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Riddle in diagnosis: When a boy recovered from rarest of rare disease

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diseaseLymph nodes are important for the proper functioning of the immune system, acting as filters for foreign particles and infectious agents. Lymph nodes are filled with white blood cells that help the body fight infections and diseases. They are everywhere in the body and in plenty especially in the upper part of the body around neck and chest.

Generalised Lymphadenitis means enlargement of more than one group of lymph nodes. It is usually due to infection.  Though not common, lymph nodes may also enlarge due to cancer. In most cases, the cause is a viral disease. Tuberculosis or blood cancer is less common cause.

Generalized lymphadenitis accompanied by weight loss, pain around the affected region, very large nodes and features associated with systemic symptoms which require systematic treatment, are often because of the disease of serious nature.

Kikuchi-Fujimoto disease (KFD)- One of the rarest disease

Ishaan (name changed) a 12-year-old child from Bhopal had a similar complicated case. He had fever and pain around his neck for over a month, swelling over left neck with flushing of face.The ultrasonography (USG) of neck showed multiple LN in cervical region with thickened cortex whereas the USG of abdomen showed enlarged liver. A contrast-enhanced computed tomography (CECT) of neck and chest showed moderate airway narrowing, multiple nodes in between lung and prominent pulmonary arteries. He had two weeks of extreme pain while eating and thus was investigated for a variety of conditions. He was recommended multiple biopsies. Despite these efforts, his condition kept worsening.

The blood culture done in our hospital, showed a kind of bacteria in blood and biopsy revealed that the child was suffering from one of the rarest disease called, Kikuchi-Fujimoto disease (KFD), or histiocytic necrotizing lymphadenitis, the cause of which is not fully understood.

It has been associated with infections and autoimmune disorder. The disease is often described case is “a sheep in wolf’s clothing” wherein the condition can easily be mistaken for fatal condition such as lymphoma, or even adenocarcinoma.

The bacteria in the child’s body causing Kikuchi-Fujimoto disease (KFD), or histiocytic necrotizing lymphadenitis was treated, with resulting decline in size of lymphadenopathy. Post eight weeks of treatment the child recovered.

Resolution of the case took over months. The disease has a recurrence rate of 3-4 per cent. Though the treatment was done with antibiotic medicines and did not require surgery, to reach to the root cause of the disease was complicated for the doctors to detect.

(Disclaimer: The writer is Dr Sunit Singhi, Chairman- Division of Pediatrics, Medanta- The Medicity. The views expressed are personal opinion.)

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