Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0023418 (leukemia)
 93,477 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A case of chronic lymphoid leukaemia complicated by a nephrotic syndrome is reported. There was no evidence of amyloidosis or renal vein thrombosis. Percutaneous renal biopsy disclosed lobular membranoproliferative glomerulonephritis with subendothelial deposits of IgG, C3, C4 and C1q. Circulating immune complexes, IgG lambda cryoglobulin and low CH50 levels due to activation of the classical pathway were demonstrated in serum. Immunosuppressive treatment of the leukaemia resulted in complete regression of the nephrotic syndrome.
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PMID:[Nephrotic syndrome associated with chronic lymphoid leukemia. Review of the literature apropos of a case]. 714 87

A case of chronic lymphocytic leukemia complicated by the nephrotic syndrome (due to membranoproliferative glomerulonephritis) is reported. There was complete remission of the proteinuria on two occasions in response to treatment of the leukemia. Possible etiological mechanisms are discussed and review of the literature is presented.
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PMID:Recurrent proteinuria in chronic lymphocytic leukemia. 727 96

An increase in the incidence and severity of bacteremia caused by group A streptococci was noted in 1993 and 1994 in the Hadassah University Medical Center, Jerusalem. During the 6-year period 1987 to 1992, 12 children with group A streptococcal bacteremia were hospitalized, whereas in 1993 and 1994 there were 17 patients, 5 of them with 1 each of the following severe clinical manifestations: meningitis and septic shock; streptococcal toxic shock syndrome; septic shock; pleural empyema; and fatal outcome. Our 29 patients with group A streptococcal bacteremia were younger than those reported in the literature: 10 (35%) were < 3 months of age; 17 (59%) were < 1 year old. Most children were previously healthy and only 3 had an underlying immunodeficiency predisposing to infection (1 case each): leukemia; Di George syndrome; and congenital nephrotic syndrome. Two children were recovering from varicella. The skin was the most common site of primary infection (16 of 29). The average white blood cell (WBC) count was 18 150 cells/mm3 (range, 2200 to 34,200). The cases were not related epidemiologically and were caused by a variety of M-protein types. Polymerase chain reaction amplification of the genes encoding exotoxins A (speA) and C (speC) was done on 19 isolates and disclosed 2 strains positive for speA and 5 positive for speC. One of the speA-positive isolates was from the single patient with toxic shock syndrome.
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PMID:Increased incidence and severity of Streptococcus pyogenes bacteremia in young children. 855 25

We report on a patient with an almost 20-year history of B chronic lymphocytic leukemia (B-CLL). During the last 2 years, the patient developed nephrotic syndrome (NS) due to membranous glomerulonephritis (MN), refractory to standard therapeutic regimens. Neither NS nor B-CLL responded objectively to systemic administration of two different combinations of corticosteroids and alkylating agents (chlorambucil, cyclophosphamide). Third-line therapy with cyclosporin A resulted in reduction of proteinuria and improvement of leukemia. Withdrawal of the drug led to an increase in leukocyte count.
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PMID:Effect of cyclosporin A in a patient with refractory nephrotic syndrome associated with B chronic lymphocytic leukemia. 885 99

Chronic lymphocytic leukemia is a common disease in the elderly but is rarely associated with a nephrotic syndrome. The rarity of this association suggests that leukemic cells may have certain properties or features that may lead to the development of glomerulonephritis. Effective medical treatment of the leukemia may not necessarily allow regression of the nephrotic syndrome; however, the effects of splenectomy on nephrotic proteinuria when associated to chronic lymphocytic leukemia have never been evaluated. We report the case of a 50-year-old male with stage C CD5+ chronic lymphocytic leukemia associated with a nephrotic syndrome due to Type I membranoproliferative glomerulonephritis. Chlorambucil and prednisone were unable to control the leukemia and the nephrotic range proteinuria, and were discontinued because of poor hematologic tolerance. A splenectomy immediately resulted in a spectacular remission of both chronic lymphocytic leukemia and the nephrotic syndrome. Spleen lymphocytes were collected and tested in quantitative flow cytometry for the expression of the main B cell associated markers. They did not exhibit any particular immunophenotypic pattern. This report of a remission of a glomerulonephritis associated with chronic leukemia following splenectomy is evidence of a possible relationship between the two diseases.
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PMID:Nephrotic syndrome associated with chronic lymphocytic leukemia resistant to immunosuppressive drugs: remission obtained by splenectomy. 904 65

In several childhood diseases which have the ensuing risk of infertility in adult life because of direct hypothalamic-pituitary-testicular axis involvement, or as a consequence of therapeutic toxicity, the role of antisperm antibodies (ASA) is rarely addressed. The aim of this study was to investigate the occurrence of ASA in a large prepubertal male population (aged 1.2-13 years) consisting of three groups: Group I, 52 patients affected by malignant diseases (lymphoblastic leukaemia, malignant lymphoma, or Wilm's tumour, n = 42), or by nephrotic syndrome (n = 10); Group II, 212 patients with either genital tract abnormalities (cryptorchidism, inguinal hernia, funicular torsion or hypospadias, n = 202), or cystic fibrosis (n = 10); Group III: 100 age-matched normal boys. Group I and II patients were investigated at diagnosis and during or after treatment (drug, radiation or surgical therapy). Group III was used as controls. ASA were detected in sera by the Tray Agglutination Test (TAT) and indirect IgG, IgA and IgM immunobead tests (iIBT). All normal boys were ASA-negative using both tests. Twenty-six out of the 264 patients (9.8%) in Groups I and II were ASA-positive: 23 (8.7%) patients had a positive TAT with a titre of 1:32 to 1:128, whilst 14 (5.3%) had IgG-ASA after iIBT. Eleven patients (4.1%) were ASA-positive in both tests. Of the 26 ASA-positive boys, 24 had genital tract abnormalities (cryptorchidism, testicular torsion, hypospadias) and two had leukaemia with testicular infiltration. Treatment did not modify antibody positivity. Our data confirm that ASA can occur in prepubertal boys, mostly among cases with urogenital pathology, but that it is rare among other cases. Therefore autoimmune reaction against spermatozoa is another factor that should be considered in the evaluation of several conditions in childhood involving reproductive tract alteration and potential impairment of the blood testis (Sertoli cell) barrier.
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PMID:Antisperm antibodies in prepubertal boys treated with chemotherapy for malignant or non-malignant diseases and in boys with genital tract abnormalities. 920 87

A correlational descriptive study was conducted to investigate self-concept and selected influencing factors among hospitalized Chinese school-age children with a chronic illness. The purposive sample was composed of 122 school-age children with chronic illnesses, such as nephrotic syndrome, leukemia, and congenital heart disease, who were admitted to three major teaching hospitals in Shanghai City. The children's medical records, demographic forms, and the Piers-Harris Self-concept Scale (PHSCS) were used to collect the data. Descriptive statistics, and stepwise multiple regression were used to analyze the data utilizing the SPSS program (SPSS Inc, Chicago, IL, USA). The results of this study showed that the majority of hospitalized Chinese school-age children with a chronic illness had at least an average level of self-concept. Academic achievement (grade point average) was a strong predictor of self-concept, as are most of its subconcepts, including social behavior, academic competence, and physical appearance and attributes. Age was a significant predictor of overall self-concept, social behavior, and popularity subconcept. Sex was a significant predictor of self-concept subconcepts such as social behavior, anxiety, and popularity. Duration of illness was a significant predictor of physical appearance and attributes. In addition, the type of illness was a significant predictor of popularity. It can be concluded that the importance of school education should be emphasized by hospital and school nurses for the purpose of helping such children build a positive self-concept concerning their age, sex, academic achievement, type of illness, and duration of illness. Recommendations for further research have been suggested.
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PMID:Selected factors influencing self-concept among hospitalized Chinese school-age children with a chronic illness. 1045 15

The proto-oncogene Frat1 was originally identified as a common site of proviral insertion in transplanted tumors of Moloney murine leukemia virus (M-MuLV)-infected Emu-Pim1 transgenic mice. Contrary to most common insertion sites implicated in mouse T cell lymphomagenesis, retroviral insertional mutagenesis of Frat1 constitutes a relatively late event in M-MuLV-induced tumor development, suggesting that proviral activation of Frat1 contributes to progression of T cell lymphomas rather than their genesis. To substantiate this notion we have generated transgenic mice that overexpress Frat1 in various organs, including lymphoid tissues. Frat1 transgenic mice develop focal glomerulosclerosis and a nephrotic syndrome, but they do not exhibit an increased incidence of spontaneous lymphomas. Conversely, these mice are highly susceptible to M-MuLV-induced lymphomagenesis, and Frat1/Pim1 bitransgenic animals develop lymphomas with increased frequency compared to Pim1 transgenic littermates. These data support a role for Frat1 in tumor progression.
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PMID:Overexpression of Frat1 in transgenic mice leads to glomerulosclerosis and nephrotic syndrome, and provides direct evidence for the involvement of Frat1 in lymphoma progression. 1055 87

The hematological malignancies associated with nephrotic syndrome are mainly Hodgkin's and non-Hodgkin's lymphomas and chronic lymphocytic leukemia. Acute myelogenous leukemia (AML) has rarely been described in association with the nephrotic syndrome. The clinical course of a 44-year-old patient with AML who presented with nephrotic syndrome is described and the clinicopathologic features of the other cases reported in the world literature are reviewed. We could not find a consistent pattern regarding the subtype of leukemia, renal pathology, and temporal relationship between the nephrotic syndrome and the leukemia or the response to treatment. The present case was unique in that the clinical course of the renal disorder correlated with the course of the leukemia responding to treatment with anti-leukemic agents. We conclude that nephrotic syndrome can also be associated with AML. In some cases there is a direct causal effect of the leukemic process on the renal pathology while in others it is exerted indirectly via other complications of the malignancy or the treatment.
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PMID:Acute myeloid leukemia associated with nephrotic syndrome: case report and literature review. 1214 97

We describe herein a case of nephrotic syndrome (NS) following allogeneic bone marrow transplantation (allo-BMT) for natural killer cell leukemia/lymphoma. Histologic studies defined the diagnosis as crescentic glomerulonephritis with massive immunoglobulin A (IgA) deposition, which has never been reported in NS cases following allo-BMT. Most of the massive infiltrated cells in the interstice were CD3(+)CD4(-)CD8(+) T cells derived from the donor. We observed mesangial deposition of Haemophilus parainfluenza outer membrane (OMHP) antigen and decreased glycosylation of the IgA1 hinge in the recipient's samples is consistent with the recently reported pathogenesis of IgA nephropathy. Further, the titer of IgA antibody against the donor serum was as high as other IgA nephropathy cases. These findings suggest that NS and crescentic glomerulonephritis in this case occurred as one of the forms of chronic graft-versus-host disease (GVHD), and that IgA deposition was associated with H parainfluenza and decreased glycosylation of the IgA1 hinge.
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PMID:Nephrotic syndrome with crescent formation and massive IgA deposition following allogeneic bone marrow transplantation for natural killer cell leukemia/lymphoma. 1254 67


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