WORLD JOURNAL OF ADVANCE
HEALTHCARE RESEARCH

*Dr. Juhi Shahab, Dr. Sufian Zaheer, Dr. Sunil Ranga

ABSTRACT

Introduction: Classical Hodgkin lymphoma (CHL) as per WHO 2016 is a clonal lymphoid neoplasm. CHL contains Reed-Sternberg (RS) cells in a background of a non-neoplastic inflammatory infiltrate including lymphocytes, eosinophils, neutrophils, histiocytes, and plasma cells.The expression of PAX5 is reduced and in rare cases of CHL, PAX5 expression is absent in RS cell. This has been postulated to be caused by compromised B-cell specific transcription machinery and inactivity of immunoglobulin promoters, which results in low levels to absent expression of several B-cell-restricted transcription factors such as PAX5 and OCT2. Hence, the PAX5 negative cases of CHL are extremely rare and pose a major diagnostic challenge for pathologists. Case report: An 8 year old male child presented to the paediatric department with the complaints of prolong fever, loss of appetite, weakness and unexplained weight loss since 3 months. The general examination was non contributory but on CECT child was suspected for mesenteric gastro intestinal stromal tumour( Mesenteric GIST) with no other palpable lymph nodes. The mesenteric lymph node was excised and sent for histopath examination which revealed the findings of a rare case i.e. a PAX-5 negative hodgkin lymphoma presenting solely as mesenteric lymphadenopathy. Discussion: PAX-5 negative Hodgkin lymphoma is itself a rare case which is associated with bad overall prognosis but in our case important point was that, that it solely presented as mesenteric lymphadenitis which made a confusion to clinico-radiological team as a possibility of mesenteric GIST. Conclusion: PAX5-negative CHL may have worse clinical outcomes, when compared to typical PAX5-positive CHL.

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