PMC4755279_01
Pathological findings of T-cell lymphomas arising in three patients with acquired generalized lipodystrophy.. Case 1. Peripheral T-cell lymphoma, NOS. Atypical lymphoid cells formed a tumor on the right lower leg. The atypical lymphoid cells replaced the dermis and extended into subcutaneous tissue. There was no epidermotropism. A. A stain for CD5 highlights the extent of infiltration. The neoplastic cells were also positive for CD3, CD4 and Beta F1, and negative for CD8 and CD30 (not shown). Cytotoxic molecules were negative. B. Nearly all cells are positive for MIB-1(Ki-67), indicating a high proliferative rate. Cells have large round to irregular nuclei and distinct basophilic nucleoli.. Case 2. Peripheral T-cell lymphoma, NOS, involving the skin of ankle C. Neoplastic cells extend from superficial dermis to subcutis. There is superficial edema but minimal epidermotropism. D. At higher power, neoplastic lymphoid cells are round to oval in shape with clear cytoplasmic borders. Cells are CD3-positive (Inset), and were also positive for CD5 (weak), CD4 and Beta F1; cells were negative for CD8, CD30, and TCR-gamma. Case 3. ALK-positive anaplastic large cell lymphoma. E. A soft tissue mass from the region of the right breast shows infiltration by atypical, pleomorphic cells with a range in cell size. F. The cells are strongly positive for CD30, and showed nuclear and cytoplasmic staining for ALK (not shown).. Original magnifications: A, C, 20x; B, D, E, F, 400x.
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F1
Biopsy revealed peripheral T-cell lymphoma (PTCL, Figure 1A and 1B). Biopsies obtained from cutaneous thick plaques and nodules from the ankle, lower leg, and right arm showed similar histological features (Figure 1C and 1D), consistent with PTCL. Core needle biopsy showed anaplastic lymphoma kinase (ALK)-positive anaplastic large cell lymphoma (ALCL) (Figure 1E and 1F).
Clinical photographs of peripheral T-cell lymphoma, NOS, in a patient with acquired generalized lipodystrophy (Case 2) before (A) and after (B) chemotherapy.
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F2
Eight months after starting metreleptin, she developed a nodular skin rash (Figure 2).
PMC4755279_02
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