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Intravascular Large B Cell Lymphoma

Chris Larsen, MD renal pathologist at arkana laboratories
By Chris Larsen, MD

Nov 02, 2017

Intravascular Large B Cell Lymphoma, stained positive for CD20
8001 MicroFIRECamera0 8002 LHH513018 8003 9.96 8004 ms 8005 1.0000 8006 1.0905 8007 1.0000 8008 1.3543 8009 Off 8009 Off 800a 24 800b 1600 800c 1200 800d 0 800d 0 800e 0 800f 0 8010 0 8011 Last Used 8012 1 8013 1.000000 8014 mm 8015 0 8016 0 8017 0 8018 0 8019 0 8019 0 801a 0 801b 50 801c 50 801d 1.0000 801e 0 801f 0 8020 0 8021 0 8022 0 8023 0 8024 0 8025 0 8026 0 8027 0 8028 0 8029 0 802a 0 802b 0 802c 0 802d 0 802e 255 802f 255 8030 255 8031 255 8032 1.4 8033 1.4 8034 1.4 8035 1.4 8036 1.4 8037 1.4 8038 8 8039 0 803a 2008/09/24 12:06:19:161 803b 2008/09/24 12:06:19:208 803c 2008/09/24 12:06:19:255 803d

A 75-year-old woman presented for her annual checkup with no complaints. She had a past medical history of hypertension and her blood pressure at this visit was 160/80. Laboratory findings were significant for an elevated serum creatinine at 2.1 mg/dL (baseline 1.0 mg/dL) as well as proteinuria of 855 mg/24 hr. All serologies, including proteinase-3, myeloperoxidase, and anti-nuclear antibodies were negative. A kidney biopsy was performed to evaluate the decreased kidney function and proteinuria. It revealed a total of five glomeruli were present, three of which showed a global endocapillary proliferation of large atypical cells with frequent mitotic figures (Photomicrographs A and B). These atypical cells were isolated to the glomeruli and stained positive for CD20. The remaining glomeruli did not contain any proliferative changes. Electron microscopy and immunofluorescence were both negative for immune deposits.

This is a rare subtype of extranodal diffuse large B cell lymphoma that has been known in the past by several different names and was originally thought to be a proliferation of endothelial cells; however, it is currently classified by the WHO as “intravascular large B-cell lymphoma” (IVBCL). IVLBCL most frequently affects adults over 60 and is typically widely disseminated in the small vessels of many organs at presentation. It most often presents without tumor masses or lymphadenopathy and lymphoma cells are usually not seen in peripheral blood smears. Clinical symptoms are highly variable and usually result from the occlusion of small vessels. The vast majority of cases are diagnosed in the bone marrow, liver, spleen, or skin. However, there are case reports of the diagnosis being made by kidney biopsy. In these cases, it usually manifests with slowly progressive renal failure and proteinuria which is frequently in the nephrotic range.

 

 

 

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