LYMPHOCYTOMA OF THE SPLEEN - A SEPARATE N OSOLOGICAL ENTITY AND SUBJECT OF SPECIFIC TREATMENT POLICY

The paper presents new findings in favor of recognition of splenic lym phocytoma (SLC). This disease was characterized by A. I. Vorobyev and M. D. Brilliant in 1982 in terms of detailed clinicomorphological feat ures, prognosis and optimal treatment policy. The study included 52 pa tients (mean age 53 years) of which 36 were females and 16 males. They were followed up for 5.7 years, on the average. SLC manifested clinic ally by splenomegaly with minimally enlarged lymph nodes, morphologica lly by nodular lymphocytic proliferates in the spleen, bone marrow and liver, diffuse or diffuse-nodular proliferation in the lymph node. Pe ripheral blood contained middle-size lymphoid cells with round nuclei. SLC immunophenotype exhibits moderate or marked expression of CD22 an d membrane immunoglobulins, the absence of CD5, CD23 and EM receptor, combination CR1-/CR2+. Paraprotein secretion was recorded in 49% of ca ses. There were frequent autoimmune reactions, especially against eryt hroid cells and platelets (42%). Optimal therapeutic policy is expecta tion and eventual splenectomy producing a persistent clinical effect i n 94% of patients. In progressive disease long-term therapy with cyclo phosphamide is recommended. Thus, SLC is a mature-cell lymphatic tumor growing as a rule in the spleen. Its prognosis in valid therapy is fa vourable.