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Revista Habanera de Ciencias Médicas

On-line version ISSN 1729-519X

Abstract

ALIAGA-CHAVEZ, Rolig A.; BENDEZU-HUASASQUICHE, Luis E.  and  CRISOL-DEZA, Diego A.. Successful treatment of Rosai-Dorfman disease with immunomodulators and metronomic chemotherapy. Rev haban cienc méd [online]. 2022, vol.21, n.2, e4457.  Epub May 10, 2022. ISSN 1729-519X.

Introduction:

Rosai-Dorfman disease is a massive lymphocytic histiocytosis characterized by emperipolesis with positive immunohistochemistry for S100 and CD68. It is a rare clinical entity of benign course most commonly presenting in males.

Objective:

The purpose of this clinical case is to demonstrate successful treatment of Rosai-Dorfman disease with immunomodulators and metronomic chemotherapy.

Case presentation:

A 57-year-old male patient presenting with bilateral cervical lymphadenopathy underwent CT scan and lymph node biopsy with positive results for S100 and CD68 with marked emperipolesis phenomenon. He received immunomodulatory scheme and successful metronomic chemotherapy with disease remission.

Conclusions:

Patients diagnosed with Rosai-Dorfman are very few due to the unusual nature of this clinical entity. It is important to mention that this disease is a non-Langerhans cell histiocytosis with benign characteristics and good response to management with corticosteroids and metronomic chemotherapy, therapy established in our patient with good evolution.

Keywords : Rosai-Dorfman disease; Histiocytosis; Non-Langerhans-Cell; Emperipolesis.

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