Rapidly Progressive Polyneuropathy in a Patient With Monoclonal Gammopathy: A Case Report of POEMS Syndrome and Beyond.
Neuropathy, the dominant clinical feature of POEMS syndrome, is typically distal, symmetric, and slowly progressive with demyelinating changes. After a gradual proximal spread, it usually results in severe muscle weakness and functional disabilities. Cases characterized by acute onset polyneuropathy are rarely described. In the present report, we describe a 32-year-old male diagnosed as POEMS syndrome, but presenting with a rapidly evolving polyneuropathy. Detailed clinical, electrophysiological, and genetic studies revealed a coexisting underdiagnosed inherited axonal neuropathy, namely Charcot-Marie-Tooth disease 2A2. The patient received lenalidomide-based chemotherapy and consolidated by autologous stem cell transplantation for his POEMS syndrome, which improved the neurological disability. In most conditions, only 1 cause is responsible for a patient's polyneuropathy. However, an insidious inherited neuropathy can be overlooked, when an acquired condition is present. The case illustrated here, to the best of our knowledge, is the first one with coexistent axonal type Charcot-Marie-Tooth disease and POEMS syndrome, suggesting that an unrecognized inherited neuropathy may change the disease course of a further acquired neuropathy.
From the Department of Hematology (CW, Q-QC, D-BZ, JL); Department of Neurology (Y-ZG); and Department of Clinical Laboratory (WS), Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.
Chen
C
Wang
Yu-Zhou
YZ
Guan
Qian-Qian
QQ
Cai
Wei
W
Su
Dao-Bin
DB
Zhou
Jian
J
Li
D000328:Adult; D003937:Diagnosis, Differential; D018450:Disease Progression; D005819:Genetic Markers; D005820:Genetic Testing; D018380:Hematopoietic Stem Cell Transplantation / Q000379:methods*; D006801:Humans; D007155:Immunologic Factors / Q000627:therapeutic use; D000077269:Lenalidomide; D008297:Male; D016878:POEMS Syndrome / Q000175:diagnosis* / Q000235:genetics / Q000628:therapy; D013792:Thalidomide / Q000031:analogs & derivatives* / Q000627:therapeutic use
D005819:Genetic Markers; D007155:Immunologic Factors; D013792:Thalidomide; D000077269:Lenalidomide