Annals of Hematology

, Volume 93, Issue 4, pp 693–697 | Cite as

Intracranial hemorrhage pattern in the patients with factor XIII deficiency

  • Majid Naderi
  • Tahereh Zarei
  • Sezaneh Haghpanah
  • Peyman Eshghi
  • Ebrahim Miri-Moghaddam
  • Mehran Karimi
Original Article

Abstract

Intracranial hemorrhage (ICH) is one of the most severe and life-threatening manifestations occurring in the patients with factor XIII (F XIII) deficiency. The aim of this study was to describe the ICH pattern in the patients suffering from F XIII deficiency. In this case series, we investigated 38 patients with severe F XIII deficiency in south of Iran from January to May 2012. ICH pattern, neurologic complications, efficacy of treatment, and incidence of recurrence were reported. The site of ICH was intraparenchymal in 35 patients (92.1 %), subdural in 2 patients (5.2 %), and epidural hemorrhage in 1 patient (2.6 %). Besides, neurologic complications occurred in 21 patients (55.2 %), including locomotor disability in 8, psychological impairment in 7, mental disorders in 5, speech impairment in 4, and visual impairment in 2. Prophylaxis was started with a dose of 10 IU/kg Fibrogammin every 4–6 weeks for all the patients, except for one. All the patients on prophylaxis showed good response without any episodes of recurrence, except for one. The most frequent site of ICH in our patients was intraparenchymal. It seems that long-term prophylactic treatment with a dose of 10 IU/kg Fibrogammin could be effective in the prevention of CNS bleeding in the patients with F XIII deficiency. Moreover, all the patients with severe F XIII deficiency even without severe bleeding symptoms are recommended to undergo prophylactic treatment.

Keywords

Factor XIII deficiency Intracranial hemorrhage Pattern Intraparenchymal hemorrhage 

Notes

Acknowledgments

The authors would like to thank Shirin Parand at the Hematology Research Center for her assistance in manuscript preparation. They are also grateful for A. Keivanshekouh at the Research Improvement Center of Shiraz University of Medical Sciences for improving the use of English in the manuscript.

Conflict of interest

The authors state that they have no interests which might be perceived as posing a conflict or bias.

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Copyright information

© Springer-Verlag Berlin Heidelberg 2013

Authors and Affiliations

  • Majid Naderi
    • 1
  • Tahereh Zarei
    • 2
  • Sezaneh Haghpanah
    • 2
  • Peyman Eshghi
    • 3
  • Ebrahim Miri-Moghaddam
    • 4
  • Mehran Karimi
    • 2
  1. 1.Ali ebn-e Abitaleb Hospital, Children and Adolescents Health Research CenterZahedan University of Medical SciencesZahedanIran
  2. 2.Hematology Research CenterShiraz University of Medical SciencesShirazIran
  3. 3.Pediatric Congenital Hematologic Disorders Research CenterShahid Beheshti University of Medical SciencesTehranIran
  4. 4.Department of Hematology and ImmunologyZahedan University of Medical SciencesZahedanIran

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