Li Yanchu, Shi Changle, Wu Yu, He Mingmin, Xia Xueming, Liu Jie, Jiang Yu
Department of Head and Neck Oncology, West China Hospital of Sichuan University, Chengdu, China.
Department of Pathology, West China Hospital of Sichuan University, Chengdu, China.
Front Med (Lausanne). 2022 May 19;9:840407. doi: 10.3389/fmed.2022.840407. eCollection 2022.
ALK-positive histiocytosis (APH) is a rare and recently described, solitary or generalized, histiocytic proliferative disorder with a characteristic gene translocation involving the fusion of the ALK gene at chromosome 2p23. To date, only 25 cases of APH have been reported. The patient presented with multiple nodules in the lung, liver, gallbladder, pancreas, kidney, and skin rashes, along with recurrent pancreatitis and cholecystitis. The histiocytes from the lesion were positive for CD68 and ALK and negative for S100 and CD1α. A reduced dose of the ALK inhibitor was administered rather than the standard dose of or chemotherapy because of recurrent pancreatitis, which has not been previously reported in APH cases. After 18 months of follow-up, the patient was maintained on , and a partial response (PR) was achieved.
ALK 阳性组织细胞增多症（APH）是一种罕见的、近期才被描述的、单发或全身性的组织细胞增殖性疾病，具有特征性的基因易位，涉及 2p23 染色体上 ALK 基因的融合。迄今为止，仅报道了 25 例 APH 病例。该患者出现肺部、肝脏、胆囊、胰腺、肾脏多发结节及皮疹，伴有复发性胰腺炎和胆囊炎。病变部位的组织细胞 CD68 和 ALK 呈阳性，S100 和 CD1α 呈阴性。由于复发性胰腺炎，给予的是降低剂量的 ALK 抑制剂，而非标准剂量的[药物名称未给出]或化疗，APH 病例中此前尚未有过这种情况的报道。经过 18 个月的随访，患者持续服用[药物名称未给出]，并实现了部分缓解（PR）。
