Indus Valley Times

53-year-old male is the first patient in India who was diagnosed with Rosai-Dorfman Disease; a rare immune disorder

There are only 8 cases of Rosai-Dorfman Heart Disease reported so far across the globe

Kolkata / Bhubaneswar , Dec 1: Fortis Hospital Anandpur has successfully treated India’s first reported case of Rosai-Dorfman Heart Disease. Dr. K. M. Mandana, Director, Department of Cardiothoracic and Vascular Surgery, Fortis Anandapur, treated this rare and challenging case. Rosai-Dorfman Disease (RDD) is a rare disorder of the immune system, which presents as a massive enlargement of the lymph nodes (lymphadenopathy) due to overproduction and accumulation of a specialized type of white blood cells called Histiocytes. RDD can happen in patients irrespective of age or gender, although some people have a genetic predisposition to contract the condition. The exact cause of this disease is still unknown.

The 53-year-old male patient was presented at Fortis Anandapur with recurrent pericardial effusion (fluid buildup) leading to swelling in the eyes, bloated face and shortness of breath due to the repeated collection of fluid around the heart and pericardial constriction. The patient’s eyes were protruding out of the eye sockets. The fluid was repeatedly being drained after hospitalization in various hospitals, where the patient had consulted earlier. Upon admission at Fortis Anandapur, the lesion in his eyes was detected by computed tomography during examination.  Further, a Biopsy was conducted of tissues behind the eyes, which revealed enlarged lymph nodes. A high-resolution CT-Scan also found a nodule in the kidney and right thyroid, after which he was diagnosed with Rosai- Dorfman disease.

According to Dr. K.M. Mandana, “This is the first reported case of Rosai-Dorfman Disease (RDD)in India that involves the heart. Currently, there are only eight reported cases in the world affecting the eye and the heart. Due to the rare reported occurrence of RDD in unusual cardiac sites and the appearance of a histiocytic fibro lesion, the patient underwent a cardiac surgery for a recurrent collection of fluid around the heart. However, as he was suffering from Rosai Dorfman’s Disease, the surgeons noticed the infiltration of the lymphatic tissue in the heart and the pericardium involvement during surgery. The surgery was successful and the patient is currently under observation and doing well.”

Dr Mandana elaborated further, “There is no permanent cure for this disease as it’s a genetic predisposition. So long-term steroid medication therapy is required (for at least ten years) for patients suffering from RDD. This treatment usually has no side effects as steroids are administered in small dosages. A surgery was conducted to remove the pericardium(pericardium is a fibrous sac that encloses the heart and great vessels)and steroid medication, the patient’s symptoms were suppressed, and his health has substantially improved.”