Palatal edema is significant for:
Correct Answer: Gamma heavy chain disease
Description: Ans: (b) Gamma heavy chain diseaseWHO, 2016, Page 235-237The heavy chain diseases (HCDs) are three rare B-cell neoplasms characterized by the production of monoclonal immunoglobulin heavy chains (IgG in gamma HCD, IgA in alpha HCD and IgM in mu HCD) and typically no light chains.Gamma Heavy Chain Disease - Also known as Franklin DiseaseThe disease involves the lymph nodes, Waldeyer ring, gastrointestinal tract predominantly.Clinical Features:Autoimmune manifestations are reported in 25-70% of cases,Involvement of the Waldeyer ring - presents with Palatal Edema*.Cell of origin - A post-germinal centre B cell that can differentiate into a plasma cell.IHCThe cells express CD79a and cytoplasmic gamma chain and are negative for CD5 and CD10.Kappa and lambda light chains are typically NOT expressed.
Category:
Pathology
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