Hemophagocytic lymphohistiocytosis as a presentation of T-cell lymphoma

Case report: Hemophagocytic lymphohistiocytosis

Authors

  • Silvina Casares Hospital Privado Universitario de Córdoba
  • Martín Milanesio Hospital Privado Universitario de Córdoba
  • Enzo Amelia Hospital Privado Universitario de Córdoba
  • Emanuel José Saad Hospital Privado universitario de Córdoba https://orcid.org/0000-0001-6244-7607
  • Luciana Guanchiale Hospital Privado Universitario de Córdoba
  • Juan Manuel Alomar Hospital Privado Universitario de Córdoba

DOI:

https://doi.org/10.31053/1853.0605.v78.n3.30373

Keywords:

lymphohistiocytosis, hemophagocytic, lymphoma, immunocompromised host, lymphadenopathy

Abstract

Introduction: Hemophagocytic lymphohistiocytosis (HHL), a severe hyperinflammatory syndrome caused by aberrant activation of macrophages and cytotoxic T cells, is clinically manifested as a febrile onset along with cytopenias, high ferritin serum level and splenomegaly. In adult patients, secondary causes of it should be looked for, such as autoimmune and infectious diseases and neoplasms. Prompt initiation of treatment is important due to the high mortality of this syndrome.

Methods: A case of a 53-year-old patient diagnosed with HHL associated with T-cell lymphoma is presented as a sudden onset of several symptoms and signs of HHL, along with infectious complications.

Results: Once the diagnosis of HHL was made, treatment with Etoposide and Dexamethasone was started, and with the diagnosis of T-cell lymphoma established, chemotherapy treatment with a CHOEP scheme was started, with a favorable initial evolution.

Main conclusion: As HHL is a rare entity associated with high mortality, initial suspicion must be high when facing an acute onset of fever, cytopenia and splenomegaly. Nonetheless, diagnosing HHL is challenging and often appear superimposed on multiple infectious diseases. The early initiation of treatment is important given the high mortality of this pathology

Key words: lymphohistiocytosis, hemophagocytic; lymphoma; immunocompromised host; lymphadenopathy

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Author Biographies

Silvina Casares, Hospital Privado Universitario de Córdoba

Second-year resident of the Internal Medicine of the Universidad Nacional de Córdoba(UNC), currently practicing in the Hospital Privado Universitario de Córdoba. Trainings carried out: BLS (2019) // National Congress of Internal Medicine (2018) // XVII National Day of Internal Medicine (2017). Volunteer work: Member of Rotaract Club Cordoba, a program of Rotary International.

Martín Milanesio, Hospital Privado Universitario de Córdoba

Fourth year resident doctor of the Internal Medicine of the Hospital Privado Universitario de Córdoba. Former assistant student in the Human Physiology chair of the Medicine career Faculty of Health Sciences of the Universidad Católica de Córdoba. Author of presentations at conferences and publication in an indexed journal

Enzo Amelia, Hospital Privado Universitario de Córdoba

First-year anesthesiology resident at the Universidad Católica de Córdoba (UCC), currently practicing in the Hospital Privado Universitario de Córdoba Hospital.

Emanuel José Saad, Hospital Privado universitario de Córdoba

Specialist in Internal Medicine, Facultad de Ciencias Médicas, Universidad Nacional de Córdoba (UNC). Member of the Internal Medicine department of Hospital privado universitario de Córdoba. Master's degree in clinical research and scientific knowledge management, Miguel de Cervantes European University. Instructor of residents physician the Hospital Privado Universitario de Córdoba. . Postgraduate teacher of the Internal medicine of UNC and the Universidad Católica de Córdoba, through the residency of the Internal Medicine in Hospital privado Universitario de Córdoba. Basic Life Support Instructor, American Heart Association. Adjunct Professor of the Department of Medicine III, IUCBC. Clinical Teaching Instructor, Chairs of Medicine I, Medina II, Medicine III, and PFO, IUCBC. Author of indexed scientific publications.

Luciana Guanchiale, Hospital Privado Universitario de Córdoba

Specialist in Internal Medicine. Specialist in Clinical Hematology. I remember the SAH lymphoma subcommittee. Physician at the Hematology and Oncology Service of Hospital Privado Universitario de Córdoba. Member of subcommittee on lymphomas of the GATLA. Former President of the SHC. Teaching instructor of the specialization course in Hematology by IUCBC. Teachcer of Medicine III at Instituto universitario de Ciencias Biomédicas de Córdoba and in Universidad Nacional de Córdoba.

Juan Manuel Alomar, Hospital Privado Universitario de Córdoba

Specialist in Internal Medicine, Facultad de Ciencias Médicas, Universidad Nacional de Córdoba (UNC). Member of the Internal medicine department of Hospital Privado Universitario de Córdoba. Head of the Department of Internado del Hospital Privado Universitario de Córdoba. Professor of postgraduate studies in Clinical Medicine at UNC and Universidad Católica de Córdoba, through residency in Internal Medicine at Hospital Privado Universitario de Córdoba. Adjunct Professor of the Obligatoria Final Practice at the Instituto universitario de Ciencias Biomédicas de Córdoba. Professor in Ciencias de la Salud awarded by Instituto Universitario de Ciencias Biomédicas de Córdoba.

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Published

2021-08-23

How to Cite

1.
Casares Diaz SA, Milanesio M, Amelia EM, Saad EJ, Guanchiale L, Alomar JM. Hemophagocytic lymphohistiocytosis as a presentation of T-cell lymphoma: Case report: Hemophagocytic lymphohistiocytosis. Rev Fac Cien Med Univ Nac Cordoba [Internet]. 2021 Aug. 23 [cited 2024 Jun. 29];78(3):317-21. Available from: https://revistas.unc.edu.ar/index.php/med/article/view/30373

Issue

Vol. 78 No. 3 (2021)

Section

Case Report

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