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  • 1
    Electronic Resource
    Electronic Resource
    Springer
    Journal of molecular medicine 52 (1974), S. 305-317 
    ISSN: 1432-1440
    Keywords: Alkaline leukocyte phosphatase ; Bone marrow biopsy ; Folic acid level ; Histamin level ; Immunoglobulins ; Myeloid metaplasia ; Myelofibrosis — clinical findings ; — course ; — experimental reproduction ; Myelosclerosis ; Alkalische Leukocyten-Phosphatase ; Bekkenkamm-Biopsie bei OMF ; Folsäure-Spiegel bei OMF ; Histamin-Spiegel bei OMF ; Immunglobuline bei OMF ; Myeloide Metaplasie ; Osteomyelofibrose — klinischer Befund ; — Verlauf ; — tierexperimentelle Erzeugung ; Osteomyelosklerose
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Bei der Osteomyelofibrose handelt es sich z.T. um autonom-proliferative Erkrankungen, z.T. um zu Nekrosen und terminaler Fibrosierung führende, durch unbekannte Stimuli ausgelöste Knochenmarks-Prozesse. Diese zweite Entstehungsmöglichkeit wird u.a. durch tierexperimentelle Untersuchungen gestützt, die die Entwicklung von generalisierten Markfibrosen bei rezidivierenden Antigen-Antikörper-Reaktionen zeigen. Díese durch die Trias „Markfibrose (evtl. mit Knochenumbau), extramedulläre Blutbildung und erythroleukämisches Blutbild“ charakterisierte Krankheitsgruppe zeigt im Einzelfall weite Unterschiede hinsichtlich der klinischen Befunde, der Labor-Untersuchungen und des Verlaufs. So ist eine sichere Diagnose nur durch die histologische Knochenmarks-Untersuchung möglich, die gelegentlich die Abgrenzung proliferativer Krankheitsformen gegenüber reaktiv-nekrotisierenden Markprozessen gestattet. Bemerkenswerterweise sind in über der Hälfte der Fälle die Immunglobuline (in der Regel γ-G-Globuline) vermehrt. Bei der Therapie werden die Indikationen zur Splenektomie hervorgehoben, während Cytostatika oder Corticoide nur ausnahmsweise Verwendung firden.
    Notes: Summary A survey is presented on the theories concerning the nosology as well as the clinical and laboratory findings in myelofibrosis. In some cases the disease is a proliferative process, while in others necrosis of myeloid tissue is provoked by unknown stimuli and terminates in fibrosis of the marrow. This concept is underscored by experimental studies which show generalized marrow fibrosis in rabbits during repeated antigen-antibody reactions. Clinical and laboratory findings as well as the course vary widely from one case to the other. Histological examination of a marrow biopsy is essential for the diagnosis; the histological pattern sometimes allow to distinguish between proliferative and reactive-necrotizing forms. The immunoglobulins (mostly γ-G-globulins) are elevated in more than 50% of the cases, which together with sometimes positive findings of antibodies against collagen point in the direction of an inflammatory process. In therapy the indications of splenectomy are stressed, whereas cytostatic drugs or corticoids are used only in selected cases.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1432-1440
    Keywords: Deoxycoformycin ; Hairy cell leukemia ; Refractory treatment
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Three patients with advanced hairy cell leukemia received low-dose deoxycoformycin treatment after failure to respond to therapy with interferon alpha. Patients 1 and 2 had progressive disease after splenectomy and subsequent treatment with recombinant interferon alpha (for 7 and 3 months, respectively). DCF was administered at 4 mg/m2 weekly for 3 weeks, and then once every week for 6 weeks. Patient 1 was in complete remission after 9 weeks of treatment and patient 2 in partial remission with normalization of peripheral blood counts. The third patient, also splenectomized, developed hepatotoxicity after therapy trial with interferon for 24 days and no objective improvement was observed at this stage. She subsequently responded to DCF treatment with improvements in blood counts and bone marrow. This report demonstrates that DCF is highly effective in hairy cell leukemia and non-cross-resistant with interferon alpha.
    Type of Medium: Electronic Resource
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