Case Report: Hematopoietic Stem Cell Transplantation to Treat Severe Acquired Aplastic Anemia in a Pediatric Kidney Transplant Recipient.

BACKGROUND

Hematopoietic stem cell transplantation (HSCT) in solid organ transplant recipients has been reported in adults. However, data on children are scarce. We report a case of an allogeneic HSCT in a 14-year-old girl to treat idiopathic very severe aplastic anemia (SAA).

CASE PRESENTATION

The girl developed end-stage renal disease at the age of 4 years following Shiga toxin hemolytic-uremic syndrome. The cadaveric kidney was grafted at the age of 7 years. Three years later, the patient was successfully treated for active humoral graft rejection and continued with tacrolimus and antihypertensive treatment. At 10 years, an absence epilepsy manifested; therefore, lamotrigine and ethosuximide were added. After 7 years of having a kidney transplant, the patient developed very severe pancytopenia and was diagnosed with SAA. Parvovirus B19 and EBV infections were documented. At the age of 14 years, she received allogeneic hematopoietic stem cells from a matched unrelated CMV-seronegative donor. Neutrophils engrafted on Day +19 and full donor chimerism was achieved. An acute graft-versus-host disease grade II regressed after the escalation of immune suppression, which aggravated arterial hypertension and triggered CMV reactivation treated with glomerular filtration rate-adjusted ganciclovir. Antiviral therapy deteriorated renal graft function. A high-risk transplantation-associated thrombotic microangiopathy was diagnosed on Day +42 and treated with eculizumab. Despite adoptive therapy with CMV-specific cytotoxic T-lymphocytes (Day +62) the pericardial effusion developed and required surgical drainage. Nevertheless, CMV viremia and polyserositis gradually progressed to multiorgan failure. The patient died on Day +95 after HSCT.

CONCLUSIONS

Despite reported favorable outcomes in children who received allogeneic HSCT after kidney transplantation, there is a lack of evidence on how to overcome numerous challenges in these ultrarare cases.