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Specific Skin Lesions in Chronic Myelomonocytic Leukemia: A Spectrum of Myelomonocytic and Dendritic Cell Proliferations. A Study of 42 Cases

Vitte, Franck MD*; Fabiani, Bettina MD; Bénet, Claire MD; Dalac, Sophie MD§; Balme, Brigitte MD; Delattre, Claire MD; Vergier, Béatrice MD, PhD#; Beylot-Barry, Marie MD, PhD**; Vignon-Pennamen, Dominique MD; Ortonne, Nicolas MD, PhD††; Algros, Marie Paule MD*; Carlotti, Agnès MD‡‡; Samaleire, Dimitri MD, PhD§§; Frouin, Eric MD∥∥; Levy, Anne MD¶¶; Laroche, Liliane MD, PhD##; Theate, Ivan MD***; Monnien, Franck MD†††; Mugneret, Francine MD‡‡‡; Petrella, Tony MD§§§,∥∥∥

American Journal of Surgical Pathology: September 2012 - Volume 36 - Issue 9 - p 1302–1316
doi: 10.1097/PAS.0b013e31825dd4de
Original Articles

Chronic myelomonocytic leukemia (CMML) is a rare clonal hematopoietic disorder that can also involve the skin. The histopathology of these skin lesions is not clearly defined, and few data are available in the literature. To better understand tumoral skin involvements in CMML we carried out an extensive, retrospective clinicopathologic study of 42 cases selected from the database of the French Study Group of Cutaneous Lymphomas. On the basis of clinical data, morphology, and phenotype we identified 4 clinicopathologic profiles representing 4 distinct groups. The first group comprised myelomonocytic cell tumors (n=18), exhibiting a proliferation of granulocytic or monocytic blast cells, which were CD68 and/or MPO positive but negative for dendritic cell markers. The second group comprised mature plasmacytoid dendritic cell tumors (n=16), denoted by a proliferation of mature plasmacytoid dendritic cells, which were CD123, TCL1, and CD303 positive but CD56, CD1a, and S100 negative. The third group comprised blastic plasmacytoid dendritic cell tumors (n=4), characterized by a proliferation of monomorphous medium-sized blast cells, which were CD4, CD56, CD123, TCL1 positive but CD1a and S100 negative. The fourth group consisted of a putatively novel category of tumor that we named blastic indeterminate dendritic cell tumors (n=4), distinguished by a proliferation of large blast cells that not only exhibited monocytic markers but also the dendritic markers CD1a and S100. These 4 groups showed distinctive outcomes. Finally, we showed, by fluorescence in situ hybridization analysis, a clonal link between bone marrow disease and skin lesions in 4 patients. Herein, we have described a novel scheme for pathologists and physicians to handle specific lesions in CMML, which correspond to a spectrum of myelomonocytic and dendritic cell proliferations with different outcomes. A minimal panel of immunohistochemical markers including CD68, CD1a, S100, Langerin, and CD123 is necessary to make the correct classification in this spectrum of cutaneous CMML tumors, in which dendritic cell lineage plays an important role.

*Department of Pathology, CHU Jean Minjoz

†††Department of Statistics, CHU, Besançon

Department of Pathology, Hôpital Saint-Antoine

Department of Pathology, Hôpital Saint-Louis

‡‡Department of Pathology, Hôpital Cochin, APHP, Paris

Departments of §Dermatology

‡‡‡Genetics

§§§Pathology, CHU Bocage

∥∥∥Centre de Pathologie, Dijon

Department of Pathology, Hôpital Lyon-Sud, Lyon

Department of Pathology, CHRU, Lille

Departments of #Pathology

**Dermatology, CHU, Bordeaux

††Department of Pathology, Hôpital Henri Mondor, APHP, Créteil

§§Department of Pathology, CHU, Grenoble

∥∥Department of Pathology, CHU, Montpellier

Departments of ¶¶Pathology

##Dermatology, Hôpital Avicennes, APHP, Bobigny, France

***Department of Pathology, CSL, Brussels, Belgium

Conflicts of Interest and Source of Funding: The authors have disclosed that they have no significant relationships with, or financial interest in, any commercial companies pertaining to this article.

Correspondence: Tony Petrella, MD, Department of Pathology, Plateau Technique de Biologie, 2 rue Angélique Ducoudray, 21000 Dijon, France (e-mail: tony.petrella@chu-dijon.fr; tony.petrella@centre-de-pathologie.fr).

© 2012 Lippincott Williams & Wilkins, Inc.