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Occurrence of Acute Leukaemia in Myeloproliferative Disorders
Author(s) -
Rosenthal David S.,
Moloney William C.
Publication year - 1977
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1111/j.1365-2141.1977.tb00661.x
Subject(s) - myeloproliferative disorders , medicine , haematopoiesis , bone marrow , acute myeloblastic leukemia , immunology , leukemia , pathology , stem cell , biology , genetics
In a series of 306 cases of myeloproliferative disorders followed over a period of 21 years, 18 cases of well‐documented acute leukaemia were encountered. Leukaemias were either acute myeloblastic or myelomonocytic and occurred from 6 months to 20 years after the initial diagnosis. Onset was relatively abrupt and the course rapidly fatal with a median survival of 4 weeks. Due to the prolonged preleukaemic phase, it was possible to carry out a variety of clinical and laboratory observations. While no consistent features were noted, dysplastic haemopoiesis, a fall in leucocyte alkaline phosphatase activity, presence of Pelger‐Hüet anomaly and other abnormalities suggest a disturbance in granulocytic maturation. These findings suggest that, following an initial injury to a pleuripotential haemopoietic stem cell, a prolonged ‘latent’period occurs and, due to exposure to additional injurious agents or to a lack of cell regulating factors, acute leukaemia develops.