Die Wertigkeit der Adenosintriphosphatase Reaktion zur Diagnostik von essentiellen Paraproteinämien

Zusammenfassung Bei 26 Fällen mit essentieller Paraproteinämie wurde die cytochemische Aktivität der Adenosintriphosphatase (ATPase) in den Plasmazellen des Knochenmarks untersucht. 15 Fälle zeigten eine erniedrigte und 11 Patienten eine normale ATPase-Aktivität. Die Untersuchungen zeigen, daß die Erniedrigung der ATPase für die Diagnostik des Plasmocytoms nicht spezifisch ist.Herrn Prof. Dr. W. Hoffmeister, Mannheim, zum 65. Geburtstag.     

Über zwei neue Fälle von hereditärer nichtsphärocytärer hämolytischer Anämie bei Glucose-6-Phosphat-Dehydrogenase-Defekt in einer norddeutschen Familie

Zusammenfassung Bei zwei Geschwistern norddeutscher Abstammung findet sich das Krankheitsbild einer hereditären nichtsphärocytären hämolytischen Anämie, dem ein Defekt der Glucose-6-Phosphatdehydrogenase der Erythrocyten zugrundeliegt. Das Protein weist von der Norm abweichende qualitative Eigenschaften auf. Die histochemisch belegbare Heterozygotie der Mutter für die anomale genetische Information wird bei biochemischer Untersuchung der Gesamtpopulation der Erythrocyten durch den relativ hohen Anteil der normalen Erythrocyten überdeckt. Das klinische Bild des einen der beiden Geschwister ist durch ein zusätzliches kongenitales Vitium cordis (Vorhofseptumdefekt vom Ostium secundum-Typ) kompliziert.

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Summary Two brothers of a family in North-Germany have a hereditary non spherocytic hemolytic anemia with glucose-6-phosphate dehydrogenase deficiency in erythrocytes. The activity of the enzyme was 20% resp. 10% of that found in normal red cells. In the patients the enzyme protein were showing not only a quantitative difference from normal activity, but also a qualitative abnormality of the protein. In the parents the behaviour of the enzyme protein is normal. The total enzyme level of the mother's red cells is in the normal range, but histochemically the heterozygosity (ie146-1) were found. One of the patients were splenectomized without response. In the other patient a congenital heart disease (ostium secundum defect) is associated with the anemia.

     

Allergoprints in Horse Allergic Children

In order to determine the allergenic activity of five purified horse allergens, 22 children allergic to horses according to history, skin test, and leukocyte histamine release were evaluated. Washed leukocytes from all patients were tested for allergen-induced histamine release utilizing four epidermal horse allergens (Ags 6, 9, 11 , and 15) and horse serum albumin. Crossed radioimmunoelectrophoresis was carried out with a standardized horse dander extract and serum from each patient.
The results showed considerable variation in the individual allergoprints. Ag 11 had the highest mean allergenic activity. Sensitivity to horse serum albumin was demonstrated three times. Our data show that the amount of serum IgE antibodies bound by horse allergens correlates significantly with the capacity of the allergens to induce histamine release from washed leukocytes.     

Syncytial organization of acanthors of Polymorphus minutus (Palaeacanthocephala), Neoechinorhynchusrutili (Eoacanthocephala), and Moniliformis moniliformis (Archiacanthocephala) (Acanthocephala)

The fine structures of immature and of developed shelled acanthors of three species belonging to the three subgroups of the Acanthocephala were investigated. Acanthors are surrounded by four eggshells (embryonic envelopes) and are composed of three syncytia: a frontal syncytium, a central syncytium, and an epidermal syncytium. Neither a sense organ nor a nervous system has been found. The central syncytium shows a mass of condensed nuclei and 12 decondensed nuclei and gives rise to 10 anterior/posterior subepidermal myofibrillar systems and 2 oblique retractor muscles. Circular muscles are missing. A single decondensed nucleus can be assigned to each of the 12 muscular systems. The epidermal syncytium embeds the other two syncytia and forms the wrinkled epidermis, which shows an extracellular glycocalyx and intrasyncytial condensations. Prominent recurved hooks, which mark the anterior end of each acanthor, and body spines are intraepidermal differentiations. Partly branched tubular infoldings of the epidermal plasma membrane of the acanthor exist and represent precursors of the pore ducts typical of the adult epidermis. Autapomorphies in the ground pattern of the monophylum Acanthocephala are the four eggshells, the early development of three syncytia, the condensed nuclei in the central syncytium, and the differentiation of ten longitudinal muscle bands and two muscle retractors and of intraepidermal hooks and spines. The syncytial organization of the epidermis with intraepidermal skeletal condensations and infoldings of the apical plasma membrane are characteristics inherited from a stem species common to Acanthocephala, Seison, and Rotifera. Received: 25 September 1996 / Accepted: 10 October 1996     

PCR-based assays for the detection of monoclonality in non-Hodgkin's lymphoma: application to formalin-fixed,paraffin-embedded tissue and decalcified bone marrow samples

In crucial cases the diagnosis of non-Hodgkin's lymphoma (NHL) still represents a challenge to the pathologist since morphological criteria do not always help to distinguish between reactive and malignant lymphoproliferations. Clonality assays are a useful supplement since monoclonal cell proliferation is strong evidence for malignancy. The polymerase chain reaction (PCR) can be utilized to establish the clonal origin of B-or T-cell lymphocyte populations by amplification of rearranged immunoglobulin and T-cell receptor (TCR) genes. In the present study DNA was isolated from a variety of neoplastic and nonneoplastic formalin-fixed, paraffin-embedded lymph nodes (n=62), cutaneous tissue (n=9), samples of miscellaneous origin (n=11), and, reported here for the first time, decalcified bone marrow samples (n=35). These samples were submitted to PCR-based assays directed against the immunoglobulin heavy-chain (IgH), immunoglobulin light-chain (IgL), and TCR chain genes. The impact of various decalcifying agents on the ability to amplify DNA was investigated by PCR-based amplification of a single copy gene. Buffered and nonbuffered EDTA was found not to impede amplification of DNA fragments up to 300 bp in length. In lymph node and cutaneous specimens monoclonality was detected in 83% of B-NHL cases using a seminested PCR approach for the amplification of IgH, whereas the same approach gave rise to monoclonal bands in 80% of bone marrow samples. The subsequent amplification of IgL helped to raise the sensitivity of detection to 94%. Monoclonality was detected in seven of nine T-cell NHLs by amplification of TCR. Most of the false-negative results were related to DNA extracted from centroblastic-centrocytic lymphoma and lymphoplasmocytic immunocytoma (37% negative each). PCR-based rearrangement analysis of immunoglobulin and TCR chain genes should be used in diagnostic pathology for cases which are histopathologically and immunohistochemically questionable. The application of clonality assays to bone marrow samples previously decalcified with EDTA provides a new tool for the detection of minimal residual disease.Abbreviations BALT bronchus-associated lymphoid tissue - dNTP deoxynucleoside triphosphate - Ig immunoglobulin - IgH immunoglobulin heavy chain - IgL immunoglobulin light chain - MALT mucosa-associated lymphoid tissue - NHL non-Hodgkin's lymphoma - PCR polymerase chain reaction - TCR T-cell receptor     

Enterococcal septicemia in patients with hematological malignancies

Thirty-six cases of enterococcal septicemia in patients with hematological malignancies were reviewed retrospectively and categorized according to their clinical significance using strict previously described definitions. Overall, most of the infected patients were males (77 %), had acute leukemia (64 %), had recently received cytotoxic drug therapy (86 %), were granulocytopenic at the onset of septicemia (77 %), and acquired the infection during hospitalization (77 %). The source of septicemia was unknown in 18 (50 %) patients, intestinal in 15 (42 %) and intravascular in three (8 %). Mortality was 19 % among 21 inpatients who had clinically significant septicemia and 30 % among patients with septicemia of uncertain clinical significance. The fatal outcome could be definitively attributed to enterococcal septicemia in only one of the nine inpatients who died. Clinically significant septicemia appeared somewhat more frequently to be polymicrobial (p=0.06), whereas septicemia of unknown significance presented more frequently as breakthrough septicemia (p=0.013). Unless associated with intravascular infection, enterococcal septicemia in patients with hematological malignancies seems to represent a marker of cytotoxic drug damage of the intestinal mucosa rather than a truly invasive infection.     

Comparative in vitro activity of Ro 40-6890, Ro 41-3399, and other antimicrobial agents against anaerobic bacteria

The in vitro activity of the ester Ro 41-3399 and its free active acid Ro 40-6890 was tested against 189 strains of anaerobic bacteria in comparison to other oral cephalosporins and to antimicrobial agents established in the treatment of anaerobic infections.Prevotella, Porphyromonas, Peptostreptococcus, Fusobacterium andClostridium spp. were susceptible to Ro 40-6890, with few exceptions. Due to its lack of activity against the major pathogens of theBacteroides fragilis group, Ro 40-6890 does not promise to be of major use in the treatment of infections caused by anaerobes.