Dr Adeena D S, Dr Praveena B,Dr C Jayakumar
Department Of Pediatrics
AIMS, Kochi
Six year old male child, 1 st born of a non consanguineous marriage, presented with persistent right cervical lympadenitis and on and off fever of 9 months duration.
Nine months back, history of low grade fever spikes of 1 day duration followed swelling of right sub-mandibular region.
However, the swelling persisted without progression over 1 month for him.
Not associated with tenderness, redness, restriction of neck movements
One month later, it was noted to descend into neck,
FNAC done showed features s/o reactive lymphadenitis.
Peripheral smear – reactive neutrophilia and eosinophilia.
USG neck -Right jugular lymphadenopathy with retained fatty hilum, no caseation, necrosis.
Third month of onset of swelling, developed tenderness, without increse in size
Labs showed CBC10,000with 68%PMN ,ESR – 100 , Mantoux negative. Repeat FNAC was suggestive of reactive lymphadenitis.
May 2024- Excision biopsy was done which was suggestive of reactive follicular hyperplasia with paracortical widening. Possibility of viral induced lymphadenopathy .IHC- Follicular hyperplasia.Child also received 1 month of Inj.Streptomycin (IM) for the same.
H/o on and off low grade fever spikes since last 2 months, fever spikes were mostly in the evening and has increased in frequency for the past 2 weeks. Due to persistence of fever and neck swelling, child was brought here for further evaluation and management.
No h/o night sweats, weight loss, appetite loss, joint or bone pain, cough, jaundice, abdominal pain or respiratory symptoms.
No h/o contact with TB.
H/o contact with animals such as cats, dogs, cattles, hens, parrots.
O/e alert, active and not sick looking.
Vitals stable
Mild pallor +
discrete cervical lymphnodes on right side-non tender, multiple, firm , largest 2.5 x 2 cm
No icterus, cyanosis, clubbing or or edema.
Height-112cm( btw 0&-2SD)
Weight-18.5kg( btw 0 & -2SD)
BMI -14.74 kg/m2( btw 0 & -2SD)
Head to foot examination
Diffuse swelling of 9X5 cm on the right neck with diffuse lymph nodes palpable.
Surgical scar seen.
No axillary, epitrochlear lymph nodes.
Few small inguinal lymph nodes palpable.
Carious teeth on the lower right side.
Systemic examination :
P/A- Soft, non tender,liver palpable 3 cm below RCM with liver span of 10 cm,
Spleen was not palpable., Bowel sounds +
Other systems WNL
DD’s consisdered were:
1.tuberculous lymphadenitis
2.EBV
3.toxoplasma
4.lymphoma
5.sarcoidosis
Labs showed normal counts, Hb 10 g/dl with indices suggestive of microcytic anemia with elevated CRP 100 and ESR 112.Chest xray was within normal limits. LFT/RFT/S.electrolytes and URE was normal.Mantoux done was negative. Gastric aspirate for MTB was negative. Peripheral smear showed microcytic hypochromic anemia, thrombocytosis with no blasts or atypical cells.LDH was normal. To rule out infective causes, IgM and IgG of Brucella, toxoplasma were send and was negative.EBV IgM negative, but IgG was positive. Child was started on IV Ceftazidime after sending relevant cultures. Child had low grade fever spikes during the hospital stay .Pre op serology was negative. Immunoglobulin profile was within normal limits. Serum ACE level send to rule out sarcoidosis was normal. Ophthal evaluation done showed no tubercles or rheumatological features.
ANA IFA was send considering rheumatological causes of the cervical lymphadenopathy, reports awaited.
Child underwent lymphnode excision biopsy, gram stain, culture and MTB panel of biopsy specimen was negative.
Biopsy came as Classic Hodkins Lymphoma-nodular sclerosis.
He underwent PET CT for staging showed Lugano Stage 2 and was started on treatment.
Hodgkins Lymphoma is a malignant process involving the lympho-reticular system, with bimodal age distribution of 15-35 years and after 50 years.Viral agents such as EBV, CMV and HHV 6 have been implicated in the pathogenesis.
Pathognomonic feature is presence of Reed Sternberg cell- large cell with multiple or multilobulated nucleoli, that arises from germinal centre B cells but typically has lost most B cell gene expression and function.Subtypes are Nodular lymphocyte predominant and classical Hodgkins. The latter includes histological subtypes of lymphocyte rich, lymphocyte depletes, mixed cellularity and nodular sclerosis.
Clinical manifestations include-painless, non tender firm rubbery cervical or supraclavicular lymphadenopathy, hepatosplenomegaly , signs of bone marrow infiltration etc.B symptoms presence or absence is important in staging.Treatment consists of combined chemotherapy with or without low dose involved –field radiation therapy.Chemotherapy regimens include – COPP(cyclophosphamide, oncovin, procarbazine and prednisolone) and ABVD( Adriamycin, bleomycin, vincristine, dacarbazine).Newer therapies-anti CD20, anti CD-30,PDL1-blocking antibodies. Relapses are common in the first 3 years and would need management with aggressive chemotherapy or stem cell transplant.