LETTERER-SIWE DISEASE

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Abstract
In 1924 Letterer1described a disease characterized by enlargement of the spleen and the liver, associated with anemia and a purpuric eruption. The patient in Letterer's case was an infant 6 months old, who died within four days after admission to the hospital. The disease was characterized histologically by a proliferation of large, pale reticuloendothelial cells which invaded and replaced the normal structure of the spleen, liver, bone marrow, skin and lymph nodes. Letterer reported his case under the title, "Aleukämische Retikulose (Ein Beitrag zu den proliferativen Erkrankungen des Retikuloendothelialapparates)." During the next nine years several similar cases were reported, and in 1933 Siwe2reported an additional case, of an infant 16 months old, and grouped all the cases together as representing a well defined entity. The term Letterer-Siwe disease was coined by Abt and Denenholz,3who considered the disease in great detail. Letterer-Siwe disease is characterized