ArticleMedicine2017
EBV lymphoproliferative-associated disease and primary cardiac T-cell lymphoma in a STK4 deficient patient: A case report.
Article in Medicine, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 18 papers.
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Who cites it
18 citing papers in PubMed, 29 citations in OpenAlex.
- Durable response of primary cardiac lymphoma after autologous stem cell transplantation and sequential CAR-T therapy: a case report and literature review.Frontiers in immunology · 2025Review
- Case Report of Two Independent Moroccan Families with Syndromic Epidermodysplasia Verruciformis and STK4 Deficiency.Viruses · 2024Article
- Epidermodysplasia Verruciformis and Vδ2 γδ T-cell Expansion in STK4 Deficiency.Journal of clinical immunology · 2024Article
- Rubella virus chronic inflammatory disease and other unusual viral phenotypes in inborn errors of immunity.Immunological reviews · 2024Review
- Primary cardiac lymphoma: a clinicopathological study of 121 cases.Frontiers in oncology · 2024Article
- A unique STK4 mutation truncating only the C-terminal SARAH domain results in a mild clinical phenotype despite severe T cell lymphopenia: Case report.Frontiers in immunology · 2024Article
- Cardiac lymphoma presenting as bradyarrhythmia.HeartRhythm case reports · 2022Article
- STK4 deficiency and EBV-associated lymphoproliferative disorders, emphasis on histomorphology, and review of literature.Virchows Archiv : an international journal of pathology · 2022Review
- Case Report: Primary Cardiac T-Cell Lymphoma With Complete Atrio-Ventricular Block Diagnosed by Endomyocardial Biopsy.Frontiers in immunology · 2022Article
- Review
- When Actin is Not Actin' Like It Should: A New Category of Distinct Primary Immunodeficiency Disorders.Journal of innate immunity · 2021Review
- Lymphoma Predisposing Gene in an Extended Family: CD70 Signaling Defect.Journal of clinical immunology · 2020Article
- A Case of STK4 Deficiency with Complications Evoking Mycobacterial Infection.Journal of clinical immunology · 2020Article
- Human inborn errors of immunity to herpes viruses.Current opinion in immunology · 2020Review
- A case report of sinusoidal diffuse large B-cell lymphoma in a STK4 deficient patient.Medicine · 2020Article
- A Novel STK4 Mutation Presenting with Juvenile Idiopathic Arthritis and Epidermodysplasia Verruciformis.Journal of clinical immunology · 2019Article
- EBV Negative Lymphoma and Autoimmune Lymphoproliferative Syndrome Like Phenotype Extend the Clinical Spectrum of Primary Immunodeficiency Caused by STK4 Deficiency.Frontiers in immunology · 2018Article
- Primary cardiac lymphoma mimicking atrial myxoma in an HIV and EBV-positive patient: a case report.International journal of clinical and experimental pathology · 2018Article
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Authors and funding
9 authors at 4 institutions in 4 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
rationalePrimary cardiac lymphoma (PLC) is an extremely uncommon malignancy. PCL is more common in secondary immunodeficient patients. In this report, we describe a unique case of PLC who had been diagnosed as a STK4 deficient patient. This case is the first Primary immunodeficiency (PID) patient developing PCL in the world. PATIENT CONCERNS: An eleven-year-old girl, a known case of PID, was referred to the pediatric cardiology department because of chest pain and dyspnea. Her CXR revealed cardiomegaly without mediastinal involvement and the echocardiography showed a mild pericardial effusion and cystic-shape echogenic masses. DIAGNOSES: After a period of missed follow up, she presented with respiratory distress following with syncope at the clinic because of a pressure effect of a large mass on the right ventricular outflow tract (RVOT) .An emergency operation was done for debulking of the tumors and resolving of RVOT obstruction. Biopsy and immunohistochemical staining was revealing "T-cell lymphoma", non-Hodgkin's type.
interventionsChemotherapy was done with cyclophosphamide, methotrexate, adriamycine, vincristine, hydrocortisone and allopurinol. OUTCOMES: The tumors shrank after chemotherapy initiation and she stayed stable for almost one month. Finally, she developed sever thrombocytopenia during her chemotherapy and died because of lung hemorrhage two months after her operation. LESSONS: Although PCL is very rare, it must be considered in the differential diagnosis of intracardiac mass or refractory pericardial effusions, especially in PIDs which are widely known for developing EBV-associated diseases such as lymphoma.
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