ABSTRACT
The type I cryoglobulins (CGs) account for 10-15% of all cryoglobulins and are found in patients with hematological disorders. We here describe the largest series of seven cases of type I cryoglobulinemia associated with multiple myeloma (MM) and provide a detailed review of the literature associated with this disorder, with the aim of improving the future diagnosis and therapeutic management of this rare disease. Six of the cases in our series were men aged 28-69 years, and most of the subject patients had an immunoglobulin G (IgG) monoclonal component and stage I indolent MM that manifested as cryoglobulin-related symptoms. The patients were all karyotypically normal. Clinical manifestations in this group were: skin lesions (five cases, 71.4%), rheumatologic failure (four cases, 57.1%), neurological abnormalities (two cases, 28.6%), mixed cutaneous/rheumatologic/renal defects (one case, 14.3%) and one case in which the cryoglobulinemia was asymptomatic. Two patients experienced acute renal failure but underwent a full recovery following treatment for MM. We conclude from our analysis that treatment approaches for severe type I cryoglobulinemia should involve plasmapheresis at the onset to achieve a rapid control of the CG-related symptoms, and that specific MM treatments should be introduced also at an early stage to avoid cryoglobulinemia relapse. In this context, bortezomib and lenalidomide are potentially the most effective therapeutic agents.
Subject(s)
Cryoglobulinemia/diagnosis , Cryoglobulinemia/etiology , Multiple Myeloma/complications , Multiple Myeloma/diagnosis , Adult , Cryoglobulinemia/therapy , Fatal Outcome , Female , Humans , Immunoglobulin G/blood , Immunoglobulin M/blood , Male , Middle Aged , Multiple Myeloma/therapy , Necrosis , Neoplasm Staging , Skin/pathology , Treatment OutcomeABSTRACT
We report a case of diffuse large B-cell lymphoma occurring in a patient with the hyperimmunoglobulinemia E syndrome, a rare immune disorder defined by elevated immunoglobulin E levels and recurrent bacterial and fungal infections often manifesting as cold abscesses. This case further supports the notion that patients with hyperimmunoglobulinemia E have an increased risk of lymphoid malignancies and should be closely monitored. Despite a theoretic risk of severe infectious complications, chemotherapy was well tolerated and resulted in a sustained complete remission.
