Rosai-Dorfman disease

3  Atlas of Rare Lymphomas

3.4  Rosai-Dorfman disease

Clinical signs:

One year later she came in with relaps in the upper jaw/paranasal region and in the splanchnocranium. Furhermore, two cystic lesion in the right lung of uncertain significance were found on the high resolution CT scan.

Considering the possibility of Erdheim-Chester disease (possible lung involvement), the diagnostic slides were send to Dr. Ronald Jaffe, Children’s Hospital of Pittsburgh for second opinion. Diagnosis of relapsing Rosai-Dorfman disease was confirmed.


Our patient has started chemotherapy according to LCH III. course 1+2 + continuation treatment).


Sheets of (sometimes atypical) histiocytes in mixture with lymphocytes.


The case was closed as recurrency of Rosai-Dorfman disease.


Lymph nodes from the relapse of the disease:
Rosai-Dorfman syndrome, HE 60x (74063)

Rosai-Dorfman syndrome, HE 60x (74064)

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