Vnitr Lek 2022, 68(8):498-507 | DOI: 10.36290/vnl.2022.107

Selected severe "haematological" syndromes in adult intensive care patients

Jaromír Gumulec^{1, 4}, Ivo Demel¹, Klára Lančová¹, Eva Drbohlavová², Alicia Piegzová³, Zdeněk Kořístek^{1, 4}, Milan Navrátil^{1, 4}, Vladimír Černý⁵

¹ Klinika hematoonkologie Fakultní nemocnice Ostrava

² Klinická hematologie Krajské nemocnice Liberec, a. s.

³ Gynekologicko‑porodnická klinika Fakultní nemocnice Ostrava

⁴ Lékařská fakulta Ostravské univerzity

⁵ Klinika anesteziologie, perioperační a intenzivní medicíny Fakulty zdravotnických studií Univerzity J. E. Purkyně v Ústí nad Labem a Krajské zdravotní, a. s. - Masarykovy nemocnice v Ústí nad Labem, o. z.

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: hemophagocytic syndrome, hemophagocytic lymphohistiocytosis, HLH, macrophage activation syndrome, cytokine storm syndrome, diffuse alveolar haemorrhage, vasculitis, thrombosis, antiphospholipid syndrome, catastrophic antiphospholipid syndrome, thrombotic microangiopathy, thrombotic thrombocytopenic purpura, haemolytic‑uremic syndrome, immunosuppressive therapy, etoposide, rituximab, plasma exchange, recombinant factor VIIa, caplacizumab, eculizumab, ravulizumab.

Accepted: December 1, 2022; Published: December 6, 2022  Show citation