PMC6333078_01
CT scan of the abdomen demonstrates hepatomegaly and splenomegaly
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He was noted to have pancytopenia and hepatomegaly, and splenomegaly (21 cm) on CT scan (Figure 1).
Bone marrow aspirate. High power view. Hypercellular for age (80%). Demonstrates involvement with T-cell lymphoma and hemophagocytes
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T-cell lymphoma, compromising about 5% of the cellularity as well as a moderate number of hemophagocytes (Figure 2). There is bone marrow involvement in most patients at diagnosis7, 11 and hemophatocytosis is a recognized but infrequent phenomenon that can develop in patients with HSTCL.7 The aspirate demonstrated a hypercellular marrow and normal erythroid number and maturation.
Bone marrow biopsy. Low power view. CD3 immunohistochemical staining consistent with lymphoma cells. Immunophenotyping was positive for CD16, and CD57 and was double negative for CD4 and 8
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CD20 was negative in neoplastic cells and positive in sparse background small B-cells. CD3 was positive in neoplastic cells (Figure 3), many within marrow sinusoids, with intermediate nuclei, open chromatin, and a variably prominent nucleolus, overall comprising about 5% of the cellularity.
Bone marrow biopsy. The IHC stain was also positive for KP1 (CD68), highlighting the nuclei of engulfed cells within macrophages
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IHC stain was also positive for KP1 (CD68), highlighting the nuclei of engulfed cells within macrophages (Figure 4). Immunophenotyping by flow cytometry demonstrated the following phenotype:
Liver biopsy. High power view. Sinusoidal infiltration of hepatic sinusoids by lymphocytes
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IL-2, and hemophagocytes bought the diagnosis of HLH. In light of the hepatomegaly, a liver biopsy was pursued which confirmed a diagnosis of HSTCL involvement in the liver (Figure 5). The lymphoma cells infiltrated the liver sinusoids (Figure 5), which is a typical finding with HSGDTL.1, 7 IHC stains were positive for CD3 and CD56.