Euler H H, Löffler H, Christophers E
Arch Dermatol. 1987 Sep;123(9):1205-10.
Long-lasting and complete remission was obtained in a 48-year-old patient with refractory pemphigus vulgaris by an experimental treatment protocol that tries to synchronize plasmapheresis with subsequent pulse cyclophosphamide therapy. The rationale of the approach tries to utilize the plasmapheresis-induced increased proliferation of pathogenic cell clones for partial deletion of these clones through application of maximum pulse immunosuppression treatment during the period of assumed maximum proliferation and, thus, maximum vulnerability of the antibody-producing cells. The treatment schedule consisted of initial withdrawal of immunosuppressive drug therapy, repeated large-volume plasmaphereses substituted with immunoglobulin-free albumin solutions, subsequent application of high-dose (36 mg/kg of body weight) cyclophosphamide therapy, and low-dose maintenance immunosuppression for several months. As a result, our patient remained disease free over a follow-up period of 40 months without any further immunosuppressive treatment. Stimulation of postexchange antibody production and subsequent application of high-dose cytotoxic drugs might be a valuable tool in the management of refractory pemphigus vulgaris and, possibly, in the management of other autoantibody-mediated diseases.
通过一种实验性治疗方案，一名48岁的寻常型天疱疮难治性患者获得了持久且完全的缓解。该方案试图使血浆置换与随后的脉冲环磷酰胺疗法同步进行。这种方法的基本原理是利用血浆置换诱导致病性细胞克隆增殖增加，在假定的最大增殖期，即抗体产生细胞最大程度易损期，通过应用最大剂量脉冲免疫抑制治疗来部分清除这些克隆。治疗方案包括最初停用免疫抑制药物治疗，用无免疫球蛋白的白蛋白溶液反复进行大容量血浆置换，随后应用高剂量（36mg/kg体重）环磷酰胺治疗，以及低剂量维持免疫抑制数月。结果，我们的患者在40个月的随访期内未接受任何进一步的免疫抑制治疗，疾病未再复发。刺激血浆置换后抗体产生并随后应用高剂量细胞毒性药物可能是治疗寻常型天疱疮难治性病例以及可能治疗其他自身抗体介导疾病的一种有价值的手段。
