Polycythemia Vera in Childhood

Abstract

We have studied a patient with polycythemia vera accompanied by thrombocythemia, massive splenomegaly, and a severe bleeding tendency. Diminished plasma levels of factors I, V, and VIII in association with increased serum

Polycythemia vera in childhood: Case report and review of the literature

“…A 13-year-old boy developed hematomas, hemarthroses, and two upper gastrointestinal hemorrhages while on no therapy 7 years after diagnosis. His hematocrit was 55-65%, platelets were 1,458,000/mm 3 , and he had low levels of fibrinogen, factors V and VIII, and elevated fibrinogen degradation products [7]. Coagulation abnormalities were present in two other children [5,8] who had occasional epistaxis.…”

“…There are only seven reported childhood cases of polycythemia vera that fulfill the diagnostic criteria of the PVSG [3][4][5][6][7][8][9]. One of these [9] developed the disease shortly be fore relapse of acute lymphocytic leukemia which is unusual for polycythemia vera.…”

“…The other complications reported in children were chromosomal abnormalities de scribed in two patients; in one [7], the interpretation of the karyotype has been ques tioned [19]; in the other, chromosomal studies were done at the time of relapse of acute leukemia [9].…”

“…It is predominantly a disease of older adults, with the mean age at diagnosis being 60 years [2]. Childhood cases are extremely rare; only nine well-documented reports have appeared in the literature or by personal communication [3][4][5][6][7][8][9][10][11]. This report describes one such patient, reviews the re ported pédiatrie cases, and discusses the diagnosis and complications of polycythemia vera in childhood.…”