Role of plasma exchange, leukocytapheresis, and plasma diafiltration in management of refractory macrophage activation syndrome

We report the case of steroid and CsA resistant s‐JIA associated MAS misdiagnosed as Kawasaki disease (KD), who was successfully treated with the combination of plasma exchange (PE) and leukocytapheresis (LCAP) followed by plasma diafiltration (PDF). PE + LCAP effectively removed proinflammatory cytokines and reduced the number of peripheral white blood cells. Furthermore, PDF also removed proinflammatory cytokines as effectively as PE + LCAP. Early diagnosis of s‐JIA is necessary to avoid developing MAS. The measurement of serum ferritin and IL‐18 levels are useful for differentiating s‐JIA from KD. Apheresis therapies are an alternative option to induce remission for severe patients with steroid‐ or CsA‐resistant MAS.
Source: Journal of Clinical Apheresis - Category: Hematology Authors: Tags: CASE REPORT Source Type: research