A Case of Sjogren Syndrome Diagnosed by Purpura on Lower Extremities Presenting Grape Cells in the Bone Marrow

semanticscholar(2010)

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Abstract
Sjogren syndrome (SJS) in childhood rarely presents a symptom of xerosis of mouth or eye. Usually the patients of SJS in childhood show recurrent parotitis or asymptomatic hypergammaglobulinemia. We report a 14-yearold-girl with recurrent purpura and erythematous rash on her lower extremities. Serum IgG was 3,724 mg/dl, anti SS-A and SS-B antibodies were positive. Biopsy on labial glands showing typical infiltration of numerous lymphocytes confirmed the diagnosis of SJS. A bone marrow specimen showed grape cells. Grape cells had been reported to be found in multiple myeloma or plasma cell neoplasms of adulthood. Grape cells reported in childhood are, to our knowledge, extremely rare. Accelerated production of immunoglobulin in autoimmune disease was considered to be the cause of the presence of grape cells. 要 旨 小児 の シェーグ レン症 候群 においては,唾 液腺や涙腺 な どの乾燥症状 を示 す ことはまれであ る.通 常,反 復性 耳下腺腫 脹や無症 状の高 ガ ンマ グロ ブ リン血症 な どを呈 す る.わ れわれの報告 す る症例 は14歳 女 児で,反 復 す る下肢 の紫斑.丘 疹 状皮疹 を呈 してい た.血 清IgGが3,724mg/dlで,抗SS-A, SS-B抗 体 が陽性で あ った.口 唇腺 の生検 によ り典型 的な多数の リンパ球浸 潤を認 め シェー グレン症候群 と確定診断 し た.骨 髄像 ではgrape cellが み られ た. Grape cellは成人 の多発性骨 髄腫 など形質細 胞性腫瘍 においてみ られ る.小 児 にお けるgrape cellの 報告 は,わ れわ れの検索 しえた限 りでは きわ めてまれで あ る.自 己免 疫疾患 にお ける免疫 グ ロブ リン産生 亢進がgrape cellの原因 と考 え られた.
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