Acquired inhibitor against factor IX in a child: Successful treatment with high-dose immunoglobulin and dexamethasone

M. G. Mazzucconi, M. Peraino, L. Bizzoni, S. Bernasconi, M. Luciani, G. De Rossi

Research output: Contribution to journalArticle

10 Citations (Scopus)

Abstract

The occurrence of acquired inhibitor against factor IX:C is infrequent in haemophilia B patients and is very rare in previously healthy subjects, in whom it is often related to underlying diseases. We describe the case of a 2-year-old girl, who was referred to our hospital with haematomas, without previous bleeding history. Prolonged APTT, normal PT and a factor IX:C level below 1% were found. An inhibitor against factor IX:C was detected (5.5 U mL-1. Her father and mother showed normal factor IX:C levels. Treatment with high-dose immunoglobulin (400 mg kg-1 day-1 for 5 consecutive days by intravenous infusion) and dexamethasone (4 mg three times a day by intravenous injection for 4 consecutive days) normalized factor IX:C levels and overcame the inhibitor. In conclusion, high-dose immunoglobulin and high-dose dexamethasone are a successful and safe immunosuppressive approach for recovery from inhibitor occurrence.

Original languageEnglish
Pages (from-to)132-134
Number of pages3
JournalHaemophilia
Volume5
Issue number2
DOIs
Publication statusPublished - 1999

Fingerprint

Factor IX
Dexamethasone
Immunoglobulins
Hemophilia B
Therapeutics
Immunosuppressive Agents
Intravenous Infusions
Intravenous Injections
Fathers
Hematoma
Healthy Volunteers
Mothers
Hemorrhage

Keywords

  • Acquired coagulation inhibitors
  • Dexamethasone
  • High-dose Ig
  • Immunosuppressive therapy
  • Inhibitors to factor IX:C

ASJC Scopus subject areas

  • Hematology

Cite this

Acquired inhibitor against factor IX in a child : Successful treatment with high-dose immunoglobulin and dexamethasone. / Mazzucconi, M. G.; Peraino, M.; Bizzoni, L.; Bernasconi, S.; Luciani, M.; De Rossi, G.

In: Haemophilia, Vol. 5, No. 2, 1999, p. 132-134.

Research output: Contribution to journalArticle

Mazzucconi, M. G. ; Peraino, M. ; Bizzoni, L. ; Bernasconi, S. ; Luciani, M. ; De Rossi, G. / Acquired inhibitor against factor IX in a child : Successful treatment with high-dose immunoglobulin and dexamethasone. In: Haemophilia. 1999 ; Vol. 5, No. 2. pp. 132-134.
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