Skip to main content
SpringerLink
Log in

A fatal case of portal hypertension complicating systemic mastocytosis in an adolescent

  • Gastroenterology/Hepatology
  • Original Paper
  • Published:
European Journal of Pediatrics Aims and scope Submit manuscript

Abstract

Portal hypertension was observed in a 17-year-old girl with urticaria pigmentosa since 2 months of age. Liver biopsies showed portal and sinusoidal infiltration with mast cells although spleen biopsies showed only fibrosis.

Conclusion

Portal hypertension is a complication of systemic mastocytosis that can lead to death. Treatment with interferon alpha might be effective.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

References

  1. Bonnet P, Smadja C, Szekely AM, et al (1987) Intractable ascites in systemic mastocytosis treated by portal diversion. Dig Dis Sci 32:209–213

    Article  PubMed  Google Scholar 

  2. Capron JP, Lebrec D, Degott C, Chivrac D, Coevoet B, Delobel J (1978) Portal hypertension in systemic mastocytosis. Gastroenterology 74: 595–597

    PubMed  Google Scholar 

  3. Ghandur-Mnaymneh L, Gould E (1985) Systemic mastocytosis with portal hypertension. Autopsy findings and ultrastructural study of the liver. Arch Pathol Lab Med 109:76–78

    PubMed  Google Scholar 

  4. Grundfest S, Cooperman AM, Ferguson R, Benjamin S (1980) Portal hypertension associated with systemic mastocytosis and splenomegaly. Gastroenterology 78:370–373

    PubMed  Google Scholar 

  5. Kettelhut BV, Metcalfe D (1991) Pediatric mastocytosis. J Invest Dermatol 96:15S-18S

    Article  PubMed  Google Scholar 

  6. Kluin-Nelemans HC, Jansen JH, Breukelman H, Wolthers BG, Kluin PM, Kroon HM, Willenke R (1992) Response to interferon alpha-2b in a patient with systemic mastocytosis. N Engl J Med 326:619–623

    PubMed  Google Scholar 

  7. Narayanan MN, Liu Yin JA, Azzawi S, Warnes TW, Turck WPG (1989) Portal hypertension and ascites in systemic mastocytosis. Postgrad Med J 65:394–396

    PubMed  Google Scholar 

  8. Pulik M, Lionnet F, Petit A, Genet P, Gaulier A (1994) Long term response to interferon alpha in a patient with systemic mastocytosis and chronic myelomonocytic leukemia. Am J Hematol 47:66

    Google Scholar 

  9. Sawers AH, Davson J, Braganza J, Geary CG (1982) Systemicmastocytosis, myelofibrosis and portal hypertension. J Clin Pathol 35:617–619

    PubMed  Google Scholar 

  10. Silvain C, Levillain P, Mouton P, Carretier M, Babin P, Beauchant M (1989) Variceal bleeding and systemic mastocytosis. Gastroenterol Clin Biol 13:834–837

    PubMed  Google Scholar 

  11. Sumpio BE, O'Leary G, Gusberg RJ (1988) Variceal bleeding, hypersplenism, and systemic mastocytosis. Arch Surg 123:767–769

    PubMed  Google Scholar 

  12. Travis WD, Li CH, Bergstralh EJ, Yam LT, Swee RG (1988) Systemic mast cell disease. Analysis of 58 cases and literature review. Medicine 67:345–368

    PubMed  Google Scholar 

  13. Vincenzi V, Costan Biedo F, Carrabba E, Benvegnu B, Furlanetto A, Basevi A (1992) Systemic mastocytosis with portal hypertension and hepatic dysfunction. Minerva Med 83:563–565

    PubMed  Google Scholar 

  14. Yam LT, Chan CH, Li CY (1986) Hepatic involvement in systemic mast cell disease. Am J Med 80:819–826

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Service de Pédiatric, Hôpital Huriez, 1 Place de Verdun, F-59037, Lille Cedex, France

    Hortense S. Fonga-Djimi, Frederic Gottrand, Michel Bonnevalle & Jean-Pierre Farriaux

Authors
  1. Hortense S. Fonga-Djimi
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Frederic Gottrand
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Michel Bonnevalle
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Jean-Pierre Farriaux
    View author publications

    You can also search for this author in PubMed Google Scholar

Rights and permissions

Reprints and permissions

About this article

Cite this article

Fonga-Djimi, H.S., Gottrand, F., Bonnevalle, M. et al. A fatal case of portal hypertension complicating systemic mastocytosis in an adolescent. Eur J Pediatr 154, 819–821 (1995). https://doi.org/10.1007/BF01959789

Download citation

  • Received:

  • Accepted:

  • Issue Date:

  • DOI: https://doi.org/10.1007/BF01959789

Key words

Search

Navigation