Neilson J R, Patton W N, Williams M D, Mayne E E, Boughton B J
Department of Haematology, Queen Elizabeth Hospital, Birmingham.
J Clin Pathol. 1994 May;47(5):471-2. doi: 10.1136/jcp.47.5.471.
Lymphoblastic transformation of polycythaemia rubra vera is an extremely rare phenomenon. A case of a 76 year old man with polycythaemia rubra vera who developed acute lymphoblastic leukaemia (ALL) 16 years after his initial diagnosis is reported. Membrane markers showed a CD10 positive (common ALL) immunophenotype. To our knowledge this association has not been previously recorded. The rare occurrence of ALL in polycythaemia rubra vera may indicate that in a minority of patients clonal expansion of an abnormal pluripotent haemopoietic stem cell is responsible for the polycythaemia rubra vera disease phenotype.
真性红细胞增多症的淋巴细胞转化是一种极其罕见的现象。本文报道了一例76岁的真性红细胞增多症男性患者，在初次诊断16年后发展为急性淋巴细胞白血病（ALL）。膜标志物显示为CD10阳性（普通ALL）免疫表型。据我们所知，这种关联此前尚未有记录。真性红细胞增多症中ALL的罕见发生可能表明，在少数患者中，异常多能造血干细胞的克隆性扩增是真性红细胞增多症疾病表型的原因。
