Primary Systemic Anaplastic Large Cell Lymphoma
Clinical
- Commonly presents with widespread systemic involvement
- lymph nodes
- extranodal sites
- especially skin, bone, soft tissues
- rarely gastrointestinal tract, CNS
- bone marrow
- immunohistochemistry for CD30, EMA or ALK1 may be required to recognize minimal involvement
- ALK1 positive cases:
- Predominance of children and young adults, male > female
- Better prognosis than ALK1 negative cases
- ALK1 negative cases:
- Older adults
- Poorer prognosis than ALK1 positive cases (similar to other peripheral T-cell lymphoma, NOS)
