SECONDARY HEMOPHAGOCYTIC LYMPHOHISTIOCYTIC SYNDROME IN SEVERE COVID-19 INFECTION: DIAGNOSTIC CHALLENGES AND A CASE DISCUSSION
DOI: 10.54647/cm32748 75 Downloads 4008 Views
Author(s)
Abstract
The hemophagocytic lymphohistiocytic syndrome (HLH) has been reported in critically ill patients with confirmed sars-cov-2 infection and contributes to their morbidity and mortality. Precise criteria for diagnosis and monitoring of HLH in these patients are essential for improving their clinical outcomes. The diagnosis of HLH depends on several clinical, laboratory, and morphologic parameters according to the HLH-2004 guidelines and its update for adult patients. Recently, the H-score tool provides a quantitative score that can be used to monitor clinical severity and detect early cases with incomplete criteria. Other differential diagnoses which should be considered in COVID-19 infection associated with 2ry HLH are sepsis and septic shock, and cytokine release syndrome (CRS) due to a wide overlap of clinicopathologic findings. The pathogenesis of COVID-19 associated 2ry HLH and whether a direct causal relationship exists between them are areas of intense investigation. A case of 65 years male with a documented COVID-19 disease and an associated 2ry HLH, 1 month following infection is reported, using both the HLH-2004 guidelines and a high H-score (>169). We recommend the use of a wide laboratory diagnostic panel for precise confirmation of HLH in all patients with early signs of clinical deterioration or underlying morbidity. These patients should be regularly monitored according to their rate of decline to allow for early intervention.
Keywords
COVID-19, HEMOPHAGOCYTOSIS, HLH, BONE MARROW MACROPHAGE, H-SCORE, PATHOGENESIS
Cite this paper
SAMIA HASSAN RIZK,
SECONDARY HEMOPHAGOCYTIC LYMPHOHISTIOCYTIC SYNDROME IN SEVERE COVID-19 INFECTION: DIAGNOSTIC CHALLENGES AND A CASE DISCUSSION
, SCIREA Journal of Clinical Medicine.
Volume 7, Issue 1, February 2022 | PP. 16-29.
10.54647/cm32748
References
[ 1 ] | EPI-WIN: WHO Information Network for Epidemics (2021). THE LATEST ON THE COVID-19 GLOBAL SITUATION & LONG-TERM SEQUELAE L. LAST UPDATE ON LONG-TERM EFFECTS OF VOVID-19. 26 March 2021. Risk communication (who. int) |
[ 2 ] | Prilutskiy, A., Kritselis, M., Shevtsov, A., Yambayev M., Vadlamudi, C., et.al. (2020). SARS-CoV-2 Infection–Associated Hemophagocytic Lymphohistiocytosis an Autopsy Series with Clinical and Laboratory Correlation. Am J Clin Pathol. ; XX:0–0 DOI: 10.1093/AJCP/AQAA124 |
[ 3 ] | Histiocytosis Society (2004). HLH-2004 (UK No: LCH 2006 02) Hemophagocytic Lymphohistiocytosis Study Group Treatment Protocol of the Second International HLH Study 2004. Histiocyte Society (bspho.be) |
[ 4 ] | Fardet L, Galicier L, Lambotte O, et al. (2014). Development and validation of the H-score, a score for the diagnosis of reactive hemophagocytic syndrome. Arthritis Rheumatol. 66:2613- 2620. doi:10.1002/art.38690. |
[ 5 ] | Ramakrishnan RK, Kashour T, Hamid Q, Halwani R, and Tleyjeh IM (2021) Unraveling the Mystery Surrounding Post-Acute Sequelae of COVID-19. Front. Immunol. 12:686029. DOI: 10.3389/fimmu.2021.686029. Front. Immunol., 30 June | https://doi.org/10.3389/fimmu.2021.686029 |
[ 6 ] | Prieto-Pérez L., Fortes J., Soto C., Vidal-González A., et.al. (2020). Histiocytic hyperplasia with hemophagocytosis and acute alveolar damage in COVID-19 infection. Modern Pathology. 33:2139–2146 https://doi.org/10.1038/s41379-020-0613-1 |
[ 7 ] | YANG K., XING M., JIANG L., et.al. (2021). Infection-associated Hemophagocytic Syndrome in Critically Ill Patients with COVID-19. Current Medical Science DOI https://doi.org/10.1007/s11596-021-2315-4 41(1):39-45 |
[ 8 ] | Korompoki, M. Gavriatopoulou, R.S. Hicklen et al. (2021). Epidemiology and organ-specific sequelae of post-acute COVID19: A narrative review. Journal of Infection 83 (2021) 1–1. https://doi.org/10.1016/j.jinf.2021.05.004 0163-4453/© 2021 The British Infection Ass |
[ 9 ] | Ladds E, Rushforth A, Wieringa S, Taylor S, Rayner C, Husain L, et al. Persistent symptoms after COVID-19: a qualitative study of 114 “long COVID” patients and draft quality principles for services. BMC Health Serv Res 2020;20(1). doi:10. 1186/s12913-020-06001-y |
[ 10 ] | Debaugnies F, Mahadeb B, Ferster A, et.al. (2016). Performances of the H-score for diagnosis of hemophagocytic lymphohistiocytosis in adult and pediatric patients. Am J Clin Pathol. 145:862-870. doi:10.1093/ajcp/aqw076. |
[ 11 ] | Opoka-Winiarska, V., Grywalska E., and Roliński J., (2020). Could hemophagocytic lymphohistiocytosis be the core issue of severe COVID-19 cases? BMC Medicine. 18:214 https://doi.org/10.1186/s12916-020-01682-y |