PDF(Pubmed)
Abstract：
A woman in her 70s with vague gastrointestinal (GI) symptoms and unintentional weight loss was referred to endoscopy clinic for investigation and consideration of GI malignancy. CT of the thorax, abdomen and pelvis showed a suspicious mass in the oesophago-gastric junction with a lytic lesion on S1-S2 sacrum. A subsequent upper GI endoscopy revealed two raised, ulcerated tumours on the lesser curvature of the stomach. By the time an MRI of the whole spine was done which revealed multiple metastases involving thoracic, lumbar and sacral skeleton, she had developed leg weakness and paraesthesias, consistent with the imaging findings. A positron emission tomography/CT scan further confirmed the above findings. The initial working diagnosis was primary GI tumour with bony metastases. However, she was later referred to the haematology team after the immunohistochemistry of the tumour showed that it was of a plasma cell origin (CD138 positive) associated with lambda light chain deposits. Serum-free light chain showed a raised lambda light chain of 272 mg/L and kappa light chain of 11.3 mg/L and involved/uninvolved light chain ratio of 24. Bone marrow biopsy confirmed a plasma cell myeloma with moderate disease burden. Monoclonal lambda chains were demonstrated on immunofixation but negative on serum protein electrophoresis and hence a diagnosis of oligosecretory myeloma with GI involvement was made. Subsequent management involved physiotherapy, pain management and chemotherapy, where this woman was commenced on Velcade (generically known as bortezomib), thalidomide and dexamethasone and she continued to experience clinical and biochemical improvement.
摘要：
一名70多岁，胃肠道（GI）症状模糊且无意中体重减轻的妇女被转诊到内窥镜检查诊所进行胃肠道恶性肿瘤的调查和考虑。胸部CT,腹部和骨盆在食管-胃交界处显示可疑肿块，在S1-S2骶骨上有溶解性病变。随后的上消化道内窥镜检查显示两个凸起，胃的小曲率上的溃疡肿瘤。到整个脊柱的MRI检查时，发现涉及胸部的多个转移瘤，腰椎和骶骨，她出现了腿部无力和感觉异常，与影像学检查结果一致。正电子发射断层扫描/CT扫描进一步证实了上述发现。最初的工作诊断是原发性胃肠道肿瘤伴骨转移。然而,在肿瘤的免疫组织化学显示它是与λ轻链沉积相关的浆细胞来源(CD138阳性)后，她后来被转诊到血液学小组。无血清轻链显示出272mg/L的升高的λ轻链和11.3mg/L的κ轻链，涉及/未涉及的轻链比率为24。骨髓活检证实浆细胞骨髓瘤具有中度疾病负担。单克隆λ链在免疫固定上得到证实，但在血清蛋白电泳上呈阴性，因此诊断为胃肠道受累的少分泌性骨髓瘤。随后的管理包括物理治疗，疼痛管理和化疗，这个女人在Velcade（通常称为硼替佐米）上开始，沙利度胺和地塞米松，她继续经历临床和生化改善。
