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  1. Article: Hemophagocytic Syndrome and COVID-19: A Comprehensive Review.

    Fadlallah, Mahdi M / Salman, Sarah M / Fadlallah, Mariam M / Rahal, Hassan

    Cureus

    2023  Volume 15, Issue 3, Page(s) e36140

    Abstract: ... COVID-19) patients, a hyperinflammatory mechanism leading to hypercytokinemia and hyperferritinemia has ... overlap is present between the clinical and laboratory features seen in COVID-19 and sHLH. However, SARS ... COVID-19 patients presenting with multiorgan failure, in whom sHLH should be considered. ...

    Abstract 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.
    Language English
    Publishing date 2023-03-14
    Publishing country United States
    Document type Journal Article ; Review
    ZDB-ID 2747273-5
    ISSN 2168-8184
    ISSN 2168-8184
    DOI 10.7759/cureus.36140
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  2. Article: [Hemophagocytic syndrome secondary to COVID-19: a case report and literature review].

    Huang, L X / Liang, Y D / Wang, Y Q / Li, J T / Xu, X M / Li, Y M / Ju, Y

    Zhonghua jie he he hu xi za zhi = Zhonghua jiehe he huxi zazhi = Chinese journal of tuberculosis and respiratory diseases

    2023  Volume 46, Issue 8, Page(s) 791–796

    Abstract: Objective: ...

    Abstract Objective:
    MeSH term(s) Adult ; Humans ; Male ; Middle Aged ; Female ; COVID-19/complications ; SARS-CoV-2 ; Lymphohistiocytosis, Hemophagocytic/etiology ; Lymphohistiocytosis, Hemophagocytic/complications ; Prognosis ; Thrombocytopenia/complications
    Language Chinese
    Publishing date 2023-05-25
    Publishing country China
    Document type Review ; Case Reports ; English Abstract ; Journal Article
    ZDB-ID 1027965-9
    ISSN 1001-0939
    ISSN 1001-0939
    DOI 10.3760/cma.j.cn112147-20230320-00141
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  3. Article: Hemophagocytic syndrome and COVID-19.

    Lima, Ricardo / Filho, Célio Cortinhas / Ferreira Filho, Carlos Martins / Vaisman, Mauricio / Cossenza, Alexandre / Rebello, Caroline Pinto / Leite, Carolina de Andrade / de Oliveira Tavares, Danilo / Ramalho, Julia Falconiere

    Respiratory medicine case reports

    2020  Volume 31, Page(s) 101162

    Abstract: The case of a woman hospitalized due to COVID-19 is presented. The patient developed a severe ... macrophage activation syndrome diagnosed by bone marrow biopsy, which did not respond to immunoglobulin therapy. ...

    Abstract The case of a woman hospitalized due to COVID-19 is presented. The patient developed a severe macrophage activation syndrome diagnosed by bone marrow biopsy, which did not respond to immunoglobulin therapy.
    Keywords covid19
    Language English
    Publishing date 2020-07-10
    Publishing country England
    Document type Case Reports
    ZDB-ID 2666110-X
    ISSN 2213-0071
    ISSN 2213-0071
    DOI 10.1016/j.rmcr.2020.101162
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  4. Article ; Online: Severe COVID-19-associated hyperinflammatory syndrome versus classic hemophagocytic lymphohistiocytosis: similarities, differences, and the way forward.

    Yadav, Geeta / Malhotra, Hardeep / Mehta, Pankti / Sachu, Ruovinuo / Rizvi, Imran / Bharti, Vipin Raj / Kumar, Manish / Kumar, Neeraj / Ali, Wahid / Verma, Shailendra Prasad / Puri, Bipin / Dandu, Himanshu

    Journal of investigative medicine : the official publication of the American Federation for Clinical Research

    2023  Volume 71, Issue 3, Page(s) 244–253

    Abstract: ... COVID-19 infection, suspected to have COVID-HIS and 22 patients of sHLH to other illnesses, to evaluate ... related hyperinflammatory syndrome (COVID-HIS). This was a retrospective study of 47 patients of severe ... Only 6.4% (3/47) of cases fulfilled ≥5/8 HLH 2004 criteria and only 40.52% (19/47) of patients showed ...

    Abstract The hyperinflammatory immune response in severe COVID-19 infection shares features with secondary hemophagocytic lymphohistiocytosis (sHLH) in the form of fever, cytopenia, elevated inflammatory markers, and high mortality. There are contrasting opinions regarding utility of HLH 2004 or HScore in the diagnosis of severe COVID-19-related hyperinflammatory syndrome (COVID-HIS). This was a retrospective study of 47 patients of severe COVID-19 infection, suspected to have COVID-HIS and 22 patients of sHLH to other illnesses, to evaluate the diagnostic utility and limitations of HLH 2004 and/or HScore in context to COVID-HIS and to also evaluate the utility of Temple criteria for predicting severity and outcome in COVID-HIS. Clinical findings, hematological, and biochemical parameters along with the predictor of mortality were compared between two groups. Only 6.4% (3/47) of cases fulfilled ≥5/8 HLH 2004 criteria and only 40.52% (19/47) of patients showed HScore >169 in COVID-HIS group. 65.9% (31/47) satisfied the Temple criteria in COVID-HIS as compared with 40.9% (9/22) in the non-COVID group (p = 0.04). Serum ferritin (p = 0.02), lactate dehydrogenase (p = 0.02), direct bilirubin (p = 0.02), and C-reactive protein (p = 0.03) were associated with mortality in COVID-HIS. Both HScore and HLH-2004 criteria perform poorly for identifying COVID-HIS. Presence of bone marrow hemophagocytosis may help to identify about one-third of COVID-HIS missed by the Temple Criteria.
    MeSH term(s) Humans ; Lymphohistiocytosis, Hemophagocytic/complications ; Lymphohistiocytosis, Hemophagocytic/diagnosis ; COVID-19/complications ; Retrospective Studies ; Syndrome ; C-Reactive Protein
    Chemical Substances C-Reactive Protein (9007-41-4)
    Language English
    Publishing date 2023-01-10
    Publishing country England
    Document type Journal Article
    ZDB-ID 1217870-6
    ISSN 1708-8267 ; 0009-9279 ; 1081-5589
    ISSN (online) 1708-8267
    ISSN 0009-9279 ; 1081-5589
    DOI 10.1177/10815589221140596
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  5. Article ; Online: Severe Visceral Leishmaniasis Associated With COVID-19 and Complicated by Hemophagocytic Syndrome.

    Pessoa, Fabrício Silva / Salgado, Izabel Athayde da Silva Cruz / Gonçalves, Valdênia Costa / Martins, Anne Caroline Rabelo Raposo / Pessoa, Leilane Silva / Braga Junior, Leônidas

    The Pediatric infectious disease journal

    2022  Volume 41, Issue 12, Page(s) e544–e545

    MeSH term(s) Humans ; Leishmaniasis, Visceral/complications ; Leishmaniasis, Visceral/diagnosis ; Leishmaniasis, Visceral/drug therapy ; Lymphohistiocytosis, Hemophagocytic/complications ; Lymphohistiocytosis, Hemophagocytic/diagnosis ; Lymphohistiocytosis, Hemophagocytic/drug therapy ; COVID-19/complications ; Antiprotozoal Agents/therapeutic use
    Chemical Substances Antiprotozoal Agents
    Language English
    Publishing date 2022-09-07
    Publishing country United States
    Document type Journal Article
    ZDB-ID 392481-6
    ISSN 1532-0987 ; 0891-3668
    ISSN (online) 1532-0987
    ISSN 0891-3668
    DOI 10.1097/INF.0000000000003699
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  6. Article ; Online: Sulfasalazine-induced drug reaction with eosinophilia and systemic symptoms (DRESS) coinfected with COVID-19 complicated by hemophagocytic lymphohistiocytosis: a case report.

    Li, Mengmeng / Li, Furong / Dai, Yang / Zeng, Yunou Zhang / Chen, Xiaomei

    Frontiers in immunology

    2024  Volume 15, Page(s) 1371490

    Abstract: ... deficiencies. Recently, COVID-19 has also been identified as one of the triggers for sHLH. Herein, we present ... a case of Sulfasalazine-induced DRESS coinfected with COVID-19 that subsequently progressed into HLH ... We hypothesize that excessive activation of the immune system and cytokine storm due to DRESS combined with COVID ...

    Abstract Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS) is characterized by a widespread maculopapular rash, lymphadenopathy, fever, and multisystem involvement. Conversely, hemophagocytic lymphohistiocytosis (HLH) is an infrequent yet critical condition presenting with fever, hepatosplenomegaly, cytopenias, coagulation abnormalities, and elevated inflammatory markers. The overlapping clinical and laboratory features between DRESS and HLH poses a significant diagnostic challenge. Secondary HLH (sHLH) typically occurs in adults triggered by viral infections, malignancies, rheumatologic diseases, or immune deficiencies. Recently, COVID-19 has also been identified as one of the triggers for sHLH. Herein, we present a case of Sulfasalazine-induced DRESS coinfected with COVID-19 that subsequently progressed into HLH. Our patient exhibited common hepatorenal and splenic involvement along with rare cholecystitis and appendicitis. However, a significant improvement was observed upon the addition of etoposide and azathioprine. We hypothesize that excessive activation of the immune system and cytokine storm due to DRESS combined with COVID-19 infection led to more extensive systemic damage resulting in HLH development. This highlights the potential for severe consequences when DRESS coincides with HLH during a COVID-19 infection.
    MeSH term(s) Humans ; Lymphohistiocytosis, Hemophagocytic/etiology ; Lymphohistiocytosis, Hemophagocytic/diagnosis ; Lymphohistiocytosis, Hemophagocytic/drug therapy ; COVID-19/complications ; COVID-19/immunology ; Drug Hypersensitivity Syndrome/etiology ; Drug Hypersensitivity Syndrome/diagnosis ; Sulfasalazine/adverse effects ; SARS-CoV-2 ; Coinfection/drug therapy ; Male ; Middle Aged ; Female
    Chemical Substances Sulfasalazine (3XC8GUZ6CB)
    Language English
    Publishing date 2024-04-15
    Publishing country Switzerland
    Document type Case Reports ; Research Support, Non-U.S. Gov't
    ZDB-ID 2606827-8
    ISSN 1664-3224 ; 1664-3224
    ISSN (online) 1664-3224
    ISSN 1664-3224
    DOI 10.3389/fimmu.2024.1371490
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  7. Article ; Online: An Unusual Case of Hemophagocytic Lymphohistiocytosis Associated with

    Venigalla, Tejaswi / Kalathil, Sheila / Bansal, Meena / Morginstin, Mark / Jorge, Vinicius / Perosio, Patricia

    Current oncology (Toronto, Ont.)

    2023  Volume 30, Issue 3, Page(s) 3529–3536

    Abstract: ... as described in our case report, it was narrowed down to hemophagocytic lymphohistiocytosis and COVID-19. Two ... COVID-19 tests were negative. His lab abnormalities and diagnostic testing revealed ... rash with eosinophilia and systemic symptoms (DRESS); and metabolic disorder, including ...

    Abstract Hemophagocytic lymphohistiocytosis (HLH) is a rare and very dangerous condition characterized by abnormal activation of the immune system, causing hemophagocytosis, inflammation, and potentially widespread organ damage. The primary (genetic) form, caused by mutations affecting lymphocyte cytotoxicity, is most commonly seen in children. Secondary HLH is commonly associated with infections, malignancies, and rheumatologic disorders. Most current information on diagnosis and treatment is based on pediatric populations. HLH is a disease that should be diagnosed and treated promptly, otherwise it is fatal. Treatment is directed at treating the triggering disorder, along with symptomatic treatment with dexamethasone and etoposide. We present a 56-year-old patient who was admitted with worsening weakness, exertional dyspnea, dry and nonproductive cough, and a 5-pound weight loss associated with loss of appetite. This is among the rare disorders that are not commonly encountered in day-to-day practice. Our differential diagnoses were broad, including infection, such as visceral leishmaniasis, atypical/tuberculous mycobacteria, histoplasmosis, Ehrlichia, Bartonella, Brucella, Adenovirus, disseminated herpes simplex virus (HSV), hematological-like Langerhans cell histiocytosis, or multicentric Castleman disease; drug reaction, such as drug rash with eosinophilia and systemic symptoms (DRESS); and metabolic disorder, including Wolman's disease (infantile lysosomal acid lipase deficiency) or Gaucher's disease. Based on our investigations as described in our case report, it was narrowed down to hemophagocytic lymphohistiocytosis and COVID-19. Two COVID-19 tests were negative. His lab abnormalities and diagnostic testing revealed hemophagocytic lymphohistiocytosis. He was empirically started on antibiotics and dexamethasone, to be continued for 2 weeks then tapered if the patient showed continued improvement. Dexamethasone was tapered over 8 weeks. He improved on just one of the Food and Drug Administration (FDA)-approved medications, proving that treatment should be tailored to the patient. In addition, in this case study, we included the background, etiology, pathogenesis, diagnosis, management, and prognosis of HLH.
    MeSH term(s) United States ; Male ; Child ; Humans ; Middle Aged ; Lymphohistiocytosis, Hemophagocytic/complications ; Lymphohistiocytosis, Hemophagocytic/diagnosis ; Lymphohistiocytosis, Hemophagocytic/drug therapy ; COVID-19/complications ; Dexamethasone/therapeutic use ; Carcinoma, Neuroendocrine
    Chemical Substances Dexamethasone (7S5I7G3JQL)
    Language English
    Publishing date 2023-03-21
    Publishing country Switzerland
    Document type Case Reports
    ZDB-ID 1236972-x
    ISSN 1718-7729 ; 1198-0052
    ISSN (online) 1718-7729
    ISSN 1198-0052
    DOI 10.3390/curroncol30030268
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  8. Article: Hemophagocytic syndrome and COVID-19

    Lima, Ricardo / Filho, Célio Cortinhas / Ferreira Filho, Carlos Martins / Vaisman, Mauricio / Cossenza, Alexandre / Rebello, Caroline Pinto / Leite, Carolina de Andrade / de Oliveira Tavares, Danilo / Ramalho, Julia Falconiere

    Respir. Med. Case Rep.

    Abstract: The case of a woman hospitalized due to COVID-19 is presented. The patient developed a severe ... macrophage activation syndrome diagnosed by bone marrow biopsy, which did not respond to immunoglobulin therapy. ...

    Abstract The case of a woman hospitalized due to COVID-19 is presented. The patient developed a severe macrophage activation syndrome diagnosed by bone marrow biopsy, which did not respond to immunoglobulin therapy.
    Keywords covid19
    Publisher WHO
    Document type Article
    Note WHO #Covidence: #642868
    Database COVID19

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  9. Article ; Online: Hemophagocytic syndrome and COVID-19

    Ricardo Lima / Célio Cortinhas Filho / Carlos Martins Ferreira Filho / Mauricio Vaisman / Alexandre Cossenza / Caroline Pinto Rebello / Carolina de Andrade Leite / Danilo de Oliveira Tavares / Julia Falconiere Ramalho

    Respiratory Medicine Case Reports, Vol 31, Iss , Pp 101162- (2020)

    2020  

    Abstract: The case of a woman hospitalized due to COVID-19 is presented. The patient developed a severe ... macrophage activation syndrome diagnosed by bone marrow biopsy, which did not respond to immunoglobulin therapy. ...

    Abstract The case of a woman hospitalized due to COVID-19 is presented. The patient developed a severe macrophage activation syndrome diagnosed by bone marrow biopsy, which did not respond to immunoglobulin therapy.
    Keywords Diseases of the respiratory system ; RC705-779 ; covid19
    Language English
    Publishing date 2020-01-01T00:00:00Z
    Publisher Elsevier
    Document type Article ; Online
    Database BASE - Bielefeld Academic Search Engine (life sciences selection)

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  10. Article ; Online: Hemophagocytic syndrome and COVID-19

    Lima, Ricardo / Filho, Célio Cortinhas / Ferreira Filho, Carlos Martins / Vaisman, Mauricio / Cossenza, Alexandre / Rebello, Caroline Pinto / Leite, Carolina de Andrade / de Oliveira Tavares, Danilo / Ramalho, Julia Falconiere

    Respiratory Medicine Case Reports

    2020  Volume 31, Page(s) 101162

    Keywords Pulmonary and Respiratory Medicine ; covid19
    Language English
    Publisher Elsevier BV
    Publishing country us
    Document type Article ; Online
    ZDB-ID 2666110-X
    ISSN 2213-0071
    ISSN 2213-0071
    DOI 10.1016/j.rmcr.2020.101162
    Database BASE - Bielefeld Academic Search Engine (life sciences selection)

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