Summary
Two patients with clinical manifestations of Sézary syndrome are reported. In both cases from an early stage of the disease in addition to characteristic Sézary cells large numbers of immunoblasts were present in skin lesions and peripheral lymph nodes and in one case also in the blood. Their relationship to the characteristic Sézary cells was shown by morphological, cytochemical and immunological methods. The infiltrates in the skin were epidermotropic in one case and nonepidermotropic in the other. Lymph node structure was effaced by diffuse infiltration of abnormal lymphoid cells. These were found to proliferate in the skin as well as in lymph nodes. Cytogenetical studies of blood lymphocytes indicated an abnormal hypodiploid clone in both cases. Immunologically the tumour cells had properties of peripheral T-lymphocytes but whereas all abnormal cells exhibited inducer/helper cell characteristics in one case, only a minority of the lymphocytes revealed these characteristics in the other case. In this case the tumour cell population changed into a more pleomorphic type. The classification of the cases is discused.
This is a preview of subscription content, log in via an institution to check access.
Similar content being viewed by others
References
Brehmer-Andersson E (1976) Mycosis fungoides and its relation to Sézary's syndrome, lymphomatoid papulosis, and primary cutaneous Hodgkins' disease. Acta Derm Venereol (Stockh) (Suppl) 56:75
Brouet JC, Flandrin G, Sasportes M, Preud'Homme JL, Seligmann M (1975) Chronic lymphocytic leukaemia of T-cell origin. Lancet II:890–893
Catovsky D, Rose M, Goolden AWG, White JM et al. (1982) Adult T-cell lymphoma — leukemia in blacks from the West Indies. Lancet I:639–643
Costello C, Catovsky D, O'Brien M, Morilla R, Varadi S (1980) Chronic T-cell leukemias. 1. Morphology, cytochemistry and ultrastructure. Leuk Res 4:463–476
Edelson RL (1980) Cutaneous T-cell lymphoma. J Dermatol Surg Oncol 6:358–368
Eimoto T, Mitsui T, Kikuchi M (1981) Ultrastructure of adult T-cell leukemia/lymphoma. Virchows Arch (Cell Pathol) 38:189–208
Goos M (1980) Pathologie und Klinik des immunoblastischen Lymphoms der Haut. Z Hautkr 55:1295–1301
Hanooka M, Sasaki M, Matsumoto H, Tankawa H, Yamabe H, Tomimoto K, Tasaka C, Fujiwara H, Uchiyama T, Takatsuki K (1979) Adult T-cell leukemia. Histological classification and characteristics. Acta Pathol Jpn 29:723–738
Kadin ME, Kamoun M (1982) Nonendemic adult T-cell leukemia/lymphoma Human Pathol 13:691–693
Kerl H (1981) Das Sézary Syndrom. Zentralbl Haut Geschlechtskr 144:359–446
Kienitz T, Burg G, Schmoeckel C, Weitz H, Braun-Falco O (1979) High grade malignant lymphoma arising from Sézary's syndrome. A case report. Dermatologica 158:126–136
Kikuchi M, Mitsui T, Matsui N, Sato E, Tokunaga M, Hasai K, Ichimara M, Kinoshita K, Kamihira S (1979) T-cell malignancies in adults: histopathological studies of lymphnodes in 110 patients. Jpn J Clin Oncol (Suppl) 9:407–422
Lawrence EC, Broder S, Jaffe ES, Braylan RJC, Dobbins WO, Young RC, Waldmann TA (1979) Evolution of a lymphoma with helper T-cell characteristics in Sézary syndrome. Blood 52:481–492
Leong A S-Y, Sage RE, Kinnear GC, Forbes IJ (1980) Preferential epidermotropism in adult T-cell leukemia/lymphoma. Am J Surg Pathol 4:421–430
Lennert K (1978) Malignant lymphomas. Springer, Berlin Heidelberg New York
Meijer CJLM, Van der Loo EM, Van Vloten WA, Van der Velde EA, Scheffer E, Comelisse CJ (1980) Early diagnosis of mycosis fungoides and Sézary syndrome by morphometric analysis of lymphoid cells in the skin. Cancer 45:2864–2871
Pinkus GS, Said JW, Hargreaves H (1979) Malignant lymphoma, T-cell type. Am J Clin Pathol 72:540–550
Schmoeckel C, Burg G, Braun-Falco O (1979) Quantitative analysis of lymphoid cells in mycosis fungoides, Sézary's syndrome and parapsoriasis in plaques. Arch Dermatol Res 264:17–28
Schmoeckel C, Burg G, Hoffmann-Frezer G, Stolz W, Weitz H, Löhrs U, Braun-Falco O (1982) Cutaneous immunoblastic T-cell lymphoma. Arch Dermatol Res 274:141–154
Shamoto M, Marakami S, Zenke T (1981) Adult T-cell leukemia in Japan: an ultrastructural study. Cancer 47:1804–1811
Takatsuki K, Uchiyama T, Ueshima Y, Hattori T (1979) Adult T-cell leukemia: further clinical observations and cytogenetic and functional studies of leukemia cells. Jpn J Clin Oncol (Suppl) 8:317–324
Tatsumi E, Tatshima M, Takiuchi Y, Sawada H, Shirakawa S, Uchino H (1969) Terminal deoxynucleotidyl transferase activity in leukaemic T-cells from Japanese adults. Br J Haematol 43:151–153
Uchiyama T, Yodoi J, Sagawa K, Takatsuki K, Uchino H (1977) Adult T-cell leukemia: Clinical and hematological features of 16 cases. Blood 50:481–491
Van der Putte, SCJ, van der Meer JB (1981) Mycosis fungoides: a morphological study. Clin Exp Dermatol 6:57–76
Van der Putte SCJ, Tan-Ranoewisastro SP (1981) Huber's stain for resin embedded cutaneous tissue: a modification. Br J Dermatol 104:721
Van der Putte SCJ, Toonstra J, de Weger RA, van Unnik JAM (1982) Cutaneous T-cell lymphoma, multilobated type. Histopathology 6:35–54
Warin AP, Roberts MM (1979) Chronic lymphocytic leukaemia with cutaneous involvement. Clin Exp Dermatol 4:241–246
Watanabe S, Shimosato Y, Shimosato M, Minato K (1982) Studies with multiple markers on malignant lymphomas and lymphoid leukemias. Cancer 50:2372–2382
Zackheim MS (1981) Cutaneous T-cell lymphomas. Arch Dermatol 117:295–304
Zucker-Franklin D, Melton III JW, Quagliata (1974) Ultrastructural, immunological, and functional studies on Sézary cells: a neoplastic variant of thymus-derived (T) lymphocytes. Proc Natl Acad Sci USA 71:1877–1881
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
van der Putte, S.C.J., Toonstra, J., van Prooyen, H.C. et al. Sézary syndrome with early immunoblastic transformation. Arch Dermatol Res 276, 17–26 (1984). https://doi.org/10.1007/BF00412557
Received:
Issue Date:
DOI: https://doi.org/10.1007/BF00412557