Abstract：
OBJECTIVE: 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.
METHODS: 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.
RESULTS: 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.
CONCLUSIONS: 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.
METHODS: 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.
RESULTS: 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.
CONCLUSIONS: 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.
摘要：
目的：严重血栓性抗磷脂综合征（APS）经常影响肾脏，心,和中枢神经系统。精确的频率，临床图片，鉴别诊断，缺乏APS相关血液学受累的结果，尤其是需要入住ICU的患者。这项研究旨在描述与危重血栓性APS患者和灾难性抗磷脂综合征相关的血液学表现。
方法：这个法语，国家,多中心，回顾性研究,进行了,从2000年1月至2018年9月,纳入了24个参与中心的ICU收治的所有APS患者,这些患者有任何新的血栓形成表现.研究了血液学表现的患病率及其相关结果。
结果：一百三十四个病人，女性72%,中位数[IQR]45[34-56]岁，包括152集。95%的患者出现贫血，93%的患者出现血小板减少。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,分别。低于20g/L的最低血小板计数与更高的ICU死亡率显着相关（50％，p<0.0001)。血栓性微血管病综合征（TMA）综合征见于16例患者（12%），与较高的住院死亡率相关（p=0.05）。ADAMTS-13的中位数水平为44%[27-74]。对11例患者进行了抗ADAMTS13抗体检测，结果均为阴性。66例患者怀疑肝素诱导的血小板减少症（HIT），但只有4例患者被归类为明确的HIT。51％的患者出现弥散性血管内凝血（DIC）。
结论：血小板减少在重度APS患者中非常常见，可能与TMA有关，命中,或DIC。在CAPS患者中，破译血小板减少症的机制是决定性的。要点•血小板减少症是CAPS的标志性实验室发现。•CAPS患者很少出现完全血栓性微血管病。•CAPS的替代诊断，尤其是肝素诱导的血小板减少症,需要充分调查。
方法：这个法语，国家,多中心，回顾性研究,进行了,从2000年1月至2018年9月,纳入了24个参与中心的ICU收治的所有APS患者,这些患者有任何新的血栓形成表现.研究了血液学表现的患病率及其相关结果。
结果：一百三十四个病人，女性72%,中位数[IQR]45[34-56]岁，包括152集。95%的患者出现贫血，93%的患者出现血小板减少。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,分别。低于20g/L的最低血小板计数与更高的ICU死亡率显着相关（50％，p<0.0001)。血栓性微血管病综合征（TMA）综合征见于16例患者（12%），与较高的住院死亡率相关（p=0.05）。ADAMTS-13的中位数水平为44%[27-74]。对11例患者进行了抗ADAMTS13抗体检测，结果均为阴性。66例患者怀疑肝素诱导的血小板减少症（HIT），但只有4例患者被归类为明确的HIT。51％的患者出现弥散性血管内凝血（DIC）。
结论：血小板减少在重度APS患者中非常常见，可能与TMA有关，命中,或DIC。在CAPS患者中，破译血小板减少症的机制是决定性的。要点•血小板减少症是CAPS的标志性实验室发现。•CAPS患者很少出现完全血栓性微血管病。•CAPS的替代诊断，尤其是肝素诱导的血小板减少症,需要充分调查。
