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

Main Article Content

Claudio Fozza

Keywords

acute myeloid leukemia, Hemophagocytic lymphohistiocytosis

Abstract

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.

Downloads

Download data is not yet available.


Abstract 504
PDF Downloads 216
HTML Downloads 69

References

1. Daitoku S, Aoyagi T, Takao S, Tada S, Kuroiwa M. Successful Treatment of Hemophagocytic Lymphohistiocytosis Associated with Low-risk Myelodysplastic Syndrome by Azacitidine. Intern Med.2018;57(20):2995-2999.
2. Delavigne K, Bérard E, Bertoli S, et al. Hemophagocytic syndrome in patients with acute myeloid leukemia undergoing intensive chemotherapy. Haematologica.2014;99(3):474-480.
3. Fozza C, Corda G, Barraqueddu F, et al. Azacitidine improves the T-cell repertoire in patients with myelodysplastic syndromes and acute myeloid leukemia with multilineage dysplasia. Leuk Res.2015; 39: 957-963.
4. Diesch J, Zwick A, Garz AK, Palau A, Buschbeck M, Götze KS. A clinical-molecular update on azanucleoside-based therapy for the treatment of hematologic cancers. Clin Epigenetics.2016;8:71.
5. Kalita P, Laishram D, Dey B, Mishra J, Barman B, Barman H. Secondary Hemophagocytic Lymphohistiocytosis in Post-COVID-19 Patients: A Report of Two Cases. Cureus.2021;13(8):e17328.
6. Kayaaslan BU et al (2021) A case of Hemophagocytic lymphohistiocytosis induced by COVID-19, and review of all cases reported in the literature. J Infect Dev Ctries.15(11):1607–1614.
7. Naous E, Nassani BM, Yaghi C, Nasr F, Medlej R. Hemophagocytic lymphohistiocytosis, a new cause of death during 'post-acute COVID-19 syndrome?' A case report. J Hematop.2021;14(3):229-233.
8. Ioannou M, Zacharouli K, Dukas SG, et al. Hemophagocytic lymphohistiocytosis diagnosed by bone marrow trephine biopsy in living post-COVID-19 patients: case report and mini-review. J Mol Histol.2022;53(4):753-762.