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Diagnostic challenges in Rosai-Dorfman-Destombes disease (case description and literature review)

Sheniz Yuzeir, Hinko Varbanov, Snejana Spasova, Liana Gercheva

Abstract

The described clinical case presents 39-year-old patient with no clinically significant accompanying diseases. Since the beginning of October 2015, the patient has complained of fever, abdominal pain and generalized enlarged lymph nodes. Biopsy of axillary lymph node evidences Rosai-Dorfman-Destombes (RDD) disease. Treatment with corticosteroids has been started but with no clear influence on paraclinical activity and lymphadenomegaly. From the additionally made examinations, clinical laboratory data for malignant histiocytosis are found. According to literary data, in 27% of patients suffering from RDD, it transforms into malignant histiocytosis. The presented clinical case is interesting not only because of the registered clinical laboratory data of malignant histiocytosis but for the unclear diagnostic and prognostic importance of this phenomenon, as well.


Keywords

Rosai-Dorfman-Destombes disease, sinus histiocytosis with massive lymphadenomegaly, malignant histiocytosis

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DOI: http://dx.doi.org/10.14748/ssm.v50i2.4418

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About The Authors

Sheniz Yuzeir
Clinic of Hematology University Hospital St. Marina – Varna
Bulgaria

Hinko Varbanov
Clinic of Hematology University Hospital St. Marina – Varna
Bulgaria

Snejana Spasova
Clinic of General and Clinical Pathology, UMHAT“Sveta Marina’’,Varna
Bulgaria

Liana Gercheva
Clinic of Hematology University Hospital St. Marina – Varna
Bulgaria

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