Abstract
A systemic and neoplastic proliferation of the primitive reticular cells or their derivatives is often termed malignant reticulosis, malignant reticuloendotheliosis or malignant reticulohistiocytosis. Because of rarity and variability of the clinical picture in this disease, no distinct clinical and pathological concept of this entity has been given.
We have reported 3 different types of so-called malignant reticulosis, which took a rapid fatal course.
The first case was a 6 year old boy, who developed a remittent fever, and hepatosplenomegaly one month prior to admission. Atypical primitive reticular cells were seen in the peripheral blood and bone marrow smears. Histologically, the bone marrow, liver, spleen and lymph nodes were mainly involved. The morphologically malignant reticular cells were infiltrated difusely, without producing tumor, and often, the cells phagocitized red blood cells. From these findings, this case might be categolized into leukemic reticulosis.
The 2nd case was a one year and 2 month old female with Letterer-Siwe's disease, in which infiltration of the more differentiated reticulum cells was observed. The 3rd case was mostly considered as a malignant reticulosis due to clinical findings but autopsy was not performed.