Malignancy-associated haemophagocytic lymphohistiocytosis Review
| Authors: | Setiadi, A.; Zoref-Lorenz, A.; Lee, C. Y.; Jordan, M. B.; Chen, L. Y. C. |
| Review Title: | Malignancy-associated haemophagocytic lymphohistiocytosis |
| Abstract: | Haemophagocytic lymphohistiocytosis (HLH) is an inflammatory syndrome that can occur with cancer (malignancy-associated HLH) or with immune-activating therapies for cancer. Patients with lymphoma appear to be at particularly high risk for malignancy-associated HLH. The familial form of HLH is characterised by uncontrolled activation of macrophages and cytotoxic T cells, which can be identified by genetics or specific immune markers. However, the pathophysiology of malignancy-associated HLH is not well understood, and distinguishing pathological immune activation from the laboratory and clinical abnormalities seen in cancer and cancer treatment is challenging. Emerging diagnostic tools, such as serum cytokine or chemokine concentrations, flow cytometry, and other functional measures, are discussed. Mortality remains high with current approaches. Targeted therapy, including blockade of specific cytokines such as IL-1, IL-6, and IFNγ, and inhibition of the JAK–STAT pathways might improve outcomes for some patients. Finally, we discuss a framework for thinking of malignancy-associated HLH within a larger umbrella concept of cytokine storm syndrome. © 2022 Elsevier Ltd |
| Keywords: | gene mutation; mortality; review; doxorubicin; flow cytometry; outcome assessment; neoplasm; neoplasms; cd8+ t lymphocyte; lymphocyte proliferation; t lymphocyte; biomarkers; biological marker; c reactive protein; cancer immunotherapy; etoposide; cyclophosphamide; vincristine; hematopoietic stem cell transplantation; cytotoxicity; acute lymphoblastic leukemia; prednisolone; t cell lymphoma; cytokine; cytokines; immune response; gamma interferon; bone marrow biopsy; cytotoxic t lymphocyte; interleukin 6; chimeric antigen receptor; allogeneic hematopoietic stem cell transplantation; natural killer cell; corticosteroid; macrophage; cytokine release; immunosuppressive treatment; chronic lymphatic leukemia; epstein barr virus; ferritin; human herpesvirus 8; fibrinogen; interleukin 1; hemophagocytic syndrome; lymphohistiocytosis, hemophagocytic; interleukin 18; ferritin blood level; cytotoxicity assay; disease activity; complication; cytokine release syndrome; diffuse large b cell lymphoma; transcription factor gata 2; humans; human; macrophage activation syndrome; jak-stat signaling; aggressive nk cell leukemia |
| Journal Title: | The Lancet Haematology |
| Volume: | 9 |
| Issue: | 3 |
| ISSN: | 2352-3026 |
| Publisher: | Elsevier Science, Inc. |
| Date Published: | 2022-03-01 |
| Start Page: | e217 |
| End Page: | e227 |
| Language: | English |
| DOI: | 10.1016/s2352-3026(21)00366-5 |
| PUBMED: | 35101205 |
| PROVIDER: | scopus |
| DOI/URL: | |
| Notes: | Review -- Source: Scopus |
