Hemophagocytic lymphohistiocytosis as a presentation of T-cell lymphoma: Case report: Hemophagocytic lymphohistiocytosis
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 c...
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| Autores principales: | , , , , , |
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| Formato: | Artículo revista |
| Lenguaje: | Español |
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Universidad Nacional Córdoba. Facultad de Ciencias Médicas. Secretaria de Ciencia y Tecnología
2021
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| Materias: | |
| Acceso en línea: | https://revistas.unc.edu.ar/index.php/med/article/view/30373 |
| Aporte de: |
| Sumario: | 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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