Hodgkin Lymphoma presenting as a complex paraneoplastic neurological syndrome in an eventually diagnosed case of Klinefelter Syndrome

Abstract

Background: Klinefelter-syndrome (KS) is recognized as 47, XXY or XXY, two or more X chromosome in males giving rise to a set of clinical signs and symptoms. These individuals are predisposed to certain malignancies. The association of Hodgkinlymphoma (HL) with KS is very rare and reported only in a single case in literature. Moreover, it is extremely rare to have Chronic- Demyelinating-Inflammatory-Polyneuropathy (CIDP) as initial presentation of HL. The association of CIDP in HL in a case of Klinefelter-syndrome has never reported together. Case Presentation: We report a case of 47-year old Indian male who presented with CIDP and was eventually diagnosed with Nodular sclerosis variant of HL. Karyotyping revealed KS. Institution of treatment of HL improved his neurological symptoms. However, administration of androgens was deferred till the completion of chemotherapy. Unfortunately, he could not tolerate Chemotherapy and died of Renal Failure during the fourth cycle. Conclusion: This case report depicts an extremely rare combination of CIDP, HL with KS. It also signifies that all CIDP cases should be evaluated for occult malignancy and if malignancy is found patient should undergo karyotyping for under-diagnosed chromosomal abnormalities such as KS as in this case.