European Journal of Pediatrics

, Volume 158, Issue 5, pp 379–383

Anaemia, thrombocytopenia and coagulopathy due to occult diffuse infantile haemangiomatosis of spleen and pancreas

Authors

  • A. S. Schulz
    • Universität Ulm, Kinderklinik, Prittwitzstrasse 43, D-89075 Ulm, Germany, Fax: +49-731-5027785
  • J. Urban
    • Universität Ulm, Kinderklinik, Prittwitzstrasse 43, D-89075 Ulm, Germany, Fax: +49-731-5027785
  • P. Gessler
    • Universität Zürich, Kinderspital, Zürich, Switzerland
  • W. Behnisch
    • Universität Ulm, Kinderklinik, Prittwitzstrasse 43, D-89075 Ulm, Germany, Fax: +49-731-5027785
  • E. Kohne
    • Universität Ulm, Kinderklinik, Prittwitzstrasse 43, D-89075 Ulm, Germany, Fax: +49-731-5027785
  • B. Heymer
    • Universität Ulm, Pathologie, Ulm, Germany
HEMATOLOGY/ONCOLOGY

DOI: 10.1007/s004310051096

Cite this article as:
Schulz, A., Urban, J., Gessler, P. et al. Eur J Pediatr (1999) 158: 379. doi:10.1007/s004310051096

Abstract

Diffuse infantile haemangiomatosis of the spleen is a very rare lesion. Large haemangiomas may cause trapping of platelets and coagulation disorders known as Kasabach-Merrit syndrome. We here report the case of an infant with splenic and pancreatic haemangiomatosis presenting with life-threatening thrombocytopenia, anaemia and intravascular coagulation. Diagnosis was hampered by reactive erythroblastosis and non-conclusive radiological findings. While treatment with corticosteroids was ineffective, administration of antithrombin III improved coagulation parameters. After splenectomy the child recovered promptly and has remained free of disease for 3 years to date.

Conclusion Occult visceral haemangiomatosis without visible cutaneous haemangiomas should be included in the differential diagnosis of thrombocytopenia, anaemia and consumption coagulopathy. Antithrombin III treatment may be considered to overcome bleeding problems in patients with Kasabach-Merrit syndrome.

Key words Antithrombin IIICoagulationHaemangiomatosisKasabach-Merrit syndrome

Copyright information

© Springer-Verlag Berlin Heidelberg 1999