臨床血液
Online ISSN : 1882-0824
Print ISSN : 0485-1439
ISSN-L : 0485-1439
症例
高IgE血症を伴つた免疫グロブリン・フラグメント病の1例
谷内 昭赤保内 良和坂本 真一和田 武雄森 道夫
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ジャーナル 認証あり

1974 年 15 巻 11 号 p. 1238-1247

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A 72-year-old male patient with immunoglobulin fragment disease accompanied by a high serum IgE level was reported. He was admitted on June, 1971, with chief complaints of systemic lymph node swelling and bronchial asthma.
Laboratory examinations revealed relative lymphocytosis with a few lymphoblastoid cells and lymphoid cells (an appearance of reticulum cells) in the peripheral blood and in the sternal marrow aspirate the same types of cells increased in number up to 40%. Cellulose-acetate electrophoresis showed diffuse hypergammaglobulinemia with simultaneous increase of IgG, IgA and IgM without the M-component. Moreover, serum IgE was detectable in Ouchterlony diffusion and immunoelectrophoresis and gave measurements of 3.2 mg/dl by the single radial immunodiffusion method. Urinalysis disclosed massive excretion of the κ-chain, λ-chain and Fc like fragments and only the κ-chain showed M-component in starch gel immunoelectrophoresis. Biopsy of the cervical lymph node revealed marked proliferation of lymphoid cells and plasmocytes, some were stained with fluorescence conjugated ε-chain antiserum, other were stained with either fluorescence conjugated κ-chain or λ-chain antiserum.
Prednisolone successfully controlled the complaints, but the patient passed away on Jan. 30, 1973, from acute pneumonia with recurring bronchitis.
Postmortem examination revealed the proliferation of lymphocytes, lymphoid cells and reticulum cells in the lymph nodes, which had penetrated into the neighboring fatty tissue of some lymph nodes. On the other hand, the proliferation of plasmocytes were not so prominent.
From these findings it was postulated that this case might come under immunoproliferative syndrome and some discussions were made on immunoglobulin fragment diseases in the literature.
著者関連情報
© 1974 一般社団法人 日本血液学会
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