Cases reported "Dysgammaglobulinemia"

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11/156. iga deficiency and autoimmune hemolytic disease.

    A case of familial selective iga deficiency associated with autoimmune hemolytic disease is reported that illustrates the therapeutic implications when these two entities coexist. Immunoglobulin screening is recommended for patients with AHD who require blood transfusions, in order to identify IgA-deficient patients who may have anti-IgA anaphylactic reactions.
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keywords = deficiency
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12/156. A family with partial and total deficiency of complement c3.

    A young White girl was found to have no detectable complement c3 or C1q. She suffered repeated attacks of pneumococcal meningitis and pneumococcal pneumonia. Her parents, and some of her siblings, had half the normal level of C3; other siblings were normal. She also had decreased IgG levels and increased IgM concentrations. These findings are correlated with a dysmorphic state of the germinal centres of the peripheral lymphoid tissues, seen after death.
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13/156. Selective immunoglobulin m (IgM) deficiency in two immunodeficient adults with recurrent staphylococcal pyoderma.

    Two adult men with recurrent pyoderma due to staphylococcus aureus and a selective deficiency of immunoglobulin m (IgM) antibody synthesis are described. An analysis of each patient's polymorphonuclear leukocyte chemotaxis, phagocytosis and killing of Staph. aureus, serum opsonizaiton of Staph. aureus, and serum and lymphocyte-mediated responses to antigenic stimulation was performed. family studies revealed a possible autosomal dominant inheritance pattern with heterogenetic expression of various dysgammaglobulinemic states in each patient's first degree relatives. In vivo studies of delayed hypersensitivity and in vitro studies of polymorphonuclear leukocyte and lymphocyte function were normal. A defect in IgM, but not in IgG (immunoglobulin G), antibody synthesis to a number of antigens, and a mild decrease in serum opsonic activity to Staph. aureus correctable by heat inactivated normal human serum were found in each patient. In these patients, the recurrent staphulococcal pyoderma prompted an investigation of host defense mechanisms and revealed low to absent IgM levels and a defect in IgM antibody synthesis.
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ranking = 1.4262166557914
keywords = iga, deficiency
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14/156. Hereditary C2 deficiency: association with skin lesions resembling the discoid lesion of systemic lupus erythematosus.

    Two patients with hereditary homozygous C2 deficiency are described. They showed many similar clinical features. Both had discoid-like skin rashes and serologic abnormalities consistent with SLE, but neither had evidence of involvement of any organ other than skin. Although biopsy of these lesions revealed expected changes by light microscopy, typical immunofluorescent findings were not present. The presence of the HL-A haplotype 10, W18 was seen in both families and was associated with the C2 deficiency gene. The 2 patients were mutually nonreactive in MLC tests and both were found to be homozygous for the LD7a type.
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keywords = deficiency
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15/156. Evidence for abnormally regulated alternative rna processing of mu chain gene in B-lymphoblastoid cells from Bloom's syndrome.

    Selective IgM deficiency is commonly found in patients with Bloom's syndrome. In this study, mu mRNA synthesis was investigated in B-lymphoblastoid cells transformed by Epstein-Barr virus (LCL) from a patient with Bloom's syndrome who showed selective IgM deficiency. LCL established from the patient with Bloom's syndrome well expressed IgM molecules in their surface, but scarcely produced secreted IgM, compared with healthy controls. The JH hybridization patterns of digested dna of LCL from the patient with Bloom's syndrome showed the rearrangement of VDJ as well as those of control LCLs. The mu mRNA was well detected, but mu s C-terminal mRNA was poorly detected compared with control LCLs, indicating that secreted mu mRNA was poorly transcribed though membrane-bound mu mRNA was well transcribed. These results suggest that alternative rna processing of mu chain gene is abnormally regulated in LCL from patients with Bloom's syndrome.
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ranking = 0.82621665579136
keywords = iga, deficiency
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16/156. A familial case of hyper-IgM immunodeficiency.

    A 22-year-old male was diagnosed as having immunodeficiency with hyper-IgM based upon recurrent pneumonia, marked elevation of serum IgM and markedly decreased level of IgG. IgG-or IgA-bearing B cells were not detected in peripheral blood while a number and a proportion of peripheral blood T lymphocytes were normal. Peripheral blood lymphocytes from this patient proliferated normally in response to T-independent and T-dependent B cell mitogens, and to T cell mitogens. Furthermore, the same type of dysgammaglobulinemia with increased IgM was found in the patient's father and brother. From these observations, it is suggested that it is a rare case of autosomal dominant or polygenal inheritance of hyper-IgM immunodeficiency.
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keywords = deficiency
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17/156. Iatrogenic IgG2 deficiency in a leukaemic child. A case report.

    A girl with acute non-lymphoblastic leukaemia was treated with immunosuppressive chemotherapy. After cessation of therapy she had three consecutive episodes of infection due to streptococcus pneumoniae from which she recovered and was shown to have developed a combined deficiency of both IgG2 and IgG4. The patient eventually relapsed and died 3 years after the initial diagnosis. The importance of measuring IgG subclasses in patients treated with immunosuppressive chemotherapy is discussed.
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keywords = deficiency
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18/156. Combined immune deficiency syndromes with primary T-cell defect and partial B-cell reactive hyperactivity. Immunological and morphological analysis of two unusual cases.

    Two cases of combined immunodeficiency with lymphopenia, thymic dysplasia, and defective immunoglobulin production are reported. Both show selective hypo-gammaglobulinemia (IgG and IgA respectively) and selective hyper-gammaglobulinemia (both IgE, IgA, and IgM respectively). The cases are classified, by correlation of clinical and histopathological data as a variant of Fireman's disease.
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keywords = deficiency
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19/156. Primary immunodeficiency in combination with transverse upper limb defect and anal atresia in a 34-year-old patient with Jacobsen syndrome.

    We describe a 34-year-old male patient with Jacobsen syndrome associated with a broad spectrum of anomalies and an increased susceptibility to infections. Features commonly seen in Jacobsen syndrome were short stature, mental retardation, congenital heart disease, cryptorchidism, strabismus, distal hypospadia glandis, and mild thrombocytopenia. Chromosome analysis disclosed a mosaic 46,XY,del(11)(q24.1)/46,XY karyotype with a very low percentage of normal cells. In addition, transverse upper limb defect, imperforate anus, and hearing impairment were noted. Cellular anomalies include functional impairment and deficiency of T-helper cells, and a low serum immunoglobulin m (IgM)-level. The presence of a transverse limb defect and primary immunodeficiency has not been reported previously in Jacobsen syndrome.
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ranking = 1.2
keywords = deficiency
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20/156. Urticarial vasculitis, iga deficiency and C1 esterase inhibitor deficiency in the presence of an IgG monoclonal gammopathy--a case report.

    A female patient, currently aged 40, has attended our Lupus Clinic for 8 years with a clinical picture of urticarial vasculitis, joint pains, severe myalgias and abdominal pain which has occurred in bouts at irregular intervals. She has been found to have consistently reduced C4 levels, C1-esterase inhibitor deficiency and a persistent monoclonal band in the gamma region on electrophoresis. Her serum IgA level has been continuously low. Her symptoms have failed to respond to antihistamines and a variety of other measures but have been partially alleviated by immunosuppressive therapy ('pulsed' cyclophosphamide). We believe that this is the first patient described showing this combination of clinical and laboratory features.
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ranking = 1.8
keywords = deficiency
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