A Case of De Novo CD5+ Disseminated Intravascular Large B-Cell Lymphoma Presenting as Multiorgan Failure
Intravascular large B-cell lymphoma is an extremely rare extranodal lymphoma that proliferates in the lumen of the blood vessels while sparing the organ parenchyma. It usually presents with CNS and skin involvement. A 65-year-old Caucasian female presented with fevers and chills of 3-4 months’ durat...
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| Format: | Article |
| Language: | English |
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Wiley
2016-01-01
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| Series: | Case Reports in Hematology |
| Online Access: | http://dx.doi.org/10.1155/2016/6239416 |
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| author | Daulath Singh Devika Kapuria Suparna Nanua Rakesh Gaur |
| author_facet | Daulath Singh Devika Kapuria Suparna Nanua Rakesh Gaur |
| author_sort | Daulath Singh |
| collection | DOAJ |
| description | Intravascular large B-cell lymphoma is an extremely rare extranodal lymphoma that proliferates in the lumen of the blood vessels while sparing the organ parenchyma. It usually presents with CNS and skin involvement. A 65-year-old Caucasian female presented with fevers and chills of 3-4 months’ duration. Bone marrow biopsy done 3 months prior showed no significant myelodysplasia or lymphoid aggregates. The patient later died due to multiorgan failure. A bone marrow biopsy showed 20–30% CD5+ B cells consistent with infiltrative large B-cell lymphoma. An autopsy performed revealed diffuse intravascular invasion by lymphoma cells. Multiorgan involvement by intravascular B-cell lymphoma is very rare. Based on our literature review and to the best of our knowledge, there are only 5 case reports describing the presentation of this lymphoma with multiorgan failure. The immunophenotypic studies performed revealed that our patient had de novo CD5+ intravascular large B-cell lymphoma which is known to be aggressive with very poor prognosis. Although it is an extremely rare lymphoma, it should be considered as a potential cause of multiorgan failure when no other cause has been identified. A prompt tissue diagnosis and high-dose chemotherapy followed by ASCT can sometimes achieve remission. |
| format | Article |
| id | doaj-art-2fb707778ec247b7be0c9c4c616e4b06 |
| institution | Kabale University |
| issn | 2090-6560 2090-6579 |
| language | English |
| publishDate | 2016-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Case Reports in Hematology |
| spelling | doaj-art-2fb707778ec247b7be0c9c4c616e4b062025-02-03T06:04:59ZengWileyCase Reports in Hematology2090-65602090-65792016-01-01201610.1155/2016/62394166239416A Case of De Novo CD5+ Disseminated Intravascular Large B-Cell Lymphoma Presenting as Multiorgan FailureDaulath Singh0Devika Kapuria1Suparna Nanua2Rakesh Gaur3Department of Internal Medicine, The University of Missouri Kansas City School of Medicine, 2301 Holmes Street, Kansas City, MO 64108, USADepartment of Internal Medicine, The University of Missouri Kansas City School of Medicine, 2301 Holmes Street, Kansas City, MO 64108, USADepartment of Laboratory Medicine, Saint Luke’s Hospital of Kansas City, 4401 Wornall Road, Kansas City, MO 64111, USADepartment of Hematology/Oncology, Saint Luke’s Hospital of Kansas City, Saint Luke’s Cancer Specialists, 4321 Washington Street, No. 4000, Kansas City, MO 64111, USAIntravascular large B-cell lymphoma is an extremely rare extranodal lymphoma that proliferates in the lumen of the blood vessels while sparing the organ parenchyma. It usually presents with CNS and skin involvement. A 65-year-old Caucasian female presented with fevers and chills of 3-4 months’ duration. Bone marrow biopsy done 3 months prior showed no significant myelodysplasia or lymphoid aggregates. The patient later died due to multiorgan failure. A bone marrow biopsy showed 20–30% CD5+ B cells consistent with infiltrative large B-cell lymphoma. An autopsy performed revealed diffuse intravascular invasion by lymphoma cells. Multiorgan involvement by intravascular B-cell lymphoma is very rare. Based on our literature review and to the best of our knowledge, there are only 5 case reports describing the presentation of this lymphoma with multiorgan failure. The immunophenotypic studies performed revealed that our patient had de novo CD5+ intravascular large B-cell lymphoma which is known to be aggressive with very poor prognosis. Although it is an extremely rare lymphoma, it should be considered as a potential cause of multiorgan failure when no other cause has been identified. A prompt tissue diagnosis and high-dose chemotherapy followed by ASCT can sometimes achieve remission.http://dx.doi.org/10.1155/2016/6239416 |
| spellingShingle | Daulath Singh Devika Kapuria Suparna Nanua Rakesh Gaur A Case of De Novo CD5+ Disseminated Intravascular Large B-Cell Lymphoma Presenting as Multiorgan Failure Case Reports in Hematology |
| title | A Case of De Novo CD5+ Disseminated Intravascular Large B-Cell Lymphoma Presenting as Multiorgan Failure |
| title_full | A Case of De Novo CD5+ Disseminated Intravascular Large B-Cell Lymphoma Presenting as Multiorgan Failure |
| title_fullStr | A Case of De Novo CD5+ Disseminated Intravascular Large B-Cell Lymphoma Presenting as Multiorgan Failure |
| title_full_unstemmed | A Case of De Novo CD5+ Disseminated Intravascular Large B-Cell Lymphoma Presenting as Multiorgan Failure |
| title_short | A Case of De Novo CD5+ Disseminated Intravascular Large B-Cell Lymphoma Presenting as Multiorgan Failure |
| title_sort | case of de novo cd5 disseminated intravascular large b cell lymphoma presenting as multiorgan failure |
| url | http://dx.doi.org/10.1155/2016/6239416 |
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