Heavy-chain diseases (HCDs)


Rare B-cell lymphoplasma-cell proliferative disorders characterized by production of truncated monoclonal immunoglobulin heavy chains without associated light chains.

  • Variant types of non-Hodgkin lymphoma

Classification

α-HCD “Seligman’s Disease” (M/C & M/uniform presentation)

  • 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
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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

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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:


Diagnosis

  • Immunoglobulin heavy chain without a bound light chain in the serum or urine
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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)
  • ABV (doxorubicin, bleomycin, and vinblastine)

 


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