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Journal of Gastrointestinal Cancer

, Volume 41, Issue 4, pp 281–284 | Cite as

A Case of Explosive Progression of Hepatocellular Carcinoma in a Patient with Common Variable Immunodeficiency (CVID)

  • Kaushang GandhiEmail author
  • Purvi Parikh
  • Wilbert S. Aronow
  • Harit Desai
  • Harshad Amin
  • Mala Sharma
  • Arye Rubinstein
Brief Communication

Abstract

Objective

While it is well known that patients with common variable immunodeficiency (CVID) are predisposed to various malignancies, primarily non-Hodgkin’s lymphoma and gastric carcinomas, to our knowledge no cases of hepatocellular carcinoma have been reported in the absence of preexisting liver disease.

Method and Results

We report a 50-year-old male patient with CVID with a B- and T-cell deficiency. The patient was on prophylactic intravenous gammaglobulin and had received several years earlier a course of rituximab for an autoimmune disorder. He had no history of hepatitis. The patient developed a rapidly progressing hepatocellular carcinoma within 3 to 4 weeks.

Conclusions

Although patients with CVID are predisposed to malignancies such as lymphoma and adenocarcinoma of the stomach, rapidly progressive hepatocellular carcinoma in the absence of any preexisting liver disease has not been described.

Keywords

common variable immunodeficiency hepatocellular carcinoma 

References

  1. 1.
    Di Renzo M, Pasqui AL, Auteri A. Common variable immunodeficiency: a review. Clin Exp Med. 2004;3:211–7.CrossRefPubMedGoogle Scholar
  2. 2.
    Schaffer AA, Salzer U, Hammarstrom L, Grimbacher B. Deconstructing common variable immunodeficiency by genetic analysis. Curr Opin Genet Dev. 2007;17:201–12.CrossRefPubMedGoogle Scholar
  3. 3.
    Cunningham-Rundles C, Bodian C. Common variable immunodeficiency: clinical and immunological features of 248 patients. Clin Immunol. 1999;92:34–48.CrossRefPubMedGoogle Scholar
  4. 4.
    Conley ME, Notarangelo LD, Etzioni A. Diagnostic criteria for primary immunodeficiencies: representing PAGID (Pan-American Group for Immunodeficiency) and ESID (European Society for Immunodeficiencies). Clin Immunol. 1999;93:190–7.CrossRefPubMedGoogle Scholar
  5. 5.
    Hermaszewski RA, Webster AD. Primary hypogammaglobulinaemia: a survey of clinical manifestations and complications. Q J Med. 1993;86:31–42.PubMedGoogle Scholar
  6. 6.
    Quinti I, Soresina A, Spadaro G, Martino S, Donnanno S, Agostini C, et al. Long-term follow-up and outcome of a large cohort of patients with common variable immunodeficiency. J Clin Immunol. 2007;27:308–16.CrossRefPubMedGoogle Scholar
  7. 7.
    Fukushima K, Ueno Y, Kanegane H, Yamagiwa Y, Inoue J, Kido O, et al. A case of severe recurrent hepatitis with common variable immunodeficiency. Hepatol Res. 2008;38:415–20.CrossRefPubMedGoogle Scholar
  8. 8.
    Deane S, Selmi C, Naguwa SM, Teuber SS, Gershwin ME. Common variable immunodeficiency. Etiological and treatment issues. Int Arch Allergy Immunol. 2009;150(4):311–24.CrossRefPubMedGoogle Scholar
  9. 9.
    Park MA, Li JT, Hagan JB, Maddox DE, Abraham RS. Common variable immunodeficiency: a new look at an old disease. Lancet. 2008;372:489–502.CrossRefPubMedGoogle Scholar
  10. 10.
    Kinlen LJ, Webster AD, Bird AG, Haile R, Peto J, Soothill JF, et al. Prospective study of cancer in patients with hypogammaglobulinaemia. Lancet. 1985;1:263–6.CrossRefPubMedGoogle Scholar
  11. 11.
    Mellemkjaer L, Hammarstrom L, Andersen V, et al. Cancer risk among patients with IgA deficiency or common variable immunodeficiency and their relatives: a combined Danish and Swedish study. Clin Exp Immunol. 2002;130:495–500.CrossRefPubMedGoogle Scholar
  12. 12.
    Cunningham-Rundles C, Cooper DL, Duffy TP, Strauchen J. Lymphomas of mucosal-associated lymphoid tissue in common variable immunodeficiency. Am J Hematol. 2002;69:171–8.CrossRefPubMedGoogle Scholar
  13. 13.
    Cunningham-Rundles C, Siegal FP, Cunningham-Rundles S, Lieberman P. Incidence of cancer in 98 patients with common varied immunodeficiency. J Clin Immunol. 1987;7:294–9.CrossRefPubMedGoogle Scholar
  14. 14.
    Mellemkjaer L, Hammarstrom L, Andersen V, Yuen J, Heilmann C, Barington T, et al. Cancer risk among patients with IgA deficiency or common variable immunodeficiency and their relatives: a combined Danish and Swedish study. Clin Exp Immunol. 2002;130:495–500.CrossRefPubMedGoogle Scholar
  15. 15.
    Zenone T, Souquet PJ, Cunningham-Rundles C, Bernard JP. Hodgkin’s disease associated with IgA and IgG subclass deficiency. J Intern Med. 1996;240:99–102.CrossRefPubMedGoogle Scholar
  16. 16.
    Sumazaki R, Matsubara T, Aoki T, Nagai Y, Shibasaki M, Takita H. Rapidly progressive hepatitis C in a patient with common variable immunodeficiency. Eur J Pediatr. 1996;155(7):532–4.CrossRefPubMedGoogle Scholar
  17. 17.
    Bonilla FA, Bernstein IL, Khan DA, et al. Practice parameter for the diagnosis and management of primary immunodeficiency. Ann Allergy Asthma Immunol. 2005;94(5 suppl 1):S1–63.CrossRefPubMedGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC 2010

Authors and Affiliations

  • Kaushang Gandhi
    • 1
    • 4
    Email author
  • Purvi Parikh
    • 2
  • Wilbert S. Aronow
    • 1
  • Harit Desai
    • 1
  • Harshad Amin
    • 1
  • Mala Sharma
    • 1
  • Arye Rubinstein
    • 3
  1. 1.Department of Internal MedicineWestchester Medical Center/New York Medical CollegeNew YorkUSA
  2. 2.Department of Internal MedicineCleveland ClinicClevelandUSA
  3. 3.Department of Pediatrics, Microbiology and ImmunologyAlbert Einstein College of Medicine and Montefiore HospitalBronxUSA
  4. 4.New YorkUSA

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