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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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