Skip to main content Accessibility help

We use cookies to distinguish you from other users and to provide you with a better experience on our websites. Close this message to accept cookies or find out how to manage your cookie settings.

Close cookie message
×
Hostname: page-component-78c5997874-dh8gc Total loading time: 0 Render date: 2024-11-06T09:06:26.880Z Has data issue: false hasContentIssue false

17 - POEMS syndrome and paraproteinemic syndromes: management and follow-up

from Section 3 - Myeloma: clinical entities

Published online by Cambridge University Press:  18 December 2013

By
Angela Dispenzieri  and
Suzanne R. Hayman
Edited by
Stephen A. Schey ,
Kwee L. Yong ,
Robert Marcus  and
Kenneth C. Anderson
Stephen A. Schey
Affiliation:
Department of Haematology, King’s College Hospital, London
Kwee L. Yong
Affiliation:
Department of Haematology, University College Hospital, London
Robert Marcus
Affiliation:
Department of Haematology, King’s College Hospital, London
Kenneth C. Anderson
Affiliation:
Dana-Farber Cancer Institute, Boston
Get access

Summary

Introduction

POEMS syndrome is an atypical plasma cell disorders, composed of a diverse group of diseases that often share little other than an underlying plasma cell clone. The pathophysiologic relationship between the plasma cell dyscrasia is often cryptic in many of these disorders. However, understanding that there may be relationships between bone marrow and distant organ systems is important to arrive at a diagnosis and management plan. A convenient means of characterizing these disorders is by their dominant clinical feature: neuropathy, dermopathy and nephropathy (Figure 17.1). Some atypical plasma cell disorders will have phenotypes that cross many systems, as will be specified in the following pages. The most common atypical plasma cell disorder, light chain (AL) amyloidosis, may affect almost any organ system but will be discussed in a separate chapter.

Atypical PCD with peripheral neuropathy as dominant phenotype

Chemotherapy-induced peripheral neuropathy, Waldenstrom’s macroglobulinemia, multiple myeloma, and light chain amyloid are important etiologies for plasma cell disorder (PCD)-associated neuropathy, but these are beyond the scope of this review. In this section the focus will be upon POEMS (polyneuropathy, organomegaly, endocrinopathy, M protein and skin changes) syndrome and MGUS (monoclonal gammopathy of undetermined significance) associated peripheral neuropathy.

Type
Chapter
Information
Myeloma
Pathology, Diagnosis, and Treatment
 , pp. 225 - 244
Publisher: Cambridge University Press
Print publication year: 2013

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

Bardwick, P. A., Zvaifler, N. J., Gill, G. N. et al. Plasma cell dyscrasia with polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes: the POEMS syndrome. Report on two cases and a review of the literature. Medicine 1980;59(4):311–22.CrossRefGoogle Scholar
Takatsuki, K., Sanada, I.Plasma cell dyscrasia with polyneuropathy and endocrine disorder: clinical and laboratory features of 109 reported cases. Jpn. J. Clin. Oncol. 1983;13(3):543–55.Google ScholarPubMed
Nakanishi, T., Sobue, I., Toyokura, Y. et al. The Crow-Fukase syndrome: a study of 102 cases in Japan. Neurology 1984;34(6):712–20.CrossRefGoogle ScholarPubMed
Singh, D., Wadhwa, J., Kumar, L. et al. POEMS syndrome: experience with fourteen cases. Leuk. Lymphoma 2003;44(10):1749–52.CrossRefGoogle ScholarPubMed
Soubrier, M. J., Dubost, J. J., Sauvezie, B. J.POEMS syndrome: a study of 25 cases and a review of the literature. French Study Group on POEMS Syndrome. Am. J. Med. 1994;97(6):543–53.CrossRefGoogle Scholar
Zhang, B., Song, X., Liang, B. et al. The clinical study of POEMS syndrome in China. Neuro. Endocrin. Lett. 2010;31(2):229–37.Google Scholar
Li, J., Zhou, D. B., Huang, Z. et al. Clinical characteristics and long-term outcome of patients with POEMS syndrome in China. Annals. Hematol. 2011;90(7):819–26.CrossRefGoogle Scholar
Kulkarni, G. B., Mahadevan, A., Taly, A. B. et al. Clinicopathological profile of polyneuropathy, organomegaly, endocrinopathy, M protein and skin changes (POEMS) syndrome. J. Clin. Neurosci. 2011;18(3):356–60.CrossRefGoogle ScholarPubMed
Dispenzieri, A.POEMS Syndrome. Blood. Reviews 2007;21(6):285–99.CrossRefGoogle ScholarPubMed
Dao, L. N., Hanson, C. A., Dispenzieri, A. et al. Bone marrow histopathology in POEMS syndrome: a distinctive combination of plasma cell, lymphoid and myeloid findings in 87 patients. Blood 2011;117(24):6438–44.CrossRefGoogle ScholarPubMed
Dispenzieri, A.Castleman disease. Cancer Treatment Res. 2008;142:293–330.Google ScholarPubMed
Kelly, J. J., Kyle, R. A., Miles, J. M., Dyck, P. J.Osteosclerotic myeloma and peripheral neuropathy. Neurology 1983;33(2):202–10.CrossRefGoogle ScholarPubMed
Koike, H., Iijima, M., Mori, K. et al. Neuropathic pain correlates with myelinated fibre loss and cytokine profile in POEMS syndrome. J. Neurol. Neurosurg. Psych. 2008;79(10):1171–9.CrossRefGoogle ScholarPubMed
Allam, J. S., Kennedy, C. C., Aksamit, T. R., Dispenzieri, A.Pulmonary manifestations in patients with POEMS syndrome: a retrospective review of 137 patients. Chest 2008;133(4):969–74.CrossRefGoogle ScholarPubMed
Lesprit, P., Authier, F. J., Gherardi, R. et al. Acute arterial obliteration: a new feature of the POEMS syndrome?Medicine 1996;75(4):226–32.CrossRefGoogle ScholarPubMed
Dupont, S. A., Dispenzieri, A., Mauermann, M. L., Rabinstein, A. A., Brown, R. D.. Cerebral infarction in POEMS syndrome: incidence, risk factors, and imaging characteristics. Neurology 2009;73(16):1308–12.CrossRefGoogle ScholarPubMed
Dispenzieri, A., Kyle, R. A., Lacy, M. Q. et al. POEMS syndrome: definitions and long-term outcome. Blood 2003;101(7):2496–506.CrossRefGoogle ScholarPubMed
Stankowski-Drengler, T., Gertz, M. A., Katzmann, J. A. et al. Serum immunoglobulin free light chain measurements and heavy chain isotype usage provide insight into disease biology in patients with POEMS syndrome. Am. J. Hematol. 2010;85(6):431–4.Google ScholarPubMed
Ghandi, G. Y., Basu, R., Dispenzieri, A. et al. Endocrinopathy in POEMS syndrome: the Mayo Clinic experience. Mayo. Clin. Proc. 2007;82(7):836–42.Google Scholar
Watanabe, O., Arimura, K., Kitajima, I., Osame, M., Maruyama, I.Greatly raised vascular endothelial growth factor (VEGF) in POEMS syndrome [letter]. Lancet 1996;347(9002):702.CrossRefGoogle Scholar
Soubrier, M., Dubost, J. J., Serre, A. F. et al. Growth factors in POEMS syndrome: evidence for a marked increase in circulating vascular endothelial growth factor. Arthritis Rheumatism 1997;40(4):786–7.CrossRefGoogle ScholarPubMed
Hashiguchi, T., Arimura, K., Matsumuro, K. et al. Highly concentrated vascular endothelial growth factor in platelets in Crow–Fukase syndrome. Muscle Nerve 2000;23(7):1051–6.3.0.CO;2-V>CrossRefGoogle ScholarPubMed
Watanabe, O., Maruyama, I., Arimura, K. et al. Overproduction of vascular endothelial growth factor/vascular permeability factor is causative in Crow-Fukase (POEMS) syndrome. Muscle Nerve 1998;21(11):1390–7.3.0.CO;2-4>CrossRefGoogle ScholarPubMed
Scarlato, M., Previtali, S. C., Carpo, M. et al. Polyneuropathy in POEMS syndrome: role of angiogenic factors in the pathogenesis. Brain 2005;128(Pt 8):1911–20.CrossRefGoogle ScholarPubMed
Tanaka, O., Ohsawa, T.The POEMS syndrome: report of three cases with radiographic abnormalities. Radiologe 1984;24(10):472–4.Google ScholarPubMed
Chong, S. T., Beasley, H. S., Daffner, R. H.POEMS syndrome: radiographic appearance with MRI correlation. Skeletal Radiol. 2006;35(9):690–5.CrossRefGoogle ScholarPubMed
Sung, J. Y., Kuwabara, S., Ogawara, K., Kanai, K., Hattori, T.Patterns of nerve conduction abnormalities in POEMS syndrome. Muscle Nerve 2002;26(2):189–93.CrossRefGoogle ScholarPubMed
Saida, K., Kawakami, H., Ohta, M., Iwamura, K.Coagulation and vascular abnormalities in Crow–Fukase syndrome. Muscle Nerve 1997;20(4):486–92.3.0.CO;2-X>CrossRefGoogle ScholarPubMed
Li, J., Zhang, W., Jiao, L. et al. Combination of melphalan and dexamethasone for patients with newly diagnosed POEMS syndrome. Blood 2011;117(24):6445–9.CrossRefGoogle ScholarPubMed
Dispenzieri, A.POEMS syndrome: 2011 update on diagnosis, risk-stratification, and management. Am. J. Hematol. 2011;86(7):591–601.CrossRefGoogle ScholarPubMed
Samaras, P., Bauer, S., Stenner-Liewen, F. et al. Treatment of POEMS syndrome with bevacizumab. Haematologica 2007;92(10):1438–9.CrossRefGoogle ScholarPubMed
Imai, N., Taguchi, J., Yagi, N. et al. Relapse of polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes (POEMS) syndrome without increased level of vascular endothelial growth factor following successful autologous peripheral blood stem cell transplantation. Neuromusc. Disord. 2009;19(5):363–5.CrossRefGoogle ScholarPubMed
Kojima, H., Katsuoka, Y., Katsura, Y. et al. Successful treatment of a patient with POEMS syndrome by tandem high-dose chemotherapy with autologous CD34+ purged stem cell rescue. Int. J. Hematol. 2006;84(2):182–5.CrossRefGoogle ScholarPubMed
Latov, N., Sherman, W. H., Nemni, R. et al. Plasma-cell dyscrasia and peripheral neuropathy with a monoclonal antibody to peripheral-nerve myelin. N. Engl. J. Med. 1980;303(11):618–21.CrossRefGoogle ScholarPubMed
Quarles, R. H., Weiss, M. D.Autoantibodies associated with peripheral neuropathy. Muscle Nerve 1999;22(7):800–22.3.0.CO;2-F>CrossRefGoogle ScholarPubMed
Suarez, G. A., Kelly, J. J.. Polyneuropathy associated with monoclonal gammopathy of undetermined significance: further evidence that IgM-MGUS neuropathies are different than IgG-MGUS. Neurology 1993;43(7):1304–8.CrossRefGoogle ScholarPubMed
Chassande, B., Leger, J. M., Younes-Chennoufi, A. B. et al. Peripheral neuropathy associated with IgM monoclonal gammopathy: correlations between M-protein antibody activity and clinical/electrophysiological features in 40 cases. Muscle Nerve 1998;21(1):55–62.3.0.CO;2-F>CrossRefGoogle ScholarPubMed
Nobile-Orazio, E., Meucci, N., Baldini, L., Di Troia, A., Scarlato, G.Long-term prognosis of neuropathy associated with anti-MAG IgM M- proteins and its relationship to immune therapies. Brain 2000;123(Pt 4):710–17.CrossRefGoogle ScholarPubMed
Dyck, P. J., Low, P. A., Windebank, A. J. et al. Plasma exchange in polyneuropathy associated with monoclonal gammopathy of undetermined significance. N. Engl. J. Med. 1991;325(21):1482–6.CrossRefGoogle ScholarPubMed
Dalakas, M. C., Quarles, R. H., Farrer, R. G. et al. A controlled study of intravenous immunoglobulin in demyelinating neuropathy with IgM gammopathy. Ann. Neurol. 1996;40(5):792–5.CrossRefGoogle ScholarPubMed
Mariette, X., Brouet, J. C., Chevret, S. et al. A randomised double blind trial versus placebo does not confirm the benefit of alpha-interferon in polyneuropathy associated with monoclonal IgM [letter]. J. Neurol. Neurosurg. Psychiatry 2000;69(2):279–80.CrossRefGoogle Scholar
Oksenhendler, E., Chevret, S., Leger, J. M. et al. Plasma exchange and chlorambucil in polyneuropathy associated with monoclonal IgM gammopathy. IgM-associated Polyneuropathy Study Group. J. Neurol. Neurosurg. Psychiatry 1995;59(3):243–7.CrossRefGoogle ScholarPubMed
Comi, G., Roveri, L., Swan, A. et al. A randomised controlled trial of intravenous immunoglobulin in IgM paraprotein associated demyelinating neuropathy. J. Neurol. 2002;249(10):1370–7.CrossRefGoogle ScholarPubMed
Pestronk, A., Florence, J., Miller, T. et al. Treatment of IgM antibody associated polyneuropathies using rituximab. J. Neurol. Neurosurg. Psychiatry 2003;74(4):485–9.CrossRefGoogle ScholarPubMed
Dispenzieri, A., Gertz, M. A., Witzig, T. E.Cryoglobulinemia and heavy chain disease. In: Greer, J. P., editor, Wintrobe’s Clinical Hematology. Philadelphia: Wolters Kluwer Health/Lippincott Williams & Wilkins; 2009, pp. 2484–96.Google Scholar
Gorevic, P. D., Kassab, H. J., Levo, Y. et al. Mixed cryoglobulinemia: clinical aspects and long-term follow-up of 40 patients. Am. J. Med. 1980;69(2):287–308.CrossRefGoogle ScholarPubMed
Dispenzieri, A., Gorevic, P. D.Cryoglobulinemia. Hematol. Oncol. Clin. North Am. 1999;13(6):1315–49.CrossRefGoogle ScholarPubMed
Monti, G., Galli, M., Invernizzi, F. et al. Cryoglobulinaemias: a multi-centre study of the early clinical and laboratory manifestations of primary and secondary disease. GISC. Italian Group for the Study of Cryoglobulinaemias. QJM 1995;88(2):115–26.Google Scholar
Pascual, M., Perrin, L., Giostra, E., Schifferli, J. A.Hepatitis C virus in patients with cryoglobulinemia type II. J. Infect. Dis. 1990;162(2):569–70.CrossRefGoogle ScholarPubMed
Ferri, C., Greco, F., Longombardo, G. et al. Antibodies to hepatitis C virus in patients with mixed cryoglobulinemia. Arthritis Rheumatism 1991;34(12):1606–10.CrossRefGoogle ScholarPubMed
Goldman, M., Renversez, J. C., Lambert, P. H.Pathological expression of idiotypic interactions: immune complexes and cryoglobulins. Springer Seminars Immunopathology 1983;6(1):33–49.CrossRefGoogle ScholarPubMed
Meltzer, M., Franklin, E. C., Elias, K., McCluskey, R. T., Cooper, N.Cryoglobulinemia–a clinical and laboratory study. II. Cryoglobulins with rheumatoid factor activity. Am. J. Med. 1966;40(6):837–56.CrossRefGoogle ScholarPubMed
Mendez, P., Saeian, K., Reddy, K. R. et al. Hepatitis C, cryoglobulinemia, and cutaneous vasculitis associated with unusual and serious manifestations. Am. J. Gastroenterol. 2001;96(8):2489–93.CrossRefGoogle ScholarPubMed
Brouet, J., Clauvel, J., Danon, F., Klein, M., Seligmann, M.Biological and clinical significance of cryoglobulins. A report of 86 cases. Am. J. Med. 1974;57:775–88.CrossRefGoogle Scholar
Donada, C., Crucitti, A., Donadon, V. et al. Systemic manifestations and liver disease in patients with chronic hepatitis C and type II or III mixed cryoglobulinaemia. J. Viral. Hepat. 1998;5(3):179–85.CrossRefGoogle ScholarPubMed
Montagnino, G.Reappraisal of the clinical expression of mixed cryoglobulinemia. Springer Seminars Immunopathology 1988;10(1):1–19.CrossRefGoogle ScholarPubMed
Cohen, S. J., Pittelkow, M. R., Su, W. P.Cutaneous manifestations of cryoglobulinemia: clinical and histopathologic study of seventy-two patients. J. Am. Acad. Dermatol. 1991;25(1 Pt 1):21–7.CrossRefGoogle ScholarPubMed
Cavaletti, G., Petruccioli, M. G., Crespi, V. et al. A clinico-pathological and follow up study of 10 cases of essential type II cryoglobulinaemic neuropathy. J. Neurol. Neurosurg. Psychiatry 1990;53(10):886–9.CrossRefGoogle ScholarPubMed
Ammendola, A., Sampaolo, S., Ambrosone, L. et al. Peripheral neuropathy in hepatitis-related mixed cryoglobulinemia: electrophysiologic follow-up study. Muscle Nerve 2005;31(3):382–5.CrossRefGoogle ScholarPubMed
Cordonnier, D., Vialtel, P., Renversez, J. C. et al. Renal diseases in 18 patients with mixed type II IgM-IgG cryoglobulinemia: monoclonal lymphoid infiltration (2 cases) and membranoproliferative glomerulonephritis (14 cases). Adv. Nephrology Necker Hospital 1983;12:177–204.Google Scholar
Tarantino, A., Campise, M., Banfi, G. et al. Long-term predictors of survival in essential mixed cryoglobulinemic glomerulonephritis. Kidney Int. 1995;47(2):618–23.CrossRefGoogle ScholarPubMed
Monteverde, A., Ballare, M., Bertoncelli, M. C. et al. Lymphoproliferation in type II mixed cryoglobulinemia. Clin. Experimental Rheumatol. 1995;13(13):S141–7.Google ScholarPubMed
Ferri, C., Sebastiani, M., Giuggioli, D. et al. Mixed cryoglobulinemia: demographic, clinical, and serologic features and survival in 231 patients. Semin. Arthritis Rheum. 2004;33(6):355–74.CrossRefGoogle ScholarPubMed
Ferri, C., Marzo, E., Longombardo, G. et al. Interferon alfa-2b in mixed cryoglobulinaemia: a controlled crossover trial. Gut 1993;34(2 Suppl):S144–5.CrossRefGoogle ScholarPubMed
Misiani, R., Bellavita, P., Fenili, D. et al. Interferon alfa-2a therapy in cryoglobulinemia associated with hepatitis C virus. N. Engl. J. Med. 1994;330(11):751–6.CrossRefGoogle ScholarPubMed
Dammacco, F., Sansonno, D., Han, J. H. et al. Natural interferon-alpha versus its combination with 6-methyl-prednisolone in the therapy of type II mixed cryoglobulinemia: a long-term, randomized, controlled study. Blood 1994;84(10):3336–43.Google ScholarPubMed
Lauta, V. M., De Sangro, M. A.Long-term results regarding the use of recombinant interferon alpha-2b in the treatment of II type mixed essential cryoglobulinemia. Med. Oncol. 1995;12(4):223–30.CrossRefGoogle ScholarPubMed
Mazzaro, C., Lacchin, T., Moretti, M. et al. Effects of two different alpha-interferon regimens on clinical and virological findings in mixed cryoglobulinemia. Clin. Exp. Rheumatol. 1995;13(13):S181–5.Google ScholarPubMed
Migliaresi, S., Tirri, G.Interferon in the treatment of mixed cryoglobulinemia. Clin. Exp. Rheumatol. 1995;13(13):S175–80.Google ScholarPubMed
Bruchfeld, A., Lindahl, K., Stahle, L., Soderberg, M., Schvarcz, R.Interferon and ribavirin treatment in patients with hepatitis C-associated renal disease and renal insufficiency. Nephrol. Dial. Transplant. 2003;18(8):1573–80.CrossRefGoogle ScholarPubMed
Alric, L., Plaisier, E., Thebault, S. et al. Influence of antiviral therapy in hepatitis C virus-associated cryoglobulinemic MPGN. Am. J. Kidney Dis. 2004;43(4):617–23.CrossRefGoogle ScholarPubMed
Pietrogrande, M., De Vita, S., Zignego, A. L. et al. Recommendations for the management of mixed cryoglobulinemia syndrome in hepatitis C virus-infected patients. Autoimmunity Reviews 2011;10(8):444–54.CrossRefGoogle ScholarPubMed
Zaja, F., De Vita, S., Mazzaro, C. et al. Efficacy and safety of rituximab in type II mixed cryoglobulinemia. Blood 2003;101(10):3827–34.CrossRefGoogle ScholarPubMed
Sansonno, D., De Re, V., Lauletta, G. et al. Monoclonal antibody treatment of mixed cryoglobulinemia resistant to interferon alpha with an anti-CD20. Blood 2003;101(10):3818–26.CrossRefGoogle ScholarPubMed
Ghobrial, I. M., Uslan, D. Z., Call, T. G., Witzig, T. E., Gertz, M. A.Initial increase in the cryoglobulin level after rituximab therapy for type II cryoglobulinemia secondary to Waldenstrom macroglobulinemia does not indicate failure of response. Am. J. Hematol. 2004;77(4):329–30.CrossRefGoogle ScholarPubMed
Frickhofen, N., Wiesneth, M., Jainta, C. et al. Hepatitis C virus infection is a risk factor for liver failure from veno-occlusive disease after bone marrow transplantation. Blood 1994;83(7):1998–2004.Google ScholarPubMed
Cokonis Georgakis, C. D., Falasca, G., Georgakis, A., Heymann, W. R.Scleromyxedema. Clin. Dermatol. 2006;24(6):493–7.CrossRef
Kucher, C., Xu, X., Pasha, T., Elenitsas, R.Histopathologic comparison of nephrogenic fibrosing dermopathy and scleromyxedema. J. Cutan. Pathol. 2005;32(7):484–90.CrossRefGoogle ScholarPubMed
Dinneen, A. M., Dicken, C. H.Scleromyxedema. J. Am. Acad. Dermatol. 1995;33(1):37–43.CrossRefGoogle ScholarPubMed
Berger, J. R., Dobbs, M. R., Terhune, M. H., Maragos, W. F.The neurologic complications of scleromyxedema. Medicine (Baltimore) 2001;80(5):313–19.CrossRefGoogle ScholarPubMed
Pomann, J. J., Rudner, E. J.Scleromyxedema revisited. Int. J. Dermatol. 2003;42(1):31–5.CrossRefGoogle ScholarPubMed
Rongioletti, F., Rebora, A.Updated classification of papular mucinosis, lichen myxedematosus, and scleromyxedema. J. Am. Acad. Dermatol. 2001;44(2):273–81.CrossRefGoogle ScholarPubMed
Lacy, M. Q., Hogan, W. J., Gertz, M. A. et al. Successful treatment of scleromyxedema with autologous peripheral blood stem cell transplantation. Arch. Dermatol. 2005;141(10):1277–82.CrossRefGoogle ScholarPubMed
Sansbury, J. C., Cocuroccia, B., Jorizzo, J. L. et al. Treatment of recalcitrant scleromyxedema with thalidomide in 3 patients. J. Am. Acad. Dermatol. 2004;51(1):126–31.CrossRefGoogle ScholarPubMed
Fernandez-Herrera, J., Pedraz, J.Necrobiotic xanthogranuloma. Semin. Cutan. Med. Surg. 2007;26(2):108–13.CrossRefGoogle ScholarPubMed
Finan, M. C., Winkelmann, R. K.Necrobiotic xanthogranuloma with paraproteinemia. A review of 22 cases. Medicine (Baltimore) 1986;65(6):376–88.CrossRefGoogle ScholarPubMed
Venencie, P. Y., Le Bras, P., Toan, N. D., Tchernia, G., Delfraissy, J. F.Recombinant interferon alfa-2b treatment of necrobiotic xanthogranuloma with paraproteinemia. J. Am. Acad. Dermatol. 1995;32(4):666–7.CrossRefGoogle ScholarPubMed
Goede, J. S., Misselwitz, B., Taverna, C. et al. Necrobiotic xanthogranuloma successfully treated with autologous stem cell transplantation. Ann. Hematol. 2007;86(4):303–6.CrossRefGoogle ScholarPubMed
Lipsker, D., Veran, Y., Grunenberger, F. et al. The Schnitzler syndrome. Four new cases and review of the literature. Medicine (Baltimore) 2001;80(1):37–44.CrossRefGoogle ScholarPubMed
de Koning, H. D., Bodar, E. J., van der Meer, J. W., Simon, A.Schnitzler syndrome: beyond the case reports review and follow-up of 94 patients with an emphasis on prognosis and treatment. Semin. Arthritis Rheum. 2007, Jun 20.CrossRefGoogle ScholarPubMed
Schneider, S. W., Gaubitz, M., Luger, T. A., Bonsmann, G.Prompt response of refractory Schnitzler syndrome to treatment with anakinra. J. Am. Acad. Dermatol. 2007;56(5 Suppl):S120–2.CrossRefGoogle ScholarPubMed
Eiling, E., Moller, M., Kreiselmaier, I., Brasch, J., Schwarz, T.Schnitzler syndrome: treatment failure to rituximab but response to anakinra. J. Am. Acad. Dermatol. 2007, Apr 27.CrossRefGoogle ScholarPubMed
Martinez-Taboada, V. M., Fontalba, A., Blanco, R., Fernandez-Luna, J. L.Successful treatment of refractory Schnitzler syndrome with anakinra: comment on the article by Hawkins et al. Arthritis Rheum. 2005;52(7):2226–7.CrossRefGoogle ScholarPubMed
de Castro, F. R., Masouye, I., Winkelmann, R. K., Saurat, J. H.Urticarial pathology in Schnitzler’s (hyper-IgM) syndrome. Dermatology 1996;193(2):94–9.CrossRefGoogle ScholarPubMed
Lipsker, D., Spehner, D., Drillien, R. et al. Schnitzler syndrome: heterogeneous immunopathological findings involving IgM-skin interactions. Br. J. Dermatol. 2000;142(5):954–9.CrossRefGoogle ScholarPubMed
Randall, R. E., Williamson, W. C., Mullinax, F., Tung, M. Y., Still, W. J.Manifestations of systemic light chain deposition. Am. J. Med. 1976;60(2):293–9.CrossRefGoogle ScholarPubMed
Gallo, G., Picken, M., Frangione, B., Buxbaum, J.Nonamyloidotic monoclonal immunoglobulin deposits lack amyloid P component. Mod. Pathol. 1988;1(6):453–6.Google ScholarPubMed
Kambham, N., Markowitz, G. S., Appel, G. B. et al. Heavy chain deposition disease: the disease spectrum. Am. J. Kidney Dis. 1999;33(5):954–62.CrossRefGoogle ScholarPubMed
Pozzi, C., D’Amico, M., Fogazzi, G. B. et al. Light chain deposition disease with renal involvement: clinical characteristics and prognostic factors. Am. J. Kidney Dis. 2003;42(6):1154–63.CrossRefGoogle ScholarPubMed
Heilman, R. L., Velosa, J. A., Holley, K. E., Offord, K. P., Kyle, R. A.Long-term follow-up and response to chemotherapy in patients with light-chain deposition disease. Am. J. Kidney Dis. 1992;20(1):34–41.CrossRefGoogle ScholarPubMed
Royer, B., Arnulf, B., Martinez, F. et al. High dose chemotherapy in light chain or light and heavy chain deposition disease. Kidney Int. 2004;65(2):642–8.CrossRefGoogle ScholarPubMed
Weichman, K., Dember, L. M., Prokaeva, T. et al. Clinical and molecular characteristics of patients with non-amyloid light chain deposition disorders, and outcome following treatment with high-dose melphalan and autologous stem cell transplantation. Bone Marrow Transplant 2006;38(5):339–43.CrossRefGoogle ScholarPubMed
Ma, C. X., Lacy, M. Q., Rompala, J. F. et al. Acquired Fanconi syndrome is an indolent disorder in the absence of overt multiple myeloma. Blood 2004;104(1):40–2.CrossRefGoogle ScholarPubMed
Mariette, X., Chastang, C., Clavelou, P. et al. A randomised clinical trial comparing interferon-alpha and intravenous immunoglobulin in polyneuropathy associated with monoclonal IgM. The IgM-associated Polyneuropathy Study Group. J. Neurol. Neurosurg. Psych. 1997;63(1):28–34.CrossRefGoogle ScholarPubMed
Dispenzieri, A., Kyle, R. A.Neurological aspects of multiple myeloma and related disorders. Best Pract. Res. Clin. Haematol. 2005;18(4):673–88.CrossRefGoogle ScholarPubMed

Save book to Kindle

To save this book to your Kindle, first ensure [email protected] is added to your Approved Personal Document E-mail List under your Personal Document Settings on the Manage Your Content and Devices page of your Amazon account. Then enter the ‘name’ part of your Kindle email address below. Find out more about saving to your Kindle.

Note you can select to save to either the @free.kindle.com or @kindle.com variations. ‘@free.kindle.com’ emails are free but can only be saved to your device when it is connected to wi-fi. ‘@kindle.com’ emails can be delivered even when you are not connected to wi-fi, but note that service fees apply.

Find out more about the Kindle Personal Document Service.

Available formats
×

Save book to Dropbox

To save content items to your account, please confirm that you agree to abide by our usage policies. If this is the first time you use this feature, you will be asked to authorise Cambridge Core to connect with your account. Find out more about saving content to Dropbox.

Available formats
×

Save book to Google Drive

To save content items to your account, please confirm that you agree to abide by our usage policies. If this is the first time you use this feature, you will be asked to authorise Cambridge Core to connect with your account. Find out more about saving content to Google Drive.

Available formats
×