Hairy cell leukaemia complicated by anti-MAG paraproteinemic demyelinating neuropathy: resolution of neurological syndrome after cladribrine treatment.
Hairy cell leukaemia (HCL) occasionally displays a monoclonal gammopathy, yet the association of HCL with paraproteinemic demyelinating neuropathy (PDN) has not been reported. We describe a HCL case complicated by PDN and high titers of monoclonal IgM against myelin associated glycoprotein (MAG). Heavy and light chains of the patient's anti-MAG monoclonal protein were consistent with those expressed by HCL cells. After treatment with cladribrine, remission of HCL strictly paralleled disappearance of the IgM monoclonal protein and of the serum anti-MAG activity, and led to PDN clinical and electrophysiological improvement. Purine analogs may represent a choice in IgM PDN associated with lymphoproliferative disorders.
Rossi
Davide
D
Division of Haematology, Department of Clinical and Experimental Medicine & IRCAD, Amedeo Avogadro University of Eastern Piedmont, Novara, Italy. rossidav@med.unipmn.it
Franceschetti
Silvia
S
Cerri
Michaela
M
Conconi
Annarita
A
Lunghi
Monia
M
Capello
Daniela
D
Cantello
Roberto
R
Gaidano
Gianluca
G
D000368:Aged; D017338:Cladribine / Q000008:administration & dosage*; D020278:Demyelinating Autoimmune Diseases, CNS / Q000150:complications* / Q000188:drug therapy* / Q000276:immunology; D015973:Gene Expression Regulation, Leukemic / Q000187:drug effects / Q000276:immunology; D006801:Humans; D007143:Immunoglobulin Heavy Chains / Q000276:immunology; D007147:Immunoglobulin Light Chains / Q000276:immunology; D007075:Immunoglobulin M / Q000378:metabolism; D007166:Immunosuppressive Agents / Q000008:administration & dosage*; D007943:Leukemia, Hairy Cell / Q000150:complications* / Q000188:drug therapy* / Q000276:immunology; D008297:Male; D018992:Myelin-Associated Glycoprotein / Q000276:immunology; D010265:Paraproteinemias / Q000150:complications* / Q000188:drug therapy* / Q000276:immunology; D012074:Remission Induction
D007143:Immunoglobulin Heavy Chains; D007147:Immunoglobulin Light Chains; D007075:Immunoglobulin M; D007166:Immunosuppressive Agents; D018992:Myelin-Associated Glycoprotein; D017338:Cladribine