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[NEJM临床医学影像]:雨滴头骨
2018年06月02日 临床影像, 临床话题 暂无评论

IMAGES IN CLINICAL MEDICINE

Raindrop Skull

Fabio Solis, Cesar Gonzalez

N Engl J Med 2018; 378:1930

DOI: 10.1056/NEJMicm1714471

A 46-year-old man presented to the emergency department with a 1-month history of fatigue, shortness of breath, and low back pain and report of a weight loss of 30 kg over the previous 10 months. On physical examination, his conjunctiva and palms were pale. Laboratory evaluation revealed a hemoglobin level of 4.9 g per deciliter (reference range, 12 to 16), a creatinine level of 5.4 mg per deciliter (480 μmol per liter; reference range, 0.4 to 1.0 mg per deciliter [35 to 88 μmol per liter]), and a calcium level of 12 mg per deciliter (3 mmol per liter; reference range, 8.9 to 10.3 mg per deciliter [2.2 to 2.6 mmol per liter]). A skeletal survey showed diffuse osteopenia, a pubic bone fracture, and numerous radiolucent lesions on the skull, which had an appearance known as “raindrop skull” — a pattern of lytic or punched-out lesions that resemble raindrops hitting a surface and splashing. These findings are characteristic of multiple myeloma. The diagnosis was confirmed by bone marrow biopsy and aspirate samples that showed 50% clonal plasma cells in the marrow; serum protein electrophoresis revealed a monoclonal IgA paraprotein. Initial management included red-cell transfusion for treatment of anemia and hydration to correct hypercalcemia. The patient did not respond well to induction therapy and died 3 months after diagnosis.

一名46岁男性患者因乏力、呼吸困难及腰背疼痛1个月到急诊就诊。患者在既往10个月中体重下降30 kg。体格检查发现,患者结膜与手掌苍白。实验室检查显示,血红蛋白4.9 g/dL(正常范围12-16),肌酐5.4 mg/dL(480 μmol/L;正常范围0.4 - 1.0 mg/dL [35 - 88 μmol/L]),钙 12 mg/dL (3 mmol/L;正常范围 8.9 - 10.3 mg/dL [2.2 - 2.6 mmol/L])。骨骼检查发现弥漫性骨质减少,耻骨骨折,颅骨多处低密度病变,呈“雨滴头骨”表现 — 这是一种溶骨性病变,如同雨滴撞击物品表面后四处飞溅。这些表现是多发骨髓瘤的特征性表现。骨髓活检及骨穿显示骨髓中50%的克隆浆细胞,因而确诊多发骨髓瘤;血清蛋白电泳显示单克隆IgA副蛋白。初始治疗包括输血治疗贫血,水化纠正高钙血症。患者对诱导化疗反应不佳,确诊后3个月去世。

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