Treatment with hydroxyurea in a patient compound heterozygote for a high oxygen affinity hemoglobin and β‐thalassemia minor

The concept that central nervous system (CNS) could be a target of graft-versus-host disease (GVHD) is controversial. There are a few case reports which support the possibility of CNS-GVHD [1,2]. Here, we describe a patient who developed unique CNS symptoms soon after cord blood transplantation with reduced-intensity conditioning (RI-CBT). On Day 7 post-transplant, a high fever, slight skin eruption, moderate diarrhea, and liver damage suddenly developed. Three days later, her white blood cell (WBC) count rapidly increased to 1,700 μl ―1 and consisted mostly of mature lymphocytes. Generalized convulsions developed on the same day. An analysis of the cerebrospinal fluid (CSF) revealed elevated proteins and pleocytosis comprising mostly mature lymphocytes. The lymphocytes found in the peripheral blood (PB) and CSF were phenotypically polyclonal T-cells that were donor derived. Extensive investigations did not detect any microorganisms or other causes for the T-cell proliferation and CNS symptoms. Considering the coexistence of CNS and systemic GVHD-like symptoms, proliferation of donor-derived polyclonal T-cells in the CSF and PB, and no microorganisms or other factors detected, CNS GVHD seems to be the most likely explanation for her clinical course.