TY - CHAP M1 - Book, Section TI - Heavy-Chain Disease A1 - Wahner-Roedler, Dietlind L. A1 - Kyle, Robert A. A2 - Kaushansky, Kenneth A2 - Lichtman, Marshall A. A2 - Prchal, Josef T. A2 - Levi, Marcel M. A2 - Press, Oliver W. A2 - Burns, Linda J. A2 - Caligiuri, Michael Y1 - 2015 N1 - T2 - Williams Hematology, 9e AB - SUMMARYThe heavy-chain diseases (HCDs) are B-cell lymphoplasmacytic proliferative disorders in which neoplastic cells produce monoclonal immunoglobulins (Ig) consisting of truncated heavy chains (HCs) without attached light chains. The complex abnormalities of HCD proteins and the usual lack of normal light chains are a result of several distinct gene alterations, including somatic mutations, deletions, and insertions. HCDs involving the three main immunoglobulin classes have been described: α-HCD is the most common and has the most uniform presentation; γ- and μ-HCDs have variable clinical presentations and histopathologic features. The diagnosis is established from immunofixation of serum, urine, or secretory fluids in the case of α-HCD or by immunohistologic analysis of the proliferating lymphoplasmacytic cells in nonsecretory disease. Treatment of α-HCD consists of antibiotics. If there is no response to antibiotics or if aggressive lymphoma is diagnosed, chemotherapy is indicated. Treatment of γ- and μ-HCDs depends on the underlying clinicopathologic features rather than on the presence of the abnormal protein. Table 110–1 summarizes the features of the HCDs. SN - PB - McGraw-Hill Education CY - New York, NY Y2 - 2024/03/28 UR - accessmedicine.mhmedical.com/content.aspx?aid=1121101639 ER -