title: A Unique Case of Erdheim-Chester Disease with Axial Skeleton, Lymph Node, and Bone Marrow Involvement.
abstract: Erdheim-Chester disease is a rare non-Langerhans-cell histiocytosis with bone and organ involvement. A 76-year-old man presented with low back pain and a history of visits for exertional dyspnea. We diagnosed him with anemia of chronic disease, cytopenia related to chronic illness, chronic renal failure due to hypertension, and hypothyroidism. However, we could not determine a definite cause or explanation for the cytopenia. Multiple osteosclerotic axial skeleton lesions and axillary lymph node enlargement were detected by computed tomography. Bone marrow biopsy revealed histiocytic infiltration, which was CD68-positive and CD1a-negative. This report describes an unusual presentation of Erdheim-Chester disease involving the bone marrow, axial skeleton, and lymph nodes.
authors.affiliation: Division of Hematology and Medical Oncology, Department of Internal Medicine, Seoul National University Hospital, Seoul, Korea.
authors.forename: Jin
authors.initials: J
authors.lastname: Lim
authors.affiliation: Division of Hematology and Medical Oncology, Department of Internal Medicine, SMG-SNU Boramae Medical Center, Seoul, Korea.
authors.forename: Ki Hwan
authors.initials: KH
authors.lastname: Kim
authors.affiliation: Division of Hematology and Medical Oncology, Department of Internal Medicine, Seoul National University Hospital, Seoul, Korea.
authors.forename: Koung Jin
authors.initials: KJ
authors.lastname: Suh
authors.affiliation: Division of Hematology and Medical Oncology, Department of Internal Medicine, Seoul National University Hospital, Seoul, Korea.
authors.forename: Kyung Ah
authors.initials: KA
authors.lastname: Yoh
authors.affiliation: Division of Rheumatology, Department of Internal Medicine, Seoul National University Hospital, Seoul, Korea.
authors.forename: Jin Young
authors.initials: JY
authors.lastname: Moon
authors.affiliation: Department of Pathology, SMG-SNU Boramae Medical Center, Seoul, Korea.
authors.forename: Ji Eun
authors.initials: JE
authors.lastname: Kim
authors.affiliation: Department of Laboratory Medicine, SMG-SNU Boramae Medical Center, Seoul, Korea.
authors.forename: Eun Youn
authors.initials: EY
authors.lastname: Roh
authors.affiliation: Division of Hematology and Medical Oncology, Department of Internal Medicine, SMG-SNU Boramae Medical Center, Seoul, Korea.
authors.forename: In Sil
authors.initials: IS
authors.lastname: Choi
authors.affiliation: Division of Hematology and Medical Oncology, Department of Internal Medicine, SMG-SNU Boramae Medical Center, Seoul, Korea.
authors.forename: Jin-Soo
authors.initials: JS
authors.lastname: Kim
authors.affiliation: Division of Hematology and Medical Oncology, Department of Internal Medicine, SMG-SNU Boramae Medical Center, Seoul, Korea.
authors.forename: Jin Hyun
authors.initials: JH
authors.lastname: Park
mesh_terms: D000368:Aged; D001853:Bone Marrow / Q000473:pathology*; D001842:Bone and Bones / Q000000981:diagnostic imaging / Q000473:pathology*; D003937:Diagnosis, Differential; D031249:Erdheim-Chester Disease / Q000175:diagnosis / Q000473:pathology*; D006646:Histiocytosis, Langerhans-Cell; D006801:Humans; D008198:Lymph Nodes / Q000000981:diagnostic imaging / Q000473:pathology*; D008297:Male
keywords: Axial skeleton; Cytopenia; Lymph nodes; Non-Langerhans-cell histiocytosis