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Observational Study
. 2024 Sep;43(9):2889-2898.
doi: 10.1007/s10067-024-07051-x. Epub 2024 Jul 29.

Hematological features and alternate diagnoses in critically ill thrombotic antiphospholipid syndrome patients

Levi-Dan Azoulay  1 , Thomas Frapard  1 , Romaric Larcher  2 , Frédéric Pène  3 , Laurent Argaud  4 , Julien Mayaux  5 , Matthieu Jamme  6 , Remi Coudroy  7 , Alexis Mathian  1 , Aude Gibelin  8 , Elie Azoulay  9 , Yacine Tandjaoui-Lambiotte  10   11   12 , Auguste Dargent  13 , François-Michel Beloncle  14 , Jean-Herlé Raphalen  15 , Nicolas Bréchot  16 , Nicolas de Prost  17 , Jérôme Devaquet  18 , Damien Contou  19 , Samuel Gaugain  20 , Pierre Trouiller  21 , Steven Grangé  22 , Stanislas Ledochowski  23 , Jérémie Lemarie  24 , Stanislas Faguer  25 , Vincent Degos  26 , Corinne Frere  27 , Paul Quentric  1   28 , Quentin Moyon  1   29   30 , Charles-Edouard Luyt  28   29 , Alain Combes  29   30 , Zahir Amoura #  1   28 , Marc Pineton de Chambrun #  31   32   33   34
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Free article
Observational Study

Hematological features and alternate diagnoses in critically ill thrombotic antiphospholipid syndrome patients

Levi-Dan Azoulay et al. Clin Rheumatol. 2024 Sep.
Free article

Abstract

Objectives: 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.

Conclusion: 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.

Keywords: ADAMTS13; Antiphospholipid syndrome; Catastrophic antiphospholipid syndrome; Heparin-induced thrombocytopenia; Intensive care unit.

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