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Heavy chain disease

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The heavy chain diseases (HCDs) are rare B-cell malignancies that are distinguished by the production of a monoclonal immunoglobulin heavy chain (HC) without an associated light chain by the malignant B-cells. There are three types of HCD defined by the class of immunoglobulin (Ig) HC produced: IgA (α-HCD), IgG (μ-HCD), and IgM (γ-HCD). α-HCD is the most common and occurs most commonly as intestinal malabsorption in a young adult from a country bordering the Mediterranean Sea. Treatment consists of antibiotics and improved nutrition and hygiene. Surgery is occasionally required for patients with bulky masses at risk for bowel perforation. If there is no response to antibiotics or if aggressive non-Hodgkin’s lymphoma (NHL) is diagnosed, the patient should be treated with chemotherapy. Gamma- and γ-HCD are rare and essentially are found in patients with a B-cell NHL that produces an abnormal Ig heavy chain. These patients occasionally may be diagnosed with a monoclonal gammopathy of undetermined significance (MGUS). Patients with MGUS with NHL should be administered chemotherapy. Screening the serum and urine of patients with lymphoplasmacytoid NHL would likely identify more patients with γ- or μ-HCD.

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References and Recommended Reading

  1. Jaffe E, Harris N, Stein H, Vardiman J: Pathology and Genetics of Tumours of Haematopoietic and Lymphoid Tissues. Edited by Kleihues P, Sobin L. Lyon, France: IARC Press; 2001.

    Google Scholar 

  2. Fine KD, Stone MJ: Alpha-heavy chain disease, Mediterranean lymphoma, and immunoproliferative small intestinal disease: a review of clinicopathological features, pathogenesis, and differential diagnosis. Am J Gastroenterol 1999, 94:1139–1152.

    CAS  PubMed  Google Scholar 

  3. Seligmann M: [Heavy chain diseases]. Rev Prat 1993, 43:317–320.

    CAS  PubMed  Google Scholar 

  4. Rambaud JC, Galian A, Matuchansky C, et al.: Natural history of alpha-chain disease and the so-called Mediterranean lymphoma. Recent Results Cancer Res 1978, 64:271–276.

    Article  CAS  PubMed  Google Scholar 

  5. Al-Bahrani Z, Al-Saleem T, Al-Mondhiry H, et al.: Alpha heavy chain disease (report of 18 cases from Iraq). Gut 1978, 19:627–631.

    Article  CAS  PubMed  Google Scholar 

  6. Rambaud JC, Halphen M, Galian A, Tsapis A: Immunoproliferative small intestinal disease (IPSID): relationships with alpha-chain disease and "Mediterranean" lymphomas. Springer Semin Immunopathol 1990, 12:239–250.

    Article  CAS  PubMed  Google Scholar 

  7. Goossens T, Klein U, Kuppers R: Frequent occurrence of deletions and duplications during somatic hypermutation: implications for oncogene translocations and heavy chain disease. Proc Natl Acad Sci U S A 1998, 95:2463–2468.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  8. Sun T, Peng S, Narurkar L, et al.: Modified immunoselection technique for definitive diagnosis of heavychain disease. Clin Chem 1994, 40:664.

    CAS  PubMed  Google Scholar 

  9. Harzic M, Girard-Pipau F, Halphen M, et al.: [Bacteriological, parasitological and virological study of the digestive flora in alpha-chain disease]. Gastroenterol Clin Biol 1985, 9:472–479.

    CAS  PubMed  Google Scholar 

  10. Fermand JP, Brouet JC: Heavy-chain diseases. Hematol Oncol Clin North Am 1999, 13:1281–1294.

    Article  CAS  PubMed  Google Scholar 

  11. Lassoued K, Picard C, Danon F, et al.: Cutaneous manifestations associated with gamma heavy chain disease: report of an unusual case and review of literature. J Am Acad Dermatol 1990, 23:988–991.

    Article  CAS  PubMed  Google Scholar 

  12. Husby G: Is there a pathogenic link between gamma heavy chain disease and chronic arthritis? Curr Opin Rheumatol 2000, 12:65–70.

    Article  CAS  PubMed  Google Scholar 

  13. Wester SM, Banks PM, Li CY: The histopathology of gamma heavy-chain disease. Am J Clin Pathol 1982, 78:427–436.

    CAS  PubMed  Google Scholar 

  14. Kyle RA, Greipp PR, Banks PM: The diverse picture of gamma heavy-chain disease: report of seven cases and review of literature. Mayo Clin Proc 1981, 56:439–451.

    CAS  PubMed  Google Scholar 

  15. Agrawal S, Abboudi Z, Matutes E, Catovsky D: First report of fludarabine in gamma-heavy chain disease. Br J Haematol 1994, 88:653–655.

    Article  CAS  PubMed  Google Scholar 

  16. Otto S, Peter I, Vegh S, Juhos E, Besznyak I: Gammachain heavy-chain disease with primary thyroid plasmacytoma. Arch Pathol Lab Med 1986, 110:893–896.

    CAS  PubMed  Google Scholar 

  17. Galanti LM, Doyen C, Vander Maelen C, et al.: Biological diagnosis of a gamma-1-heavy chain disease in an asymptomatic patient. Eur J Haematol 1995, 54:202–204.

    Article  CAS  PubMed  Google Scholar 

  18. Wahner-Roedler DL, Kyle RA: Mu-heavy chain disease: presentation as a benign monoclonal gammopathy. Am J Hematol 1992, 40:56–60.

    Article  CAS  PubMed  Google Scholar 

  19. Iwasaki T, Hamano T, Kobayashi K, Kakishita E: A case of mu-heavy chain disease: combined features of mu-chain disease and macroglobulinemia. Int J Hematol 1997, 66:359–365.

    Article  CAS  PubMed  Google Scholar 

  20. Witzens M, Egerer G, Stahl D, et al.: A case of mu heavy-chain disease associated with hyperglobulinemia, anemia, and a positive Coombs test. Ann Hematol 1998, 77:231–234.

    Article  CAS  PubMed  Google Scholar 

  21. Tracy RP, Kyle RA, Young DS, et al.: Two-dimensional gel electrophoresis as an aid in the analysis of monoclonal gammopathies. Hum Pathol 1984, 15:122–129.

    Article  CAS  PubMed  Google Scholar 

  22. O’Reilly DS, Adjukiewicz A, Whicher JT: Biochemical findings in a case of mu-chain disease. Clin Chem 1981, 27:331–333.

    PubMed  Google Scholar 

  23. Kyle RA, Garton JP: The spectrum of IgM monoclonal gammopathy in 430 cases. Mayo Clin Proc 1987, 62:719–731.

    Article  CAS  PubMed  Google Scholar 

  24. Kyle RA, Greipp PR, Gertz MA, et al.: Waldenstrom’s macroglobulinaemia: a prospective study comparing daily with intermittent oral chlorambucil. Br J Haematol 2000, 108:737–742.

    Article  CAS  PubMed  Google Scholar 

  25. Zamir A, Parasher G, Moukarzel AA, et al.: Immunoproliferative small intestinal disease in a 16-year-old boy presenting as severe malabsorption with excellent response to tetracycline treatment. J Clin Gastroenterol 1998, 27:85–89.

    Article  CAS  PubMed  Google Scholar 

  26. Martin IG, Aldoori MI: Immunoproliferative small intestinal disease: Mediterranean lymphoma and alpha heavy chain disease. Br J Surg 1994, 81:20–24.

    Article  CAS  PubMed  Google Scholar 

  27. Khojasteh A, Saalabian M, Haghshenass M: Randomized comparison of abdominal irradiation (AI) vs CHOP vs C-MOPP for the treatment of immunoproliferative small intestinal disease (IPSID) associated lymphoma. Proc ASCO 1983, 2:207.

    Google Scholar 

  28. Salimi M, Spinelli JJ: Chemotherapy of Mediterranean abdominal lymphoma: retrospective comparison of chemotherapy protocols in Iranian patients. Am J Clin Oncol 1996, 19:18–22.

    Article  CAS  PubMed  Google Scholar 

  29. Ben-Ayed F, Halphen M, Najjar T, et al.: Treatment of alpha chain disease: results of a prospective study in 21 Tunisian patients by the Tunisian-French intestinal Lymphoma Study Group. Cancer 1989, 63:1251–1256.

    Article  CAS  PubMed  Google Scholar 

  30. Hubmann R, Kaiser W, Radaszkiewicz T, et al.: Malabsorption associated with a high-grade-malignant non-Hodgkin’s lymphoma, alpha-heavy-chain disease and immunoproliferative small intestinal disease. Z Gastroenterol 1995, 33:209–213.

    CAS  PubMed  Google Scholar 

  31. Akbulut H, Soykan I, Yakaryilmaz F, et al.: Five-year results of the treatment of 23 patients with immunoproliferative small intestinal disease: a Turkish experience. Cancer 1997, 80:8–14.

    Article  CAS  PubMed  Google Scholar 

  32. Price SK: Immunoproliferative small intestinal disease: a study of 13 cases with alpha heavy-chain disease. Histopathology 1990, 17:7–17.

    Article  CAS  PubMed  Google Scholar 

  33. Shih LY, Liaw SJ, Dunn P, Kuo TT: Primary smallintestinal lymphomas in Taiwan: immunoproliferative small-intestinal disease and nonimmunoproliferative small-intestinal disease. J Clin Oncol 1994, 12:1375–1382.

    CAS  PubMed  Google Scholar 

  34. Malik IA, Shamsi Z, Shafquat A, et al.: Clinicopathological features and management of immunoproliferative small intestinal disease and primary small intestinal lymphoma in Pakistan. Med Pediatr Oncol 1995, 25:400–406.

    Article  CAS  PubMed  Google Scholar 

  35. Khojasteh A, Haghighi P, Rabhi H, et al.: Immunoproliferative small intestinal disease: portrait of a potentially preventable cancer from the Third World. Am J Med 1990, 89:483–490.

    Article  CAS  PubMed  Google Scholar 

  36. McLaughlin P, Grillo-Lopez AJ, Link BK, et al.: Rituximab chimeric anti-CD20 monoclonal antibody therapy for relapsed indolent lymphoma: half of patients respond to a four-dose treatment program. J Clin Oncol 1998, 16:2825–2833.

    CAS  PubMed  Google Scholar 

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  1. Division of Hematology, Mayo Clinic and Mayo Foundation, 200 SW First Street, 55905, Rochester, MN, USA

    Thomas E. Witzig MD & Dietlind L. Wahner- Roedler MD

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  1. Thomas E. Witzig MD
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  2. Dietlind L. Wahner- Roedler MD
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Witzig, T.E., Roedler, D.L.W. Heavy chain disease. Curr. Treat. Options in Oncol. 3, 247–254 (2002). https://doi.org/10.1007/s11864-002-0014-3

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