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Acute myelofibrosis in megakaryoblastic leukemia with translocation between chromosomes 8 and 14

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Summary

In a 45-year-old woman with severe normochromic anemia (Hb 2.8 g%) an extensive myelofibrosis and infiltration of the bone marrow with small blasts was observed histologically. Cytochemical examination of the blasts showed a negative peroxidase and a strongly positive alpha-NE reaction. PAS reaction was slightly granular positive in the cytoplasmic protuberances of the blasts and in the platelets. Marker analysis yielded no evidence of lymphatic origin of the blasts. In flow-cytometric studies of 230,000 cells a homogeneous 2c blast population could be identified. Cytogenetic analysis revealed an abnormal pseudo-diploid karyotype characterized by 2 acrocentric marker chromosomes caused by a translocation of chromosomes 8 and 14, as usually seen in Burkitt type lymphoma. Finally the reaction product of platelet-specific peroxidase could be demonstrated in the perinuclear cisternae of the endoplasmic reticulum by electron microscopy. Highly elevated β-thromboglobulin and platelet factor 4 plasma levels were also measured.

Following an ineffective treatment with daunoblastine and ARA-C, the patient died of pseudomonas aeruginosa septicemia after having received high-dose ARA-C treatment.

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Abbreviations

βTG:

β-thromboglobulin

DAB:

diaminobenzidine

ETF:

EDTA/theophyline/forskolin

IIF:

indirect immunofluorescence

PF4:

platelet factor 4

PPO:

platelet peroxidase

PPP:

platelet-poor plasma

PRP:

platelet-rich plasma

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Authors and Affiliations

  1. Abteilung für Hämatologie, Onkologie und Klinische Immunologie, Universität Düsseldorf, Germany

    M. Winkelmann, C. Aul, R. E. Scharf, J. Th. Fischer & W. Schneider

  2. Institute für Pathologie, Universität Düsseldorf, Germany

    A. Schmitt-Gräff

  3. Abteilung für Klinische Physiologie, Universität Ulm, Germany

    F. Carbonell

  4. Institute National de la Sante et de la Recherche Medical (I.N.S.E.R.M.-U.91) Creteil, France

    J. L. Villeval

  5. Institut für Immungenetik, Universität Essen, Germany

    H. Grosse-Wilde

Authors
  1. M. Winkelmann
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  2. C. Aul
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  3. R. E. Scharf
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  4. A. Schmitt-Gräff
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  5. F. Carbonell
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  6. J. L. Villeval
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  7. H. Grosse-Wilde
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  8. J. Th. Fischer
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  9. W. Schneider
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Additional information

This work was supported in part by grants from the Deutsche Forschungsgemeinschaft (Wi 806/1-1 and Scha 358/1-2)

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Winkelmann, M., Aul, C., Scharf, R.E. et al. Acute myelofibrosis in megakaryoblastic leukemia with translocation between chromosomes 8 and 14. Klin Wochenschr 65, 1034–1041 (1987). https://doi.org/10.1007/BF01726322

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  • DOI: https://doi.org/10.1007/BF01726322

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