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Case Reports
. 2018 Apr 3:2018:9413015.
doi: 10.1155/2018/9413015. eCollection 2018.

Small Cell Variant of Intravascular Large B-Cell Lymphoma: Highlighting a Potentially Fatal and Easily Missed Diagnosis

Mahboubeh Rahmani  1 , Stephanie Halene  2 , Mina L Xu  3
Affiliations
Case Reports

Small Cell Variant of Intravascular Large B-Cell Lymphoma: Highlighting a Potentially Fatal and Easily Missed Diagnosis

Mahboubeh Rahmani et al. Biomed Res Int. .

Abstract

Context: Intravascular large B-cell lymphoma (IVLBCL) is a rare non-Hodgkin B-cell lymphoma with a poor prognosis. While typically described as comprising large atypical cells restricted to the lumina of small blood vessels, it can show variability in cell size.

Objective: To report the clinicopathologic features of the IVLBCL with small cell morphology and discuss the practical implications of our findings.

Design: We searched our archives for all IVLBCL diagnosed in our institution for the last 25 years (1992-2017). Slides were reviewed independently by two hematopathologists.

Results: We found a total of 11 cases of IVLBCL. Bone marrow, brain, lymph node, pericardium, small bowel, and fallopian tube and ovary were the organs in which the lymphoma was initially diagnosed. One of the cases initially diagnosed in the marrow showed intrasinusoidal involvement by a small cell lymphoma; the diagnosis was confirmed by random skin biopsies showing intravascular large cells with the same phenotype. Retrospective review of the liver on this case also showed the intrasinusoidal involvement by the disease consisting of small cells. In another case, IVLBCL that was initially diagnosed in a small bowel biopsy was retrospectively found in a breast biopsy, but with small cell morphology.

Conclusions: Our findings suggest that, in the presence of high clinical suspicion, IVLBCL should be high in the differential diagnosis when lymphoma is predominantly intravascular, even when the tumor cells are small. A timely diagnosis of this entity can be critical. Hence, awareness of a small cell variant of IVLBCL should be increased.

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Figures

Figure 1
Figure 1
(a) Liver biopsy showing mild steatosis and dilated sinusoids, H&E stain, 4x magnification. (b) Extensive intravascular involvement of the liver by small atypical cells, H&E stain, 40x magnification. (c) Small intrasinusoidal lymphocytes highlighted by CD20, immunohistochemistry stain, 20x magnification. (d) The marrow showed involvement by atypical cells mostly of small size lymphocytes with an intrasinusoidal pattern, H&E stain, 40x magnification. (e) Small intrasinusoidal lymphocytes highlighted by CD20, immunohistochemistry stain, 20x magnification. (f, g) Subcutaneous vessel showing intravascular large atypical cells highlighted by CD20 confirming the diagnosis of IVLBCL, H&E, and immunohistochemistry stains, 40x magnification. (h, i) Small intrasinusoidal lymphocytes in the marrow in comparison with intravascular large atypical cells in the skin, H&E stains, 60x magnification.
Figure 2
Figure 2
(a) Histopathologic exam of the small bowel revealed the diagnosis of IVLBCL with intravascular large atypical cells, H&E stain, 20x magnification. (b) Placenta also showed involvement of the disc (maternal blood) by IVLBCL including large atypical cells, H&E stain, 20x magnification. (c, d). Small atypical cells were seen in the small blood vessels of the breast, highlighted by CD20, H&E, and immunohistochemistry stains, 40x magnification. (e, f) Small lymphoma cells involving breast in comparison with large cells involving placenta, H&E stains, 60x magnification.
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