Limitation of plasmapheresis in cryoglobulinemia with high levels of cryoglobulins.
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Cryoglobulins
Plasmapheresis
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The management of patients with cryoglobulins often meets with limited success. Reported here is the use of plasmapheresis and/or partial plasma exchange in the management of five patients with cryoglobulinemia. The procedure was carried out at room temperature with reinfusion through a blood warmer. Circulating levels of mixed cryoglobulins and monoclonal IgM cryoglobulins were more easily reduced than were IgG cryoproteins. Improvement in symptoms was associated with removal of the cryoprotein. Pheresis can be used as primary therapy for reduction of cryoglobulin levels in cases of symptomatic essential cryoglobulinemia. Where an etiology for cryoglobulinemia is known and specific treatment exists, pheresis can be used as effective adjunct therapy.
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Cryoglobulins
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Hematology
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WALGRAEVE, NATASCHA M.D.; VAN DEN EECKHAUT, ANJA M.D.; DE GEETER, FRANK M.D., PH.D. Author Information
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Type I cryoglobulinemia, a condition associated with lymphoproliferative disorders, is caused by monoclonal immunoglobulins that precipitate at low temperatures. It mostly involves the skin and pathology study shows no signs of vasculitis. Management is usually based on immunosuppressive drugs associated with plasmapheresis for severe disease. The use of rituximab has recently been described for resistant cases. We present an unusual case of long-standing type I cryoglobulinemia associated with a monoclonal gammopathy of uncertain significance. The patient developed extremely severe skin lesions with histological signs of vasculitis. The patient died due to the onset of noncutaneous manifestations of the cryoglobulinemia and complications of the immunosuppressive treatment. La crioglobulinemia tipo I es un trastorno producido por inmunoglobulinas monoclonales que precipitan a bajas temperaturas. Se asocia a trastornos linfoproliferativos, su clínica es predominantemente cutánea y la anatomía patológica no muestra fenómenos de vasculitis. En su manejo se emplean habitualmente fármacos inmunosupresores junto con plasmaféresis para los casos graves. Recientemente se ha descrito el uso de rituximab para los casos resistentes. Se presenta un caso poco común de crioglobulinemia tipo I de largo tiempo de evolución asociada a una gammapatía monoclonal de significado incierto. El paciente desarrolló lesiones cutáneas de extrema gravedad con fenómenos de vasculitis en la anatomía patológica, y presentó un desenlace fatal secundariamente a la aparición de manifestaciones extracutáneas de la crioglobulinemia y a las complicaciones del tratamiento inmunosupresor.
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Cryoglobulins
Plasmapheresis
Cryoglobulins
Purpura (gastropod)
Serum sickness
Rheumatoid factor
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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.
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Eculizumab
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Cryoglobulinemia is defined as the presence of an abnormal immunoglobulin that may be responsible for vasculitis of small-caliber vessels. Apheresis can be used in order to temporarily eliminate circulating cryoglobulins. The aim of this study was to assess the effectiveness of apheresis (double-filtration plasmapheresis-DFPP-) in symptomatic and/or severe cryoglobulinemias.Four male patients presenting cryoglobulinemic vasculitis and who received DFPP sessions were included.Their mean age was 57 ± 15 years. One patient had hepatitis-C virus (HCV)-related cryoglobulinemia and the other three patients were carriers of an IgM Kappa monoclonal gammopathy. Mean duration of follow-up was 15 ± 2 months. DFPP allowed healing of ulcerative skin lesions in the first patient and remission of nephrotic syndrome in the other patients after a median of 6(5-10) sessions.DFPP can be used safely in cryoglobulinemic-vasculitis and can be considered early to achieve a faster and sustained clinical-biological response.
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Purpura (gastropod)
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