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Bone pathologic fracture revealing an unusual association: coexistence of Langerhans cell histiocytosis with Rosai-Dorfman disease

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ABSTRACT

Background: The coexistence of Rosai-Dorfman disease (RDD) with Langerhans cell histiocytosis (LCH) is very rare, as to date only 17 cases have been reported in the english literature. The pathophysiology of this uncommon co-occurrence still remains enigmatic and a subject of various speculations.

Case presentation: We report a case of a 30-year-old female patient who presented with a pathologic fracture of the left proximal femur. Her medical history was unremarkable, there were no fever, skin lesions, lymphadenopathy or other organomegaly at physical examination. X-ray radiograph of the fractured femur showed an isolated and ill-defined osteolytic lesion. The histopathological analysis of biopsies from this lesion were consistent with a combined RDD-LCH of the bone.

Conclusion: Combined RDD-LCH is a very rare phenomenon, whose pathophysiology still remains unclear and a subject of various speculations.

No MeSH data available.


Mononuclear cells stained strongly positive for CD1a, whereas giant cells with emperipolesis stained negative (black arrows) (a). Some CD1a + cells are round-shaped with oval nuclei, reminiscent to RDD-cells (“Blanks cells” or transitional cells) (b)
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Fig3: Mononuclear cells stained strongly positive for CD1a, whereas giant cells with emperipolesis stained negative (black arrows) (a). Some CD1a + cells are round-shaped with oval nuclei, reminiscent to RDD-cells (“Blanks cells” or transitional cells) (b)

Mentions: After treatment with 10% nitric acid (decalcification), the histological examination of the submitted bone biopsies showed a dense inflammatory infiltrate destroying the bone tissue (Fig. 2a). It consisted of admixed numerous plasma cells, lymphocytes, neutrophils, eosinophils and histiocytes. Prominent emperipolesis figures are seen with large histiocytes that engulf inflammatory cells such as plasma cells, neutrophils and macrophages (Fig. 2b). Beside these emperipolesis figures, there are some large histiocytes with oval nuclei and granular chromatine and inconspicuous nucleoli. Also, in some areas, scattered cells with “bean-shaped” and grooved nuclei are visible. At imunohistochemistry, many mononucleated cells show intense membraneous expression of CD1a, whereas giant multinucleated cells with emperipolesis stain negative (Fig. 3a). Also, some CD1a positive cells are round-shaped with oval nuclei, and a large cytoplasm, morphologically reminiscent of RDD cells (Fig. 3b). These cells have overlapping features between RDD and LCH cells, they have been called “transitional cells” [4]. However, both cell types express CD68 and S-100 protein (Fig. 4). Finally, the diagnosis of combined RDD and LCH has been made. The patient recovered well after surgery (osteosynthesis and curetage) without any sign of the disease.Fig. 2


Bone pathologic fracture revealing an unusual association: coexistence of Langerhans cell histiocytosis with Rosai-Dorfman disease
Mononuclear cells stained strongly positive for CD1a, whereas giant cells with emperipolesis stained negative (black arrows) (a). Some CD1a + cells are round-shaped with oval nuclei, reminiscent to RDD-cells (“Blanks cells” or transitional cells) (b)
© Copyright Policy - OpenAccess
Related In: Results  -  Collection

License 1 - License 2
Show All Figures
getmorefigures.php?uid=PMC5383940&req=5

Fig3: Mononuclear cells stained strongly positive for CD1a, whereas giant cells with emperipolesis stained negative (black arrows) (a). Some CD1a + cells are round-shaped with oval nuclei, reminiscent to RDD-cells (“Blanks cells” or transitional cells) (b)
Mentions: After treatment with 10% nitric acid (decalcification), the histological examination of the submitted bone biopsies showed a dense inflammatory infiltrate destroying the bone tissue (Fig. 2a). It consisted of admixed numerous plasma cells, lymphocytes, neutrophils, eosinophils and histiocytes. Prominent emperipolesis figures are seen with large histiocytes that engulf inflammatory cells such as plasma cells, neutrophils and macrophages (Fig. 2b). Beside these emperipolesis figures, there are some large histiocytes with oval nuclei and granular chromatine and inconspicuous nucleoli. Also, in some areas, scattered cells with “bean-shaped” and grooved nuclei are visible. At imunohistochemistry, many mononucleated cells show intense membraneous expression of CD1a, whereas giant multinucleated cells with emperipolesis stain negative (Fig. 3a). Also, some CD1a positive cells are round-shaped with oval nuclei, and a large cytoplasm, morphologically reminiscent of RDD cells (Fig. 3b). These cells have overlapping features between RDD and LCH cells, they have been called “transitional cells” [4]. However, both cell types express CD68 and S-100 protein (Fig. 4). Finally, the diagnosis of combined RDD and LCH has been made. The patient recovered well after surgery (osteosynthesis and curetage) without any sign of the disease.Fig. 2

View Article: PubMed Central - PubMed

ABSTRACT

Background: The coexistence of Rosai-Dorfman disease (RDD) with Langerhans cell histiocytosis (LCH) is very rare, as to date only 17 cases have been reported in the english literature. The pathophysiology of this uncommon co-occurrence still remains enigmatic and a subject of various speculations.

Case presentation: We report a case of a 30-year-old female patient who presented with a pathologic fracture of the left proximal femur. Her medical history was unremarkable, there were no fever, skin lesions, lymphadenopathy or other organomegaly at physical examination. X-ray radiograph of the fractured femur showed an isolated and ill-defined osteolytic lesion. The histopathological analysis of biopsies from this lesion were consistent with a combined RDD-LCH of the bone.

Conclusion: Combined RDD-LCH is a very rare phenomenon, whose pathophysiology still remains unclear and a subject of various speculations.

No MeSH data available.