Yingqi Wang, Yeow-Tee Goh, Aloysius Ho, William Huang, Jeffrey Quek, Hein Than, Tertius Tuy, Yeh-Ching Linn
Response of AIC after allo-HSCT to first-line treatment is heterogenous and long-term course is variable, but even the most refractory cases can respond to a combination of treatments.
INTRODUCTION: We analysed in detail the clinical course of 16 patients with autoimmune cytopenia (AIC) after allogeneic haematopoietic stem cell transplant (allo-HSCT).
METHODS: Patients with AIC reported retrospectively by physicians were studied for their clinical characteristics, treatment, response and long-term outcome.
RESULTS: Sixteen patients including 10 with immune thrombocytopenia purpura (ITP), 3 with autoimmune haemolytic anemia (AIHA) and 3 with Evans syndrome (ES) were analysed. Recipients of unrelated (n = 6) and haploidentical (n = 8) donors, especially of partial T cell depleted grafts (n = 7) predominated. Onset occurred from months to years after transplant and cytopenia was severe. First-line treatment including glucocorticoids, iv immunoglobulin as monotherapy or in combination for all patients, as well as Eltrombopag for ITP. Eight of the 10 ITP patients, 1 of 3 AIHA and 1 of 3 ES patients responded to first line therapy, while others required further lines of treatment. Recurrence of the AIC occurred in 5 patients, where response was achieved readily or could be challenging. Overall, all 16 patients responded with no mortality due to AIC.
CONCLUSION: Response of AIC after allo-HSCT to first-line treatment is heterogenous and long-term course is variable, but even the most refractory cases can respond to a combination of treatments.