PubMed Health⌕ Search

Biomedical subjects

A Georgii

Publications and source records attributed to A Georgii.

At least 37 records · Page 2Linked to original sources

Immunophenotype of hairy-cell leukaemia after cold polymerization of methyl-methacrylate embeddings from 50 diagnostic bone marrow biopsies.

Hairy-cell leukaemia may be difficult to diagnose in bone marrow biopsies, especially in the early stages or in its residum after complete clinical remission. To consider the impact of published data on immunophenotyping hairy-cell leukaemias, a total of 50 diagnostic biopsies were systematically analysed with a panel of eight antibodies and compared with cases of chronic lymphatic leukaemia (CLL), 20 follicular centre lymphomas, 20 lympho-plasmacytoid immunocytomas, 10 small-cell T-cell non-Hodgkin lymphomas and 20 cases of benign nodular lymphatic hyperplasia. The panel of eight antibodies comprised DBA44, CD45, CD20, CD45R, CD45RO, CD43 and the CD68 antibodies KP1 and Ki-M1P. The hairy-cell leukaemias were staged histologically into four categories of bone marrow infiltration. DBA44 reacted positively in 47/50 cases. CD45 and the B-cell markers CD20 and CD45R reacted in 49/50 and 43/50 cases, respectively. One CD68 marker, KP1, was positive in 38/50 cases but the other-Ki-M1P-only in 1/50 cases. Chronic lymphatic leukaemia cases, the other B-cell NHLs and lymphatic hyperplasias showed strong positivity for CD20 and CD45R, but only the immunocytomas reacted with DBA44 in 7/20 cases. The T-cell NHLs and hyperplasias showed a strong positivity for the T-cell markers CD45RO and CD43. The CD68-marker Ki-M1P revealed a high specificity since it was negative in all NHLs and positive only in one hairy-cell leukaemia. Methyl-methacrylate embedding of bone marrow biopsies under cold polymerization produces a high quality of histo- and cytomorphology, resulting in greater diagnostic reliability and the detection of low-stage infiltration of hairy-cell leukaemia. DBA44 appears as a highly specific antibody to mark hairy-cells since only immunocytomas reacted positively in a few cases. A small panel of antibodies including DBA44. CD20, CD45R and Ki-M1P may serve to distinguish small-cell. NHL from hairy-cell leukaemia even at an early stage or when there are minimal residual tumour cells.

Adult↗

Tyramine amplification technique in routine immunohistochemistry.

Signal amplification in immunohistochemistry via binding of biotinylated tyramine to proteins near the site of peroxidase-labeled antibodies is a promising new technique, but studies investigating a wide range of markers are lacking. The tyramine amplification technique (TAT) was investigated on 85 antibodies using a simple and fast protocol, and TAT results were compared to those obtained with conventional immunohistochemistry. Using TAT, most of the markers could be 5- to 50-fold further diluted and still showed identical staining results compared with standard stainings (maximal 500-fold). However, the variable reactivity of the different markers with TAT underlines the need for individual testing of every antibody to determine the optimal dilution. Some antibodies against cell adhesion molecules could be demonstrated for the first time in archival, formalin-fixed tissue sections. TAT, if carefully evaluated, offers a revolutionary improvement for modern immunostaining, either to increase sensitivity or primary antibody dilutions (cost reduction). From a methodological point of view, immunohistochemistry has not reached its limits by far and TAT is an important progressive step in this developmental process.

Antibodies, Monoclonal↗

Lymphocyte-predominant Hodgkin's disease. An immunohistochemical analysis of 208 reviewed Hodgkin's disease cases from the German Hodgkin Study Group.

There is wide consensus that lymphocyte predominance Hodgkin's disease (LPHD) represents a distinct clinicopathological entity of B-cell origin. However, inconsistent results of immunophenotyping studies and low confirmation rates among multi-center trials pose the question of whether LPHD really expresses heterogeneous marker profiles or whether it represents a mixture of morphologically similar entities. Among 2,836 cases reviewed by the German Hodgkin Study Group, immunophenotyping was performed on 1) cases classified or confirmed as LPHD by the reference panel (n = 104) or 2) cases not confirmed as LPHD but classified as classical HD (cHD) within the reference study trial (n = 104). In most cases, immunohistochemistry revealed a phenotype either LPHD-like (CD20+, CD15-, CD30-, CD45+) or cHD-like (CD15+, CD30+, CD20-, CD45-). In 27 cases, the immunophenotype was not fully conclusive. Additional markers for Epstein-Barr virus and CD57 and in situ hybridization for mRNA light chains allowed for a more clear-cut distinction between LPHD and cHD. However, in 25 of 104 cases, immunohistochemistry disproved the morphological diagnosis of LPHD of the panel experts, whereas 13 cases originally not confirmed as LPHD showed a LPHD-like immunopattern. Immunohistochemically confirmed LPHD cases showed a significantly better freedom from treatment failure (P = 0.033) than cHD; this was not observed in the original study classification based only on morphology (P > 0.05). Significantly better survival for LPHD cases improved from P = 0.047 (original study classification) to P = 0.0071 when classified by immunohistochemistry. Our results show that LPHD is a more immunohistochemical rather than a purely morphological diagnosis. Immunophenotyping of HD biopsies suspected of being LPHD is mandatory when a modified therapy protocol, that is, one different from those used in cHD, is discussed.

Adult↗

Classical Hodgkin's disease. Clinical impact of the immunophenotype.

Antibodies against CD15, -30, and -20 are often used to support morphological diagnosis of Hodgkin's Disease (HD). The classical HD, i.e., the non-lymphocyte-predominance types, are CD15+, CD30+, and CD20- in general. However, the results for CD15 are less clear-cut in many studies, showing up to 40% of classical HD that lack positivity for this maker. Little is currently known about the relevance of antigen expression in relation to clinical outcome in HD. Therefore, the three markers were analyzed in 1751 cases from the German Hodgkin Study Group, using micro-wave epitope retrieval to optimize staining sensitivity. Eighty-three percent of the cases showed a classical immunophenotype (CD15+, CD30+, CD20-), twelve percent lacked CD15 positivity (CD15-, CD30+, CD20-), and five percent showed other combinations. For 1286 cases, clinical follow-up was available, which revealed significant differences for freedom from treatment failure (P = 0.0022) and overall survival (P = 0.0001) between cases with classical immunophenotype and CD15 negativity (CD30+, CD20-). Multivariate Cox regression using the three markers, age, sex, histology, stage, B-symptoms (fever, sweats, weight loss > 10% of body weight), hemoglobin, and erythrocyte sedimentation rate as factors showed that lack of CD15 expression in classical HD is an independent negative prognostic factor for relapses (P = 0.022) and survival (P = 0.0035). In conclusion, immunohistochemistry is able to identify classical HD cases with unfavorable clinical outcome.

Adolescent↗

Square sampling. An easy method of estimating numerical densities of cells or particles within a tissue.

OBJECTIVE: A new parametric method is presented, called "square sampling," which speeds up the estimate of the number of cells or particles that are randomly distributed within a tissue. STUDY DESIGN: The principle of square sampling is subdivision of a biopsy into at least 100 squares of the same size using a measuring ocular or computer-based morphometric system and estimating the cell number by counting "positive" squares, squares with at least one cell of interest, assuming a binomial distribution of positive squares, depending on numerical density. RESULTS: The derived estimate yielded almost identical results when compared with the exact count of pseudo-Gaucher cells within bone marrow biopsies from untreated patients with chronic myeloid leukemia (r = .97, examined area = 94 x 2 mm2, with 400 squares/2 mm2), but (1) the total time of investigation could be halved by square sampling (25.1 versus 55.3 hours, P < .00005), and (2) the estimated number of cells did not very more widely around the mean exact count than the cell numbers exactly counted (P > .05). CONCLUSION: Square sampling is an easy, fast and effective alternative to nonparametric approaches in order to quantify the numerical density of cells randomly distributed within a tissue. The method can also be applied to test hypotheses of random distribution as well as to quantify a clustering of cells in cases of nonrandom cell distribution.

Bone Marrow↗

Randomized trial with early-stage Hodgkin's disease testing 30 Gy vs. 40 Gy extended field radiotherapy alone.

PURPOSE: To evaluate whether or not a total dose (TD) of 30 Gy is sufficient for treatment of assumed subclinical Hodgkin's Disease compared to 40 Gy TD with early stage Hodgkin's Disease (ESHD). METHODS AND MATERIALS: In a prospective multicenter trial, 376 patients with laparotomy-proven ESHD stages PS IA to PS IIB without risk factors such as large mediastinum, massive splenic involvement, extranodal disease, elevated erythrocyte sedimentation rate (ESR), and/or three or more involved lymph node areas were randomly allocated either to receive (ARM A) 40 Gy TD extended field-radiotherapy (EF-RT) or (ARM B) 30 Gy TD EF-RT plus 10 Gy TD involved field-radiotherapy (IF-RT), both arms without any chemotherapy. Three hundred sixty-six of these patients were evaluable for early and long-term response, such as remission status, freedom from treatment failure (FFTF), and overall survival (OAS). For quality control, all planning and verification films as well as dose charts were prospectively reviewed by a panel of four experts, all heads of a radiotherapy department, where protocol violations (PV) were seen either with regard to errors in treatment technique, treatment volume, in TD and/or in dose/time-relationship. RESULTS: Treatment resulted in a complete remission (CR) of 98%; in a 5-year FFTF of 76%, and a 5-year OAS of 97%. There was no difference between the two arms in favor of 40 Gy EF compared to 30 Gy EF regarding FFTF and OAS, without any in field relapse throughout the EF volumes. Expectedly, 5-years FFTF was significantly influenced by the quality of radiotherapeutical procedures: 70% with protocol violations (PV) vs. 82% without PV. CONCLUSION: Subclinical involvement in ESHD without risk factors is sufficiently treated by a TD of 30 Gy without chemotherapy, leading to a 5-years FFTF of 82% and a 5-year OAS of 97% in a multicenter treatment setting, where quality assurance is mandatory.

Adolescent↗

Megakaryocytes carry the fused bcr-abl gene in chronic myeloid leukaemia: a fluorescence in situ hybridization analysis from bone marrow biopsies.

Histological examination of bone marrow biopsies shows that about one-third of chronic myeloid leukaemia (CML) patients exhibit an increase of megakaryocytes. The megakaryocytic predominance may be so striking that differentiation from other chronic myeloproliferative disorders (CMPD) may be difficult in some CML patients. Megakaryocytes in CML are clonal as demonstrated by loss of glucose-6-phosphate dehydrogenase isoenzymes. The Ph translocation, fusing the abl and bcr genes on chromosomes 9 and 22, however, obviously occurs as a second step in tumour development. So far, the Ph translocation has not been assigned explicitly to megakaryocytes. The question is whether the megakaryocytic cell lineage could harbour the bcr/abl fusion in those CML cases with striking proliferation of megakaryocytes but lack this genetic defect in cases with normal or decreased megakaryocyte counts. We therefore performed triple-colour fluorescence in situ hybridization (FISH) for portions of the bcr and abl genes flanking the breakpoint in CML in paraffin sections of CML cases with normal and with increased numbers of megakaryocytes. This method allows identification of the bcr/abl fusion in single, morphologically intact cells, whereas conventional cytogenetics requires lysis and thus destruction of the cell. Among the 21 CML patients examined by FISH, 10 were informative for bcr and abl genes and displayed distinct hybridization signals within nuclei of bone marrow cells. Besides the granulopoietic cells, megakaryocytes of all those patients (4 without and 6 with varying grades of megakaryocytic increase) displayed bcr/abl fusion signals indicative of a Ph translocation. The lack of hybridization signals in the remaining 11 cases indicates that this technique is not of value diagnostically and should be reserved for scientific questions. Positive controls consisted of conventional chromosome preparations from bone marrow aspirates demonstrating the Ph chromosome in all patients examined, and negative controls of paraffin sections of bone marrow biopsies from non-CML patients. These showed no fusion signals in bone marrow cells, including megakaryocytes, using FISH. Our results demonstrate clearly that not only the transforming event but also the Ph translocation leading to the bcr/abl fusion happens prior to the differentiation of the pluripotent stem cell into different myeloid lineages. The megakaryocytic proliferation evident in some CML cases is probably a consequence of the disease progress.

Biopsy↗

Detection of numerical karyotype changes in the giant cells of Hodgkin's lymphomas by a combination of FISH and immunohistochemistry applied to paraffin sections.

Conventional karyotyping of Hodgkin's disease (HD) has until now yielded only limited insight into karyotypic characteristics of this disease. For this reason, fluorescence in situ hybridization (FISH) using alpha-satellite chromosome-specific probes was applied to paraffin sections of HD tumors in order to verify numerical aberrations suggested to be specific for HD in the literature. The FISH technique was combined with immunohistochemical detection of the CD30 antigen to allow easier identification of the Reed-Sternberg and Hodgkin (RS&H) cells. The number of specific FISH signals per nucleus was determined both in CD30-positive RS&H cells as well as in non-malignant "bystander" cells in order to assess differences in the signal distribution. Contrasted with normal lymphoid cells, the tumor cells in HD were found to be polysomic for at least one of the chromosomes analyzed (1,2,4, and 8). The technique described is a reliable method for confirmation of results obtained from conventional cytogenetics, which is especially suited for archival material or samples not containing dividing cells.

Antigens↗

[Solitary fibrous tumor of the epicardium].

A giant Solitary Fibrous Tumor (SFT) arising in the pericardium is described. A 53 year old woman was suspected to have a lung tumor and thoracotomy was performed. Intraoperatively the child-head sized mass was found to be localized in the pericardial sac. The histopathological interpretation of a small wedge-biopsy was 'endothelioma of uncertain malignancy' and heart-transplantation was performed two months later. The patient died of postoperative infection. Post mortem examination could exclude tumor rest or metastasis. The explanted heart revealed a large bulk of 2800 grams arising from the epicardium of the left chamber and enveloping the heart without invading the underlying myocardium. The histopathological pattern varied between cell-rich and -poor fibromatous areas and well capillarized endothelioma-like zones. Immunohistochemistry revealed positive reactions with monoclonal antibodies against Vimentin and CD 34 and no reactions against Cytokeratins and Faktor VIII. This pattern was confirmed in 7 SFT's of the pleura from our archives. A second control group of 7 mesotheliomas was positive for cytokeratins and vimentin but not for CD 34 and Faktor VIII. Diagnosis of SFT might be difficult because of its variability in histopathology, sometimes mimicking a hemangiopericytoma or endothelioma. Recent reports of tumor localizations devoiding serosal surfaces illustrate the diagnostic and histogenetic dilemma of this tumor. The differentiation of SFT from mesothelioma and endothelioma can be achieved by immunophenotyping including CD 34.

Antigens, CD34↗

Microwave pretreatment improves RNA-ISH in various formalin-fixed tissues using a uniform protocol.

RNA is situ hybridization technique (RNA-ISH) is hampered by formalin fixation of tissues. This necessitates a pretreatment step of enzymatic digestion. However, to achieve the best results, digestion times and concentrations of enzymes have to be adapted for every specimen in a cumbersome procedure. Microwave pretreatment (MP) of formalin-fixed tissues has developed to become a powerful tool in immunohistochemistry (IHC) in recent years. To evaluate whether MP could also be helpful for RNA-ISH, we compared MP and conventional enzymatic pretreatment systematically using different tissues and various fixation times. As a model for RNA-ISH we chose the detection of kappa/lambda light chain mRNA in tonsils and lymph nodes with follicular hyperplasia and lymph nodes of patients with lymphocyte predominant Hodgkin's Disease (LPHD). Signal intensity obtained after MP was at least as good or dramatically enhanced as that obtained with optimized, single case adapted conventional pretreatment, the morphology being much better preserved after MP. These results confirm MP as a suitable method to unify the staining protocols in RNA-ISH, regardless of the duration of formalin fixation. Based on our results we recommend MP as a reliable and inexpensive method to enhance, standardized and simplify RNA-ISH.

Formaldehyde↗

The site of inhibin production in ovarian neoplasms.

Inhibin, a physiological product of ovarian follicle cells, normally absent in serum of postmenopausal women, is elevated in adult granulosa cell tumours of the ovary. Recently, high serum levels of inhibin were reported in carcinomas and, surprisingly, also in Krukenberg tumours of the ovary. This study attempted to determine the site of inhibin production in primary (111 cases), metastatic (13) and secondary (10) ovarian tumours by using immunohistochemistry. Positive staining in tumour cells was encountered in all cases of sex-cord- stromal cell tumours, adult (13) and juvenile (3) granulosa cell tumours, thecofibromas (10), in a lipid cell tumour (1) and a Sertoli-Leydig cell tumour (1). Primary and secondary tumours not derived from sex-cord stroma revealed no positivity in tumour cells, but in theca-like cells in the surrounding non-neoplastic ovarian stroma. A positive reaction was not observed in non-tumour-bearing ovaries of a control group. The ovarian inhibin of postmenopausal women is derived from activated sex-cord stroma or sex-cord-stromal neoplasms. Therefore, elevated serum inhibin concentrations in women with primary or secondary ovarian neoplasms with other histogenesis seem to be due to an activation of the non-neoplastic ovarian stroma. Inhibin will fail to be a tumour marker in these cases. By contrast, it will be useful in proving sex-cord differentiation by immunohistochemistry and might be used in surveillance of malignant sex-cord derived neoplasms by serum assays.

Biomarkers, Tumor↗

Fluorescence in situ hybridization (FISH) is a reliable diagnostic tool for detection of the 9;22 translocation.

The fluorescence in situ hybridization (FISH) technique for detection of the 9;22 translocation was compared with the "gold standard" of conventional cytogenetics. For this purpose, both methods were applied to 81 bone marrow aspirates and/or peripheral blood specimens comprising 50 CML cases and controls from 31 patients without CML. Independently, core biopsies of these 81 patients were investigated by three histopathologists. Conventional karyotype analysis from unstimulated bone marrow cells was successful in 71/81 cases and demonstrated the Ph-chromosome in 42/46 CML patients. With FISH, results were obtained in all 81 cases investigated, confirming fusion of the ber and abl genes in all cytogenetically Ph-positive patients. Among the five Ph-chromosome-negative specimens bcr/abl fusions were detected in only one patient. The percentage of cells found to be Ph-positive by both methods was correlated, but in individual cases considerable differences in the numbers of Ph-positive cells were observed. Different results may be due to selection of cells after in vitro cultivation predominantly. FISH proved to be a very reliable technique for specimens that do not contain dividing cells. With FISH, large numbers of cells can easily be scored which is an advantage compared to conventional cytogenetics. Therefore, this method is particularly suitable for those whose therapy is being monitored or a relapse is suspected. However, the FISH results should be evaluated critically with respect to the practical limit of sensitivity since non-specific fusion signals can also be observed in a small percentage of cells in non-CML cases. It is suggested that each laboratory define its own threshold of bcr/abl fusion signals for diagnosing Ph-positive CML by FISH.

Adolescent↗

Classification and staging of Ph-negative myeloproliferative disorders by histopathology from bone marrow biopsies.

The present study illustrates characteristic features of histopathology in the 3 non-leukemic, Ph-negative groups of chronic myeloproliferative diseases (CMPD). Attention is paid to the final outcome of CMPD, especially its transformation into acute leukemias and the occurrence of myelofibrosis from bone marrow biopsies (BMB) in a total of 1,716 CMPD patients. Essential thrombocythemia (ET), polycythemia vera (P. vera), and chronic megakaryocytic granulocytic myelosis (CMGM) can readily be distinguished by histopathology from BMB in the great majority of patients without regarding laboratory data, leaving a compartment of about 12% unclassifiable cases. Histologic patterns of staging are the increase in number and pleomorphism of megakaryocytes (MK), increase in number and density of reticulin fibers and collagen fibrosis, and excess of blasts. These 3 criteria are each graded from 0 to 3 in every biopsy. From these, a staging results by means of the histology of BMB in each of the Ph-negative CMPD. This staging provides a classification by defined criteria which permits comparative studies, the possibility of monitoring the individual patients by follow-up histology, and offers a baseline for reliable evaluation of results from therapy protocols.

Acute Disease↗

Light-chain mRNA in lymphocyte-predominant and mixed-cellularity Hodgkin's disease.

There is growing evidence for a B-cell lineage of lymphocyte-predominant Hodgkin's disease (1). To support this assumption, in situ hybridization techniques were used to detect immunoglobulin light-chain mRNA in 44 formalin-fixed specimens of Hodgkin's lymphoma (22 of lymphocyte-predominant Hodgkin's disease; 22 of mixed-cellularity Hodgkin's disease). In addition, immunoglobulin light chains were evaluated by polyclonal antisera. All specimens had been unequivocally diagnosed histologically by the referees of the German Hodgkin Trial and been immunophenotyped by monoclonal antibodies against CD15, CD20, CD30, and CD45. Light-chain mRNA coding either for kappa or for lambda could be detected by an enhanced in situ hybridization protocol using microwave heating in the lymphocytic and histiocytic cells of 14 (64%) of 22 specimens of lymphocyte-predominant Hodgkin's disease tested. None of the specimens, however, belonging to one of the classic subtypes of Hodgkin's disease (mixed-cellularity Hodgkin's disease) showed positivity for mRNA in the giant tumor cells. Our results support the idea that lymphocyte-predominant Hodgkin's disease represents a B-cell malignancy that is a entity separate from classic Hodgkin's disease. Diverging results of former studies in assessing light-chain mRNA in lymphocytic and histiocytic cells probably reflect problems with the detection threshold, i.e., the sensitivity of the techniques applied.

Hodgkin Disease↗

The bcl-2/JH gene rearrangement is undetectable in Hodgkin's lymphomas: results from the German Hodgkin trial.

Ninety-one Hodgkin's lymphomas (HD), 52 non-Hodgkin lymphomas (NHL) and 33 specimens of non-neoplastic lymphatic tissues were investigated by polymerase chain reaction (PCR) for the presence of the bcl-2/JH gene rearrangement. The majority of the HD cases were drawn from the files of the German Hodgkin trial where diagnoses are established by a panel of four independent histopathologists. Using the very sensitive PCR method which detected 1 positive among 10000 negative cells, the bcl-2/JH gene rearrangement was found in 7/52 NHL and 3/16 tonsils with follicular hyperplasia, but in none of the 91 HD. The bcl-2 protein, however, was expressed by malignant cells of B and T cell lymphomas and by the giant tumour cells in 2/13 HD lymphocyte predominant, 11/28 HD nodular sclerosing I, 14/17 HD nodular sclerosing II, 10/27 HD mixed cellularity and 3/3 HD lymphocyte depleted. The bcl-2/JH rearrangement is thus independent of protein over-expression, the latter being found in all types of lymphomas. Our results do not confirm the findings of others who have detected the bcl-2/JH rearrangement in HD. These discrepancies may be explained by differences in choice of material, the gene rearrangement actually occurring in bystander cells but not in Reed-Sternberg or Hodgkin cells, or by contamination.

Chromosomes, Human, Pair 18↗

Trisomy 1 and 8 occur frequently in hepatocellular carcinoma but not in liver cell adenoma and focal nodular hyperplasia. A fluorescence in situ hybridization study.

Conventional cytogenetic studies revealed gains and structural aberrations of chromosome 1 to be the most consistent chromosomal aberrations in hepatocellular carcinoma (HCC). We investigated touch preparations of eight HCC, five cholangiocellular carcinomas (CCC), five liver cell adenomas (LCA), four focal nodular hyperplasias (FNH) as well as nine specimens of normal liver tissue using fluorescence in situ hybridization (FISH) with centromere specific probes for chromosomes 1 and 8. Polysomies of chromosome 1, especially trisomy 1, were found in five of eight HCC and four of five CCC but in no normal liver tissue or benign tumour. Only three of seven cases of HCC revealed trisomy 8 whereas the five benign liver tumours and all normal liver tissues examined had disomy 8. Our results confirm conventional cytogenetic findings in terms of chromosome 1 aberrations in HCC although they are not specific for these types of malignant liver tumours. Since alpha-satellite probes were used in our study, only gains or losses including the centromeric regions of the chromosomes 1 and 8 could be detected. Nevertheless, our findings suggest that FISH may help in the differential diagnosis of malignant versus benign neoplasms of the liver.

Adenoma, Liver Cell↗

Comparison of scoring systems in primary myelodysplastic syndromes.

To compare the prognostic value of scoring systems in primary myelodysplastic syndromes (pMDS), four clinicohematological systems (Rennes, Bournemouth, Düsseldorf, Pavia) and the histopathological Hannover Scoring System were applied to 415 MDS patients from the Bone Marrow Registry of Hannover Medical School. According to the FAB classification, 180 patients (43%) were diagnosed as RA, 33 (8%) as RARS, 99 (24%) as RAEB, 36 (9%) as RAEBt, and 48 (12%) as CMMOL; 19 patients (4%) were not further classified (MDS.UC). All scoring systems revealed three or four groups of patients with significantly different survival times. The ranges and standard deviations in these groups were similar but high in all scoring systems. A good differentiation between short-term survivors (< 1 year survival time) and intermediate-term survivors (1-4 years) was possible with all tested scoring systems, but the differentiation between intermediate- and long-term survivors (> 4 years) was not distinctive enough. The problem of risk assessment in the single patient is furthermore elucidated by the values of specificity and sensitivity, which were relatively low in all scoring systems tested. Best results were yielded by the Bournemouth Score for long-term survivors and the Düsseldorf and Hannover Score for intermediate- and short-term survivors. Multivariate analysis of all parameters used in the scoring systems showed the highest negative impact on survival for increase of myeloblasts, anemia, myelofibrosis, high age of the patient, and abnormal localization of immature precursors (ALIPs). Therefore, the histopathological Hannover Scoring System is not only equal to clinicohematological risk assessment in pMDS, but also includes important independent prognostic parameters. Risk assessment for the individual low-risk MDS patient using only initial parameters may be rendered impossible due to the biological nature of pMDS. Therefore, sequential analysis is needed to elucidate random events which alter the prognosis.

Adult↗