ArticleThe American journal of surgical pathology2010
Development of disseminated histiocytic sarcoma in a patient with autoimmune lymphoproliferative syndrome and associated Rosai-Dorfman disease.
Article in The American journal of surgical pathology, 2010. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 20 papers, 1 of them a synthesis that pooled it.
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Who cites it
20 citing papers in PubMed, 1 synthesis or guideline pooled it, 56 citations in OpenAlex.
- Cancer Trends in Inborn Errors of Immunity: A Systematic Review and Meta-Analysis.Journal of clinical immunology · 2024Pooled it
- Natural history of autoimmune lymphoproliferative syndrome associated with FAS gene mutations.Blood · 2014Trial
- Histiocytic Sarcoma of Palatine Tonsil in a Pediatric Patient: A Case Report of a Rare and Aggressive Malignancy.Clinical case reports · 2026Article
- Atypical lymphoproliferations associated with germline genetic variants: a report of the 2024 EA4HP/SH lymphoma workshop.Virchows Archiv : an international journal of pathology · 2025Review
- Retrospective nationwide survey of pediatric RDD in Japan: a high prevalence of mutations in the kinase pathway genes.International journal of hematology · 2025Article
- Histiocytic sarcoma involving multiple abdominal sites: A rare case with KRAS mutation and response to ICE chemotherapy.Radiology case reports · 2025Article
- Apoptotic cell death in disease-Current understanding of the NCCD 2023.Cell death and differentiation · 2023Review
- Rosai-Dorfman disease in the central nervous system with two isolated lesions originated from a single clone: a case report.BMC neurology · 2021Article
- NRAS Mutations May Be Involved in the Pathogenesis of Cutaneous Rosai Dorfman Disease: A Pilot Study.Biology · 2021Article
- Case Report: Eighteen Month Relapse- Free Survival Following Radical Multidisciplinary Oncological Treatment in a 68-Year-Old Male Patient With Histiocytic Sarcoma.Frontiers in oncology · 2021Article
- Pediatric multifocal histiocytic sarcoma- a fatal diagnosis not to miss!Autopsy & case reports · 2020Article
- Is Rosai-Dorfman disease a reactve process? Detection of a MAP2K1 L115V mutation in a case of Rosai-Dorfman disease.Virchows Archiv : an international journal of pathology · 2017Article
- Bone marrow findings in autoimmune lymphoproliferative syndrome with germline FAS mutation.Haematologica · 2017Article
- Primary/Congenital Immunodeficiency: 2015 SH/EAHP Workshop Report-Part 5.American journal of clinical pathology · 2017Article
- Rosai-Dorfman Disease Harboring an Activating KRAS K117N Missense Mutation.Head and neck pathology · 2016Article
- Rosai-Dorfman disease: a report of eight cases in a tertiary care center and a review of the literature.Brazilian journal of medical and biological research = Revista brasileira de pesquisas medicas e biologica · 2015Article
- Autoimmune lymphoproliferative syndrome: an update and review of the literature.Current allergy and asthma reports · 2014Review
- Autoimmune Lymphoproliferative Syndrome and Epstein-Barr Virus-Associated Lymphoma: An Adjunctive Diagnostic Role for Monitoring EBV Viremia?Case reports in immunology · 2013Article
- Review
- Histiocytic sarcoma with two immunohistopathologically distinct populations.International journal of hematology · 2010Article
Corrections and comments
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Authors and funding
5 authors at 5 institutions in 2 countries.
Funding
Abstract
Patients with autoimmune lymphoproliferative syndrome (ALPS) have defective lymphocyte apoptosis with increased risk for lymphoid malignancies. Herein, we report a patient with ALPS who developed histiocytic sarcoma in a background of sinus histiocytosis and massive lymphadenopathy or Rosai- Dorfman disease. This patient had documented ALPS type Ia with a germline missense mutation in exon 9 of the TNFRSF6 gene (973 A>T, D244V) encoding Fas (CD95/Apo-1). He presented at 10 months with hepatosplenomegaly and autoimmune hemolytic anemia and was diagnosed with ALPS. At the age of 6 (1/2) years, he developed classic Hodgkin lymphoma which was treated using standard chemotherapy. Two years later, a biopsy of a positron emission tomography-positive axillary node showed features of ALPS and focal involvement by sinus histiocytosis and massive lymphadenopathy. Thereafter, the patient continued to have continued lymphadenopathy and progressive splenomegaly, leading to exploratory surgery at the age of 13 years for suspicion of lymphoma. Para-abdominal nodes revealed sheets of malignant- looking histiocytes with increased mitotic activity and areas of necrosis, indicative of histiocytic sarcoma. Spleen and lymph nodes also showed involvement by Rosai-Dorfman disease. Both components had an identical phenotype of S-100+/CD68+/ CD163+. The occurrence of malignancies involving 2 separate hematopoietic lineages in ALPS has not been reported earlier. Given the central role of defective Fas signaling in ALPS, histiocytes may be yet another lineage at risk for neoplastic transformation secondary to a block in apoptosis.
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