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[Recurrent hematomas and normal standard hemostasis tests]

E Wiel1, B Marciniak, B Wibaut

  • 1Département d'anesthésie-réanimation chirurgicale 2, hôpital Claude-Huriez, CHU Lille, France.

Annales Francaises D'Anesthesie Et De Reanimation
|September 29, 1998
PubMed

Insights

A child with recurrent bleeding and a large subcutaneous hematoma was diagnosed with inherited factor XIII deficiency. Treatment with factor XIII concentrate successfully managed the condition and prevented recurrence.

Area of Science:

  • Hematology
  • Pediatric Medicine
  • Genetics

Background:

  • Recurrent bleeding disorders in children can be challenging to diagnose.
  • Subcutaneous hematomas, especially following trauma, warrant thorough investigation for underlying coagulopathies.

Observation:

  • A 22-month-old boy presented with a large, recurrent subcutaneous hematoma after head trauma.
  • Initial coagulation tests were normal, despite a history of bleeding since birth and consanguineous parents.

Findings:

  • Detailed coagulation factor analysis revealed a severe inherited factor XIII deficiency (less than 2%).
  • Administration of factor XIII concentrate was followed by favorable hematoma resolution.

Implications:

  • This case highlights the importance of investigating severe factor deficiencies even with normal standard coagulation screening.
  • Factor XIII concentrate is an effective treatment for factor XIII deficiency, and prophylactic therapy can prevent recurrent bleeding episodes.

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