TY - JOUR
T1 - A case of chronic inflammatory demyelinating polyradiculoneuropathy concomitant with acquired von Willebrand syndrome
AU - Ueda, Maki
AU - Kawamura, Nobutoshi
AU - Tateishi, Takahisa
AU - Shigeto, Hiroshi
AU - Ohyagi, Yasumasa
AU - Kira, Jun Ichi
N1 - Copyright:
Copyright 2011 Elsevier B.V., All rights reserved.
PY - 2011/5
Y1 - 2011/5
N2 - We report a case of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) concomitant with acquired von Willebrand syndrome. A 33-year-old man developed motor and sensory polyneuropathy with electrophysiological conduction slowing. At this time, M-protein was absent. He was diagnosed with CIDP and received intravenous immunoglobulin and subsequent oral corticosteroids, which resulted in almost complete remission for over 10 years. At the age of 44, he presented with chronic anemia. Laboratory tests and colonoscopy revealed that he had acquired von Willebrand syndrome with monoclonal gammopathy of undetermined significance (IgG λ type) and colon cancer. Bleeding symptoms were resolved with intravenous immunoglobulin, but not with supplementation of factor VIII. Shortly after successful excision of the cancer, CIDP and acquired von Willebrand syndrome simultaneously recurred. Intravenous immunoglobulin produced rapid improvement of both neurological and hematological abnormalities. Concurring CIDP and acquired von Willebrand syndrome in the present case may indicate that the conditions have a partly common immunological background including monoclonal gammopathy and a potential common autoantibody-mediated mechanism. Alternatively, dysfunction of von Weillebrand factor may increase blood-nerve barrier permeability, inducing the recurrence of CIDP.
AB - We report a case of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) concomitant with acquired von Willebrand syndrome. A 33-year-old man developed motor and sensory polyneuropathy with electrophysiological conduction slowing. At this time, M-protein was absent. He was diagnosed with CIDP and received intravenous immunoglobulin and subsequent oral corticosteroids, which resulted in almost complete remission for over 10 years. At the age of 44, he presented with chronic anemia. Laboratory tests and colonoscopy revealed that he had acquired von Willebrand syndrome with monoclonal gammopathy of undetermined significance (IgG λ type) and colon cancer. Bleeding symptoms were resolved with intravenous immunoglobulin, but not with supplementation of factor VIII. Shortly after successful excision of the cancer, CIDP and acquired von Willebrand syndrome simultaneously recurred. Intravenous immunoglobulin produced rapid improvement of both neurological and hematological abnormalities. Concurring CIDP and acquired von Willebrand syndrome in the present case may indicate that the conditions have a partly common immunological background including monoclonal gammopathy and a potential common autoantibody-mediated mechanism. Alternatively, dysfunction of von Weillebrand factor may increase blood-nerve barrier permeability, inducing the recurrence of CIDP.
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U2 - 10.5692/clinicalneurol.51.334
DO - 10.5692/clinicalneurol.51.334
M3 - Article
C2 - 21706830
AN - SCOPUS:79959487725
VL - 51
SP - 334
EP - 337
JO - Clinical Neurology
JF - Clinical Neurology
SN - 0009-918X
IS - 5
ER -