Acute myelofibrosis in megakaryoblastic leukemia with translocation between chromosomes 8 and 14.
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 beta-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.
D001707:Biopsy, Needle; D001792:Blood Platelets / Q000473:pathology; D001853:Bone Marrow / Q000473:pathology; D002883:Chromosomes, Human, Pair 14; D002898:Chromosomes, Human, Pair 8; D005260:Female; D005434:Flow Cytometry; D006801:Humans; D007947:Leukemia, Megakaryoblastic, Acute / Q000235:genetics* / Q000473:pathology; D008854:Microscopy, Electron; D008875:Middle Aged; D010978:Platelet Factor 4 / Q000378:metabolism; D055728:Primary Myelofibrosis / Q000235:genetics* / Q000473:pathology; D014178:Translocation, Genetic; D001620:beta-Thromboglobulin / Q000378:metabolism
D001620:beta-Thromboglobulin; D010978:Platelet Factor 4