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Monoclonal gammopathies in sézary syndrome: A report of four new cases and a review of the literature

✍ Scribed by Peter M. Kövary; Ludwig Suter; Egon Macher; Helmut Niedorf; Ekkehard Grundmann; Olinde Lukitsch; Joachim Herzberg; Hans Intorp; Everhard Schürmeyer; Darab Kamanabroo; Heinz Losse


Publisher
John Wiley and Sons
Year
1981
Tongue
English
Weight
550 KB
Volume
48
Category
Article
ISSN
0008-543X

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✦ Synopsis


Clinical and laboratory findings of four patients with Sezary syndrome and monoclonal gammopathy are presented. In the literature five cases with the association of these symptoms have been reported. Among the authors' series of 13 patients with Sezary syndrome, monoclonal gammopathy was observed far more often than could be expected from its natural incidence. It appears likely that monoclonal gammopathy is related to Sezary syndrome.

Cancer 48:788-792, 1981. EZARY SYNDROME is characterized by the triad of S pruritic erythroderma, generalized lymphadenopathy, and circulating malignant lymphocytes (SCzary cells). Both SCzary syndrome and mycosis fungoides are grouped together as cutaneous T cell lymphomas.' Cutaneous T cell lymphomas may be distinguished from B cell lymphomas by the tendency of their tumor cells to invade the upper dermis,' the epidermal compartment,'-2 and T cell regions of lymph nodes.' The dermal infiltrate generally is loose and band-like and does not develop follicle-like structures. Ultrastruc-turd1 analysis provides a useful diagnostic t00I.'.~*~ The tumor cells of cutaneous T cell lymphomas have convoluted, cerebriform or serpentine nuclei best demonstrable by electron microscopy." The nuclear abnormalities may easily be recognized in 1 p m sections of epon-embedded material ("semithin sections") by light microscope Other conditions in which similar appearing cells may be found are clinically and pathologically not likely to be c o n f u ~e d . ~, ~ Sdzary cells may retain immunoregulatory activities: Frequently they display helper functions when co-cultivated with normal B cells or B cells from pa-From the Departments of Dermatology,* Pathology,t Internal Medicine (policlinic),t Internal Medicine A;$ University of Miinster, the Department of Internal Medicine, Clemenshospital, Miinster.11 and the Department of Dermato1ogy.T Stadt. Krankenhaus St.


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