CHAPTER 18 



LYMPHOPROLIFERATIVE DISORDERS 



WILLIAM DAMESHEK 



The lymphoproliferative disorders include both isolated and generalized 

 proliferations of lymphoid tissue. The generalized proliferations may be 

 benign, reactive, and self-limited — as in certain infectious disorders and in 

 the primary immune response. The localized proliferations of lymphoid 

 tissue are neoplastic, although their degree of malignancy varies greatly. In 

 varying lengths of time, they tend to become generalized. There is little if 

 any distinction between the generalized lymphosarcomatous processes and 

 lymphocytic leukemia; histologically, they are indistinguishable. 



Chronic lymphocytic leukemia is perhaps the commonest of the forms 

 of leukemia in the West. There are "aggressive" types with considerable 

 lymphadenopathy, splenomegaly, and systemic manifestions. There are 

 "benign" types without lymphadenopathy or splenomegaly and completely 

 asymptomatic. Autoimmune hemolytic anemia occurs in both these types 

 and is often by far the important feature of the leukemic process. Hyper- 

 globulinemia and hypogammaglobulinemia may be found. 



Macroglobulinemia may occur in occasional cases of generalized lympho- 

 cytic proliferation and may sometimes be the outstanding manifestation. 

 With a considerable quantity of heavy globulin, hemorrhagic disease may 

 ensue and numerous infections may develop, presumably — as in multiple 

 myeloma — because normal antibody globulin production is diverted to the 

 production of this abnormal globulin. Waldenstrom's macroglobulinemia 

 is probably best considered as a form of lymphoproliferative disease. 



The lymphocytic tissues are involved in anitbody production. Thus, it is 

 not strange that when they are proliferating in a general fashion, the produc- 

 tion of abnormal globulins is greatly increased. Some of these may be cell- 

 specific (as against red cells, i.e. autoimmune hemolytic anemia); at times 

 other manifestations are seen, including thrombocytopenic purpura of the 



