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  • 1
    ISSN: 1432-2307
    Keywords: Myelodysplastic syndrome ; Myelofibrosis ; Cytogenetics ; Histopathology ; Bone marrow biopsy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Chromosome analyses of bone marrow and peripheral blood cells were performed in a total of 51 patients with myelodysplastic syndromes (MDS) simultaneously with histopathological examination of resinembedded bone marrow biopsies. Diagnosis of MDS was established by histopathology according to the French-American-British (FAB) classification, and reassessed by haematological data and clinical course. Clonal karyotypic changes were found in 30 of the 51 patients (59%): in 15 of 19 (79%) patients with refractory anaemia, 7 of 11 (64%) with refractory anaemia and excess of blasts (RAEB), 6 of 10 (60%) with RAEB in transformation, and 2 of 11 (18%) with chronic myelomonocytic leukaemia. The following three features of the histopathology revealed positive correlations with karyotype abnormalities: all cases of myelofibrosis in MDS (7/51) were accompanied by chromosome aberrations, microforms of megakaryocytes with reduced nuclear lobulation were observed in 18 of 30 cases with karyotype changes, and hypocellularity of haematopoiesis was associated with aberrations of chromosome 7 in 2 of 4 cases. No positive correlations were revealed between abnormal karyotypes and the transformation to acute leukaemia. The survival times were significantly decreased in patients with complex (3 and more) karyotype changes, when compared with patients with single (1–2) chromosome aberrations or normal karyotype, independently of the FAB classification.
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    Springer
    Virchows Archiv 370 (1976), S. 273-289 
    ISSN: 1432-2307
    Keywords: Bone marrow biopsy ; Hairy cell leukemia ; Differential diagnosis ; Myelofibrosis ; Electron microscopy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary In 24 patients with hairy cell leukemia, histological and fine structural findings from biopsies of the bone marrow are reported and their validity is compared with other diagnostic procedures available. Diagnosis by light microscopy of anterior iliac crest biopsies obtained by the method of myelotomy is possible with a high degree of accuracy. The differentiation of hairy cell leukemia from other myelo- or lymphoproliferative disorders based on cytomorphology as well as patterns of growth is emphasized. Morphological differences between fibrosis in this entity and other lesions such as malignant lymphomas, Hodgkin's disease, osteomyelofibrosis and -sclerosis are emphasized. Electron microscopy of the bone marrow shows single fibroblastic cells with numerous slender cytoplasmic processes randomly dispersed among the hairy cells. These fibroblasts are probably responsible for the synthesis of the reticulin and collagen fibres in their surroundings. Moreover fine structure of the hairy cells demonstrates pinocytic activity but no apparent phagocytosis in contrast to the phagocytic reticulum cells (histiocytes, macrophages). In the bone marrow the precursor cells and the many immature forms of hairy cells exhibit an overall lymphocytoid appearance during their maturation, suggesting a lymphocytic origin.
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  • 3
    Electronic Resource
    Electronic Resource
    Springer
    Virchows Archiv 389 (1980), S. 269-286 
    ISSN: 1432-2307
    Keywords: Myelofibrosis ; Osteomyelosclerosis ; Histopathology ; Cytogenetics ; Bone marrow biopsy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary A combined histological and cytogenetic study was performed on the bone marrow in 33 patients with overt osteomyelofibrosis/-sclerosis (MF/OMS) and so called agnogenic myeloid metaplasia including blast crisis. Histopathology of the plastic embedded samples of bone marrow showed an abnormal proliferation of megakaryopoiesis with conspicuous atypias of growth and maturation in addition to a neoplastic neutrophilic granulopoiesis, particularly in the early stages of MF. Thus a biphasic population of neoplastic hematopoiesis is postulated and this lesion is called chronic megakaryocytic-granulocytic myelosis (CMGM) with myelofibrosis — CMGM stage III — or with osteomyelosclerosis — CMGM stage IV. Initiation of fibrillogenesis, the most striking alteration of this disorder, is partially attributed to disorganization of megakaryopoiesis with abnormal proliferation and clustering around the sinuses and intraluminal growth, with subsequent obliteration of the vascular compartment. Cytogenetic evaluation demonstrated the Philadelphia chromosome (Ph'-chromosome) in 93% of CGL and in 67% of MF/OMS, including cases with blast crisis. Unlike CGL and MF/OMS where a Ph'-chromosome is common, myelofibrosis of non-neoplastic origin and AML displayed no Ph'-chromosome. Further aberrations such as aneuploidy involved the C/D group chromosomes predominantly and were especially prominent in blast crisis (about 50%) with no significant differences in CGL and MF/OMS or in AML. Our results of chromosomal analysis, evaluated in close context with histopathology, show no fundamental differences between CGL and myeloproliferative disorders of mixed cellularity, i.e., chronic megakaryocytic-granulocytic myelosis (CMGM). For this reason the terminal stages of fibrotic and osteosclerotic lesions belong into these categories of CMGM or CGL respectively. In conclusion MF/OMS are final stages or subtypes of CML, carrying the same chromosomal marker and demonstrating remarkable atypias of the hematopoietic tissue suggestive of malignancy. The fibrotic/ osteosclerotic alteration itself is thought to represent a secondary nonneoplastic feature.
    Type of Medium: Electronic Resource
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  • 4
    Electronic Resource
    Electronic Resource
    Springer
    Virchows Archiv 420 (1992), S. 473-478 
    ISSN: 1432-2307
    Keywords: Histopathology ; Bone marrow ; Megakaryocytes ; Myeloid leukaemia
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary A histological study on sequential bone marrow biopsies in patients with chronic myeloid leukaemia (CML) was performed. We wished to answer the question as to whether a different content of megakaryopoiesis in the bone marrow of CML patients has a prognostic significance for the development of myelofibrosis during the course of disease. In addition, the significance of possible changes in the quantity of megakaryopoiesis in this process was assessed. In 186 patients who had no fibre increase at first diagnosis, the rate of subsequent myelofibrosis varied from 19% for the common or granulocytic subtype (CML.CT) to 40% for patients with features of megakaryocytic increase (CML.MI). No significant differences were found either in the rapidity of progression to fibrosis or in the final rate of osteomyelosclerosis. Whereas in CML.MI most patients (75%) showed an increase of fibres only, this was accompanied by an additional increase of megakaryocytes in CML.CT, changing the histological pattern from CML.CT to MI or MP, respectively. The data therefore revealed a correlation between fibre increase and subtyping of CML as suggested by the Hannover classification of chronic myeloproliferative diseases. Subtypes of CML with megakaryocytic increase could be shown to present a “pre-myelofibrotic” stage of disease and may therefore be conceived as a particular pathway of acceleration.
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  • 5
    ISSN: 1432-0584
    Keywords: Myeloproliferative disorders ; Megakaryopoiesis ; Histopathology ; Bone marrow
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Histologic diagnoses from bone marrow biopsies were analyzed in a total of 1165 patients presenting with thrombocythemic platelet counts at initial examination. Two cut-off points suggested by the Polycythemia Vera Study Group to define thrombocythemia by platelet counts were compared: the former limiting value of 1000×109/l platelets versus the recently proposed value of 600×109/l. The percentage of all nonproliferative disorders was 41% under the lower, dropping to 11% under the high cut-off point. The respective figures for myeloproliferative disorders increased from 49% under the lower to 74% under the high limiting value. Primary thrombocythemia was included in 72% by the lower, and in only 40% by the high limiting value when classified by its histologic pattern in bone marrow biopsy. A striking decrease of platelet counts occurs, related to fiber increase, among each of three main groups of myeloproliferative disorders: in CML with megakaryocytic predominance from 40% down to 25%, in megakaryocytic-granulocytic myelosis (primary, i.e., agnogenic myelofibrosis) from 36.6% to 10%, and in primary thrombocythemia from 72.6% to 28.6% in cases with reticulin sclerosis.
    Type of Medium: Electronic Resource
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  • 6
    ISSN: 1432-1963
    Keywords: Schlüsselwörter Myelodysplastisches Syndrom ; Knochenmark ; Zytogenetik ; Histopathologie ; Prognose ; Key words Myelodysplastic syndrome ; Bone marrow ; Cytogenetics ; Histopathology ; Prognosis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary The value of cytogenetics performed simultaneously with histopathology was evaluated in patients with myelodysplastic syndrome (MDS). Clonal karyotype changes of the bone marrow cells supporting the histological diagnosis were found in 38/69 cases (55 %). The chromosome aberrations, especially complex changes, were significantly correlated to distinct histopathological findings such as atypias of the haematopoietic cell lines and myelosclerosis. Complex karyotype changes were further associated with short survival of the MDS patients. Our results demonstrate that cytogenetic analyses are helpful in supplementing the histopathological diagnoses. Recent developments in molecular cytogenetics even allow the detection of chromosomal aberrations in non-dividing cells from cytological preparations or tissue sections which may become available for routine diagnosis.
    Notes: Zusammenfassung Die Bedeutung simultaner zytogenetischer und histologischer Untersuchungen wurde bei Patienten mit myelodysplastischem Syndrom (MDS) überprüft. Die Ergebnisse zeigen, daß klonale Karyotypveränderungen der Knochenmarkzellen bei 38 der 69 (55 %) analysierten Patienten auftraten und damit häufig eine Absicherung der histologischen Diagnose erlaubten. Die Chromosomenanomalien, insbesondere komplexe Karyotypveränderungen, korrelierten signifikant mit einer Reihe histopathologischer Befunde, darunter Atypien der einzelnen hämatologischen Zellreihen und Myelosklerose. Durch den Nachweis komplexer Karyotypveränderungen war eine unabhängige prognostische Aussage möglich. Damit zeigen unsere Ergebnisse am Beispiel des MDS, daß zytogenetische Analysen eine sinnvolle Ergänzung der histologischen Untersuchung sein können. Darüber hinaus ist durch den Einsatz der molekularen Zytogenetik die Bestimmung von Chromosomenanomalien in zytologischen Ausstrichpräparaten oder Gewebeschnitten möglich, wodurch sich solche Befunde auch für die tägliche Diagnostik verwenden lassen.
    Type of Medium: Electronic Resource
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  • 7
    ISSN: 1432-1963
    Keywords: Schlüsselwörter Chronische myeloproliferative Erkrankungen ; Philadelphia-Translokation ; Zytogenetik ; Molekulargenetik ; Fluoreszenz-in-situ-Hybridisierung ; Histopathologie ; Key words Chronic myeloproliferative disorders ; Philadelphia-translocation ; Cytogenetics ; Molecular genetics ; Fluorescence in situ hybridization ; Histopathology
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary The histopathological classification of chronic myeloproliferative disorders can be supported by applying cytogenetics and molecular genetics to the analysis of bone marrow or blood cells, as demonstrated in 253 cases evaluated. The Philadelphia translocation (9;22) is the most important genetic parameter, being specific for chronic myeloid leukemia. Conventional methods for the detection of the t(9;22) are karyotyping and Southern blot analysis of the bcr gene. The newly established technique of fluorescence in situ hybridization (FISH) allows visualization of bcr-abl fusion even in non dividing cells. Molecular cytogenetics for t(9;22) yield results that are rapid and reliable as well as easily quantifiable.
    Notes: Zusammenfassung Zytogenetische und molekulargenetische Untersuchungen von Knochenmark- oder Blutzellen sind für die histopathologische Klassifikation der chronischen myeloproliferativen Erkrankungen hilfreich, was durch die simultane Auswertung von 253 Fällen gezeigt wird. Insbesondere die Analyse der Philadelphia-Translokation (9;22) ist dabei für die Bestätigung oder den Ausschluß einer chronischen myeloischen Leukämie wichtig. Für den Nachweis der t(9;22) stehen die konventionelle Karyotypisierung mit Bestimmung des Philadelphia-Chromosoms und das Southernblotverfahren zur Analyse einer Umlagerung des bcr-Gens zur Verfügung. Durch die neuere Methode der Fluoreszenz-in-situ-Hybridisierung (FISH) kann auch eine bcr-abl-Fusion an Interphasekernen dargestellt werden. Diese molekulare Zytogenetik ist ein rasches und zuverlässiges Verfahren zum Nachweis der Philadelphia-Translokation, das zudem leicht quantifizierbare Ergebnisse liefert.
    Type of Medium: Electronic Resource
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  • 8
    ISSN: 1432-1963
    Keywords: Schlüsselwörter Morphometrie ; Histopathologie ; Megakaryozyten ; CML ; Chronische myeloproliferative Erkrankungen ; Key words Morphometry ; Histopathology ; Megakaryocytes ; CML ; Chronic myeloproliferative disorders
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary Morphometric analysis of sections of biopsy specimens from patients with chronic myeloproliferative disorders (CMPD) can complement the individual histological diagnosis and help to distinguish the four groups of CMPD. A total of 130 diagnostic biopsies from 29 cases of chronic myelocytic leukemia (CML.CT), 26 cases of (CML.MI), 28 of essential thrombocythemia (PTH), 26 cases of chronic megakaryocytic granulocytic myelosis (CMGM), and 21 of polycythemia vera (P. vera), and 30 from healthy control persons were evaluated morphometrically in sections of undecalcified plastic-embedded core biopsies. Clear distinctions were revealed in size of megakaryocytes, nuclear lobulation, clustering, and the nuclear size and shape of megakaryocytes. Nuclear size and cellular size were significantly less in CML (range of means of cellular size: 220–360 μm2) than in the other three Ph1-negative groups (range of means: 480–750 μm2). Nuclear lobulation was more distinct in PTH than in P. vera, and especially in CMGM. Clustering of megakaryocytes was more than twice as frequent in CMGM (8.0–10.5 %) as in any of the other three groups (0.1–7.0 %). Naked nuclei were more numerous in all groups of CMPD. The main topic of the study is the different size of megakaryocytes in the four main groups of CMPE, allowing a distinction between small-megakaryocytic Ph1-positive CML and large-megakaryocytic Ph1-negative forms of CMPD.
    Notes: Zusammenfassung Morphometrische Untersuchungen an histologischen Schnitten von Biopsien chronischer myeloproliferativer Erkrankungen (CMPE) können die individuelle histologische Diagnose unterstützen und zur Unterscheidung der 4 Gruppen der CMPE beitragen. Eine Gesamtzahl von 130 Biopsien, unterteilt in 29 Fälle von CML ohne Megakaryozytenvermehrung (CML.CT), 26 Fälle von CML mit Megakaryozytenvermehrung (CML.MI), 28 Fälle von essentieller Thrombozythämie (PTH), 26 Fälle von chronischer megakaryozytär-granulozytärer Myelose (CMGM) und 21 Fälle von Polycythämia vera (P. vera) sowie eine Anzahl von 30 Kontrollbiopsien gesunder Personen wurden morphometrisch untersucht in Schnitten von nicht entkalkten, in Plastik eingebetteten Knochenmarkbiopsien. Sichere Unterscheidungen der Gruppen sind möglich durch • Größe der Megakaryozyten, • Kernlobulierung und Kerngröße und • die Form der Megakaryozyten. Kern- und Zellfläche waren bei der CML signifikant kleiner (Streuung der Mittelwerte der Zellfläche: 220–360 μm2) als bei den 3 Ph1-negativen Erkrankungen (Streuung der Mittelwerte der Zellfläche: 480–750 μm2). Die Kernlobulierung war bei der PTH ausgeprägter als bei der CMGM und der P. vera. Das Clustering von Megakaryozyten war mehr als doppelt so häufig bei der CMGM (8,0–10,5 %), verglichen mit den 3 anderen CMPE (0,1–7,0 %). – Nacktkernige Megakaryozyten waren gegenüber den Kontrollen bei allen CMPE erhöht. Hauptergebnis ist die unterschiedliche Größe der Megakaryozyten zwischen der CML und den 3 Ph1-negativen Gruppen, so daß von der kleinmegakaryozytäre Ph1-positive CML und den großmegakaryozytäre Ph1-negativen CMPE gesprochen werden kann.
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  • 9
    Electronic Resource
    Electronic Resource
    Springer
    Der Pathologe 16 (1995), S. 70-74 
    ISSN: 1432-1963
    Keywords: Schlüsselwörter CML ; Histopathologie ; Knochenmark ; Megakaryozyten ; Histiozyten ; Key words CML ; Histopathology ; Bone marrow ; Megakaryocytes ; Histiocytes
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary Histopathology of the bone marrow of diagnostic biopsies prior to any therapy is described in a total of 412 Ph1-positive patients. Special attention is paid to the distribution of megakaryocytes, increase of fibres and blasts, and occurrence of storing histiocytes of pseudo-Gaucher type. Megakaryocytes were significantly increased in 31.6 % of diagnostic biopsies, myelofibrosis was found in 15.8 %, significant increase of blasts in 2.4 %. Pseudo-Gaucher cells were detected in 57.8 % of a total of 412 biopsies. These histiological features are considered as an indication of the progress of the disease. A semiquantitative specification of CML by this criteria is described which can be performed rather reliably and defines the stage of CML at diagnosis prior to substantial treatment.
    Notes: Zusammenfassung Die Histopathologie der chronischen myeloischen Leukämie (CML) wird aus den diagnostischen Biopsien des Knochenmarks von 412 Philadelphia-positiven Patienten beschrieben. Besonders berücksichtigt wird die Verteilung der Megakaryozyten, die Vermehrung von Fasern, von Blasten und von speichernden Histiozyten, nämlich der Pseudo-Gaucher-Zellen. Die Megakaryozyten waren eindeutig vermehrt bei 31,6 % der Patienten am Tag der Diagnose. Eine erkennbare Myelofibrose wiesen 15,8 % auf, eine deutliche Blastenvermehrung 2,4 % der Patienten. Speichernde Histiozyten vom Typ der Pseudo-Gaucher-Zellen zeigten 57,8 % im Knochenmark. Die Veränderungen werden als morphologische Kriterien der fortschreitenden Erkrankung interpretiert. Es bietet sich an, die CML semiquantitativ einzuteilen, was einfach und sicher durchgeführt werden kann. Damit wird die Erkrankung aus der Knochenmarkbiopsie heraus besser verständlich und für Verlaufsuntersuchungen definiert.
    Type of Medium: Electronic Resource
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  • 10
    Electronic Resource
    Electronic Resource
    Springer
    Der Pathologe 16 (1995), S. 53-61 
    ISSN: 1432-1963
    Keywords: Schlüsselwörter Hämatologie ; Histopathologie ; Leukämien ; Myelodysplastisches Syndrom ; Myeloproliferative Erkrankung ; Prognose ; Key words Hematology ; Histopathology ; Leukemia ; Myelodysplastic syndrome ; Chronic myeloproliferative disease ; Prognosis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary The histopathology of bone marrow in primary myelodysplastic syndromes (MDS) is described, with reference to the FAB classification. Variants such as hypoplastic, thrombocythemic and fibrotic MDS are recognized from their histopathology and must be incorporated in the FAB classification. The clinical significance of hypoplastic, thrombocythemic and fibrotic variants is illustrated by the survival rates and leukemic transformation in these patients. Histopathological classification according to the FAB system corresponds with cytological classification, as proven by the distribution of the subtypes, blood values, leukemic transformation rates and survival times. Finally it is even possible to elaborate a prognostic score for survival based on histological features of diagnostic biopsies, which emphasizes the importance of histopathological examination of bone marrow in MDS patients.
    Notes: Zusammenfassung Die Histopathologie des Knochenmarks bei primären Myelodysplasien (MDS) wird als Klassifikation nach dem FAB System beschrieben. Histopathologische Varianten des MDS, nämlich hypoplastische, thrombozythämische und myelofibrotische Formen werden hinzugefügt. Die histologische Klassifikation und prozentuale Verteilung der 5 FAB-Gruppen, refraktäre Anämie – RA – (45 %); refraktäre Anämie mit Ringsideroblasten – RARS – (9 %); refraktäre Anämie mit Exzeß von Blasten – RAEB – (23 %); refraktäre Anämie mit Exzeß von Blasten in Transformation – RAEB-t (9 %); und chronische myelomonozytäre Leukämie – CMMOL – (9 %), entspricht der einer zytologischen Klassifikation an Markausstrichen, weil sie deren Häufigkeitsverteilung, ebenso wie den hämatologischen Werten, der Rate der leukämischen Transformationen und der Lebenserwartung der Patienten praktisch gleich ist. Es gelingt sogar, einen prognostischen Score für die Lebenserwartung aus der diagnostischen Biopsie des Knochenmarks zu erstellen, womit der Sinn einer histologischen Untersuchung mehr als verdeutlicht wird. Die klinische Bedeutung der hypoplastischen, thrombozythämischen und myelofibrotischen Variante wird durch Angabe von Lebenserwartung und leukämischen Transformationen illustriert.
    Type of Medium: Electronic Resource
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