Asian-variant intravascular lymphoma in the African race
Intravascular large B-cell lymphoma (IVLBCL) is an exceptionally rare form of non- Hodgkin lymphoma (NHL) distinguished by the preferential growth of neoplastic cells within blood vessel lumen. Challenging to detect and deemed disseminated at diagnosis, this condition is characterized by a highly ag...
| Main Authors: | , , , |
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| Format: | Article |
| Language: | English |
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SAGE Publishing
2012-01-01
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| Series: | Rare Tumors |
| Subjects: | |
| Online Access: | http://www.pagepress.org/journals/index.php/rt/article/view/3388 |
| _version_ | 1818476634865401856 |
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| author | Brian Palen Ruben Mesa Holly Geyer Nina Karlin |
| author_facet | Brian Palen Ruben Mesa Holly Geyer Nina Karlin |
| author_sort | Brian Palen |
| collection | DOAJ |
| description | Intravascular large B-cell lymphoma (IVLBCL) is an exceptionally rare form of non- Hodgkin lymphoma (NHL) distinguished by the preferential growth of neoplastic cells within blood vessel lumen. Challenging to detect and deemed disseminated at diagnosis, this condition is characterized by a highly aggressive, inconspicuous course with a high mortality rate. We describe the case of a 48 year-old African-American female presenting with a two month history of low-grade fevers and malaise. Laboratory data was notable for anemia, thrombocytopenia, elevated liver function tests, and hematuria. An extensive workup for infectious, rheumatologic and malignant causes was negative. Her symptoms progressed and within two weeks, she was admitted for disseminated intravascular coagulation (DIC). Her course was complicated by diffuse pulmonary hemorrhage and ultimately, care was withdrawn. Autopsy identified widespread CD-20 positive intravascular large B-cell lymphoma with significant hepatosplenic involvement, characteristic of the Asian variant IVLBCL. This case uniquely highlights development of the Asian variant IVLBVL in a previously undescribed race. Identified by its intraluminal vascular growth pattern, IVLBCL generally spares lymphatic channels. Diagnosis and differentiation of this condition from other hematological malignancies via skin, visceral and bone marrow biopsy is imperative as anthracycline-containing chemotherapies may significantly improve clinical outcomes. This article outlines the common presentation, natural course, and treatment options of IVLBCL, along with the histopathology, immunohistochemistry, and chromosomal aberrations common to this condition. |
| first_indexed | 2024-12-10T09:28:23Z |
| format | Article |
| id | doaj.art-b3fa6af0f9984c809e4048f782357cee |
| institution | Directory Open Access Journal |
| issn | 2036-3605 2036-3613 |
| language | English |
| last_indexed | 2024-12-10T09:28:23Z |
| publishDate | 2012-01-01 |
| publisher | SAGE Publishing |
| record_format | Article |
| series | Rare Tumors |
| spelling | doaj.art-b3fa6af0f9984c809e4048f782357cee2022-12-22T01:54:27ZengSAGE PublishingRare Tumors2036-36052036-36132012-01-0141e10e1010.4081/rt.2012.e10Asian-variant intravascular lymphoma in the African raceBrian PalenRuben MesaHolly GeyerNina KarlinIntravascular large B-cell lymphoma (IVLBCL) is an exceptionally rare form of non- Hodgkin lymphoma (NHL) distinguished by the preferential growth of neoplastic cells within blood vessel lumen. Challenging to detect and deemed disseminated at diagnosis, this condition is characterized by a highly aggressive, inconspicuous course with a high mortality rate. We describe the case of a 48 year-old African-American female presenting with a two month history of low-grade fevers and malaise. Laboratory data was notable for anemia, thrombocytopenia, elevated liver function tests, and hematuria. An extensive workup for infectious, rheumatologic and malignant causes was negative. Her symptoms progressed and within two weeks, she was admitted for disseminated intravascular coagulation (DIC). Her course was complicated by diffuse pulmonary hemorrhage and ultimately, care was withdrawn. Autopsy identified widespread CD-20 positive intravascular large B-cell lymphoma with significant hepatosplenic involvement, characteristic of the Asian variant IVLBCL. This case uniquely highlights development of the Asian variant IVLBVL in a previously undescribed race. Identified by its intraluminal vascular growth pattern, IVLBCL generally spares lymphatic channels. Diagnosis and differentiation of this condition from other hematological malignancies via skin, visceral and bone marrow biopsy is imperative as anthracycline-containing chemotherapies may significantly improve clinical outcomes. This article outlines the common presentation, natural course, and treatment options of IVLBCL, along with the histopathology, immunohistochemistry, and chromosomal aberrations common to this condition.http://www.pagepress.org/journals/index.php/rt/article/view/3388intravascular lymphoma, Asian variant, hemophagocytic syndrome, diffuse large Bcell lymphoma, chromosome abnormalities |
| spellingShingle | Brian Palen Ruben Mesa Holly Geyer Nina Karlin Asian-variant intravascular lymphoma in the African race Rare Tumors intravascular lymphoma, Asian variant, hemophagocytic syndrome, diffuse large Bcell lymphoma, chromosome abnormalities |
| title | Asian-variant intravascular lymphoma in the African race |
| title_full | Asian-variant intravascular lymphoma in the African race |
| title_fullStr | Asian-variant intravascular lymphoma in the African race |
| title_full_unstemmed | Asian-variant intravascular lymphoma in the African race |
| title_short | Asian-variant intravascular lymphoma in the African race |
| title_sort | asian variant intravascular lymphoma in the african race |
| topic | intravascular lymphoma, Asian variant, hemophagocytic syndrome, diffuse large Bcell lymphoma, chromosome abnormalities |
| url | http://www.pagepress.org/journals/index.php/rt/article/view/3388 |
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