TY - JOUR
T1 - bcr/abl-negative, classic myeloproliferative disorders
T2 - Diagnosis and treatment
AU - Tefferi, Ayalew
AU - Barbui, Tiziano
PY - 2005/9
Y1 - 2005/9
N2 - Essential thrombocythemia, polycythemia vera, and myelofibrosis with myelold metaplasia constitute the "classic" bcr/abl-negative myeloproliferative disorders (MPDs). Each of these MPDs represents a stem cell-derived clonal myeloprollferation with the respective features of thrombocytosis, erythrocytosis, and bone marrow fibrosis. Unlike with cases of chronic myeloid leukemia, in which the bcr/abl mutation is Invariably detected, current diagnosis of essential thrombocythemia, polycythemia vera, and myelofibrosis with myelold metaplasia Is based on a consensus-driven set of clinical and laboratory criteria that have undergone substantial modification In recent times. The recent discovery of a recurrent activating Janus tyrosine kinase (JAK2) mutation (JAK2V617F) In all 3 classic MPDs offers another opportunity for refining current diagnoses and disease classifications. In this article, we outline contemporary diagnostic algorithms for each of these disorders and provide an evidence-based approach to management.
AB - Essential thrombocythemia, polycythemia vera, and myelofibrosis with myelold metaplasia constitute the "classic" bcr/abl-negative myeloproliferative disorders (MPDs). Each of these MPDs represents a stem cell-derived clonal myeloprollferation with the respective features of thrombocytosis, erythrocytosis, and bone marrow fibrosis. Unlike with cases of chronic myeloid leukemia, in which the bcr/abl mutation is Invariably detected, current diagnosis of essential thrombocythemia, polycythemia vera, and myelofibrosis with myelold metaplasia Is based on a consensus-driven set of clinical and laboratory criteria that have undergone substantial modification In recent times. The recent discovery of a recurrent activating Janus tyrosine kinase (JAK2) mutation (JAK2V617F) In all 3 classic MPDs offers another opportunity for refining current diagnoses and disease classifications. In this article, we outline contemporary diagnostic algorithms for each of these disorders and provide an evidence-based approach to management.
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U2 - 10.4065/80.9.1220
DO - 10.4065/80.9.1220
M3 - Article
C2 - 16178503
AN - SCOPUS:24144461147
SN - 0025-6196
VL - 80
SP - 1220
EP - 1232
JO - Mayo Clinic proceedings
JF - Mayo Clinic proceedings
IS - 9
ER -