severe thrombocytopenia is present. Patients
who cannot be weaned off prednisone or
relapse after prednisone should be considered for splenectomy, although these patients
are at higher risk of relapsing.80 Increasingly
rituximab is being used with success.84,85 For
patients who fail splenectomy and rituximab, aggressive immunosuppression should
be considered. Increasing data support the
benefits of sirolimus, and this should be
considered for refractory patients.86 For
patients with Evans syndrome due to underlying lymphoma, antineoplastic therapy often
results in prompt resolution of the symptoms.
Recurrence of the autoimmune cytopenias
often heralds relapse.
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