Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0021051 (immunodeficiency)
 71,517 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The proportion of T gamma and T mu lymphocytes was studied in 40 cases of B-chronic lymphocytic leukaemia (B-CLL) and six of B-prolymphocytic leukaemia (B-PLL). The significant increase in T gamma cells, previously reported in two small B-CLL series, was confirmed and shown to be directly correlated with the clinical stages of the disease (P less than 0.01 to less than 0.001). The normal T mu:T gamma ratio (2.3:1) was reversed in B-CLL (1:1.4) and B-PLL (1:1.9). The proportion of T mu cells was decreased but was not related to stage. Our findings suggest that the increase in T gamma cells may be responsible for the hypogammaglobulinaemia of B-CLL. This is supported by two sets of observations. First, serum Ig levels were more often normal in cases in Stages 0 and I than in Stages II-IV (P less than 0.05), while the levels of two or three Ig classes were below normal in Stages II-IV twice as frequently. Secondly, splenic irradiation in one case was followed by a fall in the absolute number of T gamma lymphocytes, a reversion to normal of the T mu: T gamma ratio and an improvement in serum Ig levels. Thus, the imbalance in ;the regulatory T-cell subsets may provide an important clue to understand the pathogenic mechanism of the immunodeficiency in the chronic B-cell leukaemias.
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PMID:Increase in T gamma lymphocytes in B-cell chronic lymphocytic leukaemia. II. Correlation with clinical stage and findings in B-prolymphocytic leukaemia. 697 Nov 20

Genetic factors suspected in the etiology of human hemopoietic neoplasia, such as leukemia, lymphoma and multiple myeloma, are reviewed. High incidence of consanguineous marriage was found in parents of familial leukemia in siblings. It was also noted that the age of patients with familial leukemia in children of consanguineous parents was younger than that of cases whose parents were not related. These findings suggest that genetic factors may play an important role in the etiology of familial leukemia in siblings. According to the frequencies of familial aggregations in close relatives, the genetic relationships were supposed to be important in chronic lymphocytic leukemia and acute leukemia, but not in chronic granulocytic leukemia. Increased prevalence of autoimmune diseases in relatives of leukemic patients suggests a possibility of genetic immunodeficiency as a common etiologic factor in both diseases. Immunodeficiency was found in unaffected relatives of patients with familial leukemia and lymphoma. Genetic factors were also suggested by the familial occurrences of multiple myeloma and primary macroglobulinemia, and the incidence of benign monoclonal gammopathy in relatives of patients with these diseases. HLA studies revealed the increased frequencies of A2 in acute lymphocytic leukemia, of B5 and B18 in Hodgkin's disease, and of A5 and B18 in multiple myeloma. From such relationships existing between familial immunodeficiencies and hemopoietic neoplasia, genes regulating the immune responsiveness might be involved in susceptibility to these diseases.
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PMID:[Genetics and hemopoietic neoplasia]. 718 26

The study performed in Omsk has ascertained that incidence rates of cancer among Omsk hemoblastosis patients are 8.2 times higher than those in normal population (62 cases per 2961 patients versus 25.46 cases per 10,000 normals). Most frequently, cancer develops in lymphoproliferative diseases. Acute leukemia and chronic myeloid leukemia, for the most part, arise in females with genital and breast cancer, exposed to radiotherapy. In chronic lymphoid leukemia cancer appears in established hemoblastosis. The combination of tumors with hemoblastosis is recorded with similar frequency at the age over 39, in males and females. Combination of tumors with chronic lymphoid leukemia is attributed to immunodeficiency, typical for patients with lymphoproliferative diseases.
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PMID:[Hemoblastosis and cancer]. 761 48

Graft-versus-host disease (GVHD) is a life threatening complication that may occur following allogenic bone marrow transplantation (BMT) in the patients with aplastic anemia, leukemia or genetic immunodeficiency. It has been known that GVHD occurs approximately 70% of recipients of BMT in western countries but no definite incidence has been reported in Korea. In our St. Mary's Hospital, GVHD occurs in about 30% of BMT recipients. Histopathologically the acute phase skin shows diffuse lymphocytic infiltrates in the upper dermis with extensive exocytosis. Scattered throughout the epidermis are many degenerated keratinocytes, which are often associated with one or more satellite lymphocytes (satellite cell necrosis). In the chronic phase, acanthosis, eosinophilic keratinocytes resembling colloid bodies and mononuclear cell infiltrates in the upper dermis are noted. We reviewed 5 cases of acute GVHD and 6 cases of chronic GVHD. All patients received allogenic BMT from Jan. 1, 1992 to July 1, 1993. Ten patients were male and one was female. The mean age was 34 (20-70). The pathologic diagnosis was 3 cases of CML, 2 of ALL, 2 of AML (FAB M2), 2 of aplastic anemia, 1 of CLL and 1 of AML (FAB M5). The interval from BMT to GVHD varied from 14 days to 4 years (median 220 days). The skin and GI tract were involved in all eleven cases. Ten cases were histologically proven by skin biopsies, and two cases by salivary gland and colonic biopsies, respectively. The histological findings of the skin, salivary gland and colonic biopsieds were described. Immunohistochemical stain of the skin was done using CD4, CD8, HLA DR and Leu 7 antibodies.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Graft-versus-host disease--clinical and pathological analysis of 11 biopsy proven cases. 770 86

In recent years, especially with the advent of acquired immunodeficiency syndrome, new skin disorders associated with systemic disease have been described in the literature. Eosinophilic folliculitis and pruritic papules of human immunodeficiency virus (HIV) infection are clinically similar lesions that respond to phototherapy. Bacillary angiomatosis, another HIV-related skin disease that is caused by a pleomorphic gram-negative organism, resembles Kaposi's sarcoma clinically but is curable if treated early with antibiotics. Toxic strep syndrome, a scarlatiniform, desquamative eruption associated with hypotension, fever and multiorgan system dysfunction, is caused by group A streptococcal soft tissue infection. Paraneoplastic pemphigus, a recently characterized autoimmune vesicular eruption, produces painful mucocutaneous ulcerations in patients with an occult neoplasm, such as chronic lymphocytic leukemia or malignant lymphoma.
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PMID:New cutaneous manifestations of systemic diseases. 765 17

Normal differentiation of B lineage cells has been the subject of intensive investigation over the past three decades. Current models of this process in humans are melded from the results of studies in a variety of organisms, including humans, mice and birds. Several recent developments have significantly reshaped and refined these models. The technique of homologous recombination in embryonic stem cells has allowed the production of mice with selectively disrupted genes that are important for B cell development in mice. At the same time, functional studies of human B cell differentiation, together with analysis of naturally occurring mutations that disrupt this process, have progressed rapidly. This has provided insight into the pathogenesis of lymphoproliferative and immunodeficiency diseases as well as a clearer view of normal developmental events. In this chapter we have reviewed human B cell differentiation with particular emphasis on newly emerging concepts. We also discussed CD5, a pan-T cell antigen that is expressed in low levels on a subpopulation of B cells implicated in the pathogenesis of chronic lymphocytic leukaemia (CLL). Finally, we discussed the issue of restricted variable region gene usage during B cell ontogeny and in CLL.
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PMID:Normal B lymphocyte differentiation. 803 90

Serum levels of the soluble form of the low-affinity receptor for IgE (FcERII, CD23) (sCD23) are elevated in autoimmune conditions associated with hypergammaglobulinaemia and B cell hyperactivity. Very high levels of sCD23 are found in patients with B-chronic lymphatic leukaemia (B-CLL) who are, however, frequently hypogammaglobulinaemic. We therefore compared the serum levels of sCD23 in healthy controls (n = 33) with three conditions associated with hypogammaglobulinaemia (HGG) and varying B cell numbers: X-linked agammaglobulinaemia (XLA, n = 12), common variable immunodeficiency (CVI, n = 20) and B-chronic lymphatic leukaemia (n = 33). Serum levels of sCD23 showed a significant correlation with the CD19+ B cell count in both normals and patients with CVI (r = 0.65, P < 0.0001). Amongst the different clinical groups, serum levels of sCD23 were increased in the order XLA < CVI < normals < CLL (medians 2.5, 7.7, 11.1 and 540, respectively; P < 0.001 for all comparisons except CVI versus normals P < 0.03 in a one-tailed test). In the CVI group, serum sCD23 was lowest amongst four patients with low B cell numbers. There was no overlap in sCD23 between patients with XLA and this subgroup of CVI patients. Serum sCD23 is, therefore, derived predominantly from B cells, and is significantly related to the peripheral blood B cell count.
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PMID:Serum soluble CD23 in patients with hypogammaglobulinaemia. 805 Jan 71

The nucleoside analogs fludarabine monophosphate, 2-chlorodeoxyadenosine, and 2-deoxycoformycin (pentostatin) all have activity in chronic lymphocytic leukemia. The most widely studied drug is fludarabine which is able to obtain complete or partial responses in more than 50% of previously treated patients. The response rate is 44% for 2-CDA and approximately 25% for pentostatin. Fludarabine has also been used to treat patients as initial therapy, and has resulted in overall response rate of 79% with 75% of the patients achieving complete remission. The NCI and International Working Group for CLL criteria for complete remission allow for persistent nodules or lymphoid infiltrates in the bone marrow biopsy. Studies have now demonstrated persistent lymphoid aggregates are associated with a shorter time to progression for responders but no survival disadvantage. There is a strong association of documented refractoriness to alkylating agents with probability of response to fludarabine and also survival. The major morbidity associated with the use of these drugs are infections, which, in some circumstances, are associated with neutropenia but in other circumstances are probably related to the hypogammaglobulinemia and T-cell immunodeficiency which are part of the disease. The T-cell immunodeficiency is aggravated by the nucleoside analogs. Even after discontinuation of therapy the immunodeficiency as measured by CD4 cell number is sustained for 12 to 24 months. Opportunistic organisms such as herpes simplex, herpes zoster, Listeria monocytogenes, and pneumocystis carinii are being noted in patients treated with these agents. The potency of these drugs and low incidence of toxicities to other organs suggests that they will be effectively combined with other agents.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Nucleoside analogs in treatment of chronic lymphocytic leukemia. 809 53

A 78-year-old man, who suffered from chronic lymphocytic leukaemia and diabetes mellitus, but was human immunodeficiency virus (HIV)-negative, developed disseminated angiomatous papules following a cat scratch. Bacillary angiomatosis was diagnosed by light and electron microscopic demonstration of the causative bacteria in the vascular lesions. The lesions resolved completely when he was treated with erythromycin. This case demonstrates that bacillary angiomatosis can be an important cutaneous manifestation of immunodeficiency in individuals who are not infected with the human immunodeficiency virus.
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PMID:Bacillary angiomatosis in a patient with lymphocytic leukaemia. 820 79

In a 78 years old patient with chronic lymphoid leukemia, diabetes mellitus a cat scratch induced disseminated angiomatous papules were observed. In the lesions great number of bacilluses were observed with light -and electron microscope. As a result of antibiotic treatment the lesions regressed without trace. This opportunist infection resulting general symptoms as well, may be regarded as a cutaneous manifestation of immunodeficiency. The adequate antibiotic treatment depends on the exact diagnosis.
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PMID:[Bacillary angiomatosis]. 824 15


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