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Case Reports
. 2019 Jun;6(2):55-57.
doi: 10.1016/j.ijpam.2019.05.006. Epub 2019 May 28.

Massive recurrent post-tonsillectomy bleedings revealing a transient factor XIII deficiency in a 10-year-old boy. A case report

M Jankovic  1 , M L Choucair  1 , B Hallak  2 , E Hernandez  1 , M Russo  1 , J Llor  1 , S Kayemba-Kay's  1
Affiliations
Case Reports

Massive recurrent post-tonsillectomy bleedings revealing a transient factor XIII deficiency in a 10-year-old boy. A case report

M Jankovic et al. Int J Pediatr Adolesc Med. 2019 Jun.

Erratum in

Abstract

A previously healthy 10-year-old boy was hospitalized for a left cervical abscess associated with massive tonsillar hypertrophy. He underwent abscess drainage and bilateral tonsillectomy. At H36 post-surgery, he presented with tonsillar hemorrhage requiring surgical revision. Hemorrhage relapsed 2 days later, with a total of 7 episodes, 5 of which required surgical revisions. Laboratory investigations were normal except for a markedly low factor XIII (FXIII) activity at 7%. After administration of a single dose of 40 IU/kg plasma-derived FXIII (Fibrogammin®) I.V., the bleeding stopped with no further recurrence. FXIII activity gradually normalized (75%) at 6 weeks, confirming the transient character of factor XIII deficiency. Severe congenital FXIII deficiency (FXIIID) (<1%) is very rare (1:2,000,000 births), whereas partial congenital deficiency and/or acquired deficiency may be more frequent but likely underreported. Acquired FXIIID may result from impaired synthesis (liver failure) or increased consumption (surgery, sepsis, leukemia, Henoch-Schönlein, inflammatory bowel disease, stroke, disseminated intravascular coagulation). FXIII replacement in form of fresh frozen plasma (FFP) or plasma-derived FXIII may be necessary for the presence of bleeding.

Keywords: Bleeding; Management; Tonsillectomy; Transient factor XIII deficiency.

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Figures

Fig. 1
Fig. 1
Oral cavity inflammation post surgical revision.
Fig. 2
Fig. 2
Plasma FXIII activity and therapies administered over time.
See this image and copyright information in PMC

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