Heavy Chain Disease, Mu Treatment & Management

  • Author: Ajeet Gajra, MD; Chief Editor: Emmanuel C Besa, MD   more...
 
Updated: Jan 2, 2010
 

Medical Care

  • The clinical course is variable, with survival ranging from 1 month to more than 10 years after the appearance of symptoms. Because of its low incidence, no large series of patients treated in a systematic way at a single center has been reported. Common drugs used include alkylating agents such as chlorambucil, melphalan, and cyclophosphamide, but patient responses have been inconsistent. More recently, fludarabine phosphate (Fludara) has been used.
  • No specific treatment exists. Currently, the finding of a mu-HCD protein in the serum of an apparently healthy patient should be considered a monoclonal gammopathy of unknown significance, and the patient should be monitored closely for the development of a symptomatic lymphoproliferative disorder. If this develops, chemotherapy targeted at the specific disorder is used. The same holds true if a patient presents with a treatable clinical disease such as CLL and the presence of mu-HCD.
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Surgical Care

Surgical care usually is not required, although special circumstances may require surgery (eg, surgery needed to fix a pathologic fracture).

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Consultations

  • Diagnosis of mu-HCD often can be difficult to establish. Consulting a hematopathologist is appropriate to facilitate an appropriate and adequate workup.
  • Occasionally, consultation with a radiation oncologist may be required to treat a pathologic fracture site after surgical correction or to prophylactically treat a site of bony involvement to prevent a pathologic fracture.
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Diet

No dietary restrictions are necessary.

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Activity

No activity restrictions are necessary.

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Contributor Information and Disclosures
Author

Ajeet Gajra, MD  Assistant Professor of Medicine, Director of Hematology/Oncology Fellowship Program, State University of New York Upstate Medical University; Consulting Staff, Department of Internal Medicine, Division of Hematology and Oncology, Veterans Affairs Medical Center

Ajeet Gajra, MD is a member of the following medical societies: American Association for Cancer Research, American College of Physicians-American Society of Internal Medicine, American Medical Association, and American Society of Hematology

Disclosure: Nothing to disclose.

Coauthor(s)

Sara J Grethlein, MD  Associate Dean for Graduate Medical Education, Professor, Department of Internal Medicine, Division of Hematology and Oncology, State University of New York Upstate Medical University

Sara J Grethlein, MD is a member of the following medical societies: American Society of Hematology

Disclosure: Nothing to disclose.

Specialty Editor Board

Paul Schick, MD  Emeritus Professor, Department of Internal Medicine, Thomas Jefferson University Medical College; Research Professor, Department of Internal Medicine, Drexel University College of Medicine; Adjunct Professor of Medicine, Lankenau Hospital, Wynnewood, PA

Paul Schick, MD is a member of the following medical societies: American College of Physicians, American Heart Association, American Society of Hematology, International Society on Thrombosis and Haemostasis, and New York Academy of Sciences

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Senior Pharmacy Editor, eMedicine

Disclosure: eMedicine Salary Employment

Troy H Guthrie, Jr, MD  Director of Cancer Institute, Baptist Medical Center

Troy H Guthrie, Jr, MD is a member of the following medical societies: American Federation for Medical Research, American Medical Association, American Society of Hematology, Florida Medical Association, Medical Association of Georgia, and Southern Medical Association

Disclosure: Nothing to disclose.

Rajalaxmi McKenna, MD, FACP  Southwest Medical Consultants, SC, Department of Medicine, Good Samaritan Hospital, Advocate Health Systems

Rajalaxmi McKenna, MD, FACP is a member of the following medical societies: American Society of Clinical Oncology, American Society of Hematology, and International Society on Thrombosis and Haemostasis

Disclosure: Nothing to disclose.

Chief Editor

Emmanuel C Besa, MD  Professor, Department of Medicine, Division of Hematologic Malignancies, Kimmel Cancer Center, Thomas Jefferson University

Emmanuel C Besa, MD is a member of the following medical societies: American Association for Cancer Education, American College of Clinical Pharmacology, American Federation for Medical Research, American Society of Hematology, and New York Academy of Sciences

Disclosure: Nothing to disclose.

References

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  2. Bakhshi A, Guglielmi P, Coligan JE, et al. A pre-translational defect in a case of human mu heavy chain disease. Mol Immunol. Jul 1986;23(7):725-32. [Medline].

  3. Mihaesco C, Ferrara P, Guillemot JC, et al. A new extra sequence at the amino terminal of a mu heavy chain disease protein (DAG). Mol Immunol. Aug 1990;27(8):771-6. [Medline].

  4. Bonhomme J, Seligmann M, Mihaesco C, et al. MU-chain disease in an African patient. Blood. Apr 1974;43(4):485-92. [Medline].

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  9. Maisnar V, Tichy M, Stulik J, et al. Capillary immunotyping electrophoresis and high resolution two-dimensional electrophoresis for the detection of mu-heavy chain disease. Clin Chim Acta. Mar 2008;389(1-2):171-3. [Medline].

  10. Yanai M, Maeda A, Watanabe N, et al. Successful treatment of mu-heavy chain disease with fludarabine monophosphate: a case report. Int J Hematol. Feb 2004;79(2):174-7. [Medline].

  11. O'Connell TX, Horita TJ, Kasravi B. Understanding and interpreting serum protein electrophoresis. Am Fam Physician. Jan 1 2005;71(1):105-12. [Medline].

  12. Buxbaum JN, Alexander A. Heavy Chain Diseases. In: Beutler B, Lichtman MA, Coller BS, Kipps TJ, and Seligsohn U, eds. Williams Hematology. 6th ed. New York, NY: McGraw-Hill; 2001:1327-36.

  13. Fermand JP, Brouet JC. Heavy-chain diseases. Hematol Oncol Clin North Am. Dec 1999;13(6):1281-94. [Medline].

  14. 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. Oct 1997;66(3):359-65. [Medline].

  15. Liapis H, Papadakis I, Nakopoulou L. Nodular glomerulosclerosis secondary to mu heavy chain deposits. Hum Pathol. Jan 2000;31(1):122-5. [Medline].

  16. Preud'homme JL, Bauwens M, Dumont G, et al. Cast nephropathy in mu heavy chain disease. Clin Nephrol. Aug 1997;48(2):118-21. [Medline].

  17. Wahner-Roedler DL, Kyle RA. Mu-heavy chain disease: presentation as a benign monoclonal gammopathy. Am J Hematol. May 1992;40(1):56-60. [Medline].

 
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