CHARGE association, hyper-immunoglobulin M syndrome, and conjunctival MALT lymphoma

Cornea. 2007 Aug;26(7):864-7. doi: 10.1097/ICO.0b013e31806c77d6.


Purpose: To report a case of a 5-year-old child with CHARGE association and bilateral conjunctival mucosa-associated lymphoid tissue (MALT) lymphoma treated with topical interferon-alpha.

Methods: Case report.

Results: A 5-year-old girl, diagnosed with nonclassical CHARGE association and hyper-immunoglobulin M (IgM) syndrome, was referred with a 2-month history of suspected purulent bilateral streptococcal conjunctivitis. Clinical symptoms did not resolve despite multiple antibiotic treatments, and her clinical course was attributed to underlying immunodeficiency. Biomicroscopy showed salmon-colored, nodular lesions occupying both fornices and caruncles. Inferior conjunctival biopsy was performed, and MALT lymphoma was diagnosed. Bilateral treatment was initiated with topical interferon-alpha, applied 3 times a day at a concentration of 5 x 10 U/m/d, for 4 months. Complete regression of symptoms and conjunctival lesions was achieved. No recurrences have been observed after 1-year follow-up.

Conclusions: We report the use of topical interferon-alpha as treatment for bilateral conjunctival MALT lymphoma in a young child with CHARGE association and hyper-IgM syndrome.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Abnormalities, Multiple*
  • Antineoplastic Agents / therapeutic use
  • Child, Preschool
  • Choanal Atresia / complications
  • Coloboma / complications
  • Conjunctival Neoplasms / complications*
  • Conjunctival Neoplasms / drug therapy
  • Ear / abnormalities
  • Female
  • Genitalia, Female / abnormalities
  • Growth Disorders / complications
  • Humans
  • Hyper-IgM Immunodeficiency Syndrome / complications*
  • Interferon alpha-2
  • Interferon-alpha / therapeutic use
  • Lymphoma, B-Cell, Marginal Zone / complications*
  • Lymphoma, B-Cell, Marginal Zone / drug therapy
  • Recombinant Proteins


  • Antineoplastic Agents
  • Interferon alpha-2
  • Interferon-alpha
  • Recombinant Proteins