Mu-heavy chain disease: presentation as a benign monoclonal gammopathy.
mu-Heavy chain disease (HCD) is a rare monoclonal lymphoid disorder characterized by the failure to assemble a complete IgM immunoglobulin. The mu-heavy chains analyzed to date revealed absence of the variable region and a shortened constant domain. We report the first case of mu-HCD presenting as a benign monoclonal gammopathy. The literature on the 27 reported mu-HCD cases is reviewed, and important clinical and laboratory findings are discussed. The ages of the patients ranged from 15 to 80 years (median, 57.5 years). Twenty-two of 27 patients had an associated lymphoplasma cell proliferative disorder. A monoclonal spike on routine serum protein electrophoresis was found in only 8 of 19 patients. Fourteen of 22 patients had Bence Jones proteinuria, but mu-HCD protein was reported in the urine of only two patients. The survival ranged from less than 1 month to 11 years (median, 24 months).
Wahner-Roedler
D L
DL
Division of Area Medicine, Mayo Clinic, Rochester, Minnesota 55905.
Kyle
R A
RA
D006362:Heavy Chain Disease / Q000503:physiopathology*; D006801:Humans; D007148:Immunoglobulin mu-Chains / Q000737:chemistry; D008232:Lymphoproliferative Disorders / Q000503:physiopathology; D008297:Male; D008875:Middle Aged
D007148:Immunoglobulin mu-Chains