Digital microscopy as a screening tool for the diagnosis of hereditary hemolytic anemia.

INTRODUCTION: Evaluation of red blood cell (RBC) morphology is an important first step in the differential diagnosis of hereditary hemolytic anemia. It is, however, labor intensive, expensive, and prone to subjectivity. To improve and standardize the analysis of RBC morphology as a screening tool in the diagnosis of hereditary hemolytic anemia, we studied its automated analysis by digital microscopy (DM). METHODS: Blood from 90 patients with hereditary hemolytic anemia and 32 normal control subjects was analyzed by the CellaVision DM96 Digital Microscope. RESULTS: All hemolytic RBC abnormalities could be distinguished by the presence of at least one aberrant red cell type. In particular, the percentage of microcytes was highly sensitive and specific (AUCROC  = 0.97) for RBC membrane disorders, and a cut-off of 5.7% microcytes was calculated to be optimal to distinguish patients from healthy controls. Subgroup analysis of patients with RBC membrane disorders revealed additional distinct differences according to the underlying gene defect. A number of cell types were significantly elevated in sickle cell anemia patients, such as polychromatic cells, macrocytes, and poikilocytes. The increase in helmet cells (AUCROC  = 0.96) and hypochromic cells (AUCROC  = 0.91) was specific for ß-thalassemia, whereas patients with pyruvate kinase deficiency showed a significant increased polychromatic cells, macrocytes, and ovalocytes. Patients with hereditary xerocytosis showed significantly higher numbers of polychromatic cells, macrocytes, and target cells. CONCLUSION: DM holds a promise as a useful screening tool in the diagnosis of hereditary hemolytic anemia by detecting and quantifying distinct morphological changes in RBCs in patients with various forms of hereditary hemolytic anemia.

[Determination of length, width and height of the human skull by critical use of roentgenologic craniometry methods]. / Zur Bestimmung von Länge, Breite und Höhe des menschlichen Schädels unter kritischer Anwendung der Methoden der röntgenologischen Kraniometrie.

X-ray craniometry has gained in importance for scientific and clinical examination. It enables accurate measurement of shape and structure of the human skull, its characterisation and an accurate description of the type and cause of changes of its shape and components. Roentgenological craniometry is closely related to craniometry of anatomists and anthropologists, but in a few essential points it does differ from it--rather fundamentally, in fact. On using the roentgenological method, the general laws of x-ray imaging must be strictly observed and measurements must be effected only if they are conducted from such points which are roentgenologically clearly defined. Measurements must be definite, repeatable at any time and suitable for comparative purposes. The influence exercised by these conditions becomes noticeable already on determining the three principal dimensions of the cranium. Whereas the x-ray determination of the length and breadth of the cranium is easy to perform and does not present any problem, determination of the "height" of the cranium causes considerable difficulties that are mainly based on the statement of suitable points of measurement. Detailed studies and deliberations have shown that the distance between the vault and the base of the skull can supply a suitable measure for assessing the height of the skull. On lateral x-ray film the distance between the "endobregma" and the "bony floor of the hypophyseal cavity" is a suitable criterion, whereas on the sagittal x-ray films of the skull the criterion is the distance between "bregma" and "hypophyseal basis". The "lateral hypophyseal height of the skull" and the "sagittal hypophyseal height" are largely equal, clearly defined and always repeatable.(ABSTRACT TRUNCATED AT 250 WORDS)

Extramedullary hematopoiesis. Demonstration by transmission and emission computed tomography.

Extramedullary hematopoiesis occurring in the thorax is a well-known complication of a number of various blood dyscrasias, and its appearance with planar Tc-99m sulfur colloid marrow scintigraphy and transmission computed tomography (CT) has been described previously. The authors present a case of thoracic extramedullary hematopoiesis evaluated by CT and emission computed tomography (ECT).

Mathematical analysis of 51Cr-labelled red cell survival curves in congenital haemolytic anaemias.

The parameters of 51Cr labelled red cell survival curves were calculated in 33 patients with homozygous beta-thalassaemia, 8 with sickle-cell anaemia and 3 with s -- beta-thalassaemia, using a non-linear weighted least squares analysis computer program. In thalassaemic children the calculated parameters denote that the shortening of the mean cell life is due to early senescence alone, while there is some evidence that in thalassemic adults additional extracellular destruction mechanisms participate as well. Red cell survival curves from patients with sickle-cell anaemia and s -- beta-thalassaemia resemble each other, while their parameters indicate an initial rapid loss of radioactivity, early senescence and the presence of extracellular red cell destruction factors.

[Double contours and companion shadows in the skull associated with anaemias (author's transl)]. / Doppelkonturen und Begletischatten am Schädel bei anämischen Erkrankungen.

Lateral X-ray pictures of the skull in certain several and chronic disorders of the blood as thalassaemia, congenital haemolytic anaemia, sickle cell anaemia and iron deficiency disease show frequently changes of the outlines of the cranial bones. They consist of a double contour of the outer cranial border and on the interior side below the sagittal sutura of a band-like shadow or of multiple stripes and lines running parallel to the carnial vault. They concern the parietal bones and may extend from the bregma till to the lamda or occupy only a part of this distance. The roentgenological features are due to the enlargement of the crainal bones, the bulging out of both parietal bondes, the sometimes enlarged and deepened borders of the sulcus sagittalis superior and particularly to the furrow and depression on the skull above the sagittal suture caused by the protruding parietal bones on both sides. As these different abnormal structures must be passed by the picturing X-ray, effects of superposition and interference are produced. Longitudinal ridges or bony edges which could explain the roentgenological findings could not be established. Since the peculiar alterations of the cranial bones are mainly found in the mentioned blood disorders, where they are caused by the overgrowing red marrow, they also display the same roentgenological features. These features are, therefore, a characteristic sign of these diseases.