High molecular response rate of polycythemia vera patients treated with pegylated interferon alpha-2a.

V617F JAK2 mutation is a reliable molecular marker of polycythemia vera (PV), potentially useful to monitor the effect of treatments in this disease. In a phase 2 study of pegylated (peg) IFN-alpha-2a in PV, we performed prospective sequential quantitative evaluation of the percentage of mutated JAK2 allele (%V617F) by real-time polymerase chain reaction (PCR). The %V617F decreased in 24 (89%) of 27 treated patients, from a mean of 49% to a mean of 27% (mean decrease of 44%; P < .

Priapism in a patient with unstable hemoglobin: hemoglobin Köln.

We report a case of severe priapism occurring in a patient with unstable hemoglobin, hemoglobin Köln, and underline several factors that may have contributed to this complication: abnormal plasticity of red cells, splenectomy, and cytomegalovirus infection. Since emergency treatment may prevent impotence, patients and parents should be educated about this complication.

[Bernard-Soulier thrombopathy: a diagnostic pitfall].

First described in 1948, Bernard-Soulier syndrome is an uncommon hereditary thrombopathy characterized by abnormal expression of the GPIb-IX-V complex which inhibits platelet migration to the site of endothelial trauma. Our case illustrates the pathophysiological mechanisms involved and points out the similarity with idiopathic thrombopenic purpura.