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2020, Number 1

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Rev Cubana Hematol Inmunol Hemoter 2020; 36 (1)

Hemophagocytic lymphohistiocytosis, a potentially fatal sundrome. Presentation of three cases

Valdés DKC, Hernández-Negrín H, Valdés LL, Vila TAA, Tejeda RMC

Language: Spanish
References: 20
Page: 1-11
PDF size: 757.46 Kb.


ABSTRACT

Introduction: The current evidence about hemophagocytic lymphohistiocytosis syndrome is based on case series and, therefore, clinical decisions are based on expert criteria. In Cuba, there are few published reports, which validates the efforts that may increase understanding of this entity in our environment.
Objective: To describe three cases of secondary hemophagocytic lymphohistiocytosis syndrome, an extremely rare and little suspected complication.
Clinical cases: Of the three patients studied, two presented lymphoma as subjacent disease. One of them was diagnosed with anaplastic T-cell non-Hodgkin lymphoma at autopsy. The other was diagnosed and treated early for large B-cell non-Hodgkin lymphoma, and evolved satisfactorily. The third patient presented tricuspid valve endocarditis and achieved remission after antibiotic, immunomodulatory and surgical treatment.
Conclusions: The cases presented highlight the complexity of hemophagocytic lymphohistiocytosis syndrome and reinforce the critical need for diagnosis and timely treatment of this condition in our setting.


REFERENCES

  1. Ramos-Casals M, Brito-Zerón P, López-Guillermo A, Khamashta M, Bosch X. Adult haemophagocytic syndrome. Lancet. 2014;383:1503-16.

  2. Pasvolsky O, Zoref-Lorenz A, Abadi U, Revital Geiger K, Lucille Hayman K, Vaxman I, et al. Hemophagocytic lymphohistiocytosis as a harbinger of aggressive lymphoma: a case series. Int J Hematol. 2019 May;109(5):553-62.

  3. Papo T. Síndromes hemofagocíticos, síndrome de activación macrofágica. EMC-Tratado de Medicina. 2019;23(2):1-8.

  4. Otrock ZK, Daver N, Kantarjian HM, Eby CS. Diagnostic challenges of hemophagocytic lymphohistiocytosis. Clin Lymphoma Myeloma Leuk. 2017;17(S1):S105-10.

  5. Brito-Zerón P, Kostov B, Moral-Moral P, Martínez-Zapico A, Díaz-Pedroche C, Fraile G, et al. Prognostic factors of death in 151 adults with hemophagocytic syndrome: Etiopathogenically driven analysis. Mayo Clin Proc Innov Qual Outcomes. 2018 Aug;2(3):267-76.

  6. Cárdenas Bruno M, Moreno Miravalles M. Diagnóstico postmortem de un caso con síndrome hemofagocítico secundario. Rev Cub Ped. 2019 [acceso 20/06/2019];91(1). Disponible en: http://scielo.sld.cu/scielo.php?script=sciarttext&pid=S0034-75312019000100012&lng=es&nrm=iso

  7. Blanco de la Portilla LE, Basanta Ampudia R, Blanco de la Portilla M. Síndrome de activación macrofágica en paciente pediátrico con enfermedad de Still. Univ Méd Pinareña. 2019 [acceso 20/06/2019];15(1):[155-62 pp.]. Disponible en: https://www.medigraphic.com/cgi-bin/new/resumenI.cgi?IDARTICULO=86405

  8. López-López JM, Sánchez-Guerra JA, Duany-Almira LF, Pompa-Carrazana SG, Larramendi-Benítez L. Linfohistiocitosis hemofagocítica familiar. Reporte de un caso. 16 de Abril. 2018 [acceso 20/06/2019];57(269):[195-200pp.]. Disponible en: https://www.medigraphic.com/cgi-bin/new/resumen.cgi?IDARTICULO=84158

  9. Santos Pérez L, Martínez Morales O, Milián Hernández G. Síndrome hemofagocítico. Informe de caso y revisión de la enfermedad. Acta Médica del Centro. 2017 [acceso 20/06/2019];11(4). Disponible en: http://www.revactamedicacentro.sld.cu/index.php/amc/article/view/861

  10. Morales Mesa E, Cubero Rego M, Reyes Romero O, Broche Cardó R, Pérez Moré M, González García N. Síndrome hemofagocítico secundario en el recién nacido. Rev Cub Ped. 2010 [acceso 15/06/2019];82. Disponible en: http://scielo.sld.cu/scielo.php?script=sci_arttext&pid=S0034-75312010000200012&nrm=iso

  11. Jaime Fagundo JC, Trelles Porro L, Durán Guarch G, Rodríguez Pérez JL, Gutiérrez Díaz A, Pavón Morán V, et al. Linfohistiocitosis hemofagocítica. Informe de un caso. Rev Cub Hematol Inmunol Hemoter. 2010 Dic [acceso11/06/2019];26(3):241-5. Disponible en: http://scielo.sld.cu/scielo.php?script=sci_arttext&pid=S0864-02892010000300009&lng=es

  12. González del Valle González Z, Vázquez Vigoa A, Rodríguez Fernández R, Lima Pérez LJ, Machado Puerto I. Síndrome de linfohistiocitosis hemofagocítica: a propósito de un caso. Rev Cub Med. 2003 [acceso 19/06/2019];42(4). Disponible en: http://scielo.sld.cu/scielo.php?script=sci_arttext&pid=S0034-75232003000400009&lng=es&nrm=iso

  13. Brito-Zerón P, Bosch X, Pérez-de-Lis M, Pérez-Álvarez R, Fraile G, Gheitasi H, et al. Infection is the major trigger of hemophagocytic syndrome in adult patients treated with biological therapies. Semin Arthritis Rheum. 2016;45:391-9.

  14. Roe C, Bennett J, Zhang L, Chavez J, Shah B, Sokol L, et al. Hemophagocytic Lymphohistiocytosis in Malignant Hematology: Uncommon but Should Not Be Forgotten? Clin Lymphoma Myeloma Leuk. 2015;15(S1):S147-50

  15. Henter J, Horne A, Arico M, Egeler R, Filipovich A, Imashuku S, et al. HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer. 2007;48:124-31.

  16. Naffaa M, Awad J, Oren I, Braun E, Lavi N. Group G streptococcal endocarditis-associated hemophagocytic syndrome. Int J Infect Dis. 2013;17:e1237-e9.

  17. Costescu Strachinaru D, Chaumont M, Gobin D, Sattar L, Strachinaru M, Karakike E, et al. Hemophagocytic lymphohistiocytosis associated to Haemophilus parainfluenzae endocarditis– a case report. Acta Clin Belg. 2018 Jun;73(3):220-3.

  18. Varghese B, Tinga K, Lopez-Matteib J, Iliescub C, Kimc J, Kim P. Aspergillus endocarditis of the mitral valve with ventricular myocardial invasion, cerebral vasculitis, and intracranial mycotic aneurysm formation in a patient with hemophagocytic lymphohistiocytosis. Med Mycol Case Rep. 2018;21:49-51.

  19. Xu P, Zeng H, Zhou M, Ouyang J, Chen B, Zhang Q. A patient with left-sided endocarditis and secondary hemophagocytic lymphohistiocytosis: a rare case report and literature review. Medicine (Baltimore). 2017 Dec;96(51):e9451.

  20. Sass LA, Ziemba KJ, Heiser EA, Clifford T. Mauriello, Werner AL, Aguiar MA, et al. A 1-year-old with mycobacterium tuberculosis endocarditis with mass spectrometry analysis of cardiac vegetation composition. J Pediatric Infect Dis Soc. 2016;5:85-8.




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