Syndrome Des Anticorps Anti-Phospholipides Et Autres Maladies Auto-Immunes Systémiques Rares, Sorbonne Université, Assistance Publique-Hôpitaux de Paris (AP-HP), Hôpital La Pitié-Salpêtrière, Institut E3M, Service de Médecine Interne 2, Centre de Référence National Lupus Systémique, Paris, France.
Service de Médecine Intensive-Réanimation, Hôpital Lapeyronie, Centre Hospitalier Universitaire (CHU) de Montpellier, PhyMedExp, Université de Montpellier, INSERM, CNRS, Montpellier, France.
Clin Rheumatol. 2024 Sep;43(9):2889-2898. doi: 10.1007/s10067-024-07051-x. Epub 2024 Jul 29.
Severe thrombotic antiphospholipid syndrome (APS) frequently affects the kidney, heart, and central nervous system. The precise frequency, clinical picture, differential diagnoses, and outcome of APS-related hematological involvement are lacking, especially in patients requiring ICU admission. This study aimed to describe the hematological manifestations associated with critically ill thrombotic APS patients and catastrophic antiphospholipid syndrome.
This French, national, multicenter, retrospective study, conducted, from January 2000 to September 2018, included all APS patients admitted to 24 participating centers' ICUs with any new thrombotic manifestation. The prevalence of hematological manifestations and their associated outcomes were studied.
One hundred and thirty-four patients, female 72%, median [IQR] age 45 [34-56] years, with 152 episodes were included. Anemia was present in 95% of episodes and thrombocytopenia in 93%. The lowest values for hemoglobin and platelets were 7.1 [6.3-8.8] g/dL and 38 [21-60] g/L, respectively. The lowest platelet count below 20 g/L was significantly associated with a higher in-ICU mortality rate (50%, p < 0.0001). A thrombotic microangiopathy syndrome (TMA) syndrome was seen in 16 patients (12%) and was associated with higher in-hospital mortality (p = 0.05). Median ADAMTS-13 levels were 44% [27-74]. Anti-ADAMTS13 antibodies were tested in 11 patients and found negative in all. A suspicion of heparin-induced thrombocytopenia (HIT) was raised in 66 patients but only four patients were classified as definite HIT. Disseminated intravascular coagulation (DIC) was seen in 51% of patients.
Thrombocytopenia is very frequent in severe APS patients and may be related to TMA, HIT, or DIC. Deciphering the mechanisms of thrombocytopenia is decisive in CAPS patients. Key Points • Thrombocytopenia is the hallmark laboratory finding in CAPS. • A complete thrombotic microangiopathy pattern is infrequent in CAPS patients. • Alternate diagnoses of CAPS, especially heparin-induced thrombocytopenia, need to be adequately investigated.
严重的血栓性抗磷脂综合征（APS）常累及肾脏、心脏和中枢神经系统。APS 相关血液学表现的准确频率、临床特征、鉴别诊断和预后尚不清楚，尤其是在需要入住 ICU 的患者中。本研究旨在描述与重症血栓性 APS 患者和灾难性抗磷脂综合征相关的血液学表现。
这是一项法国全国多中心回顾性研究，于 2000 年 1 月至 2018 年 9 月进行，纳入了在 24 个参与中心 ICU 住院且有任何新发血栓形成表现的所有 APS 患者。研究了血液学表现及其相关结局的发生率。
共纳入 134 例女性患者（72%），中位（IQR）年龄 45[34-56]岁，共 152 例次。95%的发作存在贫血，93%存在血小板减少症。血红蛋白和血小板的最低值分别为 7.1[6.3-8.8]g/dL 和 38[21-60]g/L。血小板计数低于 20 g/L 的最低值与 ICU 死亡率升高显著相关（50%，p<0.0001）。16 例（12%）出现血栓性微血管病综合征（TMA），住院死亡率较高（p=0.05）。ADAMTS-13 中位水平为 44%[27-74]。11 例患者检测了抗 ADAMTS13 抗体，结果均为阴性。怀疑肝素诱导的血小板减少症（HIT）的患者有 66 例，但仅 4 例患者被归类为明确的 HIT。51%的患者存在弥散性血管内凝血（DIC）。
严重 APS 患者中血小板减少症非常常见，可能与 TMA、HIT 或 DIC 有关。解析 CAPS 患者血小板减少症的机制至关重要。关键点：•血小板减少症是 CAPS 的标志性实验室发现。•CAPS 患者中完全性血栓性微血管病模式不常见。•需要充分调查 CAPS 的其他诊断，特别是肝素诱导的血小板减少症。
