Multiple myeloma with polyneuropathy and coagulopathy. A case report of the polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin change (POEMS) syndrome.
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.
Hyman
B T
BT
Westrick
M A
MA
D001778:Blood Coagulation Disorders / Q000150:complications*; D006801:Humans; D007147:Immunoglobulin Light Chains / Q000276:immunology; D007145:Immunoglobulin kappa-Chains / Q000276:immunology; D008297:Male; D008875:Middle Aged; D009101:Multiple Myeloma / Q000150:complications*; D009194:Myeloma Proteins / Q000097:blood; D010265:Paraproteinemias / Q000150:complications; D011115:Polyneuropathies / Q000150:complications*; D012871:Skin Diseases / Q000150:complications; D013577:Syndrome
D007147:Immunoglobulin Light Chains; D007145:Immunoglobulin kappa-Chains; D009194:Myeloma Proteins