Author: Dr. Vibha Naik, Dr. Manoj Mehta & Dr. C Shrinivasan

Clinical History

A 45 years old Female with bilateral cervical lymphadenopathy since 2 months came with the history Of Fever off & on patient had same complains one year back was treated with AKT and Underwent excision biopsy.

Imprint cytology showing emperipolesis

RDD:
Rosai–Dorfman disease (RDD) is a benign proliferative disorder of histiocytes. Here, the involved histiocytes exhibit emperipolesis of lymphocytes, plasma cells,neutrophils and red blood cells. Emperipolesis in a lymphnode with mixed inflammatory infiltrate is a feature suggestive of RDD.

Above two figures showing Sinus histiocytosis.

This figure showing emperipolesis in the histology section.

RDD is a rare condition in which OCT2 is 100% Positive.

Rare non Langerhans cell histiocytosis characterized by accumulation of activated histiocytes within affected tissues. It was first described in 1965 by a French pathologist Pierre Paul Louis. Four years later Juan Rosai and Ronald Dorfman analyzed 34 cases of the same entity under the name Sinus Histiocytosis with massive splenomegaly. It is a self-limiting disorder of unknown etiology.

Patients with classical RDD present with bilateral lymphadenopathy but 43% of the cases present with extra nodal disease. It can occur as an isolated disorder or in association with autoimmune, hereditary or malignant diseases .It is a rare disease with a prevalence of 1:200000 more common in children and young adults. Mean age is 20.6 years but can occur up to 75 years, more common in males with African descent and cutaneous form is common in Asian females. Studies have associated RDD with viral infections viz. Herpes, EBV, CMV and HIV.

H syndrome comprises disorders with RDD and hypertrichotic dermatosis with Insulin dependent,diabetes, ,autoimmune lymphoproliferative syndrome (ALPS) may be associated with malignancies viz. Hodgkin’s lymphoma, MDS and cutaneous clear cell sarcoma. 

RDD coexists with immunologic diseases in 10% cases viz. SLE, Idiopathic juvenile arthritis, AIHA Patients present with bilateral massive lymphadenopathy with or without intermittent fever, night sweats and weight loss, Mediastinal, axillary and inguinal nodes may be involved but retroperitoneal nodes are uncommon.

Extra nodal RDD in 43% cases Blood picture is normocytic normochromic anemia, leukocytosis, thrombocytopenia, and eosinophilia and raised ESR Treatment ranges from observation for uncomplicated Adenopathy Symptomatic RDD is treated with oral steroids – Prednisolone 1mg/ kg and then gradually tapered.

Refractory symptomatic RDD is treated with Sirolimus 2.5mg/m2/day for 18 months and then tapered over 6 months or radiotherapy.

Our patient was treated with oral steroids over 6 weeks and completely recovered.