A case of myeloproliferative syndrome manifested by fever, hepatosplenomegaly and pancytopenia was described. This 21 year-old Japanese female patient had a outcome of fatal course after nine months in spite of various treatments. The diagnosis of this case was very difficult although many investigations were done. We had suspected malignant lymphoma or malignant reticulosis, but could not diagnose definitely before autopsy.
There was marked hepatosplenomegaly, but no evidence of the swelling of lymph nodes were seen. The autopsy showed neither tumor formation nor focus of infection, and bone marrow revealed a compact red marrow. The microscopic examination revealed the marked increase of erythroblastic, granulocytic and megakaryocytic series in the bone marrow, and marked infiltration of mature neutrophils and erythroblasts in the sinusoid of the liver. This was also seen in the spleen and lymph nodes.
These findings were compatible to the myeloproliferative syndrome advocated by Dameshek in 1951. This syndrome includes acute and chronic granulocytic leukemia, polycythemia vera, acute and chronic myelosclerosis with myeloid metaplasia, Di Guglielmo syndrome, thrombocythemia and other disorders of the proliferation of one or more marrow constituents. In this context, this rare case was included into myeloproliferative syndrome but did not belong to any single entity of this syndrome.
