Case Report: Visceral Leishmaniasis and Hemophagocytic Lymphohistiocytosis: Three Clinical Cases, Three Different Patterns

Geydson Silveira Cruz Hematology Service and Transfusional Unit, University Hospital—Universidade Federal de Sergipe, Aracaju, Sergipe, Brazil;
Molecular Biology and Immunology Laboratory, University Hospital—Universidade Federal de Sergipe, Aracaju, Sergipe, Brazil;
Health Sciences Post Graduate Program—Universidade Federal de Sergipe, Aracaju, Sergipe, Brazil

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Amélia Ribeiro de Jesus Molecular Biology and Immunology Laboratory, University Hospital—Universidade Federal de Sergipe, Aracaju, Sergipe, Brazil;
Health Sciences Post Graduate Program—Universidade Federal de Sergipe, Aracaju, Sergipe, Brazil

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Roque Pacheco Almeida Molecular Biology and Immunology Laboratory, University Hospital—Universidade Federal de Sergipe, Aracaju, Sergipe, Brazil;
Health Sciences Post Graduate Program—Universidade Federal de Sergipe, Aracaju, Sergipe, Brazil

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Maria Aurélia Porto Hematology Service and Transfusional Unit, University Hospital—Universidade Federal de Sergipe, Aracaju, Sergipe, Brazil;

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Abstract.

Visceral leishmaniasis (VL) is a neglected tropical disease with more than 30,000 cases annually reported worldwide. In Brazil, about 3,700 cases are annually reported. The VL clinical presentation is variable, from asymptomatic to severe cases with a high risk of death. We reported three cases of VL with clinical sign similarities but distinct development. All cases had bone marrow hemophagocytosis and hemophagocytic lymphohistiocytosis (HLH) criteria. HLH is a rare condition that may have secondary causes, including infectious and parasitic diseases, like VL. The delayed recognition of the secondary HLH (sHLH) association to VL may cause unfavorable outcomes and death.

Author Notes

Address correspondence to Geydson Silveira Cruz, Hospital Universitário de Sergipe—Universidade Federal de Sergipe (UFS), Unidade Transfusional, Rua Cláudio Batista, 505 Palestina, Aracaju, Sergipe, Brazil 49.060-025. E-mail: geydson.cruz@ebserh.gov.br

Authors’ addresses: Geydson Silveira Cruz, Hematology Service and Transfusional Unit, University Hospital—Universidade Federal de Sergipe, Aracaju, Sergipe, Brazil, Molecular Biology and Immunology Laboratory, University Hospital—Universidade Federal de Sergipe, Aracaju, Sergipe, Brazil, and Health Sciences Post Graduate Program—Universidade Federal de Sergipe, Aracaju, Sergipe, Brazil, E-mail: geydson@hotmail.com. Amélia Ribeiro de Jesus and Roque Pacheco Almeida, Molecular Biology and Immunology Laboratory, University Hospital—Universidade Federal de Sergipe, Aracaju, Sergipe, Brazil, and Health Sciences Post Graduate Program—Universidade Federal de Sergipe, Aracaju, Sergipe, Brazil, E-mails: ameliaribeirodejesus@gmail.com and roquepachecoalmeida@gmail.com. Maria Aurélia Porto, Hematology Service and Transfusional Unit, University Hospital—Universidade Federal de Sergipe, Aracaju, Sergipe, Brazil, E-mail: maria.porto281@gmail.com.

  • 1.

    Focus E , 2016. WHO, Weekly epidemiological record: leishmaniasis in high-burden countries, an epidemiological update based on data reported in 2014. Wkly Epidemiol Rec / Heal Sect Secr Leag Nations 91: 287296.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 2.

    Pan American Health Organization—PAHO , 2019. Leishmaniasis: Epidemiological Report in the Americas. Available at: http://iris.paho.org/xmlui/handle/123456789/50505%0D. Accessed October 2019.

    • PubMed
    • Export Citation
  • 3.

    BRASIL M da S, 2019. LEISHMANIOSE VISCERAL—Casos confirmados notificados no Sistema de Informação de Agravos de Notificação. DATASUS—SINAN. Available at: http://tabnet.datasus.gov.br/cgi/tabcgi.exe?sinannet/cnv/leishvbr.def. Accessed January 16, 2019.

    • PubMed
    • Export Citation
  • 4.

    Burza S , Croft SL , Boelaert M , Maxfield L , Crane JS , 2018. Leishmaniasis. Lancet 392: 951970.

  • 5.

    Aronson N , Herwaldt BL , Libman M , Pearson R , Lopez-Velez R , Weina P , Carvalho E , Ephros M , Jeronimo S , Magill A , 2017. Diagnosis and treatment of leishmaniasis: Clinical Practice Guidelines by the Infectious Diseases Society of America (IDSA) and the American Society of Tropical Medicine and Hygiene (ASTMH). Am J Trop Med Hyg 96: 2445.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 6.

    Diamantidis MD , Palioura A , Ioannou M , Tsangalas E , Karakousis K , 2020. Hemophagocytic lymphohistiocytosis as a manifestation of underlying visceral leishmaniasis. Cureus 12: 48.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 7.

    Al-Samkari H , Berliner N , 2018. Hemophagocytic lymphohistiocytosis. Annu Rev Pathol Mech Dis 13: 2749.

  • 8.

    Koubâa M , Mâaloul I , Marrakchi C , Mdhaffar M , Lahiani D , Hammami B , Makni F , Ayedi A , Ben Jemâa M , 2012. Hemophagocytic syndrome associated with visceral leishmaniasis in an immunocompetent adult-case report and review of the literature. Ann Hematol 91: 11431145.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 9.

    Carvelli J et al. 2020. Functional and genetic testing in adults with HLH reveals an inflammatory profile rather than a cytotoxicity defect. Blood 136: 542552.

  • 10.

    La Rosée P et al. 2019. Recommendations for the management of hemophagocytic lymphohistiocytosis in adults. Blood 133: 24652477.

  • 11.

    Hayden A , Park S , Giustini D , Lee AYY , Chen LYC , 2016. Hemophagocytic syndromes (HPSs) including hemophagocytic lymphohistiocytosis (HLH) in adults: a systematic scoping review. Blood Rev 30: 411420.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 12.

    Allen CE , McClain KL , 2015. Pathophysiology and epidemiology of hemophagocytic lymphohistiocytosis. Hematology 2015: 177182.

  • 13.

    Otrock ZK , Eby CS , 2015. Clinical characteristics, prognostic factors, and outcomes of adult patients with hemophagocytic lymphohistiocytosis. Am J Hematol 90: 220224.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 14.

    Que Y et al. 2021. Cytokine release syndrome in COVID-19: a major mechanism of morbidity and mortality. Int Rev Immunol 22: 114.

  • 15.

    van Eeden C , Khan L , Osman MS , Tervaert JWC , 2020. Natural killer cell dysfunction and its role in COVID-19. Int J Mol Sci 21: 117.

  • 16.

    Duthie MS et al. 2014. Alteration of the serum biomarker profiles of visceral leishmaniasis during treatment. Eur J Clin Microbiol Infect Dis 33: 639649.

  • 17.

    Hayden A , Lin M , Park S , Pudek M , Schneider M , Jordan MB , Mattman A , Chen LYC , 2017. Soluble interleukin-2 receptor is a sensitive diagnostic test in adult HLH. Blood Adv 1: 25292534.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 18.

    La Rosee P , 2015. Treatment of hemophagocytic lymphohistiocytosis in adults. Hematology 2015: 190196.

  • 19.

    Lerolle N et al. 2016. Diversity and combinations of infectious agents in 38 adults with an infection-triggered reactive haemophagocytic syndrome: a multicenter study. Clin Microbiol Infect 22: 268.e1268.e8.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 20.

    Ahmed A et al. 2019. Ruxolitinib in adult patients with secondary haemophagocytic lymphohistiocytosis: an open-label, single-centre, pilot trial. Lancet Haematol 6: e630e637.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 21.

    Locatelli F et al. 2018. Safety and efficacy of Emapalumab in pediatric patients with primary hemophagocytic lymphohistiocytosis. Blood 132 (Suppl 1): LBA-6.

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