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Hodgkin and Reed‐Sternberg‐like cells within a recurrent primary cutaneous marginal zone lymphoma

An 88-year-old woman presented with a 1-month history of several papules on the right upper arm. Her history was notable for primary cutaneous marginal zone lymphoma (PCMZL) on the left shoulder 6 years previously, which was treated with excision. At that time, the history, physical examination, bone marrow biopsy, and computed tomography (CT) of chest, abdomen, and pelvis were negative for extracutaneous disease. Subsequent recurrences of PCMZL on the right upper arm were excised at 1, 4, and 5 years after the initial diagnosis; local radiation was also completed after one of these recurrences. On examination, there were several 0.5 to 1.0 cm pink, subepidermal papules within and adjacent to the scar from the last PCMZL excision (Figure 1A). Biopsies of two of the new lesions showed a dense dermal and subcutaneous lymphoid infiltrate (Figure 1B), including many reactive CD3-positive T-cells (Figure 1C), as well as small to medium-sized, CD20-positive B-cells (Figure 1D) and peripheral aggregates of plasma cells (Figure 1E) with lambda light chain restriction by in situ hybridization, consistent with the patient's known marginal zone lymphoma. Also noted were interspersed large cells (Figure 1F) with one to multiple large nuclei and abundant pale cytoplasm (Figure 2A), suggestive of Hodgkin and Reed-Sternberg (HRS) cells. These were positive for CD15 (Figure 2B), CD30 (Figure 2C), and MUM-1, weakly positive for PAX5 (Figure 2D), weakly positive to negative for BCL2, and negative for CD45 (Figure 2E), CD20 (Figure 2F), CD3, and BCL6. With regard to the CD20 negativity, it is noted that the patient had no history of rituximab therapy. EBER-1 in situ hybridization was negative. Prior specimens were reviewed, and each showed a dense dermal and/or subcutaneous lymphocytic infiltrate, with many reactive CD3-positive cells, and an equal or smaller proportion of CD20-positive small to medium-sized lymphocytes; several specimens showed peripheral aggregates of plasma cells with lambda light chain predominance. A few CD15+/CD30+ HRS-like cells were retrospectively noted in a specimen from one year prior; a similar population was not identified in three earlier specimens. Flow cytometry from the first cutaneous specimen showed a lambda monotypic B-cell population. Immunoglobulin gene rearrangement by polymerase chain reaction (PCR) was performed concurrently on the initial and current specimens, as well as a specimen from one year prior, and all showed the same monoclonal B-cell population. Following the current biopsy, a history, physical exam, complete blood count, comprehensive metabolic profile, lactate dehydrogenase, Epstein-Barr virus PCR on peripheral blood, and CT of the chest, abdomen, and pelvis were unremarkable. She was referred for local radiation of the new nodules on the right arm. She died approximately