Poster Display Therapy

LONG-TERM SAFETY OF FACILITATED SUBCUTANEOUS IMMUNOGLOBULIN IN PATIENTS WITH CVID ASSOCIATED TO AUTOIMMUNE THROMBOCYTOPENIA

Lecture Time
10:46 - 10:47
Presenter
  • Veronica Pedini, Italy
Room
Poster Area
Date
20.09.2019, Friday
Session Time
10:00 - 17:00
Board Number
196
Presentation Topic
Therapy

Abstract

Background and Aims

Autoimmunity in CVID patients is frequently described, with an estimated prevalence of 30% of all CVID patients. Among these complications, autoimmune thrombocytopenia (ITP) are the most commonly reported. The aim of our work was to evaluate the safety of the facilitated subcutaneous administration of immunoglobulin (f-SCIg) in patients with ITP CVID-related.

Methods

We described three women with CVID, diagnosed according to ESID criteria. All of the patients presented symptomatic ITP. In one of them ITP was the onset manifestation of the related immune disorder. The others two cases had a long-history of refractory ITP in course of CVID, treated with steroids and intravenous immunoglobulin with relapses. One patient presented residual splenic tissue after splenectomy performed many years before.

Results

All patients were treated with Immune Globulin Infusion 10% (Human) with Recombinant Human Hyaluronidase (Hyqvia ®, Shire Pharmaceuticals) at the initial dosage of 20 g every 2 weeks. All patients achieved remission of the thrombocytopenia without relapse during the follow-up period (respectively 52 months for two patients, 28 months for the latter). No hemorrhagic complications due to the subcutaneous infusions were reported. Nor hemolytic nor thrombotic events were observed. Patients did not report systemic adverse events related to the infusions, but only mild local reactions in the infusion site. Once remission was obtained, we gradually increased the interval between infusions to 4 weeks, maintaining a stable remission, with platelets values persistently > 90.000/mmc.

Conclusions

f-SCIg is an effective and safe option of treatment in CVID related ITP.

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