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.

ACQUIRED INHIBITOR AGAINST FACTOR IX IN A CHILD: SUCCESSFUL TREATMENT WITH HIGH-DOSE IMMUNOGLOBULINE AND DEXAMETHASONE / Mazzucconi, Maria Gabriella; Peraino, M; Bizzoni, L; Bernasconi, S; Luciani, M; DE ROSSI, G.. - In: HAEMOPHILIA. - ISSN 1351-8216. - 5:(1999), pp. 127-131. [10.1046/j.1365-2516.1999.t01-1-00297.x]

ACQUIRED INHIBITOR AGAINST FACTOR IX IN A CHILD: SUCCESSFUL TREATMENT WITH HIGH-DOSE IMMUNOGLOBULINE AND DEXAMETHASONE

MAZZUCCONI, Maria Gabriella;
1999

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.
1999
inhibitors to factor IX:C; acquired coagulation inhibitors; high-dose Ig; dexamethasone; immunosuppressive therapy
01 Pubblicazione su rivista::01a Articolo in rivista
ACQUIRED INHIBITOR AGAINST FACTOR IX IN A CHILD: SUCCESSFUL TREATMENT WITH HIGH-DOSE IMMUNOGLOBULINE AND DEXAMETHASONE / Mazzucconi, Maria Gabriella; Peraino, M; Bizzoni, L; Bernasconi, S; Luciani, M; DE ROSSI, G.. - In: HAEMOPHILIA. - ISSN 1351-8216. - 5:(1999), pp. 127-131. [10.1046/j.1365-2516.1999.t01-1-00297.x]
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11573/90387
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