The role of high-dose dexamethasone in auto-immune refractory thrombocytopaenia

Abstract

The therapeutic approach to Autoimmune
Thrombocytopaenic Purpura (ITP) refractory
to corticosteroids and splenectomy is controversial. Several second line therapies, including
immunosuppression, have received attention in the international literature.
We describe a case of a 36-year-old woman
with a 9-year history of ITP, refractory to corticosteroids and splenectomy, as well as to immunossupression with azathioprine for 2 years. Intravenous immunoglobulin (IVIG) was
used during the episodes of thrombocytopaenia with platelet levels below 10x109/L, with a
good, yet transient response and an increasing
number of hospital admissions (18 during 2 years). In view of the absence of a sustained response to the 1st and 2nd line therapeutic options and the transient response to IVIG, a 3rd line
regimen, described in the literature, of pulsed
high doses of oral dexamethasone (40 mg/day
for 4 days), with a 28 day interval, for 6 months, was started. After the last course and with
a follow-up of 2 years, the platelet counts have
remained persistently above 100x109/L, without evidence of haemorrhage.
This particular case provides additional evidence favouring the use of oral mega-doses of
dexamethasone as an effective and simple therapeutic option for refractory ITP, with favourable effects extended beyond the period of therapy.