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Small cell/lymphohistiocytic morphology is associated with peripheral blood involvement, CD8 positivity and retained T-cell antigens, but not outcome in adults with ALK+ anaplastic large cell lymphoma

Several morphologic variants of ALK+ anaplastic large cell lymphoma (ALCL) are recognized. The small cell (SC) and lymphohistiocytic (LH) variants are reported to be associated with poorer outcome in children with ALK + ALCL. In this study of 1 02 adults with ALK + ALCL, there were 18 (18%) cases of...

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Published in:Modern pathology 2022-03, Vol.35 (3), p.412-418
Main Authors: Khanlari, Mahsa, Li, Shaoying, Miranda, Roberto N., Iyer, Swaminathan, Konoplev, Sergej, Lin, Pei, Yin, C. Cameron, Tang, Guilin, Qiu, Lianqun, Vega, Francisco, Medeiros, L. Jeffrey, Xu, Jie
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Language:English
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Summary:Several morphologic variants of ALK+ anaplastic large cell lymphoma (ALCL) are recognized. The small cell (SC) and lymphohistiocytic (LH) variants are reported to be associated with poorer outcome in children with ALK + ALCL. In this study of 1 02 adults with ALK + ALCL, there were 18 (18%) cases of SC and/or LH variants. Patients with SC/LH ALK + ALCL more often had peripheral blood involvement than patients with non-SC/LH neoplasms (60% vs 0%, p  = 0.02). There were no other significant differences in clinical features between patients with SC/LH versus non-SC/LH ALK + ALCL. Compared with non-SC/LH cases of ALK + ALCL, neoplasms with SC/LH features were more often positive for CD2 (92% vs. 36%, p  = 0.0007), CD3 (81% vs. 15%, p  = 0.0001), CD7 (80% vs. 37%, p  = 0.03), and CD8 (54% vs. 7%, p  = 0.0006). There were no other significant differences in the immunophenotype between SC/LH and non-SC/LH ALK + ALCL cases. The initial chemotherapy regimens and the response rates were similar between patients with ALK + ALCL with SC/LH patterns versus those with non-SC/LH patterns. After a median follow-up of 30.8 months (range, 0.3–208 months), patients with high ( > 3) International Prognostic Index (IPI) scores had significantly shorter overall survival than patients with low (
ISSN:0893-3952
1530-0285
DOI:10.1038/s41379-021-00944-1