Rare B-cell lymphoplasma-cell proliferative disorders characterized by production of truncated monoclonal immunoglobulin heavy chains without associated light chains.
Presents as an extranodal marginal-zone lymphoma of mucosa-associated lymph-node tissue (MALT)
γ-HCD (variable presentation):
Presents as lymphoplasmacytoid non-Hodgkin lymphoma (NHL)
μ-HCD (variable presentation):
Presents as small lymphocytic non-Hodgkin lymphoma or chronic lymphocytic leukaemia
Characteristic Clinicopathologic Features of the Heavy Chain Diseases (HCD) | The Proteasome. The. (2020) The Heavy Chain Diseases: Clinical and Pathologic Features | Cancer Network. Retrieved February 10, 2020, from https://www.cancernetwork.com/oncology-journal/heavy-chain-diseases-clinical-and-pathologic-features
Pathophysiology
Immunoglobulin Molecule in Heavy Chain Disease | The Proteasome. The. (2020) The Heavy Chain Diseases: Clinical and Pathologic Features | Cancer Network. Retrieved February 10, 2020, from https://www.cancernetwork.com/oncology-journal/heavy-chain-diseases-clinical-and-pathologic-features
Histopathology:
Pathology of Alpha Heavy Chain Disease | Giada Bianchi, MDKenneth C. Anderson, MDNancy Lee Harris, MDAliyah R. Sohani, MD. (2014) The Heavy Chain Diseases: Clinical and Pathologic Features | Cancer Network. Retrieved February 10, 2020, from https://www.cancernetwork.com/oncology-journal/heavy-chain-diseases-clinical-and-pathologic-features
Pathology of Gamma Heavy Chain Disease | Giada Bianchi, MDKenneth C. Anderson, MDNancy Lee Harris, MDAliyah R. Sohani, MD. (2014) The Heavy Chain Diseases: Clinical and Pathologic Features | Cancer Network. Retrieved February 10, 2020, from https://www.cancernetwork.com/oncology-journal/heavy-chain-diseases-clinical-and-pathologic-features
Pathology of Mu Heavy Chain Disease | Giada Bianchi, MDKenneth C. Anderson, MDNancy Lee Harris, MDAliyah R. Sohani, MD. (2014) The Heavy Chain Diseases: Clinical and Pathologic Features | Cancer Network. Retrieved February 10, 2020, from https://www.cancernetwork.com/oncology-journal/heavy-chain-diseases-clinical-and-pathologic-features
Diagnosis
Immunoglobulin heavy chain without a bound light chain in the serum or urine
Serum Protein Electrophoresis (SPEP) and Urine Protein Electrophoresis (UPEP) With Immunofixation in Gamma Heavy Chain Disease | The Proteasome. The. (2020) The Heavy Chain Diseases: Clinical and Pathologic Features | Cancer Network. Retrieved February 10, 2020, from https://www.cancernetwork.com/oncology-journal/heavy-chain-diseases-clinical-and-pathologic-features
Management
Combination chemotherapy:
CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone)
CHVP (cyclophosphamide, doxorubicin, teniposide, and prednisone)
