BCR-ABL positive essential thrombocythaemia

A variant of chronic myelogerous leukaemia or a distinct clinical entity: A special case report

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3 Citations (Scopus)

Abstract

A 37-year-old Malay man presented initially with the clinical picture of essential thrombocythaemia (ET) without the extreme leukocytosis, marked splenomegaly and low neutrophil alkaline phosphatase characteristic of chronic myelogenous leukaemia (CML). Bone marrow examination showed massive megakaryocytic hyperplasia; cytogenetic studies showed the presence of Philadelphia chromosome. The patient was treated with hydroxyurea that resulted in reduction in the platelet count. Seventeen months later, he presented with fever associated with tender massive splenomegaly. Bone marrow finding was consistent with chronic phase CML. The presence of a rearrangement involving the major breakpoint cluster region (M-bcr) on chromosome 22 was confirmed by reverse transcriptase - polymerase chain reaction. The clinical importance of finding the Philadelphia chromosome in patients who seem to have ET is in assessing prognosis. ET generally follows a chronic, indolent course. However, this patient who had Philadelphia chromosome underwent clinical transition to chronic phase CML17 months and blast crisis 29 months after presentation.

Original languageEnglish
Pages (from-to)595-598
Number of pages4
JournalSingapore Medical Journal
Volume41
Issue number12
Publication statusPublished - Dec 2000

Fingerprint

Essential Thrombocythemia
Philadelphia Chromosome
Leukemia
Splenomegaly
Leukemia, Myeloid, Chronic Phase
Bone Marrow Examination
Blast Crisis
Chromosomes, Human, Pair 22
Hydroxyurea
Leukocytosis
Leukemia, Myelogenous, Chronic, BCR-ABL Positive
Reverse Transcriptase Polymerase Chain Reaction
Platelet Count
Cytogenetics
Hyperplasia
Alkaline Phosphatase
Neutrophils
Fever
Bone Marrow

Keywords

  • BCR-ABL fusion protein
  • Chronic myelogenous leukaemia
  • Essential thrombocythaemia
  • Philadelphia chromosome

ASJC Scopus subject areas

  • Medicine(all)

Cite this

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title = "BCR-ABL positive essential thrombocythaemia: A variant of chronic myelogerous leukaemia or a distinct clinical entity: A special case report",
abstract = "A 37-year-old Malay man presented initially with the clinical picture of essential thrombocythaemia (ET) without the extreme leukocytosis, marked splenomegaly and low neutrophil alkaline phosphatase characteristic of chronic myelogenous leukaemia (CML). Bone marrow examination showed massive megakaryocytic hyperplasia; cytogenetic studies showed the presence of Philadelphia chromosome. The patient was treated with hydroxyurea that resulted in reduction in the platelet count. Seventeen months later, he presented with fever associated with tender massive splenomegaly. Bone marrow finding was consistent with chronic phase CML. The presence of a rearrangement involving the major breakpoint cluster region (M-bcr) on chromosome 22 was confirmed by reverse transcriptase - polymerase chain reaction. The clinical importance of finding the Philadelphia chromosome in patients who seem to have ET is in assessing prognosis. ET generally follows a chronic, indolent course. However, this patient who had Philadelphia chromosome underwent clinical transition to chronic phase CML17 months and blast crisis 29 months after presentation.",
keywords = "BCR-ABL fusion protein, Chronic myelogenous leukaemia, Essential thrombocythaemia, Philadelphia chromosome",
author = "{S. Abdul Wahid}, {S Fadilah} and Cheong, {S. K.}",
year = "2000",
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language = "English",
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pages = "595--598",
journal = "Singapore Medical Journal",
issn = "0037-5675",
publisher = "Singapore Medical Association",
number = "12",

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TY - JOUR

T1 - BCR-ABL positive essential thrombocythaemia

T2 - A variant of chronic myelogerous leukaemia or a distinct clinical entity: A special case report

AU - S. Abdul Wahid, S Fadilah

AU - Cheong, S. K.

PY - 2000/12

Y1 - 2000/12

N2 - A 37-year-old Malay man presented initially with the clinical picture of essential thrombocythaemia (ET) without the extreme leukocytosis, marked splenomegaly and low neutrophil alkaline phosphatase characteristic of chronic myelogenous leukaemia (CML). Bone marrow examination showed massive megakaryocytic hyperplasia; cytogenetic studies showed the presence of Philadelphia chromosome. The patient was treated with hydroxyurea that resulted in reduction in the platelet count. Seventeen months later, he presented with fever associated with tender massive splenomegaly. Bone marrow finding was consistent with chronic phase CML. The presence of a rearrangement involving the major breakpoint cluster region (M-bcr) on chromosome 22 was confirmed by reverse transcriptase - polymerase chain reaction. The clinical importance of finding the Philadelphia chromosome in patients who seem to have ET is in assessing prognosis. ET generally follows a chronic, indolent course. However, this patient who had Philadelphia chromosome underwent clinical transition to chronic phase CML17 months and blast crisis 29 months after presentation.

AB - A 37-year-old Malay man presented initially with the clinical picture of essential thrombocythaemia (ET) without the extreme leukocytosis, marked splenomegaly and low neutrophil alkaline phosphatase characteristic of chronic myelogenous leukaemia (CML). Bone marrow examination showed massive megakaryocytic hyperplasia; cytogenetic studies showed the presence of Philadelphia chromosome. The patient was treated with hydroxyurea that resulted in reduction in the platelet count. Seventeen months later, he presented with fever associated with tender massive splenomegaly. Bone marrow finding was consistent with chronic phase CML. The presence of a rearrangement involving the major breakpoint cluster region (M-bcr) on chromosome 22 was confirmed by reverse transcriptase - polymerase chain reaction. The clinical importance of finding the Philadelphia chromosome in patients who seem to have ET is in assessing prognosis. ET generally follows a chronic, indolent course. However, this patient who had Philadelphia chromosome underwent clinical transition to chronic phase CML17 months and blast crisis 29 months after presentation.

KW - BCR-ABL fusion protein

KW - Chronic myelogenous leukaemia

KW - Essential thrombocythaemia

KW - Philadelphia chromosome

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