[Not Available]. / Allogene hämatopoetische Stammzelltransplantationen in Mecklenburg-Vorpommern von 1991-2000.

UNLABELLED: Allogeneic stem cell transplantation (alloSCT) is increasingly used in the treatment of hematological disorders. In order to demonstrate the national development of alloSCT as well as the effect of the foundation of local transplant centers, data of all alloSCT performed on patients living in Mecklenburg-Vorpommern (MV) during 1991 and 2000 were evaluated regarding date and location of the alloSCT, diagnosis and age of the patient as well as donor type. AlloSCT in patients from MV increased 6 fold from 1991 n=5 to 2000 n=30. Because of the lack of local transplant centers during the period between 1991 and 1997, alloSCT of patients from MV were exclusively performed in other locations, whereas in 2000 only 2 of 30 alloSCT were performed in transplants centers outside MV. With the establishment of local centers, the gap between the numbers of alloSCT between MV and Germany was closed (1991 Germany 4,17/Million inhabitants, MV 2,67/Million inhabitants, 2000 Germany 17,4/Million inhabitants, MV 16,7/Million inhabitants). The increase in the number of alloSCT was mainly due to an increased use of unrelated stem cell donors which increased from n=0 in 1991 to 12 out of 30 in 2000. Another important factor was the increase in the age limit with a mean age of 32 years in 1991 and 43 years in 2000 (upper range 60 years). CONCLUSION: The data reflect the dramatic increase in alloSCT during the last decade caused by factors listed above. In addition, the development of the numbers of transplants of patients in MV supports the need for local transplant centers, since with the opening of local transplant centers the difference of the frequency of alloSCT between Germany and MV was completely eradicated.

Two new missense mutations in a non-Jewish Caucasian family with type 3 Gaucher disease.

Gaucher disease is an autosomal recessive, lysosomal storage disease caused by a deficiency of the enzyme glucocerebrosidase. The prevalence of this disorder differs significantly among various populations and is highest in some Jewish populations. More than 35 disease-producing mutations of the gene have been reported. The change of the amino acid leucine at position 444 to proline (444Leu-->Pro) is the most common mutation in non-Jewish populations, and a second mutation at amino acid 370 (370Asn-->Ser) is the most common one in Jewish populations. However, most of the mutations have been reported in very few cases or even in only one pedigree. We performed a direct DNA sequencing analysis of the complete functional glucocerebrosidase gene in a 22-year-old neuronopathic non-Jewish patient with Gaucher disease type 3 with myoclonic epilepsy and slight mental disturbances and in most members of his family. After selective amplification of the complete coding region of the patient's functional glucocerebrosidase gene, we identified two hitherto unreported mutations in exon 9 (genomic nucleotide 5224G-->C; 417Val-->Leu) and in exon 11 (genomic nucleotide 6668C-->T; 510Thr-->Ile). Each parent showed one of these mutations. Once we sequenced the complete active gene, we could rule out the existence of further mutations. Data that show that in about 15 to 25% of non-Jewish patients with Gaucher disease no mutations within the glucocerebrosidase gene are found may be due to inappropriate screening methods, mostly used for the genotyping. Until we have precise information about the frequency and distribution of single-point mutations in patients with Gaucher disease, it is necessary to analyze the complete glucocerebrosidase gene.

[Lymphatic outflow scintigraphy in a case of artificial edema of the lower limb]. / Lymphabstromszintigraphie bei einem artefiziellen Odem des Unterschenkels.

The case of a young woman is reported who after injury to the left leg was three times hospitalized for diagnosing the cause of her lower limb edema. It took two months to verify its artificial origin. Scintigraphy showed an intensive acceleration of the lymphatic outflow from the left leg.

[Differences in electrophoretic mobility and cytochemistry of leukemic T-cells with identical immunologic phenotype (CD1 positive)]. / Unterschiede in der elektrophoretischen Mobilität und Zytochemie leukämischer T-Zellen mit gleichem immunologischen Phänotyp (CD 1 positiv).

Leukaemic cells of acute T-lymphoblastic leukaemia and T-lymphoblastic lymphoma with secondary leukaemic course of disease which showed the same immunological phenotype (cortical thymocytes, thymocyte stage II), could be further differentiated according to their cytochemical pattern and electrophoretic mobility (EPM). Leukaemic cells of T-ALL usually reveal a high EPM, whereas EPM of leukaemic cells of T-lymphoblastic lymphoma was significantly lowered. In cytochemical respect acid phosphatase was positive in both cases of leukaemia. An activity of acid esterase, however, could only be demonstrated in leukaemic cells of T-lymphoblastic lymphoma. The findings are interpreted as manifestation of a different stage of maturity of both T-cell clones with the same immunological phenotype.

Diffuse gastric antral vascular ectasia: cause of chronic gastrointestinal blood loss.

In a patient with long-standing iron deficiency anemia, diffuse gastric antral vascular ectasia was found endoscopically. Diagnostic problems were the misinterpretation of the macroscopic finding as hemorrhagic inflammation and the microscopic confirmation of the diagnosis from small biopsy particles. Resection of the antrum resulted in a persistent normalization of hematologic parameters. Focal ischemia due to microthrombi in mucosal vessels might be the cause of the tendency of this vascular malformation to bleed.

[Short-term lysis of venous thrombosis with ultra-high streptokinase doses]. / Kurzzeitlysen bei venösen Thrombosen mit ultrahoher Streptokinasedosierung.

11 patients with deep pelvic and leg vein thrombosis were treated with ultra-high streptokinase infusion, 1.5. 10(6) IE streptokinase per hour over a six hour period. Opening of the vein occlusion was achieved in four cases completely and in five cases partially. The frequency of complications was lower at short-time thrombolysis with ultra-high streptokinase infusion in contrast to long-time thrombolysis. Bleedings did not occur. Because of the slight side effects ultra-high streptokinase infusion is an alternative in the fibrinolytic treatment of risk patients with pelvic and leg vein thrombosis.

[Evaluation of clinical efficacy of platelet substitution in acute leukemia using resonance thrombography]. / Die Beurteilung der klinischen Effektivität der Thrombozytensubstitution bei akuter Leukämie mit der Resonanzthrombographie.

For evaluating the clinical effectiveness of thrombocyte substitution, the resonance thrombogram was registered before and after thrombocyte substitution in 50 patients with acute leukemia and the increase of thrombocytes (corrected increment) determined. Thrombocyte substitution led to a significant diminution of the platelet amplitude (p less than 0.01) and platelet value. The highest value of these alterations of parameters was reached 1 hour after transfusion, it was somewhat lower after 24 hours, yet the initial value was not reached again. Correlations could be found to exist between the platelet amplitude and the corrected thrombocyte increase 1 hour and 24 hours after transfusion. The resonance thrombography enables the successful control of thrombocyte substitution to be made and it is suitable for monitoring hemostasis in severe thrombocytopenia due to production.

Cell electrophoretic characterization of lymphoid cells from various sources in patients with malignant non-Hodgkin lymphomas.

Lymphocytes of 36 patients with malignant Non-Hodgkin lymphomas (NHL) were characterized by electrophoretic mobility (EPM) and EAC-rosette and E-rosette formation. Unimodal cytopherograms found in patients with low-grade malignant NHL are compatible with a monoclonal origin of the proliferating lymphoid cells. Within the CLL subgroup, deviations from the general mean EPM value in the intermediumrange were not unequivocally related to the B- or T-cell origin of the lymphocytes and remain to be explained. Comparing distinct entities of low-grade malignant NHL characterized by lymphocyte arrest at a certain stage of differentiation (Kiel Classification) we found an increase of the mean EPM in the direction of CLL----lymphoplasmacytoid immunocytoma----polymorphic immunocytoma. This arrest of leukemic cells at a certain EPM level may support a cytogenetically oriented subclassification of NHL. The Limitations of the diagnostic value of cytopherograms and rosette formation in malignant NHL--especially in those of high malignancy--are outlined.

[Glomerulonephritis therapy. Immunosuppression or coagulation inhibitors - a comparison]. / Therapie der Glomerulonephritis. Immunsuppression oder Gerinnungshemmer - ein Vergleich.

In 110 patients with mesangioproliferative glomerulonephritis the treatment (n = 48) with chlorambucil, warfarin, dipyridamol and prednisone (CAA) and the treatment with imuran and prednisone (CP, n = 23) are compared with the conditions of an untreated control group. The CAA-therapy is above all suited for active clinical pictures without sclerosis with short duration of the disease, even when the renal function is already restricted. The CP-therapy is suitable in non-active forms with sclerosis also in a longer duration of the disease.

[Therapy of primary glomerulonephritis. Comparative study on the effect of cytostatics-prednisone and cytostatics-anticoagulant-aggregation inhibitor combination]. / Therapie der primären Glomerulonephritis--Vergleichende Studie über den Einfluss einer Zytostatika-Prednison- und einer Zytostatika-Antikoagulantien-Aggregationshemmer-Kombination.

The result of the treatment with chlorambucil, dipyridamol, heparin/warfarin and prednisone on 50 patients as well as with imuran and prednisone on 32 patients is compared. Basis for this is a detailed morphological and clinical subdivision. With the help of therapy results is tried to derive regions of indication: the therapy with cytostatics, anticoagulants and aggregation inhibitors is above all suitable for patients with a short duration of the disease and with sign of activity, also in renal insufficiency. On the other hand the therapy with cytostatics and prednisone is also successful in patients with longer duration of the disease and missing signs of activity.

[Secretory immunoglobulin A]. / Das sekretorische Immunglobulin A.

Secretory IgA is the prevailung immunoglobulin on the mucous membranes of different tissues. It is a polymer immunoglobulin and in comparison to the serum IgA the secretory IgA has a additional polypeptide chain, the secretory component. The secretory IgA is synthesized locally in the mucosa. Secretory IgA is a very important factor in the immune defence of the mucous membranes. Secretory antibodies are regulated independently from serum antibodies. They show a virus neutralizing effect and activities against bacteria and lifeless noxa. The mechanism is not yet clear. It is possible, that the reaction of the secretory IgA with the antigen prevents settlement of microorganisms on the mucous membranes. The clinical importance of secretory IgA is undoubted especially the deference of the mucosa in cases of a virus infection.

[Quantitative immunochemical determination of human secretory IgA]. / Quantitative immunchemische Bestimmung von humanem sekretorischen IgA.

Secretory IgA from human colostrum was fragmented by mercaptoethanol. The heavy chain fraction was prepared by means of gel chromatography on Sephadex G 100 in 1 N acetic acid. After immunization of rabbits with this heavy chain fraction the resulting antiserum contained antibodies specific for the alpha-chain and antibodies against the secretory component. These antibodies were separated by means of immunosorption. The antibodies against the secretory component precipitate free secretory component as well as bound secretory component. The antiserum is suitable for quantitative determination of secretory IgA, but free secretory component reacts too. Also secretory IgM may react, because secretory IgM contains up to 70% secretory component.