Ann Lab Med.  2012 Mar;32(2):158-161. 10.3343/alm.2012.32.2.158.

A Case of Rosai-Dorfman Disease with Highly Elevated Serum Ferritin

• Rew SY
• Jang HC
• Park KH
• Ahn JS
• Kim GE
• Choi YD
• Jung SI

• KMID: 1245257
• DOI: http://doi.org/10.3343/alm.2012.32.2.158

Abstract

Sinus histiocytosis with massive lymphadenopathy, also known as Rosai-Dorfman disease is a rare disorder characterized by proliferation of distinctive histiocytes within lymph node sinuses and lymphatics, sometimes involving extranodal sites. However, clinical suspicion is difficult and there is also a lack of useful diagnostic markers for this disorder prior to histological confirmation. High elevation of serum ferritin is known to be a useful diagnostic marker for various hematologic diseases, including hemophagocytic lymphohistiocytosis and lymphoma. Here, we report a case of fever of unknown origin that presented along with highly elevated serum ferritin (5,780 ng/mL), and was finally diagnosed as Rosai-Dorfman disease by lymph node biopsy.

Keyword

Ferritin; Fever of unknown origin; Sinus histiocytosis with massive lymphadenopathy; Rosai-Dorfman disease

MeSH Terms

Adult
C-Reactive Protein/analysis
Female
Ferritins/*blood
Histiocytosis, Sinus/blood/*diagnosis/pathology
Humans
L-Lactate Dehydrogenase/blood
Lymph Nodes/pathology
Positron-Emission Tomography and Computed Tomography

Figure

• 

  Fig. 1 (A) PET/CT scan. Diffuse hypermetabolism in the spleen (maximum standardized uptake value, 4.6) and hypermetabolism in the neck, inguinal, and intra-abdominal lymph nodes (maximum standardized uptake value, 8.4-10.6) were observed. (B) Histological results of a right inguinal lymph node biopsy. Left upper image: Prominent wide sinuses are observed (shown in the circled area) (H&E stain,×100). Right upper image: These sinuses are composed of histiocytes, lymphocytes, and plasma cells. Histiocytes engulfing lymphocytes are noted (emperiopolesis, indicated by arrow) (H&E stain,×400). Left lower image: On immunohistochemistry, the inter-digitating dendritic cells are positive for S-100 (×100). Right lower image: Biopsied tissue stains negative for CD1a immunohistochemical stain (×100). Lymph node tissue also had cells positive for CD68 and CD163 (antigens positive for monocytes/macrophages). Abbreviations: PET/CT, positron emission tomography-computed tomography; CD, cluster of differentiation.

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  Fig. 2 Serum LDH, ferritin, and CRP were highly elevated initially, rapidly decreased during the initial 2 weeks of hospitalization, and then normalized gradually over time. Abbreviations: LDH, lactate dehydrogenase; CRP, C-reactive protein.

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