Late-onset acute autograft versus host disease occurring five years after autologous hematopoietic stem cell transplantation: a case report

Graft-versus-host disease (GVHD) is a well-recognized multisystemic disorder that occurs following hematopoietic stem cell transplantation (HSCT). It arises due to a dysregulated immune response elicited by graft immune cells against host antigens, leading to widespread immune-mediated damage, typically affecting the skin, liver, gastrointestinal tract, and hematopoietic system. It classically occurs following allogeneic HSCT and within one year of the transplant. This case was unusual, occurring nearly five years after an autologous HSCT. We describe a 54-year-old Sri Lankan, Sinhalese man who presented with multisystem involvement, including cutaneous lesions, ocular inflammation, pleural effusion, and neutropenia refractory to granulocyte colony-stimulating factor (G-CSF). He had undergone autologous stem cell transplantation for multiple myeloma and had previously undergone bortezomib-based chemotherapy. After excluding infections such as tuberculosis and hematological malignancies, including myeloma relapse and autoinflammatory syndromes, the patient was clinically and histologically diagnosed with late-onset auto-GVHD. The neutropenia, occurring in the context of a normal bone marrow, was attributed to immune-mediated peripheral destruction. He responded well to corticosteroids, with subsequent initiation of ruxolitinib due to steroid dependence. GVHD should be considered in the differential diagnosis of multisystem involvement following HSCT, even after autologous transplants and beyond the typical timeframe. Awareness of atypical manifestations, such as immune-mediated neutropenia, is essential for timely diagnosis and initiation of effective immunosuppressive therapy.