Open AccessA Rare Case of Rosai-Dorfman Disease in an Adult Male Associated with Auto-immune Hemolytic Anemia.
Author(s) -
Mickey Sachdeva,
Haifaa Abdulhaq
Publication year - 2013
Publication title -
mediterranean journal of hematology and infectious diseases
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.682
H-Index - 31
ISSN - 2035-3006
DOI - 10.4084/mjhid.2013.022
Subject(s) - medicine , rosai–dorfman disease , immune system , hemolytic anemia , disease , anemia , rare disease , immunology , pathology
Rosai-Dorfman disease (RDD) is a rare benign histiocytic proliferative disorder predominantly of the lymph nodes, which mostly occurs in children and young adults typically presenting with lymphadenopathy. Our case is of a 63 year-old African-American male who presented with subjective fever, weight loss, bilateral axillary and inguinal lymphadenopathy as well as auto-immune hemolytic anemia. The histological analysis showed emperipolesis and histiocytes that were positive for S-100 and CD-68 consistent with RDD. After steroid treatment and splenectomy, patient’s symptoms and hemolytic anemia had resolved. Our case is the first case of RDD reported to be associated with auto-immune hemolytic anemia in an adult.