PMC5556984_01
Abdominal and pelvic CT scan. Marked hepatomegaly and splenomegaly are identified. Spleen demonstrates numerous areas of heterogeneous hypoenhancement.
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fig1
Computed tomography (CT) scan at the time of admission revealed no evidence of mediastinal, hilar, or other systemic lymphadenopathy but showed marked enlargement of liver and spleen (Figure 1).
Histopathologic features. BM biopsy shows patchy interstitial infiltration of large lymphoma cells (arrows) without sinusoidal involvement (H&E; 400x) (a). By immunohistochemistry (IHC), the tumor cells are positive for CD20 (400x) (b) and negative for CD30 (400x) (c). CD34 highlights the capillary endothelial cells and confirms the lack of intravascular involvement by tumor cells (400x) (d). Lymphoma cells are large in size with large nuclei, high nuclear to cytoplasmic ratios, and open chromatin with one to several prominent nucleoli (Wright Geimsa; 100x) (e). BM aspirate smear shows histiocytic hemophagocytosis characterized by RBCs engulfing by histiocytes (Wright Geimsa; 100x) (f).
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fig2
The BM core biopsy demonstrated a marrow cellularity of 60% with scattered large atypical lymphoid cells (Figure 2(a)). These cells were either scattered individually or formed loose clusters and represented approximately 10% of the total marrow cellularity (Figure 2(b)). TdT, glycophorin, MPO, and CD117 (Figures 2(b) and 2(c)). CD20 and CD79a highlighted the interstitial distribution of the tumor cells. Tumor cells inside the marrow sinusoids were not seen (Figure 2(b)). The lack of intrasinusoidal and intravascular tumor excluded the diagnosis of IVLBCL (Figures 2(d) and 3(a) and Table 2). Individual large, atypical lymphoid cells were also recognized on the aspirate smears (Figure 2(e)). In addition, there were numerous histiocytes demonstrating hemophagocytosis throughout the aspirate smears (Figure 2(f)).
Histopathologic features. BM biopsy shows patchy interstitial infiltration of large, PAX5 positive lymphoma cells without sinusoidal (CD34 positive) involvement (400x) (a). By IHC, less than 30% of tumor cells are positive for CD10 (400x) (b) and more than 30% positive for MUM1 (400x) (c).
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IHC showed CD10 reactivity in less than 30% of tumor cells and MUM1 reactivity in more than 30% of tumor cells (Figures 3(b) and 3(c)).
Histopathologic features. BM biopsy shows a subset of the tumor cells positive for MYC (100x) (a) and for BCL2 (100x) (b).
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Additionally, BM biopsy was stained for MYC and for BCL2 and showed a subset of positive tumor cells (Figures 4(a) and 4(b)).