Secondary Hemophagocytic Lymphohistiocytosis in a Post-COVID-19 Patient

Bandaru, Sai Samyuktha; Capace, Ashley; Busa, Vishal; Williams, Aaron M.

doi:10.7759/cureus.22620

Cited by 10 publications

(9 citation statements)

References 16 publications

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“…In contrast to the earlier mentioned reports [85,88,91,99], a high incidence of sHLH in severely ill COVID-19 patients was shown in other studies (Table 2) [42,[86][87][88]90,95,96]. Furthermore, various reports of sHLH in recovered COVID-19 patients have been made [10,[99][100][101][102]. A more severe disease progression and higher mortality were seen in patients with higher H-scores and suspected HLH [86,87,91] Immune dysregulation and an unrecognized persistent inflammatory state appear to play a role in the pathogenesis of COVID-19-associated HLH [100].…”

Section: Covid-19-associated Hlhmentioning

confidence: 67%

Hemophagocytic Syndrome and COVID-19: A Comprehensive Review

Fadlallah¹,

Salman²,

Fadlallah³

et al. 2023

Cureus

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Hemophagocytic lymphohistiocytosis (HLH), a hyperinflammatory hyperferritinemic syndrome, is triggered by various etiologies and diseases and can lead to multiorgan dysfunction and death. There are two types of HLH: primary and secondary. Primary HLH (pHLH) is caused by a genetic mutation resulting in dysfunction in cytotoxic T lymphocytes (CTLs), natural killer (NK) cells, hyperactivated immune cells, and hypercytokinemia. In secondary HLH (sHLH), an underlying etiology is the cause of the disease. Infections, malignancy, and autoimmune diseases are well-known triggers for sHLH. Infectious triggers for sHLH are most frequently viruses, where different mechanisms, including dysregulated CTLs and NK cell activity and persistent immune system stimulation, have been reported. Similarly, in severe coronavirus disease 2019 (COVID-19) patients, a hyperinflammatory mechanism leading to hypercytokinemia and hyperferritinemia has been demonstrated. A similar dysfunction in CTLs and NK cells, persistent immune system stimulation with increased cytokines production, and severe end-organ damage have been reported. Therefore, a significant overlap is present between the clinical and laboratory features seen in COVID-19 and sHLH. However, SARS-CoV-2, similar to other viruses, can trigger sHLH. Hence, a diagnostic approach is needed in severe COVID-19 patients presenting with multiorgan failure, in whom sHLH should be considered.

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Section: Covid-19-associated Hlhmentioning

confidence: 67%

Hemophagocytic Syndrome and COVID-19: A Comprehensive Review

Fadlallah¹,

Salman²,

Fadlallah³

et al. 2023

Cureus

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“…In recent years, since the start of the COVID-19 pandemic, cases of SARS-CoV-2-induced secondary HLH during and post-infection have been sparsely reported [10][11][12][13][14][15]. In these case reports, the cause of HLH has been postulated to be secondary to subclinical inflammation and dysregulation of the immune system in patients of a wide age range with PCR-proven recovery [15][16][17]. At the beginning phase of the severe infectious stage of COVID-19, it is difficult to attribute inflammatory reaction solely to HLH vs. COVID-19 infection without other evidence, especially from a bone marrow biopsy.…”

Section: Discussionmentioning

confidence: 99%

Coronavirus Does It Again: Post-COVID-19 Hemophagocytic Lymphohistiocytosis (HLH)

Jeyakanthan¹,

Ladel²,

Khandpur³

et al. 2023

Cureus

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Hemophagocytic lymphohistiocytosis (HLH) is a hematological disorder that results from an uncontrolled activation of the immune system, which can then lead to multisystem organ failure. Given the nonspecific nature of this illness, it can go undetected for too long, thereby causing permanent damage to organ systems. In adults, HLH has been associated with a number of infectious etiologies, particularly viral infections. Coronavirus disease 2019 (COVID-19), caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), has led to a global pandemic and has been associated with acute respiratory distress syndrome (ARDS). Among its other manifestations, COVID-19 has also been linked to HLH. In this report, we describe a case of a male patient who presented with multisystem organ failure and was found to have HLH. Since no clear etiology for his HLH could be elicited, it was determined to be a result of his recent COVID-19 infection.

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“…Αs an entity, it may affect patients of all age groups and a causal relationship between a number of factors and HLH has been reported, including viral infections (Herpes simplex virus (HSV), EBV, CMV, HIV, Influenza A and parvovirus B19), bacterial and fungal infection (Candida, Aspergillus), hematological malignancies and solid tumors, connective tissue disorders, immunodeficiency syndromes, and cellular therapies [12][13][14]. 8,13,[21][22][23]. The main characteristics, treatment and outcome of the aforementioned cases are presented in Supplementary Table 1.…”

Section: Discussionmentioning

confidence: 99%

“…Immune-mediated macrophage activation is the hallmark of sHLH. Both Sars-Cov-2 infection [8,13,[21][22][23]25] and MDS are associated with dysregulation of the immune system. In MDS, the association between cytotoxic T-lymphocytes and apoptosis has been implicated in the pathogenesis of refractory cytopenias.…”

Section: Discussionmentioning

confidence: 99%

Hemophagocytic Lymphohistiocytosis in A Post Covid-19 Patient with Mds: A Case Report

Vasilopoulos¹,

P²,

Kalliope³

et al. 2022

Int J Blood Res Disord

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Primary or secondary hemophagocytic lymphohistiocytosis (HLH) is characterized by immune activation and lifethreatening cytopenias. Causative relationship between a number of pathogens, autoimmune diseases and even hematologic malignancies with secondary HLH (sHLH) have been reported; however, correlation with myelodysplastic syndromes (MDS) is exceedingly unusual. HLH in COVID-19 positive patients has been described. Nevertheless, patients who have recovered after COVID-19 infection and test negative for the virus by RT-PCR may develop sHLH due to immune system dysregulation. We present here a rare case of sHLH manifestation after COVID-19 infection in a patient with a history of MDS. The recently revised H-score contributed to diagnosis.

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Secondary Hemophagocytic Lymphohistiocytosis in a Post-COVID-19 Patient

Cited by 10 publications

References 16 publications

Hemophagocytic Syndrome and COVID-19: A Comprehensive Review

Hemophagocytic Syndrome and COVID-19: A Comprehensive Review

Coronavirus Does It Again: Post-COVID-19 Hemophagocytic Lymphohistiocytosis (HLH)

Hemophagocytic Lymphohistiocytosis in A Post Covid-19 Patient with Mds: A Case Report

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