Pyroglobulin，Cryoglobulinがともに陽性でかつ著明なHyperviscosity Syndromeを呈した原発性マクログロブリン血症の1剖検例

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タイトル別名
  • An Autopsy Case of Waldenström's Macroglobulinemia with Pyroglobulin, Cryoglobulin and Hyperviscosity Syndrome
  • Pyroglobulin,Cryoglobulinがともに陽性でかつ著明なHyperviscosity Syndromeを呈した原発性マイクログロブリン血症の1剖検例
  • Pyroglobulin Cryoglobulin ガ トモニ ヨウセイ デ

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A 76 year-old man was admitted with complaints of nasal bleeding, Raynaud's symptome and dyspnea in the cold, which were related to serum hyperviscosity. Physical examination demonstrated neither lymphadenopathy nor hepatosplenomegaly, but large lymphoid cells were increased in the peripheral blood and bone marrow, most of those lymphoid cells carried the monoclonal IgM (λ) on the surface. The patient serum showed hyperproteinemia (8.2 g/dl) and positive Sia test. Pathological immunoglobulin was identified as IgM (λ). Bence Jones protein in urine was also demonstrated.<br>Serum viscosity was as high as 111 times water at room temperature by simplified method using white blood cell pipette. Its viscosity was increased at 0∼4°C. Both pyroglobulin and cryoglobulin were demonstated in the serum, serum gelation was occured upon heating to 56°C over 60 minutes.<br>When pyroglobulin and cryoglobulin were removed from the serum, the M component decreased of the resulting supernatant, but it was found in the cryoglobulin. Further examination showed that cryoglobulin consists of mixtuers of monoclonal IgM (λ) and IgG. Plasmapheresis of about 1,600 ml/week was effective in reducing the hyperviscosity syndrome.<br>After 7 months from the onset, the patient died of pneumonia and autopsy was done. Autopsy showed that the cause of death was cytomegalovirus pneumonia. Diffuse lymphoplasmacytoid cell proliferation was in the bone marrow but not in the spleen, liver and lymph nodes. “Multiple lymphatic thrombosis” was demontrated, thought to be related to serum hyperviscosity.

収録刊行物

  • 臨床血液

    臨床血液 21 (2), 240-250, 1980

    一般社団法人 日本血液学会

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