Splenic Neutropenia in the Felty Syndrome

Abstract

Abstract A 59 year old woman with chronic rheumatoid arthritis of twenty years' duration developed chronic granulocytopenia and excessive susceptibility to infection. Mild anemia and symptomless thrombocytopenia were also present. Eight marrow punctures over two and one half years all showed what appeared to be maturation arrest affecting the neutrophils so that segmented polymorphs alone were reduced in number. Throughout this period no form of therapy, including a five day course of ACTH, was effective in promoting normal granulopoiesis; only at the end of this period did the spleen become palpable. Splenectomy resulted in complete symptomatic cure which has been maintained for one year but hematologic cure was not fully obtained. It is believed that this case of splenic neutropenia is really an exaggerated form of Felty's syndrome for the literature shows that malignant neutropenia is not rare in this disease. Furthermore analysis of the literature on splenic neutropenia reveals other cases of an essentially similar nature and the suggestion is put forward that Felty's syndrome may be a fairly common cause of what has been regarded as primary splenic neutropenia. The possible integration of these findings with the immunologic leukopenic mechanism of Moeschlin is briefly discussed.