Abstract
Rosai-Dorfman Disease (RDD) is also known as Sinus Histiocytosis of Massive Lymphadenopathy(SHML). Clinically Rosai-Dorfman Disease is characterized by massive, painless, bilateral cervicallymph node enlargement, often mimics lymphoma. Microscopically, it shows dilatation of lymphaticsinuses occupied by numerous lymphocytes and proliferation of histiocytes with abundant paleeosinophilic cytoplasm containing engulfed lymphocytes or plasma cells- emperipolesis.
Highlights
Rosai Dorfman disease (RDD), known as sinus histiocytosis with massive Lymphadenopathy (SHML), is a rare benign self-limiting disorder of histiocytic proliferation that commonly involves lymph nodes.[1]
SHML is mainly characterized by massive, painless, bilateral cervical lymph node enlargement, associated with fever, leukocytosis, elevated erythrocyte sedimentation rate, and polyclonal hypergammaglobulinemia
Fine needle aspiration cytology plays a significant role in primary diagnosis
Summary
Rosai Dorfman disease (RDD), known as sinus histiocytosis with massive Lymphadenopathy (SHML), is a rare benign self-limiting disorder of histiocytic proliferation that commonly involves lymph nodes.[1]. SHML is mainly characterized by massive, painless, bilateral cervical lymph node enlargement, associated with fever, leukocytosis, elevated erythrocyte sedimentation rate, and polyclonal hypergammaglobulinemia. The patient had bilaterally enlarged, soft to firm, mobile, non-tender cervical lymph nodes. On haematological examination her haemoglobin was 7.9 gm/dl, total WBC count 15800/mm, differential count- Neutrophils 48%,lymphocytes 46%,Monocytes 01%,Eosinophils05% and platelet count 4,84,000/ mm.Peripheral smear examination showed microcytic, hypochromic RBCs. Erythrocyte sedimentation rate was 38 mm/hour. They showed histiocytes predominantly engulfing lymphocytes-lymphophagocytosis (Emperipolesis). Based on this characteristic cytomorphology, a diagnosis of RosaiDorfman disease was made
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