Pulsed high-dose dexamethasone treatment of polyneuropathy associated with monoclonal gammopathy

Notermans, Nicolette C.; Vermeulen, M.; Lokhorst, Henk M.; Doorn, Pieter A. van; Berg, Leonard H van den; Teunissen, Laurien L.; Wokke, John H. J.

doi:10.1007/s004150050124

Cited by 15 publications

(4 citation statements)

References 9 publications

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“…7 8 10 12-14 17 19 20 We regard the >25% fall in the paraprotein concentration coincident with treatment seen in three of our four patients as meaningful and relevant.…”

Section: Discussionmentioning

confidence: 57%

“…Four of five patients treated with pulsed high dose intravenous dexamethasone improved but the incidence of psychiatric side effects was unacceptably high 19. Steroids have been used in combination with azathioprine, cyclophosphamide, chlorambucil, and plasma exchange in several small studies, with improvement in 0% to 100% of patients at 14 to 54 months of follow up 7…”

Section: Discussionmentioning

confidence: 99%

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Successful treatment of IgM paraproteinaemic neuropathy with fludarabine

Wilson

Lunn

Schey

et al. 1999

Journal of Neurology, Neurosurgery & Psychiatry

102

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Objectives-To evaluate the response of four patients with IgM paraproteinaemic neuropathy to a novel therapy-pulsed intravenous fludarabine. Background-The peripheral neuropathy associated with IgM paraproteinaemia usually runs a chronic, slowly progressive course which may eventually cause severe disability. Treatment with conventional immunosuppressive regimens has been unsatisfactory. Fludarabine is a novel purine analogue which has recently been shown to be eVective in low grade lymphoid malignancies. Methods-Four patients were treated with IgM paraproteinaemic neuropathy with intravenous pulses of fludarabine. Two of the four patients had antibodies to MAG and characteristic widely spaced myelin on nerve biopsy and a third had characteristic widely spaced myelin only. The fourth had an endoneurial lymphocytic infiltrate on nerve biopsy and a diagnosis of Waldenström's macroglobulinaemia. Results-In all cases subjective and objective clinical improvement occurred associated with a significant fall in the IgM paraprotein concentration in three cases. Neurophysiological parameters improved in the three patients examined. The treatment was well tolerated. All patients developed mild, reversible lymphopenia and 50% mild generalised myelosuppression, but there were no febrile episodes. Conclusion-Fludarabine should be considered as a possible treatment for patients with IgM MGUS paraproteinaemic neuropathy. (J Neurol Neurosurg Psychiatry 1999;66:575-580)

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“…7 8 10 12-14 17 19 20 We regard the >25% fall in the paraprotein concentration coincident with treatment seen in three of our four patients as meaningful and relevant.…”

Section: Discussionmentioning

confidence: 57%

Section: Discussionmentioning

confidence: 99%

Successful treatment of IgM paraproteinaemic neuropathy with fludarabine

Wilson

Lunn

Schey

et al. 1999

Journal of Neurology, Neurosurgery & Psychiatry

102

View full text Add to dashboard Cite

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“…None of the patients received treatment before the diagnosis of a hematological malignancy or MGUS, after the hematological evaluation including bone marrow examination. Treatment during follow‐up consisted of intermittent cyclophosphamide and prednisone,23 dexamethasone,24 and melphalan. Treatment response was evaluated using motor and sensory symptoms, and a positive response was defined as improvement or stabilization of symptoms during the year following treatment 23…”

Section: Methodsmentioning

confidence: 99%

Risk factors for hematological malignancy in polyneuropathy associated with monoclonal gammopathy

et al. 2001

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Polyneuropathy associated with monoclonal gammopathy of undetermined significance (MGUS) is a well-known disease entity. Of the patients with monoclonal gammopathy without neuropathy, 25% develop a hematological malignancy during long-term follow-up. Whether the frequency of hematological malignancy is similar in patients with polyneuropathy associated with monoclonal gammopathy and whether hematological screening is necessary in these patients is unknown. To determine the frequency of and risk factors for a hematological malignancy, we investigated 104 patients with polyneuropathy and monoclonal gammopathy. Potential diagnostic variables were obtained from medical history, physical and neurological examination, and laboratory analysis. The associations between potential diagnostic variables and outcome, hematological malignancy, were evaluated by univariable and multivariable logistic-regression analysis. Among our patients, 23 had a hematological malignancy (8 multiple myeloma, 10 low-grade lymphoma, 3 plasmacytoma, 1 Castleman's disease and 1 POEMS syndrome [polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes]). Weight loss, progression of the neuropathy, and an M-protein level > 1 g/L were independent risk factors for malignancy. Extensive screening is indicated in patients with these features.

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“…La corticothérapie a montré une amélioration d'un point sur les scores fonctionnels chez environ 50% des 27 patients recensés (schéma thérapeutique non disponible) mais surtout en association avec d'autres traitements dans une revue de séries et de cas rapportés 70 . Les effets indésirables de la dexaméthasone à haute dose par voie intraveineuse, notamment psychiatriques, chez trois des six patients traités pour une gammapathie monoclonale n'étaient pas acceptables au regard de l'efficacité 82 . Aucun patient sur huit n'a été amélioré par une corticothérapie orale à la dose de 60 mg par jour pendant au moins deux mois dans une étude rétrospective 81 .…”

Section: B) Corticostéroïdesunclassified