胃シグネット環状細胞腺癌に続く抗リン脂質症候群の一例

A Case of Antiphospholipid Syndrome Following Gastric Signet Ring Cell Adenocarcinoma.

• Mohammad Shayestehpour
• Majid Ehsani
• Davood Dadkhah
• Batool Zamani

抄録

背景 抗リン脂質症候群（APS）は、動脈血栓症、静脈血栓症、小血管血栓症、妊娠に伴う罹患率、抗カルジオリピン抗体や抗β2糖タンパク質Iなどの抗リン脂質抗体の存在を特徴とするまれな自己免疫疾患である。CASE REPORT 53歳女性が入院6ヵ月前から疼痛と左下肢浮腫を呈した。ドップラー超音波検査で眼窩静脈に深部静脈血栓症（DVT）と診断され，ヘパリンとワルファリンの投与を受けた．硬膜下血腫の後，抗凝固療法を中止し，開頭手術を行った。1ヵ月後に右足に痛みとDVTと診断されて再来院した。臨床検査では、ループス抗凝固抗体、IgM抗体、IgG抗体が高値を示した。アルカリホスファターゼ高値の後、全身の骨スキャンでびまん性の骨髄病変が認められた。原発性腫瘍を探していたところ、内視鏡画像で胃内に大きな濾過可能な病変が認められ、生検組織検査ではシグネット環細胞腺癌と診断され、化学療法を拒否して死去した。患者は化学療法を拒否し、診断から6ヵ月後に死亡した。結論 APSは胃シネット環状細胞腺癌と関連している。

BACKGROUND Antiphospholipid syndrome (APS) is a rare autoimmune disease characterized by arterial, venous, and small-vessel thrombosis, pregnancy-related morbidity and the presence of antiphospholipid antibodies such as anticardiolipin antibody, and/or anti-beta2-glycoprotein I. In the recent years, APS was observed in patients with solid tumors and the renal cancer, lung carcinoma and breast tumors were the most common tumors linked with APS. CASE REPORT A 53-year-old female presented with pain and pitting edema of left lower extremity that had begun 6 months prior to hospitalization. Deep vein thrombosis (DVT) in the popliteal vein diagnosed by Doppler ultrasonography and the patient was treated with heparin followed by warfarin. Following subdural hematoma, anticoagulant therapy was stopped, and the patient underwent craniotomy. One month later, the patient returned with pain and DVT diagnosed in its right leg. Laboratory tests showed high levels of lupus anticoagulant, IgM and IgG anticardiolipin antibodies. Following a high alkaline phosphatase, diffuse bone marrow involvement was found by whole body bone scan. Looking to find primary tumor, a large infilterable lesion in gastric was seen by endoscopic images, and biopsy histopathology showed a signet ring cell adenocarcinoma. The patient refused chemotherapy and died 6 months after diagnosis. CONCLUSIONS APS is associated with gastric signet ring cell adenocarcinoma.