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Final diagnosis:  Nodular sclerosis classic Hodgkin lymphoma (CHL) with foci of Langerhans cell histiocytosis (LCH)


This case demonstrated a concurrent involvement of a lymph node by CHL and LCH, which was exceedingly rare. It has been controversial if LCH associated with lymphoma represents a true neoplasm, highlighted by a recent finding that there were no BRAF or MAP2K1 mutations in LCH. Our case may represent the first demonstration of BRAF V600E mutation in a typical case of LCH associated with lymphoma, supporting that at least some of the LCHs associated with lymphoma are true neoplasm. The twice-confirmed low allele frequency for the BRAF V600E mutation despite microdissection could point to an early clonal event in the background of an initially reactive Langerhans cell proliferation.

List of References

[1]        H. Bosmuller, A. Fischer, D. L. Pham, T. Fehm, D. Capper, A. von Deimling, I. Bonzheim, A. Staebler, and F. Fend, "Detection of the BRAF V600E mutation in serous ovarian tumors: a comparative analysis of immunohistochemistry with a mutation-specific monoclonal antibody and allele-specific PCR," Human pathology, vol. 44, pp. 329-35, Mar 2013.

[2]        L. J. Christie, A. T. Evans, S. E. Bray, M. E. Smith, N. M. Kernohan, D. A. Levison, and J. R. Goodlad, "Lesions resembling Langerhans cell histiocytosis in association with other lymphoproliferative disorders: a reactive or neoplastic phenomenon?," Human pathology, vol. 37, pp. 32-9, Jan 2006.

[3]        A. L. Feldman, F. Berthold, R. J. Arceci, C. Abramowsky, B. M. Shehata, K. P. Mann, S. J. Lauer, J. Pritchard, M. Raffeld, and E. S. Jaffe, "Clonal relationship between precursor T-lymphoblastic leukaemia/lymphoma and Langerhans-cell histiocytosis," The Lancet. Oncology, vol. 6, pp. 435-7, Jun 2005.

[4]        W. O. Greaves, C. Bueso-Ramos, and L. Fayad, "Classical Hodgkin's lymphoma associated with Langerhans cell histiocytosis: multiagent chemotherapy resulted in histologic resolution of both the classical Hodgkin's lymphoma and Langerhans cell proliferation components," Journal of clinical oncology : official journal of the American Society of Clinical Oncology, vol. 29, pp. e76-8, Feb 1 2011.

[5]        S. Pina-Oviedo, L. J. Medeiros, S. Li, J. D. Khoury, K. P. Patel, K. Alayed, R. C. Cason, C. J. Bowman, and C. C. Yin, "Langerhans cell histiocytosis associated with lymphoma: an incidental finding that is not associated with BRAF or MAP2K1 mutations," Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc, vol. 30, pp. 734-744, May 2017.

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