Optical Coherence Tomography Angiography of Macular Microangiopathy in Children With Sickle Cell Disease.

In this study, we identified the presence of sickle cell maculopathy and determined correlations between hemolysis indicators and systemic and ocular manifestations in children with sickle cell disease (SCD). Thirty-three patients with SCD 5 to 18 years of age underwent optical coherence tomography angiography (OCTA) as part of a thorough eye examination. The hematological indices and clinical data (hemoglobin, fetal hemoglobin, mean corpuscular volume, and frequency of sickle cell-associated complications and therapy) were collected. The SCD group contained 33 patients (66 eyes), and the control group contained 36 healthy participants (72 eyes). The SCD group had significantly thinner parafoveal thickness (P=0.041) and significantly smaller parafoveal volume (P=0.041) than the controls. The SCD group had lower deep capillary plexus density than the controls (P=0.029). The SCD group had significantly smaller flow areas than the controls (P<0.001). The foveal avascular zone (FAZ) perimeter, foveal density, and FAZ area in the entire retina did not differ significantly between the groups (P>0.05). The platelet level was negatively correlated with parafoveal and perifoveal thickness and density. OCTA detected early macular microvascular changes in children and adolescents with asymptomatic SCD.

Abatacept-based Graft-Versus-Host Disease Prophylaxis in Haplo-identical Hematopoietic Cell Transplant in a High-risk Cohort.

BACKGROUND: There is insufficient guidance in using posttransplant cyclophosphamide in patients with organ dysfunctions. Abatacept (Aba), a T cell costimulation blockade, has recently been shown to prevent severe acute graft-versus-host disease (GVHD). OBSERVATION: We report adding Aba as GVHD prophylaxis in 4 pediatrics patients who received haplo-hematopoietic cell transplantation. Two patients had grade 2 acute GVHD and 2 had mild chronic GVHD. All 4 patients are alive with full donor chimerism, and 3 are off immunosuppressants. CONCLUSION: An Aba-based regimen can result in reliable engraftment and acceptable GVHD when concerns of organ dysfunction prevents the use of posttransplant cyclophosphamide in haplo-hematopoietic cell transplantation.

Central nervous system relapse in a child with anaplastic large cell lymphoma: potential for new therapeutic strategies.

BACKGROUND: Central nervous system (CNS) relapse is rare in childhood anaplastic large cell lymphoma (ALCL) and is associated with a poor prognosis. CASE: We describe an 8-year-old boy with ALCL who developed an early CNS relapse without initial CNS disease. Despite aggressive medical management, the patient's neurological status deteriorated rapidly and he died shortly after. CONCLUSION: Optimal treatment for children with relapsed ALCL involving the CNS remains unclear. Novel agents, including ALK inhibitors, that have CNS-penetration might be helpful and pediatric studies are warranted.