دورية أكاديمية

T Cell Histiocyte Rich Large B Cell Lymphoma Presenting as Hemophagocytic Lymphohistiocytosis: An Uncommon Presentation of a Rare Disease

التفاصيل البيبلوغرافية
العنوان: T Cell Histiocyte Rich Large B Cell Lymphoma Presenting as Hemophagocytic Lymphohistiocytosis: An Uncommon Presentation of a Rare Disease
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
الوصف
تدمد:20906706
20906714
DOI:10.1155/2017/6428461