A case report: isolated a heavy chain monoclonal gammopathy in a patient with polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin change syndrome.
A 45-year-old South-Korean man presented with abdominal distension, progressive paresthesia and motor weakness of both lower extremities. Our case was identified as polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin change (POEMS) syndrome based on diagnostic criteria. Circulating M components of POEMS syndrome consist mainly of IgG or IgA-lambda and rarely IgM-lambda, IgG-kappa or isolated light chains. In this case, the M-band on serum protein electrophoresis and isolated IgA heavy chain on serum immunofixation electrophoresis were demonstrated, but there was no abnormal light chain. We suggest that this case may be associated with a pattern of abnormal secretion of monoclonal protein or a coincidence of a heavy chain disease in POEMS syndrome, even though the latter possibility may be very rare.
Kim
S K
SK
Department of Internal Medicine, The Hospital for Rheumatic Diseases, Seoul, South Korea.
Park
I K
IK
Park
B H
BH
Park
W
W
Lee
H S
HS
Kim
T H
TH
Jun
J B
JB
Bae
S C
SC
Yoo
D H
DH
Uhm
W S
WS
D001853:Bone Marrow / Q000000981:diagnostic imaging; D006362:Heavy Chain Disease / Q000175:diagnosis*; D006801:Humans; D007070:Immunoglobulin A / Q000097:blood; D007137:Immunoglobulin alpha-Chains / Q000097:blood; D008297:Male; D008875:Middle Aged; D016878:POEMS Syndrome / Q000175:diagnosis*; D010996:Pleural Effusion / Q000000981:diagnostic imaging; D001261:Pulmonary Atelectasis / Q000000981:diagnostic imaging; D011859:Radiography; D011877:Radionuclide Imaging
D007070:Immunoglobulin A; D007137:Immunoglobulin alpha-Chains