title: Gamma-heavy chain disease: review of 23 cases.
abstract: We report the cases of 23 patients with gamma-heavy chain disease seen at our institution (8 patients previously reported, 15 new patients). There were 15 women and 8 men; the median age at diagnosis was 68 years (range, 42-87 yr). Sixteen patients had an associated lymphoplasma cell proliferative disorder, 3 had a lymphoplasma cell proliferative disorder and an autoimmune disorder, another 3 had an autoimmune disorder only, and 1 had no underlying disease. The lymphoplasma cell proliferative disorder was disseminated in 10 patients and localized in 6. Patients with localized lymphoplasma cell proliferative disorder included 3 with plasmacytoma (1 tongue, 1 submandibular area, and 1 thyroid), 2 with lymphoplasma cell proliferative disorder involving the bone marrow only, and 1 with amyloid of the skin. At the time of diagnosis, lymphadenopathy was present in 8 patients, splenomegaly in 7, and hepatomegaly in 1. A monoclonal spike on serum protein electrophoresis was documented in 19 patients. gamma-Heavy chain was documented by immunofixation in the serum of all patients; 2 had an additional immunoglobulin M-lambda. gamma-Heavy chain was present in the urine in 19 of 22 patients. Sixteen patients were treated for lymphoplasma cell proliferative disorder or autoimmune disorder (14 with chemotherapy, 1 splenectomy, and 1 thyroidectomy followed by radiation therapy). For 5 patients, treatment was not felt to be necessary; 2 patients were thought to be too sick for treatment. Of the 16 patients treated, 6 had a complete clinical response (in 2, gamma-heavy chain disappeared; in 2, gamma-heavy chain persisted; and for 2, no serologic follow-up was available); in 10 patients, clinical disease persisted (in 3, gamma-heavy chain disappeared; in 6, it persisted; and for 1, no serologic follow-up was available). Of 7 patients not treated, 2 died within 5 months; 1 died after 15 months; 2 had no clinical disease at latest follow-up, although gamma-heavy chain persisted; and 2 had no change in clinical and serologic status. The median duration of follow-up was 33 months (range, 1-261 mo). Median survival was 7.4 years.
authors.affiliation: Mayo Clinic, Rochester, Minnesota 55905, USA.
authors.forename: Dietlind L
authors.initials: DL
authors.lastname: Wahner-Roedler
authors.forename: Thomas E
authors.initials: TE
authors.lastname: Witzig
authors.forename: Laura L
authors.initials: LL
authors.lastname: Loehrer
authors.forename: Robert A
authors.initials: RA
authors.lastname: Kyle
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001327:Autoimmune Diseases / Q000150:complications / Q000453:epidemiology; D001402:B-Lymphocytes / Q000276:immunology; D005260:Female; D006362:Heavy Chain Disease / Q000150:complications / Q000276:immunology* / Q000401:mortality / Q000628:therapy; D006801:Humans; D007122:Immunoelectrophoresis; D007142:Immunoglobulin gamma-Chains / Q000097:blood / Q000276:immunology / Q000652:urine; D008232:Lymphoproliferative Disorders / Q000150:complications / Q000453:epidemiology / Q000276:immunology; D008297:Male; D008875:Middle Aged
chemical_list: D007142:Immunoglobulin gamma-Chains