Center for Hematology, Massachusetts General Hospital Cancer Center, 55 Fruit St., Boston, MA, 02114, USA.
Dana-Farber/Boston Children's Cancer and Blood Disorders Center, Boston, USA.
J Thromb Thrombolysis. 2018 Jul;46(1):81-83. doi: 10.1007/s11239-018-1647-9.
Acquired thrombotic thrombocytopenic purpura (TTP) is an autoimmune disorder resulting in potentially life-threating systemic thrombotic microangiopathy due to production of antibodies directed against the von Willebrand factor-cleaving protease ADAMTS13. Typically managed with plasma exchange, glucocorticoids, and the first-generation anti-CD20 monoclonal antibody rituximab, patients with multiple relapses or refractory disease present unique management challenges. We describe a case of a young woman with multiple relapses of TTP despite standard therapy who was treated with ofatumumab, a second-generation anti-CD20 monoclonal antibody, after developing a severe hypersensitivity reaction to rituximab precluding its use. Ofatumumab was effective for the treatment of an acute relapse of TTP in combination with plasmapheresis and as a single-agent for prophylaxis. The patient has had no evidence of relapse 2 years after completion of acute treatment and 1 year after completing prophylactic therapy. Hypersensitivity to ofatumumab did not develop.
获得性血栓性血小板减少性紫癜（TTP）是一种自身免疫性疾病，由于产生针对 von Willebrand 因子裂解蛋白酶 ADAMTS13 的抗体，导致潜在危及生命的全身性血栓性微血管病。该病通常采用血浆置换、糖皮质激素和第一代抗 CD20 单克隆抗体利妥昔单抗治疗，但对于多次复发或难治性疾病的患者，存在独特的管理挑战。我们描述了一例年轻女性，尽管采用标准治疗，仍多次复发 TTP，在对利妥昔单抗产生严重过敏反应而不能使用后，使用第二代抗 CD20 单克隆抗体奥法妥珠单抗进行治疗。奥法妥珠单抗联合血浆置换治疗 TTP 急性复发有效，且作为单一药物进行预防也有效。在完成急性治疗后 2 年和完成预防治疗后 1 年，该患者均无复发迹象。未发生对奥法妥珠单抗的过敏反应。
