[Unclassified chronic myeloproliferative Ph(-); i(17q); +8 syndrome with mixed myelo-megakaryoblastic crisis--case report].
We present a case of a 17-year old patient with extreme hepatosplenomegaly, hyperthrombocytosis, hyperleucocytosis and the presence of myelo- and megakaryoblasts in the peripheral blood film. Numerous complications that occurred in the course of the disease made cytostatic treatment difficult. Since Ph chromosome and hybrid gene bcr/abl were absent, the diagnosis of unclassified chronic myeloproliferative syndrome in the phase of blast crisis was established. Immunophenotyping confirmed a mixed myelo- megakaryoblastic character of the crisis. In the differential diagnosis other myeloproliferative syndromes were taken into account including i(17q) syndrome. The patient died after a 13-month observation due to neoplasm progression and sepsis.
Mariańska
B
B
Kliniki Hematologicznej Instytutu Hematologii i Transfuzjologii w Warszawie.
Apel
D
D
Seferyńska
I
I
Maj
S
S
D000293:Adolescent; D001752:Blast Crisis; D002869:Chromosome Aberrations / Q000175:diagnosis*; D025063:Chromosome Disorders; D002886:Chromosomes, Human, Pair 17; D002908:Chronic Disease; D017809:Fatal Outcome; D006801:Humans; D016130:Immunophenotyping; D008297:Male; D009196:Myeloproliferative Disorders / Q000175:diagnosis* / Q000235:genetics; D010677:Philadelphia Chromosome; D013577:Syndrome