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A Case of Spontaneously Improving Secondary Hemophagocytic Lymphohistiocytosis in an Adult Associated with T-Cell Histiocyte-Rich Large B-Cell Lymphoma.

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العنوان: A Case of Spontaneously Improving Secondary Hemophagocytic Lymphohistiocytosis in an Adult Associated with T-Cell Histiocyte-Rich Large B-Cell Lymphoma.
المؤلفون: Askin C; Department of Internal Medicine, San Antonio Military Medical Center, San Antonio, TX, USA., Burris A; Department of Pathology, San Antonio Military Medical Center, San Antonio, TX, USA., Layman C; Department of Hematology and Oncology, San Antonio Military Medical Center, San Antonio, TX, USA., Haney B; Department of Hematology and Oncology, San Antonio Military Medical Center, San Antonio, TX, USA., Hall J; Department of Pathology, San Antonio Military Medical Center, San Antonio, TX, USA.
المصدر: Case reports in oncological medicine [Case Rep Oncol Med] 2018 Feb 22; Vol. 2018, pp. 8069182. Date of Electronic Publication: 2018 Feb 22 (Print Publication: 2018).
نوع المنشور: Case Reports
اللغة: English
بيانات الدورية: Publisher: Hindawi Pub. Corp Country of Publication: United States NLM ID: 101581035 Publication Model: eCollection Cited Medium: Print ISSN: 2090-6706 (Print) NLM ISO Abbreviation: Case Rep Oncol Med Subsets: PubMed not MEDLINE
أسماء مطبوعة: Original Publication: New York, NY : Hindawi Pub. Corp.
مستخلص: Secondary hemophagocytic lymphohistiocytosis (HLH) in adults is a rare, often fatal syndrome characterized by widespread immune dysregulation. It is seen as a complication of infections, autoimmune diseases, and malignancies. Among the malignancy-related causes, aggressive T-cell or NK-cell neoplasms are most notable, while B-cell lymphomas are less commonly implicated. We present the case of a 32-year-old male transferred to our facility with concern for HLH. During the first week of his hospitalization, his diagnosis was confirmed and the patient demonstrated spontaneous improvement in his symptoms prompting us to delay therapy while searching for a primary cause. In the second week, the patient deteriorated, leading us to initiate steroid monotherapy in the absence of a cause for his HLH. Meanwhile, pathology results from an excisional lymph node biopsy confirmed a diagnosis of T-cell/histiocyte-rich large B-cell lymphoma (TCHRLBCL). Subsequently, we initiated therapy with dose-adjusted R-EPOCH. The patient achieved a complete remission of both HLH and TCHRLBCL as well as a complete return to his prior functional status. In our review of the literature, this represents only the second documented case of HLH associated with TCHRLBCL and the only documented case of an adult experiencing significant spontaneous recovery in this context.
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تواريخ الأحداث: Date Created: 20180424 Latest Revision: 20201001
رمز التحديث: 20240628
مُعرف محوري في PubMed: PMC5842713
DOI: 10.1155/2018/8069182
PMID: 29682376
قاعدة البيانات: MEDLINE
الوصف
تدمد:2090-6706
DOI:10.1155/2018/8069182