Hemophagocytic lymphohistiocytosis as a presentation of T-cell lymphoma.

Autor: Casares Diaz SA; Hospital Privado Universitario de Córdoba. silvi.casaresdiaz@gmail.com., Milanesio M; Hospital Privado Universitario de Córdoba. mmilanesio5@gmail.com., Amelia EM; Hospital Privado Universitario de Córdoba. enzomarceloamelia@gmail.com., Saad EJ; Hospital Privado universitario de Córdoba. emanuelsaad@hotmail.com., Guanchiale L; Hospital Privado Universitario de Córdoba. lucianaguanchiale@hotmail.com., Alomar JM; Hospital Privado Universitario de Córdoba. juan.alomar@hospitalprivado.com.ar.
Jazyk: Spanish; Castilian
Zdroj: Revista de la Facultad de Ciencias Medicas (Cordoba, Argentina) [Rev Fac Cien Med Univ Nac Cordoba] 2021 Aug 23; Vol. 78 (3), pp. 317-321. Date of Electronic Publication: 2021 Aug 23.
DOI: 10.31053/1853.0605.v78.n3.30373
Abstrakt: 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: 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 treatmbent is important given the high mortality of this pathology
(Universidad Nacional de Córdoba)
Databáze: MEDLINE