The Spectrum of Atypical Graft-Versus-Host Disease in Pediatric Hematopoietic Stem Cell Transplantation: A Twenty-Year Follow up Study from India
摘要
With the increasing numbers of alternate donor hematopoietic stem cell transplantations (HSCT), graft versus host disease (GVHD) manifestations involving organs including the bone marrow, central nervous system (CNS), and kidneys are increasingly reported. We aimed to analyze data on GVHD involving atypical organs and the variables impacting outcomes. A retrospective study was performed where children up to 18 years of age diagnosed with atypical GVHD from January 2002 to June 2023 were included. A total of 1340 children underwent HSCT during the study period, with 26 (2%) developing atypical GVHD. Twenty-three (88%) had non-malignant disorders, while 3 (12%) had malignancies. Haploidentical HSCT was the most common donor type in 18 (69%) cases, followed by matched unrelated donor (MUD) transplants in 5 (19%) cases, and matched family donor (MFD) transplants in 3 (12%) cases. The most common atypical GVHD manifestation was autoimmune hemolytic anemia (AIHA), observed in 16 (61%) patients. CNS and renal involvement were noted in 4 (15%) patients each, while 2 (8%) patients developed myasthenia gravis respectively. Overall survival in the cohort was 21/26 (80%). GVHD specific overall survival was 13/16 (81.2%) in AIHA, 3/4 (75%) in renal GVHD, 3/4 (75%) in CNS GVHD, and 100% in myasthenia gravis. The study reports atypical GVHD in 2% of the cohort, particularly in alternate donor HSCT. A high index of suspicion and early interventions may help improve outcomes in these children.