原發性低丙球蛋白血症併發類似類風濕性單關節炎之一病例報告

Primary Hypogammaglobulinemia Complicated with Rheumatoid Like Arthritis a Case Report

謝貴雄(Kue-Hsiung Hsieh) ； 蔡淳娟(Tsuen-Chiuan Tsai) ； 謝宏忠(Hong-Chung Hsieh)

《Acta Paediatrica Sinica》 24卷1期 (1983/03) Pp. 52-63

摘要

A case of primary hypogammaglobulinemia complicated with rheumatoid-like arthritis is reported. The patient was a sx-yearolc1 boy who had repeated bacterial infections since 11 months of age, Feeder and painful swelling of the left knee joint with morning stiffness and limitation of motion developed when he was 2 years old and persisted until now. Immunological studies revealed abnormally low serum immunoglobulins (IgG 275mg/dl, IgA＜4.5m/dl, IgM 52mg/dl, IgE 11.5IU/ml), absence of anti-A isohemagglutinin (patient was B group) and inability to mount the specific antibody response to Salmonella typhosa after vaccination with typhoid vaccine Although B cell was detectable in hi circulating blood by EAC rosette, surface immunoglobulins and OKIal monoclonal antibody in vitro immunogiobulin biosynthesis study showed that it was mainly due to the intrinsic defect of B cell to synthesize adequate a1nouut of the immunoglobulins, Normal number of 1gM-bearing B cell was found in the mucosa of the small intestine, but IgG or IgA-bearing B cells could not be seen, Furthermore, there was no plasma cells seen in the bone marrow smear and the antigen-stimulated lymph node, C3 and C4 in the synovial fluid were decreased and only mononuclear cells but no plasma cells were seen in the synovial membrane. After gammaglobulin replacement (100mg/Kg/3 weeks), the arthritis moderately improved with regard to the range of motion and morning stiffness and the frequency of respiratory tract infection also much decreased.

並列摘要

A case of primary hypogammaglobulinemia complicated with rheumatoid-like arthritis is reported. The patient was a sx-yearolc1 boy who had repeated bacterial infections since 11 months of age, Feeder and painful swelling of the left knee joint with morning stiffness and limitation of motion developed when he was 2 years old and persisted until now. Immunological studies revealed abnormally low serum immunoglobulins (IgG 275mg/dl, IgA＜4.5m/dl, IgM 52mg/dl, IgE 11.5IU/ml), absence of anti-A isohemagglutinin (patient was B group) and inability to mount the specific antibody response to Salmonella typhosa after vaccination with typhoid vaccine Although B cell was detectable in hi circulating blood by EAC rosette, surface immunoglobulins and OKIal monoclonal antibody in vitro immunogiobulin biosynthesis study showed that it was mainly due to the intrinsic defect of B cell to synthesize adequate a1nouut of the immunoglobulins, Normal number of 1gM-bearing B cell was found in the mucosa of the small intestine, but IgG or IgA-bearing B cells could not be seen, Furthermore, there was no plasma cells seen in the bone marrow smear and the antigen-stimulated lymph node, C3 and C4 in the synovial fluid were decreased and only mononuclear cells but no plasma cells were seen in the synovial membrane. After gammaglobulin replacement (100mg/Kg/3 weeks), the arthritis moderately improved with regard to the range of motion and morning stiffness and the frequency of respiratory tract infection also much decreased.

並列關鍵字

hypogammaglobulinemia ； primary ； rheumatoid-like arthritis