Clonal and non-clonal karyotypically abnormal cells in haemophagocytic lymphohistiocytosis

We studied chromosomes in bone marrow (BM) or peripheral blood cells of nine patients with haemophagocytic lymphohistiocytosis (HLH); three of them had a family history of HLH and four others underwent concurrent Epstein-Barr virus (EBV) infection. In addition to a large population of normal mitotic cells, karyotypically abnormal clonal cells were found in two patients, abnormal clonal cells and a nonclonal (single) abnormal cell in one, and nonclonal abnormal cells in three. All the six patients with chromosome abnormalities died of progressive disease; one of them also had EBV infection and EBV-associated clonal proliferation. Two of three patients with EBV infection and only normal mitotic cells in BM completely recovered from the disease.
Although HLH did not show histological and/or haema-tological evidence of a neoplastic disease, clonal chromosome abnormalities and the fatal clinical outcome found in some of the patients suggest that the disease may be heterogenous and include malignancy. HLH patients with karyotypically abnormal clonal cells in BM should warrant more intensive chemotherapy than that presently being applied to them and should be considered as candidates for BM transplantation.     

Drug Metabolism: Activation of 11 β-Hydroxysteroid Dehydrogenase by Dehydroepiandrosterone Sulphate as an Anti-hypertensive Agent in Spontaneously Hypertensive Rats

The anti-hypertensive properties of dehydroepiandrosterone sulphate (DHEAS) have been investigated by studying its effects on blood pressure, on serum concentrations of corticosterone and dehydrocorticosterone, and on 11 β-hydroxysteroid dehydrogenase (11 β-HSD) activity in spontaneously hypertensive rats (SHR). SHR were given intraperitoneal injections of DHEAS (10 mg day^?1 for 70 days) from six to 16 weeks of age. The blood pressure–time curve was significantly (P<0.05) suppressed immediately after administration of DHEAS. There was no difference between the heart rates of control and DHEAS groups. Serum concentrations of corticosterone and dehydrocorticosterone in the DHEAS group were significantly (P < 0.05) lower than those of the control group. The dehydrocorticosterone/corticosterone concentration ratio was, however, significantly (P < 0.05) higher in the DHEAS group, suggesting that treatment with DHEAS enhanced the overall interconversion of corticosterone to dehydrocorticosterone. The activity of 11 β-HSD in specific organs of the DHEAS group was affected, characteristic changes being increases in the kidney (14–58%), decreases in the liver (11–27%) and no change in the testis. Direct addition of DHEAS to 11 β-HSD preparations from the kidneys of control SHR had the same effect as that observed in the in-vivo experiments. The fall in serum corticosterone in the DHEAS group is considered to be related, at least partly, to increased activity of kidney 11 β-HSD. The inverse correlation of kidney 11 β-HSD activity with serum corticosterone and blood pressure (—r = 0.628, P < 0.01, and —r = 0.478, P < 0.05, respectively) suggest that DHEAS delayed the development of hypertension in SHR by selective promotion of kidney 11 β-HSD activity which in turn resulted in lower serum concentrations of corticosterone and its minimal aldosterone-like activity.     

Chronic expanding hematoma in the psoas muscle

We report an unusual case of chronic expanding hematoma in the psoas muscle. A 53-year-old man was admitted for evaluation of a mass shadow in the left lower lung field on chest X-rays. He had also been suffering from dull left back pain. A computed tomography scan showed a cystic lesion with a rim enhancement in the left retroperitoneal space. Mixed signal intensity in a mosaic pattern was seen on a T2-weighted magnetic resonance image. We could not rule out a suspicion of a neoplastic intratumoral hemorrhage. Due to increased pain and the definite diagnosis, surgery was performed. Histopathological examination confirmed the diagnosis of chronic expanding hematoma. The expansion process is thought to be due to the irritant effects of blood and its breakdown products, which cause repeated exudation and bleeding from capillaries in the granulation tissues.     

A new oral margarine loading test in children

Margarine (2g/kg) was orally administered to 39 children with normal fat digestion and absorption (control group) and 17 children with abnormal fat digestion and absorption (malabsorbed group). The serum triglyceride (TG) level was serially determined by capillary blood sampling. In the control group, the TG level was significantly elevated after 2, 3 and 4 h as compared with the level before margarine ingestion (basal TG). The maximum TG elevation rate [MTER - (maximum TG-basal TG)/basal TG x 100] was > 50% in all but three children. In contrast, a significant increase in TG was not observed in the malabsorbed group after margarine ingestion, and the MTER was < 50% in all but two children. The MTER was significantly lower in children with a high microscopic score of fecal fat than that in children with a low score. This oral margarine loading test is a simple and semi-quantitative evaluation of fat digestion and absorption, and is considered to be useful for the assessment of the presence or severity of fat maldigestion and malabsorption in children.     

A study of verbal and spatial information processing using event-related potentials and positron emission tomography

Abstract  The activated cerebral regions and the timing of information processing in the hemispheres was investigated using event-related potentials (ERP) and regional cerebral blood flow (rCBF) as the neurophysiological indicators. Seven men and one woman (age 19–27 years) were asked to categorize two-syllable Japanese nouns (verbal condition) and to judge the difference between pairs of rectangles (spatial condition), both tests presented on a monochrome display. In the electroencephalogram (EEG) session, EEG were recorded from 16 electrode sites, with linked earlobe electrodes as reference. In the positron emission tomography (PET) session, rCBF were measured by the ¹⁵O-labeled H₂O bolus injection method. Regions of interest were the frontal, temporal, parietal, occipital and central lobes, and the entire cerebral hemispheres. When the subtracted voltages of the ERP in homologous scalp sites were compared for the verbal and spatial conditions, the significant differences were at F7-F8 and T5-T6 (the 10–20 system). The latencies of the differences at T5–T6 were around 200, 250 and 320 ms. A significant difference in rCBF between the verbal and spatial conditions was found only in the temporal region. It was concluded that early processing of information, that is, registration and simple recognition, may be performed mainly in the left temporal lobe for verbal information and in the right for spatial information.     

A CASE OF FOAMY MYOCARDIAL TRANSFORMATION OF INFANCY

A case of Infantile cardiomyopathy in a year and four months old girl, which was clinically characterized by tachycardia resistent to any drugs and marked hyperplasia of mitochondria in their cytoplasms on electron microscopic observation, was presented. In the present case light and electron microscopical proof of the diagnosis was obtained by right endomyocardial biopsy, while most of this condition has not been recognized prior to autopsy. In Japan this newly recognized infantile cardiomyopathy has not been reported, and we proposed that this condition could be designated as mitochondrial cardiomyopathy caused by unknown etiology. The literatures described previously in the world have been reviewed.     

Demonstration of Somatostatin Production in Medullary Carcinoma of the Thyroid

The localization and release of somatostatin in seven casesof medullary carcinoma of the thyroid were investigated by meansof specific anti-somatostatin antibody. In all four patientsexamined high concentrations of plasma somatostatin (125–400pg/ml) and calcitonin (1,917–82,000 pg/ml) were shown.Primary and metastatic tumor tissues obtained from three patientscontained higher amounts of somatostatin (13.1–350.0 ng/gwet wt) than the surrounding tissues. Immunohistochemically,somatostatin-immuno-reactive (IR) cells and calcitonin-IR cellswere shown to compose the majority of tumor cells, leaving asmall number of nonreactive cells in primary and metastatictumors of the seven cases, but in general calcitonin-IR cellswere more numerous than somatostatin-IR cells. The numbers ofcalcitonin-IR and somatostatin-IR cells were not markedly differentfrom case to case, but their population density did not alwayscorrespond to the concentrations of plasma and tissue somatostatin.These data indicate that medullary carcinoma of the thyroidis one of the somatostatin-producing tumors.