Biomaterial Database

Philadelphia chromosome positive essential thrombocythemia evolving into lymphoid blast crisis. 1987

E Paietta, and N Rosen, and M Roberts, and P Papenhausen, and P H Wiernik

The Philadelphia chromosome with the classic translocation t(9;22) was detected in all metaphases of a patient with essential thrombocythemia who presented without hematologic signs of chronic myeloid leukemia (CML). After 12 months of treatment with hydroxyurea followed by 6 months of busulfan, the disease transformed into lymphoid blast crisis with the immunophenotype of pre-pre-B cells frequently seen in lymphoid blast crisis of CML. This progression was not associated with karyotypic evolution because lymphoblasts contained only the Philadelphia chromosome. Further clinical course in this patient confirmed the development of a Philadelphia chromosome positive stem cell with predominantly megakaryocytic committment through partially megakaryocytic/lymphoid differentiation to purely lymphoid committment, a previously unreported transformation.

UI	MeSH Term	Description	Entries
D007621	Karyotyping	Mapping of the KARYOTYPE of a cell.	Karyotype Analysis Methods,Analysis Method, Karyotype,Analysis Methods, Karyotype,Karyotype Analysis Method,Karyotypings,Method, Karyotype Analysis,Methods, Karyotype Analysis
D007945	Leukemia, Lymphoid	Leukemia associated with HYPERPLASIA of the lymphoid tissues and increased numbers of circulating malignant LYMPHOCYTES and lymphoblasts.	Leukemia, Lymphocytic,Lymphocytic Leukemia,Lymphoid Leukemia,Leukemias, Lymphocytic,Leukemias, Lymphoid,Lymphocytic Leukemias,Lymphoid Leukemias
D010677	Philadelphia Chromosome	An aberrant form of human CHROMOSOME 22 characterized by translocation of the distal end of chromosome 9 from 9q34, to the long arm of chromosome 22 at 22q11. It is present in the bone marrow cells of 80 to 90 per cent of patients with chronic myelocytic leukemia (LEUKEMIA, MYELOGENOUS, CHRONIC, BCR-ABL POSITIVE).	Ph1 Chromosome,Ph 1 Chromosome,1 Chromosomes, Ph,Chromosome, Ph 1,Chromosome, Ph1,Chromosome, Philadelphia,Chromosomes, Ph 1,Chromosomes, Ph1,Ph 1 Chromosomes,Ph1 Chromosomes
D011289	Preleukemia	Conditions in which the abnormalities in the peripheral blood or bone marrow represent the early manifestations of acute leukemia, but in which the changes are not of sufficient magnitude or specificity to permit a diagnosis of acute leukemia by the usual clinical criteria.	Preleukemias
D001752	Blast Crisis	An advanced phase of chronic myelogenous leukemia, characterized by a rapid increase in the proportion of immature white blood cells (blasts) in the blood and bone marrow to greater than 30%.	Blast Phase,Blast Crises,Blast Phases,Crises, Blast,Crisis, Blast,Phase, Blast,Phases, Blast
D005260	Female		Females
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000328	Adult	A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available.	Adults
D013920	Thrombocythemia, Essential	A clinical syndrome characterized by repeated spontaneous hemorrhages and a remarkable increase in the number of circulating platelets.	Hemorrhagic Thrombocythemia,Thrombocythemia, Hemorrhagic,Thrombocythemia, Idiopathic,Thrombocythemia, Primary,Primary Thrombocythemia,Thrombocytosis, Autosomal Dominant,Thrombocytosis, Primary,Autosomal Dominant Thrombocytoses,Autosomal Dominant Thrombocytosis,Dominant Thrombocytoses, Autosomal,Dominant Thrombocytosis, Autosomal,Essential Thrombocythemia,Essential Thrombocythemias,Hemorrhagic Thrombocythemias,Idiopathic Thrombocythemia,Idiopathic Thrombocythemias,Primary Thrombocythemias,Primary Thrombocytoses,Primary Thrombocytosis,Thrombocythemias, Essential,Thrombocythemias, Hemorrhagic,Thrombocythemias, Idiopathic,Thrombocythemias, Primary,Thrombocytoses, Autosomal Dominant,Thrombocytoses, Primary