Protein A immunoadsorption therapy for refractory, mitomycin C-associated thrombotic microangiopathy.
Ključne riječi
Sažetak
BACKGROUND
Mitomycin C-associated thrombotic microangiopathy (TMA) has a poor prognosis with limited therapeutic options. Most patients die within 4 months of diagnosis due to pulmonary or renal failure. Here, a patient resistant to total plasma exchange (TPE) and immunosuppressive therapy with glucocorticoids, rituximab, vincristine, and splenectomy who was successfully treated with protein A immunoadsorption is described.
METHODS
A 29-year-old woman developed a TMA after chemotherapy with mitomycin C. She presented with thrombocytopenia, pulmonary edema, hemolytic anemia with presence of schistocytes, and renal failure. Immediate TPE (>120 times) and immunosuppressive therapy with glucocorticoids, however, did not improve her clinical situation. Furthermore, she was refractory to subsequent immunosuppressive therapy with rituximab and vincristine and laparoscopic splenectomy. Finally, after 12 cycles of extracorporeal protein A immunoadsorption with a commercially available immunoadsorption system (Immunosorba, Fresenius AG), platelet counts increased with disappearance of hemolytic syndromes.
CONCLUSIONS
Extracorporeal protein A immunoadsorption with the Immunosorba system emerges as a potentially effective and safe treatment for refractory mitomycin C-associated TMA with only moderate side effects. This therapeutic option may be considered at an early state of the disease to prevent extensive immunosuppression.
