Runaway Train: A Leaky Radiosensitive SCID with Skin Lesions and Multiple Lymphomas

Figure 2

Affection of facial muscles and internal organs. (a) MR of the neck showing a large ulcerating tumor on the left side with infiltration into nearby muscles. (b) CT of the abdomen showing massive thickening of the ventricular wall due to lymphoma with infiltration also into the spleen and left adrenal gland. (c, d) Skin biopsy from the neck lesion showing sheets of lymphoid cells throughout dermis, with large pale gray nuclei with prominent nucleoli (H&E, 100x and 400x, resp.). On immunohistochemistry, the cells were positive for CD20, Pax5, Bcl-2, MUM1, and EBERISH and negative for CD3, CD4, CD5, CD8, CD30, CD10, Bcl-6, and cyclin D1. Ki-67 showed a proliferative fraction of 70–80% of tumor cells. The lesion was classified as diffuse large B-cell lymphoma.

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