دورية أكاديمية
T Cell Histiocyte Rich Large B Cell Lymphoma Presenting as Hemophagocytic Lymphohistiocytosis: An Uncommon Presentation of a Rare Disease
العنوان: | T Cell Histiocyte Rich Large B Cell Lymphoma Presenting as Hemophagocytic Lymphohistiocytosis: An Uncommon Presentation of a Rare Disease |
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المؤلفون: | Uroosa Ibrahim, Gwenalyn Garcia, Amina Saqib, Shafinaz Hussein, Qun Dai |
المصدر: | Case Reports in Oncological Medicine, Vol 2017 (2017) |
بيانات النشر: | Hindawi Limited, 2017. |
سنة النشر: | 2017 |
المجموعة: | LCC:Neoplasms. Tumors. Oncology. Including cancer and carcinogens |
مصطلحات موضوعية: | Neoplasms. Tumors. Oncology. Including cancer and carcinogens, RC254-282 |
الوصف: | T cell histiocyte rich large B cell lymphoma (THRLBCL) is a rare subtype of non-Hodgkin’s lymphoma characterized by malignant B cells with reactive T lymphocytes. The pathophysiology is thought to involve cytokine-mediated evasion of T cell immune response by malignant B cells. It usually presents at an advanced stage with extranodal involvement. An extremely unusual manifestation of the disease is hemophagocytic lymphohistiocytosis (HLH) which is a hyperinflammatory disorder. We present a case of a 43-year-old male who presented with recurrent fever and recent radiologic imaging showing splenomegaly and right inguinal lymphadenopathy. On presentation, he had a fever of 105°F. Laboratory work-up was consistent with pancytopenia, elevated lactate dehydrogenase, elevated D-dimer, and a ferritin of 24,247 ng/mL. The patient was started on steroid therapy. An excisional biopsy of the right inguinal lymph node was consistent with a diagnosis of THRLBCL and the patient subsequently received six cycles of chemotherapy with R-CHOP (Rituximab, Cyclophosphamide, Doxorubicin, Vincristine, and Prednisone) after which a PET-CT scan showed no evidence of biologically active disease and ferritin was down to 822 ng/mL. We discuss the clinical manifestations and diagnostic and therapeutic considerations of this rare disease along with a review of reported cases in the literature. |
نوع الوثيقة: | article |
وصف الملف: | electronic resource |
اللغة: | English |
تدمد: | 2090-6706 2090-6714 |
Relation: | https://doaj.org/toc/2090-6706; https://doaj.org/toc/2090-6714 |
DOI: | 10.1155/2017/6428461 |
URL الوصول: | https://doaj.org/article/3ffedbd19bc74cf58ebe2d6c2c478642 |
رقم الأكسشن: | edsdoj.3ffedbd19bc74cf58ebe2d6c2c478642 |
قاعدة البيانات: | Directory of Open Access Journals |
تدمد: | 20906706 20906714 |
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DOI: | 10.1155/2017/6428461 |