CORRECT DIAGNOSIS:
Diffuse large B-cell non-Hodgkin’s lymphoma, high grade
DISCUSSION:
Primary cutaneous B-cell lymphomas are rare, making up the minority of primary cutaneous lymphomas. Classification schemes for cutaneous lymphomas have been controversial and continue to be disputed. Multiple classification schemes have been proposed, and have led to confusion in the terminology of cutaneous lymphomas.
Cutaneous lymphomas typically present with erythematous to violaceous plaques and nodules on the skin. Primary versus secondary disease must be distinguished. Physical exam, laboratory workup, and imaging help to exclude systemic involvement. Patients may complain of fever, weight loss, night sweats, and fatigue. Lymphadenopathy, as well as hepatosplenomegaly, may be present, and an exam should also include lymph nodes and palpation of the liver and spleen.
Laboratory workup should include CBC, chemistries to evaluate liver and renal function, and LDH. Evaluation of systemic involvement should include a peripheral blood smear, bone marrow evaluation, CT or PET scan, and a lymph node biopsy if lymphadenopathy is found on the exam. Histology will reveal large, atypical lymphoid cells in a diffuse pattern of growth. These cells have hyperchromatic nuclei with prominent nucleoli. Mitoses may be abundant. Immunophenotype will stain positively for lymphoid markers, such as LCA (CD45), and B lymphocyte markers, such as CD20 and CD79a.
Low-grade, indolent B cell lymphomas do not require aggressive chemotherapy, and may be observed, and localized lesions may benefit from radiation therapy. Rituximab, an anti-CD20 antibody that targets CD20 on B cells, is generally used for low or high-grade B-cell lymphomas. The standard treatment for aggressive, high-grade B-cell lymphomas includes multiple-agent chemotherapy (CHOP) in combination with rituximab (CHOP-R), which may be followed by radiation.
TREATMENT:
CHOP chemotherapy with rituximab, followed by radiation.
REFERENCES:
Bosly, A., Bron, D., Van Hoof, A., De Bock, R., Berneman, Z., Ferrant, A., Kaufman, L., Dauwe, M., & Verhoef, G. (2007). Achievement of optimal average relative dose intensity and correlation with survival in diffuse large B-cell lymphoma patients treated with CHOP. Annals of Hematology.
Fink-Puches, R., Wolf, I. H., Zalaudek, I., Kerl, H., & Cerroni, L. (2005). Treatment of primary cutaneous B-cell lymphoma with rituximab. Journal of the American Academy of Dermatology, 52(5), 847-853.
Fung, M. A., Murphy, M. J., Hoss, D. M., & Grant-Kels, J. M. (2002). Practical evaluation and management of cutaneous lymphoma. Journal of the American Academy of Dermatology, 46(3), 325-357.
Geelen, F. A., Vermeer, M. H., Meijer, C. J., Van der Putte, S. C., Kerkhof, E., Kluin, P. M., & Willemze, R. (1998). bcl-2 protein expression in primary cutaneous large B-cell lymphoma is site-related. Journal of Clinical Oncology, 16(6), 2080-2085.
Grange, F., Beylot-Barry, M., Courville, P., Maubec, E., Bagot, M., Vergier, B., Souteyrand, P., Machet, L., Dalac, S., Esteve, E., Templier, I., Delaporte, E., Avril, M. F., Robert, C., Dalle, S., Laroche, L., Delaunay, M., Joly, P., Wechsler, J., & Petrella, T. (2007). Primary cutaneous diffuse large B-cell lymphoma, leg type: clinicopathologic features and prognostic analysis in 60 cases. Archives of Dermatology, 143(9), 1144-1150.
Gajra, A., Vajpayee, N., & Grethlein, S. (2007). Lymphoma, B-cell. eMedicine.
Heinzerling, L. M., Urbanek, M., Funk, J. O., Peker, S., Bleck, O., Neuber, K., Burg, G., von Den Driesch, P., & Dummer, R. (2000). Reduction of tumor burden and stabilization of disease by systemic therapy with anti-CD20 antibody (rituximab) in patients with primary cutaneous B-cell lymphoma. Cancer, 89(8), 1835-1844.
Liu, Q., Ohshima, K., & Kikuchi, M. (2000). Primary cutaneous B-cell lymphoma in Japanese patients. Pathology International, 50(12), 960-966.
Newman, M. K., Zemmel, N. J., Bandak, A. Z., & Kaplan, B. J. (2007). Primary breast lymphoma in a patient with silicone breast implants: a case report and review of the literature. Journal of Plastic, Reconstructive & Aesthetic Surgery.
Pandolfino, T. L., Siegel, R. S., Kuzel, T. M., Rosen, S. T., & Guitart, J. (2000). Primary cutaneous B-cell lymphoma: review and current concepts. Journal of Clinical Oncology, 18(10), 2152-2168.
Wiesner, T., Streubel, B., Huber, D., Kerl, H., Chott, A., & Cerroni, L. (2005). Genetic aberrations in primary cutaneous large B-cell lymphoma: A fluorescence in situ hybridization study of 25 cases. American Journal of Surgical Pathology, 29(5), 666-673.
Willemze, R., et al. (2005). WHO-EORTC classification for cutaneous lymphomas. Blood, 105(10), 3768-3785.
Pandolfino, T. L., Siegel, R. S., Kuzel, T. M., Rosen, S. T., & Guitart, J. (2000). Primary cutaneous B-cell lymphoma: review and current concepts. Journal of Clinical Oncology, 18(10), 2152-2168.
Smith, B. D., Smith, G. L., Cooper, D. L., & Wilson, L. D. (2005). The cutaneous B-cell lymphoma prognostic index: A novel prognostic index derived from a population-based registry. Journal of Clinical Oncology, 23(15), 3390-3395.
Cerroni, L., Gatter, K., & Kerl, H. (2004). An illustrated guide to skin lymphoma (2nd ed.). Malden, MA: Blackwell Publishing.