomal hepatosplenomegaly in Egyptian school children and the HLA antigens. Two groups of schistosome-infected children with similar fecal egg counts were examined: one group (23 children) had no clinically demonstrable hepatosplenomegaly whereas all the children (28) in the second group suffered from liver enlargement. Furthermore, 13 of the 28 individuals in the latter group had splenomegaly as well. Our results show that hepatosplenomegaly was related to the presence of two HLA antigens: HLA AI and B5. The average relative risk of developing hepatomegaly is 29 for HLA AI and 18.9 for 55.6. Furthermore, the severity of hepatomegaly was correlated with the presence of these two HLA antigens. These findings represent a step toward elucidating the factors controlling the pathogenic mechanisms in human schistosomiasis mansoni.
 ...
PMID:Histocompatibilty-linked susceptibility for hepatospleenomegaly in human schistosomiasis mansoni. 47 1

The effect of polytransfusion regimen is studied in two patients with thalassaemia major over a period of 74 and 56 months respectively. In both cases we have observed an improvement of the general condition in the growth as well as a reduction of hepatosplenomegaly and cardiomegaly. Furthermore a decrease of reticulocytes, erythroblasts and fetal hemoglobin values was obtained. The consequences of these frequent transfusions on iron storage metabolism are discussed. The advantage of giving HLA compatible blood is demonstrated by only a weal alloimmunization in one patient having received 77 HLA compatible transfusions and the lack of immunization in the second patient after 52 transfusions. In 6 other patients affected with thalassaemia major and 3 more with bone marrow aplasia, transfusion with incompatible HLA blood was followed by immunization of variable importance.
 ...
PMID:Multiple transfusions of HLA compatible blood in thalassaemia major. 63 9

Twenty-one patients with ATL were assessed. The predominant physical findings were lymph node and bone marrow involvement, skin involvement, hepatosplenomegaly and leukemic manifestations. The predominant histopathological findings in both skin and lymph node specimens were the diffuse medium-sized cell type and the diffuse mixed cell type. Some phenotypic discrepancy was found between the neoplastic cells in the peripheral blood, lymph nodes and skin of patients with ATL with respect to CD45RA and CD45RO, and CD7, CD29, CD25 and HLA-DR. That is, the predominant neoplastic cell phenotype was the helper T-cell, which was CD3+, CD4+, CD7+, CD25+, CD45RA+ and HLA-DR+, and CD29- and CD45RO- in peripheral blood and lymph nodes, and CD3+, CD4+, CD7+, CD29+, CD45RO+ and HLA-DR+, and CD45RA- in the skin. In other words, we have described the phenotypic heterogeneity of ATL cells and demonstrated the heterogeneity of CD45R isoform expression on ATL cells in different organs--the skin, peripheral blood and lymph nodes--of the same patient.
 ...
PMID:Adult T-cell leukemia/lymphoma (ATL)--clinical, histopathological, immunological and immunohistochemical characteristics. 136 26

A case is reported of an adult male patient with acute leukemia characterized by the presence of the novel cytogenetic abnormality, t(2;9)(p12;p23), in addition to a t(4;11)(q21;q23). The immunophenotype of the blast cell population was consistent with immature early pre-B cell acute lymphoblastic leukemia (ALL) (TdT+,HLA-DR+,CD19+,CD24 +/-,CD10-) expressing myelo-monocytic antigens (CDw65,CD15). The genotype showed a clonal rearrangement of the immunoglobulin heavy chain locus. Because the immunoglobulin kappa (kappa) light chain gene is located on chromosome 2 at band p12 and interferon alpha (alpha) and beta (beta) map to chromosome 9p21-p22, rearrangements of these loci as a result of the t(2;9) were studied. There was no evidence for rearrangement of the region covering about 40 kilobases around the kappa locus when hybridized to C(kappa), the 3' kappa enhancer or the kappa deleting element. Only germline size restriction fragments were also found for the interferon alpha and beta genes. The patient's clinical features were typical for ALL associated with the t(4;11), including a high white blood cell count at presentation, hepatosplenomegaly, and a poor outcome. The potential significance of 2p and 9p abnormalities in addition to t(4;11) is discussed.
 ...
PMID:Translocation (2;9)(p12;p23) in a case of acute leukemia with t(4;11)(q21;q23). Lack of rearrangement of the kappa and interferon gene loci. 137 31

A 2-year-old girl showed exaggerated skin reactions to mosquito bites and associated general symptoms, including a high temperature, lymphadenopathy, and hepatosplenomegaly. Peripheral blood lymphocytes contained a high percentage of CD2+, CD3-, CD4-, CD8-, CD11b+, CD16+, CD38+, CD56+, CD57-, and HLA-DR+ large granular lymphocytes that exhibited a marked natural killer cell activity. Immunohistochemically, biopsy specimens taken from the lesional skin demonstrated an infiltrate of the cells bearing the natural killer cell phenotype, indicating a role of these cells in the development of the abnormal skin reactions to mosquito bites and other systemic manifestations. Our case suggests that natural killer cell lymphocytosis may show severe hypersensitivity to mosquito bites as the most outstanding manifestation.
 ...
PMID:Severe hypersensitivity to mosquito bites associated with natural killer cell lymphocytosis. 168 90

We have characterized CD4-CD8- double-negative (DN) alpha beta TCR+ T cells from a patient with immunodeficiency, lymphocytosis, lymphadenopathy, and hepatosplenomegaly. The majority of peripheral blood lymphocytes were DN alpha beta TCR+ T cells as evaluated by FACS and biochemical analysis. The DN T cells showed the following phenotype: alpha beta TCR+, gamma delta TCR-, CD2+, CD3+, CD4-, CD5+, CD7-, CD8-, CD16-, CD25-, CD26-, CD28+, CD45RO-, CD45RA+, CD57+, and HLA-DR+. Both southern blot analysis of TCR genes and FACS analysis applying a panel of V beta and V alpha monoclonal antibodies (MoAbs) indicated a polyclonal T-cell expansion. Thymic biopsy showed normal histology, whereas lymph node biopsy samples showed altered histological and immunohistological patterns with markedly expanded paracortical areas containing the DN T cells of the same phenotype as found in peripheral blood T cells. In functional studies, the DN T cells showed a profoundly reduced proliferative response upon stimulation with mitogens as well as MoAbs against the TCR/CD3 complex, CD2, and CD28, respectively. Addition of exogenous interleukin-2 (IL-2) only minimally augmented the proliferative response. In contrast, the addition of a combination of Ca2+ ionophore and phorbol 12-myristate 13-acetate (PMA) restored the proliferative response of the DN T cells to almost normal levels. This observation strongly suggests that the protein kinase C activity of the DN T cells was intact, but that the normal mechanism for transmembrane signal transduction was impaired in these unusual DN T cells.
 ...
PMID:Phenotypical and functional characterization of double-negative (CD4-CD8-) alpha beta T-cell receptor positive cells from an immunodeficient patient. 183 26

A study was made of the cellular and molecular characteristics of nine Chinese infants, consecutively presenting with acute leukemia. Five cases were acute lymphoblastic leukemia (ALL); four were acute nonlymphoblastic leukemia (ANLL). Hyperleukocytosis, hepatosplenomegaly, and poor response to conventional therapy were common features, and CNS involvement was detected at diagnosis in three cases. The blast cells from all five cases with ALL expressed early B-cell markers, i.e., HLA-DR+, CD19+, but CD10-. Terminal deoxynucleotidyl transferase (TdT) was present in blasts from four of the five cases and periodic acid-Schiff staining in blasts from two patients only. The leukemic cells of one patient also showed positive nonspecific esterase activity and expressed myeloid-associated antigens CD33 (My9), CD11 (OkM1), and CD14 (My4 and Mo2). Molecular analysis of leukemic cell DNA from this and two other patients showed rearrangement of the immunoglobulin (Ig) heavy-chain genes, but without any evidence of kappa light-chain gene rearrangement. T-cell receptor (TCR) genes remained in the germline configuration in these cases. Cytogenetic analysis showed translocation t(4;11) (q21;q23) in all four cases studied. In the group of ANLL, three cases belonged to the M4 and one to the M2 subtype. Chromosomal abnormality involving 11q23 was also detected in two patients: t(11;17)(q23;q11) and del(11)(q14q23) in each case respectively. Neither Ig nor TCR gene rearrangement was present in blast cells from patients with ANLL. The data indicate that chromosomal rearrangement of band 11q23 was quite common in Chinese infants with either form of leukemia, a finding that may have pathogenetic implications.
 ...
PMID:Infant leukemia: an analysis of nine Chinese patients. 236 92

Patients with simple and/or complicated intestinal bilharziasis and normal controls were chosen for CBC, complete urine and stool analysis, liver function tests, liver biopsy, sigmoidoscopy and rectal biopsy. HLA typing was done by microcytotoxicity test. A statistically significant increased frequency of HLA-B8 antigen was present in with or without polyposis and with or without hepatosplenomegaly, as compared to controls (P less than 0.01). Also HLA-B8 showed a statistically significant increased frequency in those with or without polyposis and with hepatosplenomegaly and without polyposis (P less than 0.05). The HLA-A25 and A26 showed a significant increased frequency among all groups (P less than 0.05). HLA-A1, A9, B18 were variable in frequency among all groups. It was concluded that patients with bilharzial hepatosplenomegaly specially with intestinal polyposis have a high frequency of HLA-B8.
 ...
PMID:HLA typing intestinohepatic schistosomiasis. 250 21

Two cases of childhood Ki-1 lymphoma occurred with the expression of Ki-1+/HLA-DR+/IL-2R+/EMA+/Leu-M1-/pan-T antigens-/pan-B antigens- in neoplastic cells. Patient 1 with nodular skin lesions expressed Leu-2a+ in the neoplastic cells and died 14 months later. Patient 2 with lymph node swelling and hepatosplenomegaly exhibited Leu-3a+ in the neoplastic cells and remains free of disease. The Leu-2a+ (case 1) or Leu-3a+ (case 2) findings suggest that the neoplastic cells in both cases were derived from T-lymphocyte lineage. However, Southern's blot analysis did not reveal any clonal rearrangements of T-cell receptor genes in the autopsy material from case 1. The Leu-3a+/Mcs-2+ finding in case 2 may indicate that the neoplastic cells were derived from monocyte/macrophage lineage.
 ...
PMID:Childhood Ki-1 lymphoma. A report of two cases. 267 33

The present study was designed to examine the possible association of HLA antigens and schistosomal hepatosplenomegaly and colonic polyposis in a large number of patients and controls. The results show that hepatosplenomegaly was significantly associated with the presence of two HLA antigens: HLA-A1 and B5; and colonic polyposis to HLA-B5 and B8. Furthermore, asymptomatic infection was significantly seen more in subjects with CW2. These results point to possible gentic factors controlling the pathogenesis of disease and susceptibility to infection in human schistosomiasis.
 ...
PMID:Association of HLA class I antigens (A1, B5, B8 and CW2) with disease manifestations and infection in human schistosomiasis mansoni in Egypt. 308 19


1 2 3 4 5 6 Next >>