Intravascular large B‐cell lymphoma presenting as Richter's syndrome with cerebral involvement in a patient with chronic lymphocytic leukemia

Puckrin, Robert; Pop, Paula; Ghorab, Zeina; Keith, Julia; Chodirker, Lisa; Lin, Yulia; Callum, Jeannie

doi:10.1002/ccr3.1087

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“…In general, larger blood vessels are not involved, although cases have been reported that showed involvement as a tumour thrombus or a mass attached to large vessel walls. 31 Emergence of IVLBCL following small B-cell lymphoma or diffuse large B-cell lymphoma has been reported; 32,33 whether these cases represent transformation or de novo disease is not clear and these cases are not further discussed here.…”

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confidence: 99%

Diffuse large B-cell lymphoma variants: an update

et al. 2020

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Section: Definitionmentioning

confidence: 99%

Diffuse large B-cell lymphoma variants: an update

et al. 2020

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Pure intravascular recurrence of CD5-positive diffuse large B-cell lymphoma primarily arising from the nasal cavities

et al. 2018

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BackgroundCD5-positive diffuse large B-cell lymphoma (DLBCL) and intravascular large B-cell lymphoma (IVL) are recognized as rare subsets of large B-cell lymphoma with poor prognosis. These two categories have similar clinicopathological features suggesting that they might overlap.Case presentationWe present a case of a 72-year-old man with submental tumors. Positron emission tomography/computed tomography (PET/CT) showed tumors in the nasal and paranasal region and multiple submental and jugular swollen lymph nodes with abnormal uptake of 18F-fluorodeoxyglucose (FDG). Histology of biopsy from nasal tumors showed diffuse infiltration of large lymphoid cells, which showed positive expressions for CD20, CD79a, CD5 and negative for CD3 on immunohistochemistry. Thus, a CD5-positive DLBCL was diagnosed. After administration of 8 cycles of R-THPCOP (rituximab, pirarubicin, cyclophosphamide, vincristine and prednisolone), complete remission was achieved. Eight months after the first chemotherapy dose, local recurrence occurred. After salvage chemotherapy, nasal and paranasal tumors and lymph node swelling disappeared on PET/CT images, although the patient suffered from respiratory disturbance. A random skin biopsy revealed IVL, which was consistent with intravascular recurrence of CD5-positive DLBCL. Bone marrow smears showed hemophagocytosis.ConclusionWe present a rare case of primary CD5-positive DLBCL that relapsed as pure IVL after chemotherapy. Our case suggests that CD5-positive DLBCL is closely related to IVL.

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Intravascular large B‐cell lymphoma presenting as Richter's syndrome with cerebral involvement in a patient with chronic lymphocytic leukemia

Cited by 2 publications

References 35 publications

Diffuse large B-cell lymphoma variants: an update

Diffuse large B-cell lymphoma variants: an update

Pure intravascular recurrence of CD5-positive diffuse large B-cell lymphoma primarily arising from the nasal cavities

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