Poster Display Therapy

OUTCOME OF BACILLUS CALMETTE-GUÉRIN DISEASE AFTER HEMATOPOIETIC STEM CELL TRANSPLANTATION IN CHILDREN WITH PRIMARY IMMUNODEFICIENCY

Lecture Time
10:26 - 10:27
Presenter
  • Monia Ouederni, Tunisia
Room
Poster Area
Date
20.09.2019, Friday
Session Time
10:00 - 17:00
Board Number
192
Presentation Topic
Therapy

Abstract

Background and Aims

Disseminated BCG disease is often fatal and results from impaired immunity. The aim of our study is to describe the outcome of BCG disease in immune-compromised (PIDs) children receiving haematopoietic stem cell transplantation (HSCT).

Methods

We included all patients how underwent HSCT for PIDs in whom BCG infection occurred before or after HSCT. Isoniazide and rifampycine were discontinued before conditioning to ovoid drug interactions and switched to ethambutol and ciprofloxacin.

Results

Eleven included patients received HSCT for CMH class II deficiency (n= 2), Wiskott Aldrich syndrome (n= 2), chronic granulomatous disease (n=1), Interferon-gamma receptor deficiency (n=1) and severe combined immunodeficiency (n= 5). BCG disease onset was prior to HSCT in 10 patients and after HSCT in one patient. Before HSCT, BCG disease was loco-regional in four patients and disseminated in six patients. Five patients had a sustained response to treatment, two patients relapsed and three patients had persistent disease. After HSCT, one patient developed regional BCG disease at one month. One patient showed persistent disseminated BCGite requiring splenectomy at 12 months post HSCT. BCG disease treatment was continued for at least 6 months in all patients with improvement in clinical signs and progressive regression of lymphadenitis. Good immune reconstitution was acheived in 10 patients without recurrence of BCG disease. One patient died from severe graft versus host disease one month after HSCT.

Conclusions

BCG disease is lethal in severe PIDs if not treated. Combined with prolonged medical therapy, HSCT has significantly improved survival once immune reconstitution was achieved.

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