T Cell Histiocyte Rich Large B Cell Lymphoma Presenting as Hemophagocytic Lymphohistiocytosis: An Uncommon Presentation of a Rare Disease
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 a...
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| Format: | Article |
| Language: | English |
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Wiley
2017-01-01
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| Series: | Case Reports in Oncological Medicine |
| Online Access: | http://dx.doi.org/10.1155/2017/6428461 |
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| author | Uroosa Ibrahim Gwenalyn Garcia Amina Saqib Shafinaz Hussein Qun Dai |
| author_facet | Uroosa Ibrahim Gwenalyn Garcia Amina Saqib Shafinaz Hussein Qun Dai |
| author_sort | Uroosa Ibrahim |
| collection | DOAJ |
| description | 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. |
| format | Article |
| id | doaj-art-3ffedbd19bc74cf58ebe2d6c2c478642 |
| institution | Kabale University |
| issn | 2090-6706 2090-6714 |
| language | English |
| publishDate | 2017-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Case Reports in Oncological Medicine |
| spelling | doaj-art-3ffedbd19bc74cf58ebe2d6c2c4786422025-02-03T01:31:04ZengWileyCase Reports in Oncological Medicine2090-67062090-67142017-01-01201710.1155/2017/64284616428461T Cell Histiocyte Rich Large B Cell Lymphoma Presenting as Hemophagocytic Lymphohistiocytosis: An Uncommon Presentation of a Rare DiseaseUroosa Ibrahim0Gwenalyn Garcia1Amina Saqib2Shafinaz Hussein3Qun Dai4Department of Hematology/Oncology, Staten Island University Hospital, 475 Seaview Avenue, Staten Island, NY 10305, USADepartment of Hematology/Oncology, Staten Island University Hospital, 475 Seaview Avenue, Staten Island, NY 10305, USADepartment of Pulmonary/Critical Care, Staten Island University Hospital, 475 Seaview Avenue, Staten Island, NY 10305, USADepartment of Pathology, Staten Island University Hospital, 475 Seaview Avenue, Staten Island, NY 10305, USADepartment of Hematology/Oncology, Staten Island University Hospital, 475 Seaview Avenue, Staten Island, NY 10305, USAT 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.http://dx.doi.org/10.1155/2017/6428461 |
| spellingShingle | Uroosa Ibrahim Gwenalyn Garcia Amina Saqib Shafinaz Hussein Qun Dai T Cell Histiocyte Rich Large B Cell Lymphoma Presenting as Hemophagocytic Lymphohistiocytosis: An Uncommon Presentation of a Rare Disease Case Reports in Oncological Medicine |
| title | T Cell Histiocyte Rich Large B Cell Lymphoma Presenting as Hemophagocytic Lymphohistiocytosis: An Uncommon Presentation of a Rare Disease |
| title_full | T Cell Histiocyte Rich Large B Cell Lymphoma Presenting as Hemophagocytic Lymphohistiocytosis: An Uncommon Presentation of a Rare Disease |
| title_fullStr | T Cell Histiocyte Rich Large B Cell Lymphoma Presenting as Hemophagocytic Lymphohistiocytosis: An Uncommon Presentation of a Rare Disease |
| title_full_unstemmed | T Cell Histiocyte Rich Large B Cell Lymphoma Presenting as Hemophagocytic Lymphohistiocytosis: An Uncommon Presentation of a Rare Disease |
| title_short | T Cell Histiocyte Rich Large B Cell Lymphoma Presenting as Hemophagocytic Lymphohistiocytosis: An Uncommon Presentation of a Rare Disease |
| title_sort | t cell histiocyte rich large b cell lymphoma presenting as hemophagocytic lymphohistiocytosis an uncommon presentation of a rare disease |
| url | http://dx.doi.org/10.1155/2017/6428461 |
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