Visceral Leshmaniasis Associated Hemophagocytic Syndrome: A Case Report
DOI:
https://doi.org/10.60591/crspmi.48Keywords:
Amphotericin B/therapeutic use, Leishmaniasis, Visceral/diagnosis, Leishmaniasis, Visceral/drug therapy, Lymphohistiocytosis, Hemophagocytic/diagnosis, Lymphohistiocytosis, Hemophagocytic/drug therapyAbstract
Hemophagocytic syndrome is a rare severe systemic hyperinflammatory state which results in multiorgan failure, being potentially life threatening. It can be genetic or acquired in the setting of infections, malignancies, autoimmune disorders and immunodeficiency. The authors describe a case of a 19-year-old male admitted to the Emergency Department due to an infected sacrococcygeal pilonidal sinus with pancytopenia in addition to persistent fever. After an apparent initial improvement, there was a clinical deterioration related to severe pancytopenia and massive splenomegaly. The investigation showed high probability of hemophagocytic syndrome and there was high clinical suspicion of visceral leishmaniasis infection. Despite the absence of microorganism tests results, amphotericin B was empirically started. However, hemorrhagic dyscrasia occurred, requiring hospitalization in the intensive care unit and splenectomy, with full recovery. Anatomopathological study of the spleen confirmed visceral leishmaniasis diagnosis.
Downloads
References
Hayden A, Park S, Giustini D, Lee AY, Chen LY. Hemophagocytic syndromes (HPSs) including hemophagocytic lymphohistiocytosis (HLH) in adults: A systematic scoping review. Blood Rev. 2016;30:411-20. doi: 10.1016/j.blre.2016.05.001.
Rosado FG, Kim AS. Hemophagocytic lymphohistiocytosis: an update on diagnosis and pathogenesis. Am J Clin Pathol. 2013;139:713-27. doi: 10.1309/AJCP4ZDKJ4ICOUAT.
Janka GE, Lehmberg K. Hemophagocytic syndromes--an update. Blood Rev. 2014;28:135-42. doi: 10.1016/j.blre.2014.03.002.
La Rosee P, Horne A, Hines M, von Bahr Greenwood T, Machowicz R, Berliner N, et al. Recommendations for the management of hemophagocytic lymphohistiocytosis in adults. Blood. 2019;133:2465-77. doi: 10.1182/blood.2018894618.
Aronson N, Herwaldt BL, Libman M, Pearson R, Lopez-Velez R, Weina P, et al. 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). Clin Infect Dis. 2016;63:e202-64. doi: 10.1093/cid/ciw670.
World Health Organization. Leishmaniasis. 2022 [cited 2022 Oct 1]. Available from: http://www.who.int/news-room/fact-sheets/detail/leishmaniasis
Fardet L, Galicier L, Lambotte O, Marzac C, Aumont C, Chahwan D, et al. Development and validation of the HScore, a score for the diagnosis of reactive hemophagocytic syndrome. Arthritis Rheumatol. 2014;66:2613-20. doi: 10.1002/art.38690.
Henter JI, Horne A, Arico M, Egeler RM, Filipovich AH, Imashuku S, et al. HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer. 2007;48:124-31. doi: 10.1002/pbc.21039.
Riviere S, Galicier L, Coppo P, Marzac C, Aumont C, Lambotte O, Fardet L. Reactive hemophagocytic syndrome in adults: a retrospective analysis of 162 patients. Am J Med. 2014;127:1118-25. doi: 10.1016/j.amjmed.2014.04.034.
George MR. Hemophagocytic lymphohistiocytosis: review of etiologies and management. J Blood Med. 2014 ;5:69-86. doi: 10.2147/JBM.S46255.
Scalzone M, Ruggiero A, Mastrangelo S, Trombatore G, Ridola V, Maurizi P, et al. Hemophagocytic lymphohistiocytosis and visceral leishmaniasis in children: case report and systematic review of literature. J Infect Dev Ctries. 2016;10:103-8. doi: 10.3855/jidc.6385.
Hutchinson M, Tattersall RS, Manson JJ. Haemophagocytic lymphohisticytosis-an underrecognized hyperinflammatory syndrome. Rheumatology. 2019;58:vi23-vi30. doi: 10.1093/rheumatology/kez379.
Singh G, Shabani-Rad MT, Vanderkooi OG, Vayalumkal JV, Kuhn SM, Guilcher GM, et al. Leishmania in HLH: a rare finding with significant treatment implications. J Pediatr Hematol Oncol. 2013;35:e127-9. doi: 10.1097/MPH.0b013e318286d619.
Parikh SA, Kapoor P, Letendre L, Kumar S, Wolanskyj AP. Prognostic factors and outcomes of adults with hemophagocytic lymphohistiocytosis. Mayo Clin Proc. 2014;89:484–92. doi: 10.1016/j.mayocp.2013.12.012
Ozdemir N, Koc B, Arslantas E, Odaman Al I, Kelleci C, Uysalol EP, et al. Hemophagocytic lymphohistiocytosis associated with visceral leishmaniasis. J Pediatr Hematol Oncol. 2018;40:395. doi: 10.1097/ MPH.0000000000001178.
