Abstract:

We would like to share a challenging and educational case that spans a timeframe of 8 years. At the age of 16 our patient presented with regional lymphadenopathy. Lymph node biopsy showed large atypical Reed Sternberg-like cells in an inflammatory background. The cells were positive for CD30, CD15, CD4, CD2, and granzyme B and negative for CD20, PAX-5, CD8, CD7, ALK and EBV. Molecular studies were negative. A variant of classical Hodgkin lymphoma with expression of T-cell markers was favored by hematopathology. Six years later the patient presented with skin nodules. Radiologic workup revealed some regional lymphadenopathy but no evidence of systemic disease. Multiple biopsies of skin and nodal tissue over a 2 year period all showed similar findings- a CD30-positive T-cell lymphoproliferative disorder, favoring the diagnosis of primary cutaneous anaplastic large cell lymphoma. All of the patients pathology specimens to date were subsequently sent to the NIH for expert consultation. Molecular studies were performed and showed no T-cell, B-cell or DUSP22-IRF4 rearrangement. FISH studies with an ALK-specific probe showed increased copy number. Ultimately, based on the indolent clinical history, histology, immunohistochemical and molecular studies, a unifying diagnosis was rendered: primary cutaneous anaplastic large cell lymphoma (c-ALCL), initially presenting as regional nodal disease. As this case demonstrates, it can be very difficult to distinguish c-ALCL with nodal involvement from ALK-negative systemic ALCL or a T-cell variant of classical Hodgkin lymphoma, especially when the initial presentation is that of node-limited disease. It is important for dermatopathologists, hematopathologists, dermatologists, and oncologists to be aware that primary cutaneous anaplastic large cell lymphoma can initially manifest as nodal disease preceding skin involvement.

Financial Disclosure:
No current or relevant financial relationships exist.

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Feifan Chen, MD