Theml H, Burger A, Keiditsch E, Enne W, Eggert K, Dietzfelbinger H, Gräff L, Zsida L
Med Klin. 1977 Jun 10;72(23):1019-32.
In ten patients with predominant splenomegaly the clinical development and progress of disease following the course of some years are described. The hematological values showed a moderate relative lymphocytosis with a slight but inconstant increase in white blood cell counts. A more or less small number of a morphologically distinctive population of plasmacytoid lymphocytes was an important diagnostic criterion and a decrease of at least one class of immunoglobulins could be shown. Only one patient presented a paraproteinemia (IgM). In the serum of six patients atypical unspecific antibodies were found. The histologic and cytologic feature of the spleens which were removed therapeutically was characterized by a mixed infiltration of lymphocytes and plasmocytoid or plasmacellular elements. By this pattern it was possible to classify these cases as lymphoplasmocytoid (resp. lymphoplasmocytic) immunocytomas according to the new Kiel-lymphoma-classification. From the clinical point of view they represented a subunit with markedly prevalent splenic proliferation. Liver and/or bone-marrow however showed in all cases primarily circumscribed infiltrations of pathognomonic cells or did develop them during the course of disease, even after splenectomy. According to the presented observations this disease is to be placed between chronic lymphocytic leukemia and M. Waldenström not only by pathological but also by clinical criteria.
