An autopsy case of Niemann‐Pick disease whichμ was characterized by hepatosplenomegaly and lymphadenopathy is reported. On microscopic examination, numerous foamy cells were observed in the liver, spleen, kidneys, bone marrow, lungs, thymus, intestine and general lymph nodes. These cells were generally negative for periodic acid‐Schiff (PAS) stain and positive for staining of phospholipid. Based upon the morphologic characteristics of Niemann‐Pick disease, its relation to other lipidosis is discussed.

Biopsy materials obtained from cervical lymph nodes and liver tissues of two male siblings, 5 years and 10 months old and 1 year and 11 months old, were studied electron microscopically. The results were summarized as follows: 1) Marked accumulation of lipid droplets, probably neutral fat, and cholesterol clefts in reticulum cells of lymph node, Kupffer cells and hepatic cells was the most prominent feature in these biopsies. 2) These lipids were found in both lysosomes and cytoplasmic matrices. Many of the lysosomes were considered to be autolysosomes or autophagosomes, because they contained fibrillar components, endoplasmic reticulum, glycogen particles and degraded organelles. It was suggested that these lipids might have been synthesized in lysosomes. 3) This disorder seems not to be included in the category of inborn lysosomal disease, and is considered as an unclassified one.ACTA PATH. JAP. 21 : 4 4 5~4 5 8 , 1971. thra 8%.&!J, ~L L I bk, ffl% Ha3 446 446 SPEUIFIC LEIDOSIS Hepatosplenomegaly gradually reached the height of the umbilicus. cervical lymph node was also noted. Recent blood chemistry : Serum triglycerides 200-270 mg% (normal value 66-163 mg%) ; total serum cholesterol 138-240mg yo (normal value 130-250 mg%) ;GOT 65-196 SFE.; GPT 39-139 SFE.; total bilirubin 0.2-0.6 mg%. The younger boy, at the age of 10 months, was noticed to have hepatomegaly and diarrhea. Hepatomegaly became remarkable whereas spleen and superficial lymph node were not palpable.Recent blood chemistry: Serum triglycerides 303-296 mgyo ; total serum cholesterol 199438 mg%; GOT 81-175 SFE. ; GPT 61-119 SFE. ; total bilirubin 0.3 mg%. X-ray photographs of the abdomen revealed no calcification of the adrenals in both siblings. Both siblings showed no xanthomata of the skin. No emaciation was noted, and intelligence appeared to be normal. A bean-sized Light Microsc~pic FindingsCervical lymph node of the elder sibling: Through the cortex and medullary cord of the lymph node many or few groups of relatively large cells were scattered. These cells had clear, somewhat eosinophilic broad cytoplasm and oval or spindleshaped nuclei, Nucleoli were sometimes recognized. Germinal centers were found in a few areas but sinuses were not prominent. Necrofiisand fibrosis were absent (Figs. 1, 2). With oil red 0 stain, these cells are stained homogenously or granularly red. Liver tissues of the two siblings: The liver architecture of the elder brother