AS MEDICINE advances, our concepts of disease entities also must change as experience dictates. Letterer-Siwe disease is a malignant histiocytosis of infants which is generally considered neither hereditary nor infectious.1,2
The disease today is characterized by an acute onset with hepatosplenomegaly, generalized lymphadenopathy, progressive anemia, neutropenia, lymphocytosis, and often hemorrhagic phenomena. Multiple focal, osteolytic lesions of the skull, vertebrae, pelvis, and ribs are present. Histopathology is characterized by nonlipoid hyperplasia of the reticuloendothelial system. The disease in the infant is usually rapidly fatal within a few months.
Report of Cases
In January 1949, 8-month-old male twins were seen with a presumptive diagnosis of acute lymphatic leukemia, which subsequently was proved by biopsy to be nonlipoid reticuloendotheliosis (Letterer-Siwe disease)3 (Figure).Multiple antibiotic therapy to the point of stool sterilization led to dramatic improvement of twin A. Antibiotics were then prescribed for twin B, who promptly showed the same
Bierman HR. Apparent Cure of Letterer-Siwe Disease: Seventeen-Year Survival of Identical Twins With Nonlipoid Reticuloendotheliosis. JAMA. 1966;196(4):368–370. doi:10.1001/jama.1966.03100170110041
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