Selected severe „haematological“ syndromes in adult intensive care patients
Authors:
Jaromír Gumulec 1,4; Ivo Demel 1; Klára Lančová 1; Eva Drbohlavová 2; Alicia Piegzová 3; Zdeněk Kořístek 1,4; Milan Navrátil 1,4; Vladimír Černý 5
Authors‘ workplace:
Klinika hematoonkologie Fakultní nemocnice Ostrava
1; Klinická hematologie Krajské nemocnice Liberec, a. s.
2; Gynekologicko‑porodnická, klinika Fakultní nemocnice Ostrava
3; Lékařská fakulta Ostravské univerzity
4; 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.
5
Published in:
Vnitř Lék 2022; 68(8): 498-507
Category:
Main Topic
doi:
https://doi.org/10.36290/vnl.2022.107
Overview
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:
vasculitis – thrombotic thrombocytopenic purpura – thrombotic microangiopathy – plasma exchange – thrombosis – immunosuppressive therapy – diffuse alveolar haemorrhage – rituximab – eculizumab – hemophagocytic lymphohistiocytosis – antiphospholipid syndrome – hemophagocytic syndrome – HLH – macrophage activation syndrome – cytokine storm syndrome – catastrophic antiphospholipid syndrome – haemolytic‑uremic syndrome – etoposide – recombinant factor VIIa – caplacizumab – ravulizumab
Sources
1. Yildiz H, Van Den Neste E, Defour JP, Danse E, Yombi JC. Adult haemophagocytic lymphohistiocytosis: a Review. QJM: monthly journal of the Association of Physicians. 2020 Jan 14. eng. Epub 2020/01/17. doi:10.1093/qjmed/hcaa011. Cited in: Pubmed; PMID 31943120.
2. La Rosée P, Horne A, Hines M, von Bahr Greenwood T, Machowicz R, Berliner N, Birndt S, Gil‑Herrera J, Girschikofsky M, Jordan MB, Kumar A, van Laar JAM, Lachmann G, Nichols KE, Ramanan AV, Wang Y, Wang Z, Janka G, Henter JI. Recommendations for the management of hemophagocytic lymphohistiocytosis in adults. Blood. 2019 Jun 6;133(23):2465-2477. eng. Epub 2019/04/18. doi:10.1182/blood.2018894618. Cited in: Pubmed; PMID 30992265.
3. Debaugnies F, Mahadeb B, Ferster A, Meuleman N, Rozen L, Demulder A, Corazza F. Performances of the H‑Score for Diagnosis of Hemophagocytic Lymphohistiocytosis in Adult and Pediatric Patients. Am J Clin Pathol. 2016 Jun;145(6):862-70. eng. Epub 2016/06/15. doi:10.1093/ajcp/aqw076. Cited in: Pubmed; PMID 27298397.
4. Collard HR, Schwarz MI. Diffuse alveolar hemorrhage. Clinics in chest medicine. 2004 Sep;25(3):583-92, vii. eng. Epub 2004/08/28. doi:10.1016/j.ccm.2004. 04. 007. Cited in: Pubmed; PMID 15331194.
5. Levy JB, Turner AN, Rees AJ, Pusey CD. Long‑term outcome of anti‑glomerular basement membrane antibody disease treated with plasma exchange and immunosuppression. Annals of internal medicine. 2001 Jun 5;134(11):1033-42. eng. doi:10.7326/0003-4819- 134-11-200106050-00009. Cited in: Pubmed; PMID 11388816.
6. Alexandre AT, Vale A, Gomes T. Diffuse alveolar hemorrhage: how relevant is etiology? Sarcoidosis Vasc Diffuse Lung, DiS. 2019;36(1):47-52. eng. Epub 2019/01/01. doi:10.36141/ svdld.v36i1.7160. Cited in: Pubmed; PMID 32476936.
7. Lara AR, Schwarz MI. Diffuse alveolar hemorrhage. Chest. 2010 May;137(5):1164-71. eng. Epub 2010/05/06. doi:10.1378/chest.08-2084. Cited in: Pubmed; PMID 20442117.
8. Maldonado F, Parambil JG, Yi ES, Decker PA, Ryu JH. Haemosiderin‑ laden macrophages in the bronchoalveolar lavage fluid of patients with diffuse alveolar damage. Eur Respir J. 2009 Jun;33(6):1361-6. eng. Epub 2009/01/09. doi:10.1183/09031936.00119108. Cited in: Pubmed; PMID 19129275.
9. de Prost N, Parrot A, Cuquemelle E, Picard C, Antoine M, Fleury‑Feith J, Mayaud C, Boffa JJ, Fartoukh M, Cadranel J. Diffuse alveolar hemorrhage in immunocompetent patients: etiologies and prognosis revisited. Respir Med. 2012 Jul;106(7):1021-32. eng. Epub 2012/05/01. doi:10.1016/j.rmed.2012. 03. 015. Cited in: Pubmed; PMID 22541718.
10. Newsome BR, Morales JE. Diffuse alveolar hemorrhage. South Med J. 2011 Apr;104(4):269- 74. eng. Epub 2011/05/25. doi:10.1097/SMJ.0b013e3182126d3b. Cited in: Pubmed; PMID 21606695.
11. Park JA. Treatment of Diffuse Alveolar Hemorrhage: Controlling Inflammation and Obtaining Rapid and Effective Hemostasis. International journal of molecular sciences. 2021 Jan 14;22(2). eng. Epub 2021/01/21. doi:10.3390/ijms22020793. Cited in: Pubmed; PMID 33466873.
12. Szczepiorkowski ZM, Winters JL, Bandarenko N, Kim HC, Linenberger ML, Marques MB, Sarode R, Schwartz J, Weinstein R, Shaz BH. Guidelines on the use of therapeutic apheresis in clinical practice--evidence‑based approach from the Apheresis Applications Committee of the American Society for Apheresis. J Clin Apher. 2010;25(3):83-177. eng. Epub 2010/06/23. doi:10.1002/jca.20240. Cited in: Pubmed; PMID 20568098.
13. Stone JH, Merkel PA, Spiera R, Seo P, Langford CA, Hoffman GS, Kallenberg CG, St Clair EW, Turkiewicz A, Tchao NK, Webber L, Ding L, Sejismundo LP, Mieras K, Weitzenkamp D, Ikle D, Seyfert‑Margolis V, Mueller M, Brunetta P, Allen NB, Fervenza FC, Geetha D, Keogh KA, Kissin EY, Monach PA, Peikert T, Stegeman C, Ytterberg SR, Specks U. Rituximab versus cyclophosphamide for ANCA‑associated vasculitis. N Engl J Med. 2010 Jul 15;363(3):221- 32. eng. Epub 2010/07/22. doi:10.1056/NEJMoa0909905. Cited in: Pubmed; PMID 20647199.
14. Park JA, Kim BJ. Intrapulmonary recombinant factor VIIa for diffuse alveolar hemorrhage in children. Pediatrics. 2015 Jan;135(1):e216-20. eng. Epub 2014/12/31. doi:10.1542/ peds.2014-1782. Cited in: Pubmed; PMID 25548333.
15. Garcia D, Erkan D. Diagnosis and Management of the Antiphospholipid Syndrome. N Engl J Med. 2018 May 24;378(21):2010-2021. eng. doi:10.1056/NEJMra1705454. Cited in: Pubmed; PMID 29791828.
16. Asherson RA. The catastrophic antiphospholipid syndrome. J Rheumatol. 1992 Apr;19(4):508-12. eng. Epub 1992/04/01. Cited in: Pubmed; PMID 1593568.
17. Mesa CJ, Rife EC, Espinoza LR. Catastrophic antiphospholipid syndrome: is life‑ long anticoagulation therapy required? Clin Rheumatol. 2020 Jul;39(7):2115-2119. eng. Epub 2020/02/29. doi:10.1007/s10067-020-04997-6. Cited in: Pubmed; PMID 32107665.
18. Chang JC. TTP‑ like syndrome: novel concept and molecular pathogenesis of endotheliopathy‑associated vascular microthrombotic disease. Thromb J. 2018;16:20. doi:10.1186/s12959-018-0174-4. Cited in: Pubmed; PMID 30127669.
19. Tsai HM, Lian EC. Antibodies to von Willebrand factor‑cleaving protease in acute thrombotic thrombocytopenic purpura. N Engl J Med. 1998 Nov 26;339(22):1585-94. doi:10.1056/ NEJM199811263392203. Cited in: Pubmed; PMID 9828246.
20. Sarode R, Bandarenko N, Brecher ME, Kiss JE, Marques MB, Szczepiorkowski ZM, Winters JL. Thrombotic thrombocytopenic purpura: 2012 American Society for Apheresis (ASFA) consensus conference on classification, diagnosis, management, and future research. J Clin Apher. 2014 Jun;29(3):148-67. doi:10.1002/jca.21302. Cited in: Pubmed; PMID 24136342.
21. Loirat C, Garnier A, Sellier‑Leclerc AL, Kwon T. Plasmatherapy in atypical hemolytic uremic syndrome. Seminars in thrombosis and hemostasis. 2010 Sep;36(6):673-81. doi:10.1055/s-0030-1262890. Cited in: Pubmed; PMID 20865645.
Další literatura u autorů a na www.casopisvnitrnilekarstvi.cz
Labels
Diabetology Endocrinology Internal medicineArticle was published in
Internal Medicine
![Issue 8](https://www.prolekare.cz/media/cache/resolve/journal_issue/media/image_pdf/02c416bcf5ba7de43c2257f7d2133dbe.jpg)
2022 Issue 8
Most read in this issue
- Selected severe „haematological“ syndromes in adult intensive care patients
- Today's view of hereditary thrombophilia
- Antithrombotic therapy and digestive endoscopy
- Allergen immunotherapy in treating allergic eosinophilic asthma