Case Presentation
We report a case of a 1 year and 10 months old girl, who is the 5th child born to non- consanguineous parents. She presented with fever and anterior neck swelling for 3 months. Fevers were high grade in nature, non-specific periodicity and relieved by paracetamol. The neck swelling initially started on the right side, gradually increased to involve the whole neck. This was associated with difficulty in swallowing solid foods. She had lost 2 kg over the course of illness. There was no history of difficulty in breathing or chest pains, or night sweats. No history of vomiting, abdominal distensions, painful micturition, headache or convulsions and no TB contact. She had attained normal developmental milestones and had received all immunization according to our national schedule. This was her first admission to the hospital.
On examination she was alert, febrile with temperature spiking from 38 – 39 degrees, some palmar pallor, not jaundiced, not cyanosed, multiple smooth surfaced preauricular, submandibular, anterior and posterior cervical and supraclavicular lymphadenopathy which was firm and matted. [ Figure 1 ] and no hepatosplenomegaly. Other systemic findings were normal. By this time we doubted the diagnosis of Tb adenitis, since she had persistent symptoms despite of completely more than 1 month of Anti Tb treatment.
In the ward a thorough septic work and re- evaluation of TB diagnosis was done. We did a Full blood picture which showed WBC ( leukocytosis) 34.8, Neutrophil 36.2 ( 93.5%), Lymphocytes 0.766 (2.2%), Hb 8.65 MCV 73 MCH 22 Platelets 654 (thrombocytosis). Peripheral smear showed anis poikilocytosis, No blast cells.
She had raised CRP 296, ESR 245, ADA 218 and LDH 295 levels. We also tested for HIV test [ bio line] which was Negative and Gastric aspirate for Gene Expert – Negative
Viral screening for EBV IgM and IgG, CMV IgM and IgG, Herpes I and II IgM and IgG was done to know if there was any viral etiology for the lymphadenopathy. All tests were negative.
We performed 2 Blood cultures one week apart which showed no growth. Similar urine culture also showed no growth. Cerebrospinal fluid analysis had normal protein and glucose levels and culture was normal.
CT scan Head and neck revealed multiple matted supraclavicular, cervical, submandibular and sublingual lymph nodes.
We further did an Abdominal Ultrasound which also revealed multiple para aortic lymph nodes.
At this point we thought, this could be lymphoma. Flow cytometry showed no leukemic infiltration
And fine needle aspiration [FNAC] was done from the left anterior cervical lymph node which was reviewed by a team of haemato-pathologist.
While waiting for FNAC results, she was treated with 3rd generation cephalosporin ( ceftriaxone) or one week, the fevers were controlled with antipyretics (paracetamol) when required.
At this point the fevers and the swelling still persisted, her CRP And ESR continued to raise and there was persistent leukocytosis, neutrophilia and thrombocytosis in her CBC.
After 2 weeks the results of the FNAC revealed partial effacement of lymph nodes architecture by marked expansion of Sinuses by Large Histiocytic cells. Reminiscent granulomatous process and no necrosis. Immunohistochemistry was strong for CD 68 AND S-100 POSITIVITY.CD 1 a – Negative (R/o Langerhans histiocytosis). ZN STAIN was negative.
A diagnosis of ROSAI DORFMANN disease was made.
Anti TB medication and all other antibiotics were stopped. She started Tabs Prednisolone 10 mg twice daily for 4 weeks and then tapered off.
She was followed up for one month, the fevers subsided and the swelling reduced and started feeding well. Currently she is followed up every 3 months to assess the possibility of relapse for at least 2 years She was discharged with proton pump inhibitor (pantoprazole) and Calcium D [calcium Sandoz] supplements once daily to prevent the side effects of prolonged steroid exposure which was gastritis and osteoporosis respectively. The mother was advised to check the baby’s body weight every week and test for random blood glucose at least three times in a week at a nearby health center.