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heavy chain disease; Franklin disease

Lymphocytes secrete a defective heavy chain with an intact Fc fragment & a deletion in the Fd region. Alpha, gamma & mu heavy chain disease has been described. Epsilon & delta heavy chain disease has not been reported.

Specific

alpha-chain disease gamma-chain disease mu-chain disease

General

lymphoproliferative disorder paraproteinemia (monoclonal gammopathy)

Properties

PREVALENCE: EXTREMELY-RARE

References

  1. Cotran et al Robbins Pathologic Basis of Disease, W.B. Saunders Co, Philadelphia, PA 1989 pg 744
  2. Harrison's Principles of Internal Medicine, 13th ed. Isselbacher et al (eds), McGraw-Hill Inc. NY, 1994, pg 1624-255