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Multiple Myeloma With Polyneuropathy and Coagulopathy

Bradley T. Hyman

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Abstract

A patient with the syndrome of polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes (POEMS) had multiple myeloma and IgA-kappa monoclonal gammopathy. To our knowledge, of over 50 cases in the literature, this is the only instance of kappa light chains noted in a patient with the complete syndrome. Sural nerve biopsy revealed increased immunofluorescence for IgA and kappa chains. Furthermore, this patient had a hemorrhagic diathesis characterized by a prolonged bleeding time and a prolonged thrombin time due to a qualitative platelet dysfunction and an apparent inhibition of fibrin monomer polymerization. In none of the other cases of POEMS syndrome was a coagulopathy reported. A short course of plasmapheresis improved the bleeding time but did not alter the polyneuropathy.

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What this paper is about

A patient with the syndrome of polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes (POEMS) had multiple myeloma and IgA-kappa monoclonal gammopathy. To our knowledge, of over 50 cases in the literature, this is the only instance of kappa light chains noted in a patient with the complete syndrome. Sural nerve biopsy revealed increased immunofluorescence for IgA and kappa chains. Furthermore, this patient had a hemorrhagic diathesis characterized by a prolonged bleeding time and a prolonged thrombin time due to a qualitative platelet dysfunction and an apparent inhibition of fibrin monomer polymerization. In none of the other cases of POEMS syndrome was a coagulopathy reported. A short course of plasmapheresis improved the bleeding time but did not alter the polyneuropathy.

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Available abstract

A patient with the syndrome of polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes (POEMS) had multiple myeloma and IgA-kappa monoclonal gammopathy. To our knowledge, of over 50 cases in the literature, this is the only instance of kappa light chains noted in a patient with the complete syndrome. Sural nerve biopsy revealed increased immunofluorescence for IgA and kappa chains. Furthermore, this patient had a hemorrhagic diathesis characterized by a prolonged bleeding time and a prolonged thrombin time due to a qualitative platelet dysfunction and an apparent inhibition of fibrin monomer polymerization. In none of the other cases of POEMS syndrome was a coagulopathy reported. A short course of plasmapheresis improved the bleeding time but did not alter the polyneuropathy.

Key concepts: Polyneuropathy, Coagulopathy, Medicine, Bleeding diathesis, Organomegaly, Fibrin, Plasmapheresis, Multiple myeloma

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