A long course of leukocytopenia and splenomegaly with extramedullary hematopoiesis in the absence of clinically manifested rheumatoid arthritis.

A 70-year-old female with a long history of progressive leukocytopenia and giant splenomegaly is described. She had no clinically manifested rheumatoid arthritis, although she complained of slight arthralgia in the digital joints, wrists and ankles at irregular intervals. Repeated bone marrow aspirations showed no cellular atypism, chromosomal abnormalities, or phenotypical abnormalities. Just before splenectomy, both anti-neutrophil antibody positivity and anti-cyclic citrullinated peptide antibody positivity were shown. Histology of the splenectomized spleen showed follicular hyperplasia with plasmacyte infiltration and extramedullary hematopoeisis. After splenectomy, leukocyte counts returned to normal with normal leukocyte differentials and anti-neutrophil antibodies disappeared. She was almost free of arthralgia one year after splenectomy, although the anti-cyclic citrullinated peptide antibody titers remained high.