CD5-Positive Intravascular Large B-Cell Lymphoma in a Patient with Wilson’s Disease: Case Report and Review of the Literature
Intravascular large B-cell lymphoma (IVLBCL) is a rare extra-nodal B-cell lymphoma that proliferates within small/intermediate blood vessels and capillaries while sparing large blood vessels and organ parenchyma. Clinical presentation is highly variable and may include B symptoms, neurological defic...
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| Format: | Article |
| Language: | English |
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Wiley
2018-01-01
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| Series: | Case Reports in Pathology |
| Online Access: | http://dx.doi.org/10.1155/2018/5140586 |
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| author | Neha Gupta Chrystalle Katte Carreon Filiz Sen Peter Farmer Xinmin Zhang Silvat Sheikh-Fayyaz Nina Haghi |
| author_facet | Neha Gupta Chrystalle Katte Carreon Filiz Sen Peter Farmer Xinmin Zhang Silvat Sheikh-Fayyaz Nina Haghi |
| author_sort | Neha Gupta |
| collection | DOAJ |
| description | Intravascular large B-cell lymphoma (IVLBCL) is a rare extra-nodal B-cell lymphoma that proliferates within small/intermediate blood vessels and capillaries while sparing large blood vessels and organ parenchyma. Clinical presentation is highly variable and may include B symptoms, neurological deficits, and/or cutaneous findings. The diagnosis of IVLBCL is difficult due to multiorgan involvement and nonspecific symptoms. We describe the case of a 68-year-old male who presented with progressive weakness, confusion, and falls. He had a past medical history of liver cirrhosis secondary to Wilson’s disease. Physical exam and laboratory results revealed a lethargic man with jaundice, hepatic encephalopathy, and abnormal liver/kidney function tests. He expired after a short hospital course in the setting of hepatic and renal failure. Postmortem examination revealed large neoplastic lymphoid cells involving multiple organ blood vessels; however skin and neurologic involvement was absent. The neoplastic cells demonstrated B-cells positive for CD5, rendering a diagnosis of IVLBCL. Our case represents the occurrence of IVLBCL with CD5-positivity in a patient with Wilson’s disease, diagnosed at autopsy demonstrating the challenging nature of diagnosing IVLBCL. |
| format | Article |
| id | doaj-art-76660e87981a4e91b5fb89886380ea35 |
| institution | OA Journals |
| issn | 2090-6781 2090-679X |
| language | English |
| publishDate | 2018-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Case Reports in Pathology |
| spelling | doaj-art-76660e87981a4e91b5fb89886380ea352025-08-20T02:07:13ZengWileyCase Reports in Pathology2090-67812090-679X2018-01-01201810.1155/2018/51405865140586CD5-Positive Intravascular Large B-Cell Lymphoma in a Patient with Wilson’s Disease: Case Report and Review of the LiteratureNeha Gupta0Chrystalle Katte Carreon1Filiz Sen2Peter Farmer3Xinmin Zhang4Silvat Sheikh-Fayyaz5Nina Haghi6Department of Pathology and Laboratory Medicine, Donald and Barbara Zucker School of Medicine at Hofstra/Northwell, 6 Ohio Drive, Suite 202, Lake Success, New York, 11042, USADepartment of Pathology, Boston Children’s Hospital/Harvard Medical School, 300 Longwood Ave. Boston, MA 02115, USADepartment of Pathology, Memorial Sloan Kettering Cancer Center, 1275 York Avenue, New York, 10065, USADepartment of Pathology and Laboratory Medicine, Donald and Barbara Zucker School of Medicine at Hofstra/Northwell, 6 Ohio Drive, Suite 202, Lake Success, New York, 11042, USADepartment of Pathology and Laboratory Medicine, Donald and Barbara Zucker School of Medicine at Hofstra/Northwell, 6 Ohio Drive, Suite 202, Lake Success, New York, 11042, USADepartment of Pathology and Laboratory Medicine, Donald and Barbara Zucker School of Medicine at Hofstra/Northwell, 6 Ohio Drive, Suite 202, Lake Success, New York, 11042, USADepartment of Pathology and Laboratory Medicine, Donald and Barbara Zucker School of Medicine at Hofstra/Northwell, 6 Ohio Drive, Suite 202, Lake Success, New York, 11042, USAIntravascular large B-cell lymphoma (IVLBCL) is a rare extra-nodal B-cell lymphoma that proliferates within small/intermediate blood vessels and capillaries while sparing large blood vessels and organ parenchyma. Clinical presentation is highly variable and may include B symptoms, neurological deficits, and/or cutaneous findings. The diagnosis of IVLBCL is difficult due to multiorgan involvement and nonspecific symptoms. We describe the case of a 68-year-old male who presented with progressive weakness, confusion, and falls. He had a past medical history of liver cirrhosis secondary to Wilson’s disease. Physical exam and laboratory results revealed a lethargic man with jaundice, hepatic encephalopathy, and abnormal liver/kidney function tests. He expired after a short hospital course in the setting of hepatic and renal failure. Postmortem examination revealed large neoplastic lymphoid cells involving multiple organ blood vessels; however skin and neurologic involvement was absent. The neoplastic cells demonstrated B-cells positive for CD5, rendering a diagnosis of IVLBCL. Our case represents the occurrence of IVLBCL with CD5-positivity in a patient with Wilson’s disease, diagnosed at autopsy demonstrating the challenging nature of diagnosing IVLBCL.http://dx.doi.org/10.1155/2018/5140586 |
| spellingShingle | Neha Gupta Chrystalle Katte Carreon Filiz Sen Peter Farmer Xinmin Zhang Silvat Sheikh-Fayyaz Nina Haghi CD5-Positive Intravascular Large B-Cell Lymphoma in a Patient with Wilson’s Disease: Case Report and Review of the Literature Case Reports in Pathology |
| title | CD5-Positive Intravascular Large B-Cell Lymphoma in a Patient with Wilson’s Disease: Case Report and Review of the Literature |
| title_full | CD5-Positive Intravascular Large B-Cell Lymphoma in a Patient with Wilson’s Disease: Case Report and Review of the Literature |
| title_fullStr | CD5-Positive Intravascular Large B-Cell Lymphoma in a Patient with Wilson’s Disease: Case Report and Review of the Literature |
| title_full_unstemmed | CD5-Positive Intravascular Large B-Cell Lymphoma in a Patient with Wilson’s Disease: Case Report and Review of the Literature |
| title_short | CD5-Positive Intravascular Large B-Cell Lymphoma in a Patient with Wilson’s Disease: Case Report and Review of the Literature |
| title_sort | cd5 positive intravascular large b cell lymphoma in a patient with wilson s disease case report and review of the literature |
| url | http://dx.doi.org/10.1155/2018/5140586 |
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