Acute auto-aggression syndrome following autologous hematopoietic stem cell transplant and lenalidomide for multiple myeloma.

INTRODUCTION

Autologous hematopoietic stem cell transplant is an important treatment modality used to achieve long-term remission in people with multiple myeloma. Complications include chemotherapy-related toxicity or infection. Rarely, clinical features consistent with autologous graft-versus-host disease, otherwise known as auto-aggression syndrome, is possible. Auto-aggression syndrome appears more commonly in patients with multiple myeloma, hypothesized to be a result of underlying immune dysregulation, conditioning chemotherapy, or treatment with immunomodulating agents.

CASE REPORT

A 66-year-old female with multiple myeloma underwent an autologous stem cell transplant with melphalan conditioning chemotherapy followed by maintenance therapy with lenalidomide. Transplant was complicated by engraftment syndrome versus auto-aggression syndrome. After lenalidomide maintenance therapy initiation, she required hospitalization for auto-aggression syndrome.

MANAGEMENT AND OUTCOME

Auto-aggression syndrome with gastrointestinal, hepatic, and dermatologic involvement as demonstrated by skin punch biopsy, elevated reg3α, ST2, elafin, eosinophilia, transaminitis, and persistent diarrhea beyond the engraftment period were noted. Topical and systemic steroids with a prolonged taper resulted in symptom resolution.

DISCUSSION

Acute graft-versus-host disease is a complication once considered unique to allogeneic stem cell transplant recipients, but a similar syndrome termed "auto-aggression syndrome" may be seen following autologous transplant. Auto-aggression syndrome should be suspected when complications extend beyond the normal engraftment syndrome period following autologous transplant, particularly in people with multiple myeloma, and/or those who have received prior immunomodulating therapy. There should be a low threshold for obtaining biopsies in the setting of suspected auto-aggression syndrome. Early recognition and prompt initiation of corticosteroids with prolonged tapers may prevent auto-aggression syndrome relapse and readmissions.