Rare Diagnosis of CD4⁺56⁺ Leukemia from Dendritic Cells Type DC2
Authors:
M. Klabusay 1; M. Pevná 1; J. Kissová 2; M. Doubek 1; M. Heidekerová 1; J. Mayer 1; J. Vorlíček 1
Authors‘ workplace:
interní hematoonkologická klinika LF MU a FN, Brno
oddělení klinické hematologie FN, Brno
Published in:
Čas. Lék. čes. 2008; 147: 511-515
Category:
Review Article
Overview
CD4⁺56⁺ hematodermic neoplasm or leukemia from early plasmocytoid dendritic cells type DC2 was recognized by WHO-EORTC classification of cutaneous lymphomas as a separate entity related to the plasmacytoid precursor dendritic cell (pDC). This diagnosis is based on expression of CD4 and CD56 antigens and absence of B, T or myeloid lineage markers. Immunohistochemistry and flow cytometry are the only methods, which allow identification of this disease, either in isolated skin lesions or in a leukemic form. Although the co-expression of CD4 and CD56 is rare and the number of described cases is low, this group bears similar characteristics in a clinical course of disease. It is a very aggressive leukemia/lymphoma, usually with primary skin involvement, in half of the cases infiltrating bone marrow or lymph nodes. Despite high rate of initial response to treatment, early and widespread relapses occur and patients die of disease progression. Although the physiological counterpart of tumour cells was identified, the origin of the disease is still discussed because of aberrant expression of cell markers. Optimal treatment is not known. However, this aggressive disease requires radical approach with intensive chemotherapy regimens, prophylaxis of CNS involvement and early indication of allogeneic bone marrow transplantation. Two case reports are described.
Key words:
CD4⁺CD56⁺ malignancy, DC2 leukemia, hematodermic neoplasm.
Sources
1. Brody, J. P., Allen, S., Schulman, P. et al.: Acute agranular CD4-positive natural killer cell leukemia. Cancer, 1995, 75, s. 2474–2483.
2. DiGiuseppe, J. A., Louie, D. C., Williams, J. E. et al.: Blastic natural killer cell leukemia/lymphoma: a clinicopathologic study. Am. J. Surg. Pathol., 1997, 21, s. 1223–1230.
3. Bagot, M., Bouloc, A., Charue, D. et al.: Do primary cutaneous non-T non-B CD4⁺CD56⁺ lymphomas belong to the myelo-monocytic lineage? J. Invest. Dermatol., 1998, 11, s. 1242–1244.
4. Petrella, T., Dalac, S., Maynadié, M. et al.: CD4⁺ CD56⁺ cutaneous neoplasms: a distinct hematological entity? Am. J. Surg. Pathol., 1999, 23, s. 137–146.
5. Chaperot, L., Bendriss, N., Manches, O. et al.: Identification of a leukemic counterpart of the plasmacytoid dendritic cells. Blood, 2001, 97, s. 3210–3217.
6. Hayashi, K., Nakamura, S., Koshikawa, T. et al.: A case of neural cell adhesion molecule-positive peripheral T-cell lymphoma associated with human T-cell lymphotrophic virus type 1 showing an unusual involvement of the gastrointestinal tract during the course of the disease. Hum. Pathol., 1994, 25, s. 1251–1253.
7. Savoia, P., Fierro, M. T., Novelli M. et al.: CD56-positive cutaneous lymphoma: a poorly recognized entity in the spectrum of primary cutaneous disease. Br. J. Dermatol., 1997, 137, s. 966–971.
8. Reimer, P., Rűdiger, T., Kreamer, D. et al.: What is CD4⁺CD56⁺ malignancy and how should it be treated? Bone Marrow Transplant., 2003, 32, s. 637–646.
9. Feuillard, J., Jacob, M. C., Valensi, F. et al.: Clinical and biologic features of CD4⁺CD56⁺ malignancies. Blood, 2002, 99, s. 1556–1563.
10. Kohrgruber, N., Halanek, N., Groger, M., et al.: Survival, maturation, and function of CD11c– and CD11c+ peripheral blood dendritic cells are differentially regulated by cytokines. J. Immunol., 1999, 163, s. 3250–3259.
11. Petrella, T., Comeau, M. R., Maynadié, M. et al.: ’Agranular CD4⁺ CD56⁺ hematodermic neoplasm (blastic NK-Cell lymphoma) originates from a population of CD56⁺ precursor cells related to plasmacytoid monocytes. Am. J. Surg. Pathol., 2002, 26, s. 852–862.
12. Khoury, J. D., Medeiros, L. J., Manning, J. T. et al.: CD56+ TdT+ blastic natural killer cell tumor of the skin: a primitive systemic malignancy related to myelomonocytic leukemia. Cancer, 2002, 94, s. 2401–2408.
13. Dzionek, A., Fuchs, A., Schmidt, P. et al.: BDCA-2, BDCA-3, and BDCA-4: three markers for distinct subsets of dendritic cells in human peripheral blood. J. Immunol., 2000, 165, s. 6037–6046.
14. Urosevic, M., Conrad, C., Kamarashev, J., et al.: CD4⁺ CD56⁺ hematodermic neoplasms bear a plasmacytoid dendritic cell phenotype. Hum. Path., 2005, 36, s. 1020–1024.
15. Meyer, N., Petrella, T., Poszepczynska-Guigné, E. et al.: CD4⁺ CD56⁺ blastic tumor cells express CD101 molecules. J. Invest. Dermatol., 2005, 124, s. 668–669.
16. Scott, A. A., Head, D. R., Kopecky, K. J. et al.: HLA-DR–, CD33⁺, CD56⁺, CD16– myeloid/natural killer cell acute leukemia: a previously unrecognized form of acute leukemia potentially misdiagnosed as French-American-British acute myeloid leukemia M3. Blood, 1994, 84, s. 244–255.
17. Van Camp, B., Durie, B. G. M., Spier, C. et al.: Plasma cells in multiple myeloma express a natural killer cell-associated antigen: CD56(NKH-1; Leu-19). Blood, 1990, 76, s. 377–382.
18. Knudsen, H., Gronbaek, K., Straten, P.: et al. A case of lymphoblastoid natural killer (NK)-cell lymphoma: association with NK-cell receptor complex CD94/NKG2 and TP53 intragenic deletion. Br. J. Dermatol., 2002, 146, s. 148–153.
19. Franck, T. et al.: The CD4⁺ CD56⁺ CD116– CD123⁺ CD45RA⁺ CD45RO– profile is specific to DC2 malignancies. Haematologica, 2007, 88, s. e50–e51.
20. Herling, M., Teitell, M. A., Shen, R. R. et al.: TCL1 expression in plasmacytoid dendritic cells (DC2s) and the related CD4+ CD56+ blastic tumors of skin. Blood, 2003, 101, s. 5007–5009.
21. Ko, Y. H., Kim, S. H., Park, K. et al.: CD4⁺CD56⁺CD68⁺ hematopoietic tumor of probable plasmacytoid monocyte derivation with weak expression of cytoplasmic CD3. J. Korean. Med. Sci., 2002, 17, s. 833–839.
22. Anargyrou, K., Paterakis, G., Boutsis, D. et al.: An unusual case of CD4⁺ CD7⁺ CD56⁺ acute leukemia with overlapping features of type 2 dendritic cell (DC2) and myeloid/NK cell precursor acute leukemia. Eur. J. Haematol., 2003, 71, s. 294–398.
23. Garnache-Ottou, F., Chaperot, L., Biichle, S. et al.: Expression of the myeloid-associated marker CD33 is not an exclusive factor for leukemic plasmacytoid dendritic cells. Blood, 2005, 105, s. 1256–1264.
24. Kazakov, D. V., Mentzel, T., Burg G. et al.: Blastic natural killer-cell lymphoma of the skin associated with myelodysplastic syndrome or myelogenous leukaemia: a coincidence or more? Br. J. Dermatol., 2003, 149, s. 869–876.
25. Leroux, D., Mugneret, F., Callanan, M. et al.: CD4⁺, CD56⁺ DC2 acute leukemia is characterized by recurrent clonal chromosomal changes affecting 6 major targets: a study of 21 cases by the Groupe Franćais de Cytogénétique Hématologique. Blood, 2002, 99, s. 4154–4159.
26. Amor, R. B., Hicheri, Y., Pautas, C. et al.: Successful non-myeloablative allogenic HLA-identical stem cell transplantation for CD4/CD56 positive acute leukemia. Transplant., 2007, 84, s. 1066–1067.
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Addictology Allergology and clinical immunology Angiology Audiology Clinical biochemistry Dermatology & STDs Paediatric gastroenterology Paediatric surgery Paediatric cardiology Paediatric neurology Paediatric ENT Paediatric psychiatry Paediatric rheumatology Diabetology Pharmacy Vascular surgery Pain managementArticle was published in
Journal of Czech Physicians
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