Luong Tai C, Scrigni Adriana, Paglia Marcela, Garavaglia Mariano, Aisenberg Nuria, Rowensztein Hernán, Sampor Claudia
Servicio de Clínica Médica, Hospital de Pediatría Juan P. Garrahan, Ciudad Autónoma de Buenos Aires, Argentina.
Servicio de Hematooncología, Hospital de Pediatría Juan P. Garrahan, Ciudad Autónoma de Buenos Aires, Argentina.
Arch Argent Pediatr. 2016 Aug 1;114(4):e256-9. doi: 10.5546/aap.2016.e256.
Langerhans cell histiocytosis is a heterogeneous disease of unknown etiology characterized by proliferation of Langerhans cells. It is a rare disease. Bone involvement is common but vertebral disease is rare. We present a 4 year old patient with abdominal pain and neurologic symptoms. Magnetic resonance showed vertebra plana in D9 with involvement of paravertebral soft tissues. The child underwent surgery for decompression and biopsy. Biopsy confirmed Langerhans cell histiocytosis. She was treated with vinblastine and prednisone during 6 months following LCH-III with complete recovery of neurologic symptoms.
Langerhans cell histiocytosis is a differential diagnosis in a radiograph with vertebra plana or collapse of vertebral body.
朗格汉斯细胞组织细胞增多症是一种病因不明的异质性疾病,其特征为朗格汉斯细胞增殖。这是一种罕见疾病。骨骼受累常见,但脊椎疾病罕见。我们报告一名4岁有腹痛和神经症状的患者。磁共振成像显示第9胸椎椎体扁平并累及椎旁软组织。该患儿接受了减压手术和活检。活检确诊为朗格汉斯细胞组织细胞增多症。她在采用LCH-III治疗方案后的6个月内接受了长春花碱和泼尼松治疗,神经症状完全恢复。
在X线片显示椎体扁平或椎体塌陷时,朗格汉斯细胞组织细胞增多症是一种鉴别诊断。