Paraneoplastic syndrome(s)(PNSs); A rare phenomenon charcterizes HL occurring months/years before diagnosis-typically an advanced stage with B symptoms-or preceding relapse.Aims:To evaluate the available evidence regarding PNSs in HL.
A systematic review of literature on paraneoplastic syndromes in HL according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. The online database PubMed was searched using wth key words “paraneoplastic syndromes and Hodgkin lymphoma”, “vanishing bile duct syndrome in Hodgkin lymphoma” and “neurological manifestations in Hodgkin lymphoma”. Studies done on humans, published in the last 10 years were included.
112 studies met the inclusion criteria. 23 review articles and 83 case reports were identified. The most common PNSs are the neurologic manifestations including paraneoplastic cerebellar degeneration (PCD),limbic encephalitis,Guillain-Barré syndrome,autonomic neuropathy and motor neuron disease.An immune related phenomenon is the proposed pathogenesis of PNSs supported by the presence of anti-Tr antibodies against Purkinje cells and anti-mGluR5 antibodies in the case of PCD and in HL and limbic encephalopathy (Ophelia syndrome) respectively. The most serious hepatic affection:Vanishing Bile Duct syndrome (VBDS) .Reported cases of toxic epidermal necrolysis, Stevens Johnson syndrome and angiokeratoma corpora diffusum were the most severe dermatologic manifestations. Early initiation of chemotherapy with high dose steroids and IVIG is the mainstay of tratment. Prognosis is unfavorable sometimes fatal as in VBDS progressing to liver cell failure.
The management of PNSs is challenging. Most available evidence is centered on the use of steroids, IVIG concomitantly with chemotherapy based on case reports and case series. Establishing evidence-based guidelines is mandatory to improve outcome.