Cancer Res Treat.  2016 Jan;48(1):415-421. 10.4143/crt.2014.160.

A Unique Case of Erdheim-Chester Disease with Axial Skeleton, Lymph Node, and Bone Marrow Involvement

• Lim J
• Kim KH
• Suh KJ
• Yoh KA
• Moon JY
• Kim JE
• Roh EY
• Choi IS
• Kim JS
• Park JH

• KMID: 2152302
• DOI: http://doi.org/10.4143/crt.2014.160

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.

Keyword

Non-Langerhans-cell histiocytosis; Cytopenia; Axial skeleton; Lymph nodes

MeSH Terms

Aged
Anemia
Biopsy
Bone Marrow*
Chronic Disease
Dyspnea
Erdheim-Chester Disease*
Histiocytosis, Non-Langerhans-Cell
Humans
Hypertension
Hypothyroidism
Kidney Failure, Chronic
Low Back Pain
Lymph Nodes*
Skeleton*

Figure

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  Fig. 1. Result of bone marrow examination showed slightly increased foamy histiocytosis (H&E staining, ×200).

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  Fig. 2. Magnetic resonance imaging of the spine showed compression fractures of the T5 (A), L3, and L4 (B) vertebral bodies on a T1-weighted image. Involvement of the metaphysis and diaphysis, with relative sparing of the epiphysis, was observed.

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  Fig. 3. (A, B) Compression fractures were observed by a computed tomography scan of the patient’s chest at T5, T7-8, T12, and L3-4 spines. (C) In addition, computed tomography imaging identified cardiomegaly and multiple prominent lymph nodes in both axillae. (D) Irregular sclerotic change was observed in both femur necks.

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  Fig. 4. Histopathological examination of axillary lymph nodes revealed diffuse infiltration of lipid-laden histiocytes (A, H&E staining, ×200; B, H&E staining, ×400).

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  Fig. 5. Immunohistochemical examination of the previous bone marrow biopsy in March 2009 showed that the infiltrating histiocytes were positive for CD68 (A) and negative for S100, CD1a (B, C), and BRAF (D).

Reference

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