• Journal of Chest Surgery
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• v.50 no.2
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• pp.119-122
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• 2017

Pure red cell aplasia (PRCA) and hypogammaglobulinemia are paraneoplastic syndromes that are rarer than myasthenia gravis in patients with thymoma. Good syndrome coexisting with PRCA is an extremely rare pathology. We report the case of a 50-year-old man with thymoma and PRCA associated with Good syndrome who achieved complete PRCA remission after thymectomy and postoperative immunosuppressive therapy, and provide a review of the pertinent literature.

• Pediatric Infection and Vaccine
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• v.14 no.1
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• pp.116-119
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• 2007

Patients with primary immunodeficiencies often have symptoms of their disease for months or years before diagnosis and treatment. This is partly because these disorders are relatively uncommon and the infections typical of immunodeficienciey, for example otitis, sinusitis, and pneumonia, are common. We report a case of agammaglobulinemia in an 8-year-old boy with recurrent and severe infection. He was first seen in our hospital for bacterial meningitis in 2006. His immune status revealed panhypogammaglobulinemia and deficiency in mature B lymphocyte. His elder brother also showed deficiency in mature B lymphocyte but mild hypogammaglobulinemia. Some X-linked agammaglobulinemia (XLA) cases may remain undiagnosed because they only show mild hypogammaglobulinemia and they lack repeated infections in childhood.

• Journal of Chest Surgery
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• v.39 no.1 s.258
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• pp.85-89
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• 2006

Good's syndrome (thymoma with immunodeficiency) is a rare cause of combined B and T cell immunodeficiency in adults. The clinical characteristics are increased susceptibility to bacterial infection and opportunistic viral and fungal infections. The most consistent immunological abnormalities are hypogammaglobulinemia and reduced or absent B cells. This syndrome should be treated by resecting the thymoma and replacing the immunoglobulin to maintain adequate IgG values. The author experienced one case of Good's Syndrome. The patient was a 64-year-old female who had a history of frequent sinopulmonary infection. Chest CT showed Pneumonia and anterior mediastinal mass and PCNB was taken and biopsy result was thymoma. She received thymectomy and replacement of immunoglobulin to control hypogammaglobulinemia.

• Applied Microscopy
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• v.12 no.2
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• pp.1-10
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• 1982

P. carinii is a protozoan which induces an often fatal pneumonitis in a variety of compromised patients. The ultrastructure of P. carinii was studied in a male infant with pneumocystitis pneumonia associated with hypogammaglobulinemia. Four principal structural varieties-small trophozoites, large trophozoites, mature cyst and empty cyst were identified. The ultrastructure of these organisms was similar to the cases previously reported. Relevance of the morphologic findings to the functional aspect were discussed.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.18 no.1
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• pp.60-65
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• 2015

We report a pediatric patient admitted with abdominal pain, diffuse lower extremity edema and watery diarrhea for two months. Laboratory findings including complete blood count, serum albumin, lipid and immunoglobulin levels were compatible with protein losing enteropathy. Colonoscopic examination revealed diffuse ulcers with smooth raised edge (like "punched out holes") in the colon and terminal ileum. Histopathological examination showed active colitis, ulcerations and inclusion bodies. Immunostaining for cytomegalovirus was positive. Despite supportive management, antiviral therapy, the clinical condition of the patient worsened and developed disseminated cytomegalovirus infection and the patient died. Protein losing enteropathy and disseminated cytomegalovirus infection a presenting of feature in steroid-naive patient with inflammatory bowel disease is very rare. Hypogammaglobulinemia associated with protein losing enteropathy in Crohn's disease may predispose the cytomegalovirus infection in previously healthy children.

• The Journal of Internal Korean Medicine
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• v.25 no.3
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• pp.573-581
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• 2004

Evans syndrome, as originally described, refers to autoimmune hemolytic anemia accompanied by thrombocytopenia. The autoantibodies in Evans syndrome are directed specifically against red cells, platelets, or neutrophils and are not crossreacting. The incidence of autoimmune hemolytic anemia is estimated to be approximately 10 cases per million people. Many patients have associated disorders, such as lupus erythematosus and other autoimmune disease, chronic lymphadenopathy, or hypogammaglobulinemia. In Oriental Medicine, the approach to Evans syndrome is made in view of deficiency of blood. The ailment was treated through methods of Oriental Medicine. Acupucture and herbal medicine were administered to patients diagnosed with Evans Syndrome by ecchymosis at the lower limb after continued gingival bleeding. As a result significant improvement in RBC, Hb, Hct, and PLT were observed and complaints abated.

• The Korean Journal of Cytopathology
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• v.5 no.2
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• pp.189-193
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• 1994

Malignant thymoma is usually manifested as a mediastinal mass. Occasiomally, these tumors may be associated with a variety of systemic syndromes, such as myasthenia gravis, pure red cell aplasia or hypogammaglobulinemia However, it is generally accepted that thymomas rarely metastasize and the frequency of extrathoracic metastasis has been reported as 1 to 15%. In general, nuclear changes of tumor cells such as enlarged size, irregular shape and altered textures are determining factors in the assessment of the grade of malignancy. We experienced a case of malignant thymoma of anterior mediastinum in a 35-year-old woman. After 3 years, she developed recurrent malignant thymoma in the right supraclavicular area, diagnosed by aspiration biopsy cytology.

• Tuberculosis and Respiratory Diseases
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• v.63 no.5
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• pp.454-457
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• 2007

Thymomas are associated with different paraneoplastic syndromes, with the most clinically important association being with myasthenia gravis, hypogammaglobulinemia, and pure red cell aplasia (PRCA). The optimal therapy for PRCA that complicates a thymoma is unknown, given the rarity of the clinical situation. It has been suggested that remission following surgery alone is uncommon and most patients will require some other form of therapy. We report a case of PRCA with a thymoma in a 59-year-old man who remained in complete remission of the PRCA after surgical resection of the thymoma.

• Childhood Kidney Diseases
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• v.9 no.2
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• pp.143-148
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• 2005

Purpose : Hypogammaglobulinemia has been observed in nephrotic syndrome, but its pathophysiology remains unknown. We evaluated serum immunoglobulins, IgG subclasses, and vaccine-induced viral antibodies(anti-hepatitis B surface IgG and anti-measles IgG) in children with minimal change nephrotic syndrome(MCNS). Methods : Using the stored sera, the levels of immunoglobulin(IgC, IgM, IgA, and IgC) and IgG subclasses(IgG 1, 2, ,3, and 4), anti-HBs Ab and anti-measles IgG of 21 children with MCNS were analyzed and compared to those of 25 age-matched healthy children. Results : The mean values of IgG and IgG1 were $390{\pm}187\;mg/dL$ and $287{\pm}120\;mg/dL$ in nephrotic children, and $1,025{\pm}284\;mg/dL$ and $785{\pm}19\;mg/dL$ in control children, respectively. The values of the total IgG and the 4 IgG subclasses in nephrotic children were all significantly depressed(P<0.001), but the IgM($251{\pm}183\;mg/dL\;vs. 153{\pm}55\;mg/dL$, P=0.02) and IgE values(P=0.01) were elevated, and the IgA values were not changed. The seropositivity of anti-HBs IgG was 42.9$\%$(9 of 21 cases) in the MCNS group and 52$\%$(13/25) in the control group, and that of anti-measles IgG was 75$\%$(16/21) and 92$\%$(23/25), respectively, but there was no statistical difference between the two groups. Conclusion : IgG and IgG subclass levels in MCNS children are all depressed without significant seronegativity of the vaccine-induced viral antibodies. Further studies are needed to resolve the cause of hypogammaglobulinemia in MCNS. (J Korean Soc Pediatr Nephrol 2005;9:143-148)

• Tuberculosis and Respiratory Diseases
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• v.65 no.3
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• pp.207-211
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• 2008

X-linked agammaglobulinemia is the most common type of primary immunodeficiency disorder. Mutation ofthe cytoplasmic tyrosine kinase gene, Btk (Bruton's tyrosine kinase), is known to be the etiology of X-linked agammaglobulinemia. The patients with this disease manifest a B-cell deficiency and low levels of serum immunoglobulin; due to the deficient antibodies, they suffers from recurrent upper and lower respiratory infections. We report here a 24-year-old male with an initial clinical impression of recurrent pneumonia and bronchiectasis. The patient presented with marked pan-hypogammaglobulinemia and the absence of circulating B-lymphocytes on the immunologic study, and he carried a splicing mutation of intron 2 in the Btk gene (IVS2 -3C>G).