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  • 1
    Electronic Resource
    Electronic Resource
    Springer
    Pediatric surgery international 15 (1999), S. 437-439 
    ISSN: 1437-9813
    Keywords: Key words Joint chondromatosis ; Recurrence ; Differential diagnosis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A case of chondromatosis of the upper ankle joint in childhood is described. It is a monoarticular disease with a good prognosis, frequently without known prior trauma or inflammation, although often free fragments of cartilage are seen in the joint cavities. It originates from the synovium of the joint, and is known in the literature as Reichel syndrome.
    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.
    Type of Medium: Electronic Resource
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  • 3
    Electronic Resource
    Electronic Resource
    Springer
    Virchows Archiv 389 (1980), S. 287-305 
    ISSN: 1432-2307
    Keywords: Myeloproliferative disease ; Osteomyelofibrosis ; Bone marrow biopsy ; Blast crisis ; Ultrastructure ; Basophilic leukaemia
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The clinical and morphological findings are presented in two patients suffering from myelofibrosis and osteomyelosclerosis which terminated in an acute blastic crisis. Clinical follow-up data and light microscopy of the bone marrow however, revealed a chronic megakaryocytic-granulocytic myelosis (CMGM) with progression into myelofibrosis during the course of disease. In one patient the blastic transformation involved predominantly basophils, and in the other, neutrophils, with an accompanying abnormal proliferation of megakaryocytes in both cases. Electron microscopy of this cell population demonstrated remarkable atypicalities of the neutrophilic, basophilic and megakaryocytic cell lines. These abnormalities consisted of a nuclear-cytoplasmic asynchrony and a partial arrest of maturation, sometimes resulting in bizarre cell forms. Our investigations support the hypothesis of a mixed cellularity type of myelosis with a gradual and insiduous progression into osteomyelofibrosis/-sclerosis and a potential blastic crisis. In the evolution of blastic crisis all cell lines may be transformed, but with predominance of one population — basophils and neutrophils in our two cases — in addition to atypicalities of megakaryocytes.
    Type of Medium: Electronic Resource
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  • 4
    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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  • 5
    Electronic Resource
    Electronic Resource
    Springer
    Virchows Archiv 373 (1977), S. 191-211 
    ISSN: 1432-2307
    Keywords: Myeloproliferative disorder ; Thrombocytes ; Megakaryocytic-granulocytic myelosis ; Bone marrow biopsy ; Megakaryocyte lineage ; Electron microscopy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The fine structure of the bone marrow in chronic megakaryocytic-granulocytic myelosis (CMGM) was studied in 5 nontreated patients to investigate possible malignant proliferation of megakaryocytes and the role of megakaryopoiesis in fibrillogenesis, terminating in osteomyelofibrosis. In comparison with normal megakaryopoiesis there is an enormous increase of the megakaryocytic cell line and many immature and atypical forms are seen. Most conspicuous are microforms, nuclear-cytoplasmic disorganization and nuclear inclusions. Asynchrony of maturation causes abnormal thrombocytogenesis with premature detachment of platelets resulting in immature and peculiar giant forms of thrombocytes. Besides megakaryocytes appearing superficially normal the maturation anarchy of many cells is so severe that by analogy with observations in other leukaemic cells these abnormalities are thought to be representative of a malignant growth. Moreover, there is a striking accumulation of microfibrils and single collagen fibres around megakaryoblasts. Since these cells contain all those organelles commonly associated with fibre production the initial step for fibrillogenesis may therefore arise from the megakaryoblasts prior to platelet release, or any fibroblast proliferation.
    Type of Medium: Electronic Resource
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  • 6
    ISSN: 1432-0584
    Keywords: Key words Chronic myeloproliferative disorders ; Erythroid precursors ; Neutrophil granulopoiesis ; Megakaryocytes ; Macrophages ; Myelofibrosis ; Enzyme-immunohistochemistry ; Morphometry ; Bone marrow biopsy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract  The aim of this review is to evaluate morphological characteristics of the different subtypes of chronic myeloproliferative disorders (MPDs) derived by applying immunohistochemical and morphometric techniques to bone marrow biopsies and to combine these results with relevant clinical parameters. In comparison to control specimens, a significant decrease in erythroid precursors is determinable in chronic myeloid leukemia (CML), while this cell lineage is most prominent in polycythemia vera (PV) and moderately to markedly reduced in idiopathic myelofibrosis (IMF). On the other hand, neutrophilic granulopoiesis shows a predominance in CML and a relevant increase in PV, but no conspicuous changes are detectable in essential thrombocythemia (ET). CML is characterized by a prevalent growth of dwarflike micromegakaryocytes, occurring in particular in the so-called megakaryocyte-rich subtypes (about 30%). This finding differs significantly from the pleomorphous aspect, i.e., clusters of small to giant-sized megakaryocytes in PV and the grossly abnormal (dysplastic) appearance of this cell lineage in patients with IMF. Similar cytological abnormalities of megakaryopoiesis consistent with maturation defects are never encountered in ET. The incidence of mature (resident) macrophages (phagocytic reticular cells) is significantly enhanced in IMF in comparison to the other MPDs and controls. Moreover, there is a striking difference in the density of reticulin-collagen fibers, ranging from normal (ET) to extreme values (IMF). In IMF more than 80% of the patients present with some degree of myelofibrosis-osteosclerosis at diagnosis, while the rest show an initial prefibrotic, hypercellular stage. This feature deserves special attention since, when accompanied by thrombocythemia, it may simulate ET. Sequential bone marrow biopsies in patients with IMF disclose that evolution of myelofibrosis is progressive, but occurs at a variable and unpredictable speed. A synoptical approach regarding clinical diagnosis and histological subtyping of MPDs is explicitly recommended and demonstrated by sets of diagnostic criteria. This rationale requires equal consideration of laboratory data and morphology by clinicians to include well-defined subtypes of MPDs into prospective management studies. Furthermore, it may even warrant follow-up studies and repeated bone marrow examinations in initially unclassifiable cases.
    Type of Medium: Electronic Resource
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  • 7
    Electronic Resource
    Electronic Resource
    Springer
    Annals of hematology 39 (1979), S. 177-190 
    ISSN: 1432-0584
    Keywords: Chronische myelo-monozytäre Leukämie ; Seeblaue Histiozyten ; Knochenmarksbiopsie ; Elektronenmikroskopie ; Chronic myelomonocytic leukemia ; Sea-blue histiocytes ; Bone marrow biopsy ; Electron microscopy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary Clinical data and light and electron microscopic findings are presented in a patient with chronic myelomonocytic leukemia of about 5 years' duration and no need for specific therapy. Cytogenetic studies failed to demonstrate a Philadelphia-chromosome. The leading clinical symptoms were anemia, moderate hepatomegaly, and leukocytosis with monocytes in the peripheral blood count. Light microscopy of bone marrow cores showed hypercellularity of neutrophil granulocytic and monocytic cell lines including some precursor forms. Electron microscopy confirmed the existence of a biphasic myelomonocytic cell proliferation with predominance of mature forms in both lineages; there were no gross cellular abnormalities and no “hiatus leukaemicus”. Conspicuous were cells of an undeterminated origin apparently neither belonging to the neutrophil granulocytic nor monocytic series and large histiocytic cells, possibly corresponding to the so-called sea-blue histiocytes of light microscopy. The high degree of maturation of both cell lines in the bone marrow is in accordance with the relatively benign and prolongated course of this rare type of leukemia.
    Notes: Zusammenfassung Von einem Patienten mit einer chronischen myelo-monozytären Leukämie von etwa 5 Jahren Dauer und ohne spezifische Therapie werden klinische sowie licht- und elektronenmikroskopische Befunde vorgestellt. Zytogenetische Untersuchungen ließen ein Philadelphia-Chromosom nicht erkennen. Die führenden Symptome waren eine geringe Anämie, eine mäßige Lebervergrößerung und Leukozytose mit Monozyten im peripheren Blutausstrich. Lichtmikroskopische Untersuchungen des Knochenmarkes zeigten eine Zellvermehrung der neutrophilen granulozytären und der monozytären Zellreihen einschließlich einiger Frühformen. Die Elektronenmikroskopie bestätigte das Vorhandensein einer biphasischen myelo-monozytären Zellproliferation mit Vorwiegen der reifen Formen in beiden Zellinien. Es bestanden keine bemerkenswerten zellulären Anomalien und kein „hiatus leucaemicus“. Auffallend waren Zellen unbestimmbarer Herkunft, die weder der neutrophilgranulozytären oder monozytären Reihe sicher zuzuordnen waren, und große histiozytäre Zellen, die wahrscheinlich den sogenannten „seeblauen Histiozyten“ der Lichtmikroskopie entsprechen. Der hohe Grad der Ausreifung in beiden Zellinien im Knochenmark steht in Übereinstimmung mit dem relativ gutartigen und langen Verlauf dieses seltenen Types einer Leukämie.
    Type of Medium: Electronic Resource
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  • 8
    Electronic Resource
    Electronic Resource
    Springer
    Der Pathologe 16 (1995), S. 106-119 
    ISSN: 1432-1963
    Keywords: Schlüsselwörter Maligne Lymphome ; Fokale reaktive lymphoide Hyperplasie ; Knochenmark ; Differentialdiagnose ; Histotopographie ; Fasergehalt ; Immunhistochemie ; Key words Malignant lymphomas ; Reactive lymphoid hyperplasia ; Bone marrow ; Differential diagnosis ; Histotopography ; Fiber content ; Immunohistochemistry
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary The purpose of this study was to provide criteria for the differentiation of reactive lymphoid hyperplasia (RLH) and focal involvement of the bone marrow by malignant lymphoma (ML). Using trephine bone-marrow biopsy specimens embedded in paraffin wax and unequivocally established samples with ML for comparison, all patients with questionable lymphoid or lymphohistiocytic marrow aggregates were re-examined, together with obviously reactive lesions. Following this procedure, a number of characteristics were found that differed in validity with regard to diagnosis. In addition to cytology, which is preferably assessed in Giemsa-stained specimens and evaluated by the Kiel classification, histotopography, fiber content, and immunohistochemical reactions are the most valuable tools for differential diagnosis. RLH is consistent with a central-perivascular localization, a distinctive border and the presence of germinal centers, no or only minimal reticulin fibrosis and a polyclonal reaction pattern with a mixed population of B- and T-lymphocytes, following staining with appropriate antibodies. In uncertain cases (i. e., extensive lymphoproliferations in HIV-myelopathy) the results of immunohistochemical staining are of definite importance for the diagnostic evaluation of these lesions.
    Notes: Zusammenfassung Die vorliegende Studie verfolgt das Ziel, Kriterien für die differentialdiagnostische Abgrenzung zwischen fokaler reaktiver lymphatischer Hyperplasie (RLH) und nodulären Infiltraten von malignen Lymphomen (ML) im Knochenmark festzulegen. Im Vergleich zu klinisch und histologisch gesicherten Fällen von ML und offensichtlich reaktiven Veränderungen wurden alle in ihrer diagnostischen Zuordnung fraglichen lymphoiden bzw. lymphohistiozytären Läsionen anhand von Beckenkammbiopsien nach Paraffineinbettung noch einmal untersucht. Als wesentliches Ergebnis konnte eine Reihe von diagnostischen Merkmalen herausgearbeitet werden, die allerdings von sehr unterschiedlicher Wertigkeit waren. Neben der Zytologie, welche besonders gut in nach Giemsa gefärbten Präparaten auswertbar ist und sich problemlos nach den entsprechenden Maßgaben der Kiel-Klassifikation zuordnen läßt, sind Histotopographie, Fasergehalt und Immunhistochemie von besonderer nosologischer Bedeutung. Für eine RLH sprechen eine zentral-perivaskuläre Lokalisation mit scharfer Abgrenzung sowie Keimzentren, keine oder allenfalls eine minimale Retikulinfibrose sowie schließlich nach Anwendung immunhistochemischer Verfahren ein polyklonales Reaktionsmuster mit einer Mischpopulation aus B- und T-Lymphozyten. Im Zweifelsfall (z. B. bei ausgedehnter Lymphoproliferation im Rahmen einer HIV-Myelopathie) ist alleine die Immunhistochemie in der Lage, diagnostisch wegweisende Anhaltspunkte zu geben.
    Type of Medium: Electronic Resource
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  • 9
    ISSN: 1432-2307
    Keywords: Myeloproliferative disorder ; Megakaryocytic-granulocytic myelosis ; Granulocyte lineage ; Bone marrow biopsy ; Freeze-fracture ; Electron microscopy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary In 5 patients with chronic megakaryocytic-granulocytic myelosis (CMGM) bone marrow specimens were studied by electron microscopy to investigate possible abnormalities of the granulocytic cell lineage. Thin sections were compared with freeze-fracture replicas to elucidate further aspects of leucocyte cytology. The atypia exhibited in these cells (eosinophils, basophils and neutrophil granulocytes) consisted mostly of a disorganization of granulopoiesis with hyper- and hypogranulation, a pathological increase in the number of nuclear blebs and a maturation asynchrony sometimes leading to Pelger-like cell forms. Moreover, a presumptive stem cell was demonstrated in the erythopoietic and granulocytic cell lines resembling CFU cells. In conclusion, granulopoiesis in CMGM exhibited abnormalities as generally observed in chronic myelogenous leukaemia. When considered with our previous finding of malignancy in megakaryopoiesis, CMGM has to be classified as a myelosis of mixed cellularity.
    Type of Medium: Electronic Resource
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  • 10
    Electronic Resource
    Electronic Resource
    Springer
    Virchows Archiv 389 (1980), S. 307-324 
    ISSN: 1432-2307
    Keywords: Polycythemia vera ; Secondary polycythemia ; Histopathology ; Ultrastructure ; Cytogenetics ; Bone marrow biopsy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Clinical and morphological studies including light microscopy, electron microscopy and karyotyping of the bone marrow, were performed on a total of 164 patients with polycythemic conditions. A final diagnosis was obtained from clinical findings and histopathology of plastic embedded core biopsies of the bone marrow including sequential examinations. 51 patients revealed a secondary polycythemia whereas 113 displayed polycythemia vera (P. vera). In this last group 83 cases have persisting P. vera. 30 showed a transgression towards chronic myeloid leukemia with or without accompanying myelofibrosis — osteomyelosclerosis (so called chronic megakaryocytic-granulocytic myelosis — CMGM). The histopathology of the bone marrow in P. vera revealed consistent alterations which are useful in distinguishing this disorder from secondary polycythemia (SP) and CMGM: depletion of iron storage, increased neutrophilic granulopoiesis but no gross atypia in maturation, polymorphism of megakaryocytes with conspicuous giant forms and dilatation and increased branchings of venous sinusoids. Electron microscopic findings were in agreement and showed further abnormalities of cytological maturation in the erythrocytic and granulocytic lineage. Cytogenetic studies in 27 non-treated patients with P. vera revealed the Philadelphia chromosomes in 2 cases, whereas in SP only minor chromosomal anomalies have been encountered in a few patients. It is concluded that histopathology of trephine biopsies of the bone marrow is an invaluable aid to establish a correct diagnosis, differentiating P. vera from the other potentially polycythemic disorders and helping to detect a possible progression towards leukemia at an early stage. Cytogenetic investigations may show early structural and numerical abnormalities of the karyotype and possibly precede a presumptive transgression towards myeloid leukemia (CMGM). A simultaneously performed histological and chromosomal examination of bone marrow samples is therefore desirable in each case of a polycythemic condition.
    Type of Medium: Electronic Resource
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