TY - JOUR
T1 - γ-heavy chain disease review of 23 cases
AU - Wahner-Roedler, Dietlind L.
AU - Witzig, Thomas E.
AU - Loehrer, Laura L.
AU - Kyle, Robert A.
PY - 2003/7
Y1 - 2003/7
N2 - We report the cases of 23 patients with γ-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. γ-Heavy chain was documented by immunofixation in the serum of all patients; 2 had an additional immunoglobulin M-λ. γ-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, γ-heavy chain disappeared; in 2, γ-heavy chain persisted; and for 2, no serologic follow-up was available); in 10 patients, clinical disease persisted (in 3, γ-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 γ-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.
AB - We report the cases of 23 patients with γ-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. γ-Heavy chain was documented by immunofixation in the serum of all patients; 2 had an additional immunoglobulin M-λ. γ-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, γ-heavy chain disappeared; in 2, γ-heavy chain persisted; and for 2, no serologic follow-up was available); in 10 patients, clinical disease persisted (in 3, γ-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 γ-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.
UR - http://www.scopus.com/inward/record.url?scp=0037823442&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=0037823442&partnerID=8YFLogxK
U2 - 10.1097/00005792-200307000-00002
DO - 10.1097/00005792-200307000-00002
M3 - Review article
C2 - 12861101
AN - SCOPUS:0037823442
SN - 0025-7974
VL - 82
SP - 236
EP - 250
JO - Medicine
JF - Medicine
IS - 4
ER -