Three Kinds of Foamy Cells in the Spleen: Comparative Histochemical and Ultrastructural Studies

By light and electron microscopy, we observed foamy cells in the spleens from a patient with hemolytic anemia due to red cell adenosine deaminase (ADA) overproduction, a patient with rheumatoid arthritis (RA) treated with gold, and patients with idiopathic thrombocytopenic purpura (ITP)

The foamy cells associated with red cell ADA overproduction were essentially similar to Gaucher-like cells described in patients with thalassemia, and it was suggested that the accelerated destruction of red cells was one of the factors responsible for the development of foamy cells. Foamy cells in ITP and RA were closely associated with an increased destruction of platelets in the spleen. Morphologic transitions between phagocytosed platelets and myelinlike materials were traced in these disorders. In RA, however, foamy cells were heterogeneous from an ultrastructural standpoint, with different cytoplasmic inclusions. In addition to myelinlike materials, dense bodies, vacuoles with flocculent materials, and gold were noted in most of foamy cells. As gold compounds are known to inhibit lysosomal enzymes, we surmise that an acquired disturbance in lysosomal digestion is partially responsible for the accumulation of intermediate metabolites.

In the pathogenesis of foamy cells associated with blood cell dyscrasia, the accelerated destruction of blood cells and/or acquired disorders in catabolic pathways within the macrophages are suggested to be the underlying mechanism of an intralysosomal accumulation of incompletely degraded cellular debris.     

Bone resorption versus estrogen loss following oophorectomy and menopause

OBJECTIVES: To investigate if menopause and oophorectomy may represent different risk factors for bone resorption/loss. METHODS: The urinary levels of pyridinoline (Pyr) and deoxypyridinoline (D-pyr), the serum levels of type I carboxy-terminal pyridinoline cross-linked telopeptide (ICTP), and lumbar bone mineral density (BMD), were compared in 80 Japanese women after menopause or oophorectomy. These women were divided into four groups of 20 women each as follows: early postmenopausal stage (early physiologic menopause < 3 years before study entry); late postmenopausal stage (physiologic menopause > or = 3 years before study entry); early postoophorectomy stage (oophorectomy < or = 03 years before study entry); or late oophorectomy stage (oophorectomy > 3 years before study entry). RESULTS: Lumbar BMD was significantly lower in the late groups compared to their respective early groups and was lowest in the late postoophorectomy group. The ratio of D-pyr/creatinine (Cr) was not significantly different among the four groups. The ratio of Pyr/Cr was significantly higher in the early postoophorectomy subjects compared with either late group. The serum level of ICTP was significantly higher in the early postoophorectomy group compared to all other groups. CONCLUSIONS: These findings suggest that serum ICTP may be useful in detecting changes in bone resorption after oophorectomy and that women are at greater risk for bone resorption after oophorectomy than after physiologic menopause, although this difference appears to diminish with time.     

RMRP mutations in Japanese patients with cartilage-hair hypoplasia

We examined 12 Japanese patients with metaphyseal chondrodysplasia (MCD) for mutations in the ribonuclease mitochondrial RNA processing gene (RMRP), and identified four novel mutations in two patients with typical and atypical cartilage-hair hypoplasia (CHH), a form of MCD characterized by extra-skeletal manifestations including hypoplastic hair and defective immunity. A patient with typical CHH had a 17-bp duplication at +3 and a de novo 182G > A. The other patient with atypical CHH had a 17-bp insertion at -20 and a 218A > G. Expression analysis revealed that the allele with this insertion mutation in the promoter region silenced the gene. Spectrum analysis of the mutations and polymorphisms in RMRP showed marked difference between the Japanese and other ethnic groups. Such ethnic and phenotypic difference should be taken into account in mutation analysis of the gene.     

A novel synthetic route to poly(β-ketone)s via poly(alkoxyethyne)s

Isopropoxyethyne (1a) and tert-butoxyethyne (1b) were polymerized using group 5 and 6 transition metal catalysts to give poly(isopropoxyethyne) (2a) and poly(tert-butoxyethyne) (2b) , respectively. The weight-average molecular weight (M?_w) of the resulting poly(alkoxyethyne)s was up to 1.0 × 10⁴. Among the transition metal catalysts, a tungsten alkoxide or a molybdenum alkoxide having low Lewis acidity were found to effectively promote the polymerization without causing side reactions. Poly(tert-butoxyethyne) was successfully converted to poly(β-ketone) 3 by acid hydrolysis of the tert-butyl vinyl moiety.     

Polymer synthesis based on 2-(hydroxyphenyl)-2-oxazolines

2-(Hydroxyphenyl)-2oxazolines(p- and n-isomers 1a and 1b ) were used for the synthesis of various polymers. Cationic polymerization of 2-methyl-2-oxazoline initiated with the N-methyl 2 -oxazolinium species 3 , Derived from 1 , Gave telechelic poly(N-acetylethylenimine) ( 5 ) having a hydroxyl group of different reactivity at both ends. Anionic polymerization of ethylene oxide induced by the lithium salt of 1, 7 , produced a poly(ethylene oxide) macromer, 9 , having a ringopening polymerizable 2-oxazoline group. The copolymer from 9 and 2-phenyl-2oxazoline, 10 , has been shown to have good properties as a nonionic polymeric surfactant in which the N-benzoylethylenimine main-chain behaves as a lipophilic group whereas the oxyethylene graftchains act as a hydrophilic group. Di-salt 11 derived from 1 was polymerized to give polymide 13 (“amphi-ionic polymerization”).     

Abnormal distribution of cathepsins in the brain of patients with Alzheimer's disease

Formalin-fixed paraffin-embedded hippocampal sections of brains with early-onset and late-onset Alzheimer's disease were studied immunohistochemically with antisera against cathepsin D and cathepsin B. In addition to the staining of neuronal perikarya, some of the senile plaques visualized by Bielshowsky silver staining and some of reactive astrocytes were positively stained with the antisera against cathepsin D and cathepsin B in brains with Alzheimer's disease. Abnormal localization of cathepsin D and cathepsin B immunoreactivity in neuronal perikarya was observed in brains with early-onset Alzheimer's disease. These findings demonstrate that the distribution of lysosomal proteases was altered in brains with Alzheimer's disease, suggesting the primary and/or secondary involvement of the lysosomal proteases in the pathological process of Alzheimer's disease.     

Intriguing Case: Endolymphatic Stromal Myosis Coexisting with Adenocarcinoma of the Uterus

Carcinosarcoma of the uterine corpus containing endolymphatic stromal myosis (ESM) is extremely rare. This report describes the light- and electron-microscopic findings of ESM coexisting with adenocarcinoma of the uterus in a 58-year-old female. The polypoid tumor originated from the fundus uteri and filled the uterine cavity. In addition to papillary and medullary acinous adenocarcinoma at the apex of the polypoid mass, the major portion of the tumor specimen was composed of cells resembling endometrial stromal cells that infiltrated the myometrium and lymphatic channels, and a diagnosis of ESM was made due to the relative cell uniformity, rare mitoses, and the presence of invasive growth. There have been few reports on the ultra-structure of ESM and endometrial stromal sarcoma, and there are no reports on the ultrastructural difference between these tumors. In addition to the ultrastructural observations of our case, the electron-microscopic findings of previous reports are discussed.     

IMMUNOELECTRON MICROSCOPIC LOCALIZATION OF THYROGLOBULIN IN HUMAN FOLLICULAR ADENOMA

An electron microscopic immunohistochemical localization of thyroglobulin (TG) using PAP methods has been made in 15 cases of cold follicular adenoma. All cases of follicular adenoma showed organ specific functions such as synthesis, storage, reabsorption, and hydrolysis of thyroglobulin except for an area composed of follicular cells with trabecular arrangement. Immuno-reaction product for TG was precisely demonstrated in follicular lumina, subapical vesicles and reabsorbed colloid droplets. The reaction product observed in the follicular lumen was clearly demarcated from the cytoplasm of the follicular cells by the apical plasma membrane. The subapical vesicles ranging approximately from 50 mμ to 300 mμ in diameter were rarely observed in follicular adenoma and some of them fused with the reabsorbed colloid droplets. The reabsorbed colloid droplets usually had the intense reaction product and hydrolyzed colloid droplets had a vacuole containing floccular low electron dense materials. There is no reaction product in rough endoplasmic reticulum and Golgi complexes.     

Functional evaluation of lung by Xe-133 lung ventilation scintigraphy before and after lung volume reduction surgery (LVRS) in patients with pulmonary emphysema

We evaluated the respiratory functions of patients with pulmonary emphysema who underwent lung volume reduction surgery (LVRS) by the mean transit time (MTT) with Xe-133 lung ventilation scintigraphy, forced expiration volume in 1 sec (FEV1.0), residual volume (RV), distance walked in 6 min (6-min walk), and the Hugh-Jones classification (H-J classification) before and after LVRS. In 69 patients with pulmonary emphysema (62 men, 7 women; age range, 47-75 years; mean age, 65.4 years +/- 6.1, preoperative H-J classification, III (two were II)-V) who underwent LVRS, all preoperative and postoperative parameters (MTT 3 weeks after LVRS and the others 3 months after LVRS) were judged statistically by the Wilcoxon signed-ranks test and Odds ratio. Every postoperative parameter was improved with a significant difference (P < 0.05) compared to preoperative parameters. MTT at 3 weeks after LVRS was not associated with %FEV1.0 and the H-J classification at 3 months after LVRS, but was associated with RV and a 6-min walk at 3 months after LVRS. MTT was useful for the clinical evalution of aerobic capability after LVRS.