Dr Theresa, Dr Praveeena Bhaskar (pediatrics ID), Dr Rema (pediatrics hematology), Dr C Jayakumar, Amrita Institute of Medical Sciences , Kochi
Six years old male child presented with persistent cervical lymphadenitis and on and off fever of 9 months duration. Initially, the swelling was in the submandibular region, later seemed to descend to the neck region, not associated with tenderness, redness, restriction of neck movements.
USG was suggestive of cervical lymphadenopathy.
He underwent 2 FNACs and excision biopsy, which were reported as reactive cervical lymphadenitis.
Child also received 1 month off InjStreptomycin(IM) for the same.
History of on and off low grade fever spikes, persistent since last 2 months,
Fever spikes were mostly in the evening and had increased frequency in the past 2weeks. Due to persistence of fever and neck swelling, child was referred
No history of night sweats, weight loss, joint pain, loss of appetite, cough, jaundice, generalized body ache, haematuria, abdominal pain
Past history:
In November 2023, the child had low grade fever spikes followed swelling in the right submandibular region.
One month later(in December 2023), the swelling was noted to descend into the neck which was ? fluctuant.
FNAC did showed features suggestive of reactive lymphadenitis. Peripheral smear did showed reactive neutrophilia and eosinophilia.
In January 2024, USG neck was did which showed right jugular lymphadenopathy with retained fatty hilum, no caseation, necrosis.
The child was evaluated where Mantoux did was negative. FNAC done was suggestive of reactive lymphadneitis. Repeat USG neck did showed cervical lymphadenopathy
In May 2024- the child underwent excison biopsy which was suggestive of reactive follicular hyperplasia with paracortical widening, possibily viral induced
At admission, child was alert , afebrile with stable vitals. Mild pallor present
Discrete cervical lymph nodes on right side which is non tender, multiple, firm, largest measuring 2.5 x 2cm. No icterus, cyanosis, clubbing, edema
Auxiology was normal. Head to foot examination showed diffuse swelling on the right side with diffuse lymph nodes palpable of size 9 x 5 cm. Few small inguinal lymph nodes palpable. No epitrochlear or axillary lymph nodes
Systemic examination revealed soft , non tender palpable liver 3cm belowe the right costal margin with liver span of 10cm. Spleen not palapbale. Other systemic were within normal limits.
Labs did showed normal counts with indices suggestive of microcytic anemia with elevated CRP and ESR. Chest Xray was normal.
RFT/LFT/Serum electrolytes di were normal. Mantoux and gastric aspirate for gene xpert did were negative.
Peripheral smear did showed microcytic hypochromic anemia, thrombocytosis with no blasts or atypical cause.Investigations to rule out EBV, Toxoplasma Brucella were send and were negative. Child was started on InjCeftazidime after sending blood culture. Child had low grade fever spikes during hospital stay. Pre op serology did was negative. Immunoglobulin profile was within normal limits. Serum ACE level to rule out sarcoidosis was negative. ECHO done to rule out infective endocarditis was normal. Child underwent lymph node excision biopsy, gram stain and MTB panel from biopsy all were negative.
Sections from lymph node showed partial effacement with few reactive follicles in the cortex. Paracortical area and rest of the node shows cells arranged in nodular pattern with thin fibrous septae. There is a polymorphous infiltrate composed of lymphocytes , histiocytes, plasma cells and many large cells. The large cells are mono/binucleate and have moderate cytoplasm and large vesicular nucli with macronucleoli.
Perinodal spread noted.
IHC did showed large cells positive for CD 30, weakly positive for PAX 5 and negative for CD15, CD3, CD20 and ALK.
Background shows CD3 positive lymphocytes and CD20 positive follicles- classical hodgkins lymphoma- nodular sclerosis type
Whole body PET CT did showed abnormal increased FDG uptake noted in following supradiaphragmatic lymph nodes: a) conglomerate nodal mass involving right level II,III,IV and V lymph nodal station compressing the right internal jugular vein at level of body of hyoid bone. B) Multiple discrete bilateral level II lymph nodes. C) multiple discrete bilateral level V lymph nodes and bilateral axillary lymph node
FDG PET CT staging of lymphoma according to Lugano’s classification is stage II
DISCUSSION:
Hodgkins lymphoma is a lymphoproliferative disorder primarly of B cell lineage involving lymph nodes and the lymphatic system. The condition is uncommon below 5 years
Pathology: lymph nodes are the most common tissue on which the diagnosis of hodgkins lymphoma is made. WHO classification has 2 subtypes- nodular lymphocytic predominant hodgkins lymphoma((NLPHL) and the commoner classical hodgkins lymphoma. The hall mark of hodgkins lymphoma is Reed Stern berg cells
Clinical features- Children present with painless cervical or supraclavicular lymphadenopathy, the nodes are firm and rubbery in consistency. Cervical lymph nodes are the most frequent site of primary involvement. About 20-30% patients present with systemic B symptoms , as defined Ann Arbor staging criteria , with fever over 38C, night sweats and unexplained weight loss of more than 10% of body weight at presentation.
Management:
Most children are treated with combination therapy alone or in combination with radiotherapy. AVBD is the preferred front line regimen of hodgkins lymphoma. The dose of radiation therapy ranges between 15 and 25Gy with modifications based on patient age, response to chemotherapy and presence of residual tumour. Other combinations include OPPA(oncovin, procarbazine, prednisone, doxorubicin), OEPA(Oncovin, etoposide, prednisone and doxorubicin and BEACOPP.
Hematopoietic stem cell transplantation and immune therapy is offered to patients who relapse or for those who are refractory to primary therapy