Hemophagocytic lymphohistiocytosis secondary to refractory acute myeloid leukemia resolved after second line treatment with azacitidine plus venetoclax

Hemophagocytic lymphohistiocytosis (HLH), also defined as hemophagocytic syndrome (HPS), represents a potentially life-threatening hyperinflammatory syndrome, characterized by impaired function of cytotoxic T lymphocytes, natural killer cells and macrophages. The main clinical features of HLH are p...

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Main Author: Claudio Fozza
Format: Article
Language:English
Published: PAGEPress Publications 2024-01-01
Series:Mediterranean Journal of Hematology and Infectious Diseases
Subjects:
Online Access:https://mjhid.org/mjhid/article/view/5564
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author Claudio Fozza
author_facet Claudio Fozza
author_sort Claudio Fozza
collection DOAJ
description Hemophagocytic lymphohistiocytosis (HLH), also defined as hemophagocytic syndrome (HPS), represents a potentially life-threatening hyperinflammatory syndrome, characterized by impaired function of cytotoxic T lymphocytes, natural killer cells and macrophages. The main clinical features of HLH are prolonged fever, hepatosplenomegaly, cytopenia, hypertriglyceridemia, hyperferritinemia and hemophagocytosis in bone marrow, liver, spleen or lymph nodes. Secondary HLH typically occurs in conjunction with severe infections, malignancies or autoimmune disorders and intensive chemotherapy, potentially complicating treatment of acute myeloid leukemia (AML) in around 10% of cases. Herein we report for the first time a case of HLH secondary to refractory/relapsed AML resolved after a second line treatment with azacitidine plus venetoclax, thus offering a new potential therapeutic perspective in the context of a life-threatening clinical scenario.
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spelling doaj-art-03e4fbadd5be440586795c8ca104e7662025-02-03T10:29:30ZengPAGEPress PublicationsMediterranean Journal of Hematology and Infectious Diseases2035-30062024-01-0116110.4084/MJHID.2024.011Hemophagocytic lymphohistiocytosis secondary to refractory acute myeloid leukemia resolved after second line treatment with azacitidine plus venetoclaxClaudio Fozza0University of Sassari Hemophagocytic lymphohistiocytosis (HLH), also defined as hemophagocytic syndrome (HPS), represents a potentially life-threatening hyperinflammatory syndrome, characterized by impaired function of cytotoxic T lymphocytes, natural killer cells and macrophages. The main clinical features of HLH are prolonged fever, hepatosplenomegaly, cytopenia, hypertriglyceridemia, hyperferritinemia and hemophagocytosis in bone marrow, liver, spleen or lymph nodes. Secondary HLH typically occurs in conjunction with severe infections, malignancies or autoimmune disorders and intensive chemotherapy, potentially complicating treatment of acute myeloid leukemia (AML) in around 10% of cases. Herein we report for the first time a case of HLH secondary to refractory/relapsed AML resolved after a second line treatment with azacitidine plus venetoclax, thus offering a new potential therapeutic perspective in the context of a life-threatening clinical scenario. https://mjhid.org/mjhid/article/view/5564acute myeloid leukemiaHemophagocytic lymphohistiocytosis
spellingShingle Claudio Fozza
Hemophagocytic lymphohistiocytosis secondary to refractory acute myeloid leukemia resolved after second line treatment with azacitidine plus venetoclax
Mediterranean Journal of Hematology and Infectious Diseases
acute myeloid leukemia
Hemophagocytic lymphohistiocytosis
title Hemophagocytic lymphohistiocytosis secondary to refractory acute myeloid leukemia resolved after second line treatment with azacitidine plus venetoclax
title_full Hemophagocytic lymphohistiocytosis secondary to refractory acute myeloid leukemia resolved after second line treatment with azacitidine plus venetoclax
title_fullStr Hemophagocytic lymphohistiocytosis secondary to refractory acute myeloid leukemia resolved after second line treatment with azacitidine plus venetoclax
title_full_unstemmed Hemophagocytic lymphohistiocytosis secondary to refractory acute myeloid leukemia resolved after second line treatment with azacitidine plus venetoclax
title_short Hemophagocytic lymphohistiocytosis secondary to refractory acute myeloid leukemia resolved after second line treatment with azacitidine plus venetoclax
title_sort hemophagocytic lymphohistiocytosis secondary to refractory acute myeloid leukemia resolved after second line treatment with azacitidine plus venetoclax
topic acute myeloid leukemia
Hemophagocytic lymphohistiocytosis
url https://mjhid.org/mjhid/article/view/5564
work_keys_str_mv AT claudiofozza hemophagocyticlymphohistiocytosissecondarytorefractoryacutemyeloidleukemiaresolvedaftersecondlinetreatmentwithazacitidineplusvenetoclax