Thrombotic storm: clinical profile and differential diagnosis of catastrophic antiphospholipid syndrome in pregnancy

The objective is to identify and systematize the characteristic clinical, laboratory, and instrumental features of catastrophic antiphospholipid syndrome (CAPS) in pregnant women, thereby improving its timely diagnosis and differentiation from other thrombotic microangiopathies (TMAs). Materials and Methods: A cohort study of 12 patients diagnosed with CAPS that developed during pregnancy was conducted. A detailed analysis of the clinical picture, obstetric and perinatal outcomes, biochemical and hemostatic markers, as well as dopplerometry data was performed. Results: It was established that the clinical profile of CAPS is characterized by the rapid development of multi-organ failure with predominant kidney involvement (median creatinine 74.90 μmol/L). The syndrome is associated with extremely adverse perinatal outcomes. Conclusion: CAPS in pregnancy has a unique clinical and laboratory pattern, the key elements of which are a triad: acute kidney injury, an exceedingly high D-dimer level, and a confirmed high titer of aPL. The identification of this triad is crucial for differential diagnosis and the immediate initiation of life-saving therapy.

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