Involved tissues usually include mucosa-associated lymphoid tissues and evidence a histology of lymphoplasmacytoid infiltrates accompanied by large numbers of plasma cells and small lymphocytes. The plasma cells therein express the monoclonal α chain and therefore are clonal in nature and the sole or contributing producer of the α chain myeloma protein. Some 57% to 66% of patients present with disseminated lymphoma, 17% to 36% of patients present with a localized lymphoma, and 9% to 17% of patients lack any evidence of a lymphoplasmacytic neoplasm. A majority of the latter patients have an autoimmune disease or a chronic infection which may be responsible for, or contribute to, production of the α heavy chain. Studies indicated that a sub-set particularly of the digestive form of heavy chain disease is caused by infection. This is based on findings that the majority of α heavy chain disease patients are in the lower economic class living under unsanitary conditions, that gastrointestinal bacterial and parasitic infections have been documented in many of these patients, and that long-term (>6 months) appropriately selected antibiotic therapy has improved the condition in 33% to 71% of patients who are at an early stage of the disease and documented to be infected. However, these patients frequently relapse. Patients resistant to antibiotic trials have been treated with multiple drug chemotherapy to obtain complete remission rates of 64% and an overall 5 year survival of 67%.
Necrolytic acral erythema is a cutaneous condition that is a manifestation of hepatitis C viral infection or zinc deficiency.It is a papulosquamous and sometimes vesiculobullous eruption bearing clinical and histologic similarity to other necrolytic erythemas such as necrolytic migratory erythema, pseudoglucagonoma and nutritional deficiency syndromes. See also
List of cutaneous conditions

References


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