Haemophagocytic syndrome, diffuse alveolar haemorrhage, catastrophic antiphospholipid syndrome and various types of thrombotic microangiopathies are rare conditions with significant morbidity and mortality. A common feature is late diagnosis, which can affect the success of treatment. The aim of this review article is to summarize the basic diagnostic and therapeutic steps of the present subpopulation of critically ill patients.
Keywords: HLH; Lens-Associated Uveitis; antiphospholipid syndrome; anti‑glomerular basement membrane (anti‑GBM) antibodies; caplacizumab; catastrophic antiphospholipid syndrome; cytokine storm syndrome; diffuse alveolar haemorrhage; eculizumab; eculizumab; etoposide; haemolytic‑uremic syndrome; hemophagocytic lymphohistiocytosis; hemophagocytic syndrome; immunosuppressive therapy; macrophage activation syndrome; plasma exchange; ravulizumab; recombinant factor VIIa; rituximab; thrombosis; thrombotic microangiopathies; thrombotic microangiopathy; thrombotic thrombocytopenic purpura; vasculitis.