Kejian Zhang, Lauren K Meyer, Rafal Machowicz, Maria Luisa Coniglio, Elena Sieni, Kim E Nichols
{"title":"Genetics of Familial Hemophagocytic Lymphohistiocytosis (HLH).","authors":"Kejian Zhang, Lauren K Meyer, Rafal Machowicz, Maria Luisa Coniglio, Elena Sieni, Kim E Nichols","doi":"10.1016/j.hoc.2025.02.002","DOIUrl":"https://doi.org/10.1016/j.hoc.2025.02.002","url":null,"abstract":"<p><p>Familial hemophagocytic lymphohistiocytosis (fHLH) represents a group of rare, inherited immune system disorders characterized by uncontrolled inflammatory responses. fHLH results from genetic mutations that impair CD8 T cell and natural killer cell cytotoxicity. Without treatment, fHLH is commonly fatal, but early diagnosis and treatment, including immunosuppressive therapy, and in many cases, an allogeneic hematopoietic stem cell transplant (HSCT), can improve overall outcomes. Genetic testing is critical for confirming the diagnosis, identifying specific gene mutations, assessing family members for carrier status or disease risk, and informing donor selection for HSCT.</p>","PeriodicalId":55060,"journal":{"name":"Hematology-Oncology Clinics of North America","volume":" ","pages":""},"PeriodicalIF":2.5,"publicationDate":"2025-04-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143812972","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Histiocytic Disorders in Pediatric and Adult Patients.","authors":"Nancy Berliner, Barbara Degar","doi":"10.1016/j.hoc.2025.03.003","DOIUrl":"https://doi.org/10.1016/j.hoc.2025.03.003","url":null,"abstract":"","PeriodicalId":55060,"journal":{"name":"Hematology-Oncology Clinics of North America","volume":" ","pages":""},"PeriodicalIF":2.5,"publicationDate":"2025-03-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143756045","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Jerry C Lee, William T Johnson, Melissa Hines, Nirali N Shah
{"title":"Immune Effector Cell-associated Hemophagocytic Lymphohistiocytosis-like Syndrome (IEC-HS).","authors":"Jerry C Lee, William T Johnson, Melissa Hines, Nirali N Shah","doi":"10.1016/j.hoc.2025.02.005","DOIUrl":"https://doi.org/10.1016/j.hoc.2025.02.005","url":null,"abstract":"<p><p>Immune effector cell-associated hemophagocytic lymphohistiocytosis-like syndrome (IEC-HS) is a complication of adoptive cell therapy. Presenting with clinical manifestations of hyperinflammation and surrogate indicators of hyperinflammation such as elevations in serum ferritin and hepatic transaminases, decreasing cell counts, and hypofibrinogenemia, IEC-HS resembles primary and other forms of secondary hemophagocytic lymphohistiocytosis. Nonetheless, this is an iatrogenic complication resulting from the induction of hyperinflammatory pathways during T-cell-mediated anticancer targeting. Distinct from cytokine release syndrome, IEC-associated neurotoxicity syndrome, and IEC-associated hematotoxicity, IEC-HS can be life-threatening. Identification of IEC-HS, optimization of treatment strategies, and use of supportive care are critical to improving outcomes.</p>","PeriodicalId":55060,"journal":{"name":"Hematology-Oncology Clinics of North America","volume":" ","pages":""},"PeriodicalIF":2.5,"publicationDate":"2025-03-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143756046","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Clinical Characteristics and Treatment of Familial Hemophagocytic Lymphohistiocytosis.","authors":"Lauren K Meyer, Camille Keenan, Kim E Nichols","doi":"10.1016/j.hoc.2025.02.003","DOIUrl":"https://doi.org/10.1016/j.hoc.2025.02.003","url":null,"abstract":"<p><p>Familial hemophagocytic lymphohistiocytosis (fHLH) comprises a group of autosomal recessive disorders characterized by germline loss-of-function variants that negatively impact lymphocyte cytotoxicity. These disorders exhibit variable clinical presentations, most often in association with severe hyperinflammation. fHLH is diagnosed through clinical and laboratory assessments as well as genetic testing and immunologic assays. In the absence of therapy to control the hyperactive immune system, fHLH is generally fatal. Treatment has historically taken the form of cytotoxic chemotherapy and/or immunosuppressive therapy, although targeted inhibitors of inflammatory cytokines and their downstream signaling are increasingly being utilized. Definitive treatment requires allogeneic hematopoietic cell transplantation.</p>","PeriodicalId":55060,"journal":{"name":"Hematology-Oncology Clinics of North America","volume":" ","pages":""},"PeriodicalIF":2.5,"publicationDate":"2025-03-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143712197","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Macrophage Activation Syndrome: Not Just for Rheumatologists Anymore.","authors":"Sonia Iosim, Lauren A Henderson","doi":"10.1016/j.hoc.2025.02.004","DOIUrl":"https://doi.org/10.1016/j.hoc.2025.02.004","url":null,"abstract":"<p><strong>Synopsis: </strong>Macrophage activation syndrome (MAS) is a term that was originally used to describe a hyperinflammatory syndrome that developed in some patients with rheumatologic diseases. It is now clear that MAS and hemophagocytic lymphohistiocytosis (HLH) are defined by the same core pattern of clinical symptoms and share an underlying pathophysiology of impaired cytolytic activity and IFNγ-driven cytokine storm. Given that these disorders are highly related, lessons learned from the management of MAS can provide insights into effective approaches for HLH, particularly the strategy to employ anti-cytokine therapies early in the disease course.</p>","PeriodicalId":55060,"journal":{"name":"Hematology-Oncology Clinics of North America","volume":" ","pages":""},"PeriodicalIF":2.5,"publicationDate":"2025-03-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143712200","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Nitya Gulati, Erin Peckham-Gregory, D Williams Parsons, Carl E Allen
{"title":"Genomic Alterations in Langerhans Cell Histiocytosis.","authors":"Nitya Gulati, Erin Peckham-Gregory, D Williams Parsons, Carl E Allen","doi":"10.1016/j.hoc.2025.02.001","DOIUrl":"https://doi.org/10.1016/j.hoc.2025.02.001","url":null,"abstract":"<p><p>Langerhans cell histiocytosis (LCH) is a myeloid neoplastic disorder characterized by inflammatory lesions with clonal histiocytes. LCH is driven by activating mitogen-activated protein kinase (MAPK) pathway mutations. BRAF<sup>V600E</sup> is the most common mutation and is associated with more extensive disease at presentation and risks of front-line treatment failure, liver disease, and LCH-associated neurodegeneration. Genetic ancestry influences LCH with highest incidence in Hispanic populations. MAPK inhibitors are effective, but do not achieve cure in most cases. Clinical trials prospectively testing risk-stratification based on somatic mutation and/or detectable mutation in peripheral blood may improve outcomes for LCH patients.</p>","PeriodicalId":55060,"journal":{"name":"Hematology-Oncology Clinics of North America","volume":" ","pages":""},"PeriodicalIF":2.5,"publicationDate":"2025-03-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143712199","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Dukagjin M. Blakaj (Editor), Mauricio E. Gamez (Editor)
{"title":"Innovation and Emerging Technologies in Radiation Oncology","authors":"Dukagjin M. Blakaj (Editor), Mauricio E. Gamez (Editor)","doi":"10.1016/S0889-8588(25)00004-8","DOIUrl":"10.1016/S0889-8588(25)00004-8","url":null,"abstract":"","PeriodicalId":55060,"journal":{"name":"Hematology-Oncology Clinics of North America","volume":"39 2","pages":"Page i"},"PeriodicalIF":2.5,"publicationDate":"2025-02-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143487980","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Contributors","authors":"","doi":"10.1016/S0889-8588(25)00006-1","DOIUrl":"10.1016/S0889-8588(25)00006-1","url":null,"abstract":"","PeriodicalId":55060,"journal":{"name":"Hematology-Oncology Clinics of North America","volume":"39 2","pages":"Pages iii-vii"},"PeriodicalIF":2.5,"publicationDate":"2025-02-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143487982","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Copyright","authors":"","doi":"10.1016/S0889-8588(25)00005-X","DOIUrl":"10.1016/S0889-8588(25)00005-X","url":null,"abstract":"","PeriodicalId":55060,"journal":{"name":"Hematology-Oncology Clinics of North America","volume":"39 2","pages":"Page ii"},"PeriodicalIF":2.5,"publicationDate":"2025-02-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143487981","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Forthcoming Issues","authors":"","doi":"10.1016/S0889-8588(25)00008-5","DOIUrl":"10.1016/S0889-8588(25)00008-5","url":null,"abstract":"","PeriodicalId":55060,"journal":{"name":"Hematology-Oncology Clinics of North America","volume":"39 2","pages":"Page xiii"},"PeriodicalIF":2.5,"publicationDate":"2025-02-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143487983","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}