Unusual clinical manifestations and complications of Hodgkin lymphoma

Haema 2012; 3(3):307-320

by Argiris S. Symeonidis

Hematology Division, Dept of Int.Medicine, University Hospital of Patras, Greece

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In addition to the classical or conventional clinical manifestations of Hodgkin lymphoma, altogether consisting a frequency of about 80-85% of all the cases, in this type of lymphoma many rare and atypical clinical manifestations, as well as some rarer paraneoplastic syndromes, have been described. Among the former, primary extranodal disease, which is rarer, than that developing in Non Hodgkin’s Lymphomas, can originate in any tissue or organ of the body. Among the atypical or uncommon clinical manifestations, alcohol-induced nodal or bone pain is a well-recognized, but rarer impressive manifestation. Various hematological findings may be encountered, including anemia of different severity, leukocytosis with neutrophilia, thrombocytosis, eoasinophilia, and pancytopenia sometimes with increased marrow fibrosis. Autoimmune manifestations, either organ-specific or not, are not uncommon, and may be clustered in some patients. The paraneoplastic syndromes are organ- or system-specific manifestations, which cannot be interpreted by the infiltration or the participation of the affected organ, but result from a long-acting hormonal, humoral, immunologic or other as yet non-clarified pathogenetic mechanism. Among these diseases, neurological syndromes, such as subacute cerebellar degeneration, progressive multifocal leucoencephalopathy, stiff-person syndrome, various peripheral, axonal poly-radiculoneuronopathies, and other rarer syndromes are included. Among the remaining paraneoplastic syndromes, the vanishing bile-duct syndrome, idiopathic cholestasis, minimal change nephrotic syndrome, various bullous skin disorders, granulomatous vasculitis of the CNS and the orbit, uveitis chorioretinitis, amyloidosis, clubbing, hypercalcemia, myasthenia gravis, ichthyasis, polymyalgia rheumatica, and hyperprolactinemic galactorrhea are included. Awareness about these atypical and uncommon cli nical manifestations and their prompt recognition is particularly important for any clinical hematologist, and might be crucial and life-saving for the patient, since the majority of these syndromes and manifestations are at least stabilized and may be completely reversed, following the administration of the appropriate anti-lymphoma treatment.