Multimodal management, including precisely targeted irradiation, in a severe refractory case of Evans syndrome.
A challenging case of acute autoimmune thrombocytopenia (ITP) which evolved into a chronic refractory case of Evans syndrome over a period of more than 23 years is presented and may illustrate current therapeutic dilemmas now perplexing patients and clinicians. Newer modalities are being developed and their eventual role in the scheme of clinical management remains to be established. While this development unfolds, highly targeted radiotherapy was applied in this case to reduce platelet uptake by a refractory recurrent splenule with the goal of stabilizing the platelet count until promising investigational thrombopoietic agents or other newer, less toxic therapies might become available for wider application.
Miale
Thomas D
TD
Pediatric Hematology-Oncology Section, Fort Sanders Regional Medical Center, Knoxville, Tennessee 37950-0642, USA. TomMiale@aol.com
Wong
Jeffrey Y C
JY
Ahmed
Imran
I
Wagman
Lawrence D
LD
D000328:Adult; D000744:Anemia, Hemolytic, Autoimmune / Q000188:drug therapy / Q000532:radiotherapy* / Q000601:surgery; D019468:Disease Management; D005260:Female; D006801:Humans; D010976:Platelet Count; D016553:Purpura, Thrombocytopenic, Idiopathic / Q000188:drug therapy / Q000532:radiotherapy* / Q000601:surgery; D011878:Radiotherapy / Q000295:instrumentation / Q000379:methods*; D012008:Recurrence; D012720:Severity of Illness Index; D013156:Splenectomy; D013577:Syndrome; D013921:Thrombocytopenia / Q000188:drug therapy / Q000601:surgery; D016896:Treatment Outcome