Disease Information for Alpha Heavy Chain Disease: Definition

  • IgA Heavy Chain Disease

    differentiate from IgG Gamma Heavy Chain Disease (Franklin"s Disease), Alpha Heavy Chain Disease (Seligmann"s Disease), and IgM Mu Heavy Chain Disease;

    The heavy chain diseases are rare lymphoplasmacytic malignancies; Their clinical manifestations vary with the heavy chain isotype; Patients secrete a defective heavy chain that usually has an intact Fc fragment and a deletion in the Fd region; Gamma, alpha, and mu heavy chain diseases have been described, but no reports of delta or epsilon heavy chain diseases have appeared; This disease affects people of widely different age groups and countries of origin; It is characterized by lymphadenopathy, fever, anemia, malaise, hepatosplenomegaly, and weakness; Its most distinctive symptom is palatal edema, resulting from node involvement of Waldeyer"s ring, and this may progress to produce respiratory compromise; The diagnosis depends on the demonstration of an anomalous serum M component [often <20 g/L (<2 g/dL) that reacts with anti-IgG but not anti-light chain reagents; The M component is typically present in both serum and urine; Most of the paraproteins have been of the gamma1 subclass; ---------[Harrisons online 2007]------

    Alpha heavy chain disease: A clonal disorder, also known as immunoproliferative small intestinal disease or Mediterranean lymphoma, characterised by the secretion of a defective alpha heavy chain; It predominantly affects young people in the Mediterranean region; It involves the small intestine, and patients usually present with malabsorption syndrome, abdominal pain, weight loss, and fever; There is extensive villous atrophy of the small intestinal mucosa, which is heavily infiltrated by small lymphocytes and plasma cells; The small intestinal morphologic changes are consistent with a mucosa-associated lymphoid tissue lymphoma (MALT lymphoma); Alpha heavy chain disease frequently progresses to large B-cell lymphoma, and normally has a poor prognosis; -- 2004 -------------[wrong diagnosis online 2007]--------------------------------