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绝对未成熟血小板计数提示复杂型复发性血栓性血小板减少性紫癜时血小板生成受抑制。

Absolute Immature Platelet Counts Suggest Platelet Production Suppression during Complicated Relapsing Thrombotic Thrombocytopenic Purpura.

机构信息

Department of Pathology, University Hospitals Cleveland Medical Center and Case Western Reserve University, Cleveland, Ohio, USA.

Department of Pathology, University Hospitals Cleveland Medical Center and Case Western Reserve University, Cleveland, Ohio, USA,

出版信息

Acta Haematol. 2021;144(4):465-469. doi: 10.1159/000510913. Epub 2020 Nov 25.

Abstract

Absolute immature platelet counts (A-IPC) aid in diagnosis and treatment follow-up in thrombotic thrombocytopenic purpura (TTP). A-IPC was used to follow a patient on mycophenolate mofetil (MMF) maintenance therapy treated with a prolonged therapeutic plasma exchange (TPE) regimen for relapsing TTP. On admission, the platelet (PLT) count was 95 × 109/L declining to 14 × 109/L in 5 days. Daily TPE was initiated for suspected TTP, and MMF was discontinued. A-IPC and PLT count were 1 × 109/L and 14 × 109/L, respectively, prior to first TPE. A-IPC improved to 3.2 × 109/L with 1 TPE, and on day 5, A-IPC and PLT count were 7.5 × 109/L and 218 × 109/L, respectively. On day 6, A-IPC and PLT count decreased to 4.8 × 109/L and 132 × 109/L further worsening to 0.4 × 109/L and 13 × 109/L, respectively. ADAMTS13 activity remained <5% with an inhibitor; counts did not recover. Initial improvement followed by rapidly declining A-IPC despite therapy suggested production suppression. In TTP, A-IPC may aid in establishing early therapy effects over PLT production.

摘要

绝对未成熟血小板计数 (A-IPC) 有助于诊断和治疗随访血栓性血小板减少性紫癜 (TTP)。A-IPC 用于监测接受麦考酚酸酯(MMF)维持治疗的患者,该患者接受了延长的治疗性血浆置换 (TPE) 方案治疗复发性 TTP。入院时,血小板 (PLT) 计数为 95×10^9/L,5 天内降至 14×10^9/L。怀疑 TTP 发生,开始每日 TPE,并停用 MMF。首次 TPE 前,A-IPC 和 PLT 计数分别为 1×10^9/L 和 14×10^9/L。A-IPC 在进行 1 次 TPE 后改善至 3.2×10^9/L,第 5 天,A-IPC 和 PLT 计数分别为 7.5×10^9/L 和 218×10^9/L。第 6 天,A-IPC 和 PLT 计数分别降至 4.8×10^9/L 和 132×10^9/L,进一步恶化至 0.4×10^9/L 和 13×10^9/L。ADAMTS13 活性仍<5%且存在抑制剂;计数未恢复。尽管进行了治疗,但 A-IPC 先改善后迅速下降,提示产生抑制。在 TTP 中,A-IPC 可能有助于在 PLT 生成之前建立早期治疗效果。

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