EZH2mutation in an adolescent with Weaver syndrome developing acute myeloid leukemia and secondary hemophagocytic lymphohistiocytosis

2016 ◽  
Vol 170 (5) ◽  
pp. 1274-1277 ◽  
Author(s):  
Jakob Usemann ◽  
Thomas Ernst ◽  
Vivien Schäfer ◽  
Kai Lehmberg ◽  
Karl Seeger
Keyword(s):  
Acute Myeloid Leukemia ◽  
Hemophagocytic Lymphohistiocytosis ◽  
Myeloid Leukemia ◽  
Weaver Syndrome ◽  
Secondary Hemophagocytic Lymphohistiocytosis ◽  
Acute Myeloid

scholarly journals Hemophagocytic Lymphohistiocytosis Syndrome As Presenting Sign For Acute Monocytic Leukaemia- A Case Report.

2020 ◽  
Vol 7 (08) ◽  
pp. 4918-4924
Author(s):  
Gal Sahaf Levin ◽  
Gida Ayada ◽  
Moshe Yeshurun ◽  
Oleg Rogach ◽  
Shaul Lev
Keyword(s):  
Acute Myeloid Leukemia ◽  
Organ Failure ◽  
Hemophagocytic Lymphohistiocytosis ◽  
Myeloid Leukemia ◽  
Case Reports ◽  
Transitional Cell ◽  
Interleukin 2 ◽  
Clinical Manifestations ◽  
Multi Organ Failure ◽  
Acute Myeloid

Background: Hemophagocytic lymphohistiocytosis (HLH) is a rare aggressive syndrome of excessive immune activation. Clinical manifestations of this syndrome mimic various other clinical conditions making the diagnosis harder to achieve. These manifestations are believed to be a result of cytokine storm which leads, eventually, to a multi-organ failure and eventually death. The latter two might be prevented if HLH was diagnosed early. HLH is classified into primary consist of monogenic disorders and secondary occurs as a complication in various settings such as infection, autoimmune disease, and malignancy. In hematologic malignancies, HLH is classically associated with specific entities, mainly, lymphoma or induced by treatment-related infections. Acute myeloid leukemia, on the other hand, is less common trigger with only few case reports.   Case presentation: An 83-years-old, 5 years free of transitional cell man, presented with unstable atrial fibrillation was intubated and shortly after that he developed a multi-organ failure. Bicytopenia and a high level of ferritin aroused a clinical suspicion of HLH syndrome. Further evaluation revealed high levels of soluble interleukin 2 receptors and no activity of natural killers cells. A bone marrow was performed and it did not show phagocytosis, however, acute myeloid leukemia (AML) was diagnosed. AML was suggested to be associated with chemotherapy that our patient received 5 years earlier.   Conclusion: Hemophagocytic lymphohistiocytosis can be present as a multi-organ failure requiring a high index of suspicion. Chemotherapy related-AML can be a trigger for HLH. 

Risk of Etoposide-Related Acute Myeloid Leukemia in the Treatment of Epstein-Barr Virus—Associated Hemophagocytic Lymphohistiocytosis

2002 ◽  
Vol 75 (2) ◽  
pp. 174-177 ◽  
Author(s):  
Shinsaku Imashuku ◽  
Tomoko Teramura ◽  
Kikuko Kuriyama ◽  
Junichi Kitazawa ◽  
Etsuro Ito ◽  
...  
Keyword(s):  
Acute Myeloid Leukemia ◽  
Hemophagocytic Lymphohistiocytosis ◽  
Myeloid Leukemia ◽  
Epstein Barr Virus ◽  
Barr Virus ◽  
Epstein Barr ◽  
Acute Myeloid

scholarly journals Hemophagocytic lymphohistiocytosis as an unexpected complication of Venetoclax+Azacitidine in Acute Myeloid Leukemia

2021 ◽  
Vol 9 (1) ◽  
pp. 17-19
Author(s):  
Ovilla-Martinez Roberto ◽  
Perez-Lozano Uendy ◽  
Cota-Rangel Xochitl ◽  
Baez-Islas Pamela
Keyword(s):  
Acute Myeloid Leukemia ◽  
Hemophagocytic Lymphohistiocytosis ◽  
Myeloid Leukemia ◽  
Early Recognition ◽  
Case Series ◽  
Lymphocytic Leukemia ◽  
Acute Phase Reactants ◽  
Hematologic Disorder ◽  
Serious Infections ◽  
Acute Myeloid

Background: Venetoclax is a drug that targets BCL-2 protein in cancer cells, first approved for chronic lymphocytic leukemia, this drug has showed efficacy also in acute myeloid leukemia in non-intense chemotherapy candidates in combination with hypomethylating agents as azacitidine and decitabine. This scheme has shown efficiency in acute myeloid leukemia reporting overall response rate (CR) in 61% in untreated elderly patients combined with azacitidine or decitabine. Febrile neutropenia was reported in 30%, thrombocytopenia in 47%, and serious infections in 33%. Hemophagocytic lymphohistiocytosis (HLH) is an uncommon hematologic disorder caused by a proinflammatory state manifested by cytopenias and elevation of acute phase reactants; it is a severe complication of some diseases and to our knowledge it has never been reported secondary to venetoclax plus azacitidine. Early treatment is fundamental for success in HLH. Case series: Three cases of HLH secondary to venetoclax plus azacitidine have appeared in our medical group in patients treated for acute myeloid leukemia. One elderly woman and elderly men with previously untreated acute myeloid leukemia presented HLH with laboratory and bone marrow findings, both responded to dexamethasone plus ruxolitinib. The third case was documented in a male diagnosed with blast phase chronic myeloid leukemia who also responded to dexamethasone plus ruxolitinib. No patient died from HLH. Conclusion: Here we report three cases of patients with HLH after the treatment with azacitidine plus venetoclax. We suspect that the great effect of venetoclax in synergy with azacitidine can liberate enough proinflammatory cytokines in the medullar niche to induce HLH. Early recognition is vital for soon treatment and successful management of this potential complication.

Therapy-Related Acute Myeloid Leukemia with KMT2A-SNX9 gene fusion associated with a hyperdiploid karyotype after Hemophagocytic Lymphohistiocytosis

2021 ◽  
Author(s):  
Ingrid Sardou-Cezar ◽  
Bruno A. Lopes ◽  
Francianne Gomes Andrade ◽  
Teresa Cristina Cardoso Fonseca ◽  
Teresa de Souza Fernandez ◽  
...  
Keyword(s):  
Acute Myeloid Leukemia ◽  
Hemophagocytic Lymphohistiocytosis ◽  
Myeloid Leukemia ◽  
Gene Fusion ◽  
Acute Myeloid

scholarly journals Acute myeloid leukemia with new complex t(8;21;22) induced hemophagocytic lymphohistiocytosis

Medicine ◽  
2018 ◽  
Vol 97 (44) ◽  
pp. e12762
Author(s):  
Yuling Wu ◽  
Juan Xu ◽  
Kai Shen ◽  
Jie Ji ◽  
Chenlu Yang ◽  
...  
Keyword(s):  
Acute Myeloid Leukemia ◽  
Hemophagocytic Lymphohistiocytosis ◽  
Myeloid Leukemia ◽  
Acute Myeloid
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