Treatment
Initial treatment is with glucocorticoid corticosteroids or intravenous immunoglobulin, a procedure that is also used in ITP cases. In children, good response to a short steroid course is achieved in approximately 80 percent of cases. Although the majority of cases initially respond well to treatment, relapses are not uncommon and immunosuppressive drugs (e.g. ciclosporin, mycophenolate mofetil, vincristine and danazol) are subsequently used, or combinations of these.
The off-label use of rituximab (trade name Rituxan) has produced some good results in acute and refractory cases, although further relapse may occur within a year. Splenectomy is effective in some cases, but relapses are not uncommon.
The only prospect for a permanent cure is the high-risk option of an allogeneic hematopoietic stem cell transplantation (SCT).
Read more about this topic: Evans Syndrome
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“Any important disease whose causality is murky, and for which treatment is ineffectual, tends to be awash in significance.”
—Susan Sontag (b. 1933)
“[17th-century] Puritans were the first modern parents. Like many of us, they looked on their treatment of children as a test of their own self-control. Their goal was not to simply to ensure the child’s duty to the family, but to help him or her make personal, individual commitments. They were the first authors to state that children must obey God rather than parents, in case of a clear conflict.”
—C. John Sommerville (20th century)
“I feel that any form of so called psychotherapy is strongly contraindicated for addicts.... The question “Why did you start using narcotics in the first place?” should never be asked. It is quite as irrelevant to treatment as it would be to ask a malarial patient why he went to a malarial area.”
—William Burroughs (b. 1914)