“Pre HSCT SARS-CoV2 infection in an ADA deficiency SCID patient successfully treated with remdesivir: a case report”

SARS-CoV2 infection before hematopoietic stem cell transplant (HSCT) is lifethreating in severe combined immunodeficiency (SCID) patients.
In 2020, Remdesivir proved its effectiveness in adult patients; later it proved safe in children and by April 2022, its use was expanded in the US.
We report a girl, first child of healthy unrelated parents, full term born with no pathologies, who received standard national immunization. She presented at 6 months old with a rhino/enterovirus infection and one month later, with a severe pneumonia requiring ICU and mechanical ventilation (MV). Bronchoalveolar lavage showed E. coli, P. jirovecii and BCG. She had lymphopenia and abnormal proliferation. Genetic test: compound heterozygous ADA mutation. She improved under antimycobacterial and Co-Trimoxazole treatment. She was discharged with immunoglobulins, antifungal and anti-BCG prophylaxis. No enzymatic or genetic treatments were available. She had no match donors; we decided on a haploidentical HSCT from her father. She evolved with a SARS-CoV2 infection before HSCT. She received a 10 days Remdesivir cycle and remained asymptomatic but with persistent virus shedding. She evolved with norovirus diarrhea and Burkholderia cepacia bacteremia. The conditioning regimen was Treosulfan, Fludarabine, ATG and PTCy. Prophylaxis with Cyclosporine and Mofetil Mycophenolate. CD34: 6,5 x10e6/Kg. She evolved with fluid overload and needed MV. She engrafted with donor chimerism >95%. Day +30: CD3: 328 xmm3 CD4 268 x mm3 IgG 771. She was discharged and by day +65 she had a negative SARS-CoV2 PCR. She evolved with Epstein Barr Virus reactivation treated with Rituximab and waxing and waning skin BCG granulomas. No GvHD. On day +180, CD4 278 CD19 0 NK 436 IgG 1599.
Remdesivir in children is safe and provides clinical stability or improvement.
Remdesivir should be used in SCID with SARS-CoV2 infection before HSCT.

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