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.
Subject(s)
Heavy Chain Disease/immunology , Adult , Aged , Aged, 80 and over , Autoimmune Diseases/complications , Autoimmune Diseases/epidemiology , B-Lymphocytes/immunology , Female , Heavy Chain Disease/complications , Heavy Chain Disease/mortality , Heavy Chain Disease/therapy , Humans , Immunoelectrophoresis , Immunoglobulin gamma-Chains/blood , Immunoglobulin gamma-Chains/immunology , Immunoglobulin gamma-Chains/urine , Lymphoproliferative Disorders/complications , Lymphoproliferative Disorders/epidemiology , Lymphoproliferative Disorders/immunology , Male , Middle AgedSubject(s)
Hemangiosarcoma/therapy , Intestinal Neoplasms/therapy , Intestine, Small , Adult , Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Combined Modality Therapy/methods , Female , Heavy Chain Disease/mortality , Heavy Chain Disease/therapy , Hemangiosarcoma/mortality , Humans , Immunoglobulin alpha-Chains , Intestinal Neoplasms/mortality , Lymphatic Metastasis , Lymphoma, Large B-Cell, Diffuse/mortality , Lymphoma, Large B-Cell, Diffuse/therapy , Lymphoma, Non-Hodgkin/mortality , Lymphoma, Non-Hodgkin/therapy , Male , Middle Aged , Palliative CareABSTRACT
Making the diagnosis of alpha-chain disease in the laboratory is difficult. Immunologic and structural characteristics of the abnormal protein were shown in results of cellular studies. There are clinicopathological features associated with the protein abnormality. The peculiar geographic origin of patients affected with alpha-chain disease and the possible reversibility of the hyperplastic process at its early stage lead to some considerations of cause.