Juvenile myelomonocytic leukaemia: A case series

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2 Citations (Scopus)

Abstract

Juvenile myelomonocytic leukaemia (JMML), previously known as juvenile chronic myeloid leukaemia (JCML) is a rare, myelodysplastic - myeloproliferative disease typically presenting in early childhood. This disorder is difficult to distinguish from other myeloproliferative syndrome such as chronic myeloid leukaemia (CML) because of the similarities in their clinical and bone marrow findings. However, because of its unique biological characteristics such as absolute monocytosis with dysplasia, absence of Philadelphia chromosome or BCR-ABL fusion protein, hypergammaglobulinaemia and raised fetal haemoglobin level, this disorder does not satisfy the criteria for inclusion in the CML or chronic myelomonocytic leukaemia (CMML) group, as seen in adult patients. We describe a series of three patients with JMML, who had almost similar clinical and laboratory findings, and discuss the difficulty in the classification and treatment of the disease.

Original languageEnglish
Pages (from-to)121-128
Number of pages8
JournalMalaysian Journal of Pathology
Volume31
Issue number2
Publication statusPublished - Dec 2009

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Juvenile Myelomonocytic Leukemia
Leukemia, Myelogenous, Chronic, BCR-ABL Positive
Myelodysplastic-Myeloproliferative Diseases
Leukemia, Myelomonocytic, Chronic
Hypergammaglobulinemia
Fetal Hemoglobin
Philadelphia Chromosome
Bone Marrow
Proteins
Therapeutics

Keywords

  • Juvenile myelomonocytic leukaemia
  • Myelodysplastic - myeloproliferative disease

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Cell Biology
  • Histology

Cite this

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title = "Juvenile myelomonocytic leukaemia: A case series",
abstract = "Juvenile myelomonocytic leukaemia (JMML), previously known as juvenile chronic myeloid leukaemia (JCML) is a rare, myelodysplastic - myeloproliferative disease typically presenting in early childhood. This disorder is difficult to distinguish from other myeloproliferative syndrome such as chronic myeloid leukaemia (CML) because of the similarities in their clinical and bone marrow findings. However, because of its unique biological characteristics such as absolute monocytosis with dysplasia, absence of Philadelphia chromosome or BCR-ABL fusion protein, hypergammaglobulinaemia and raised fetal haemoglobin level, this disorder does not satisfy the criteria for inclusion in the CML or chronic myelomonocytic leukaemia (CMML) group, as seen in adult patients. We describe a series of three patients with JMML, who had almost similar clinical and laboratory findings, and discuss the difficulty in the classification and treatment of the disease.",
keywords = "Juvenile myelomonocytic leukaemia, Myelodysplastic - myeloproliferative disease",
author = "{Raja Sabudin}, {Raja Zahratul Azma} and {Abdul Latiff}, Zarina and Hamidah Alias and {A. Jamal}, {A. Rahman} and Sharifah, {N. A.} and O. Ainoon and Hussin, {Noor Hamidah}",
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T1 - Juvenile myelomonocytic leukaemia

T2 - A case series

AU - Raja Sabudin, Raja Zahratul Azma

AU - Abdul Latiff, Zarina

AU - Alias, Hamidah

AU - A. Jamal, A. Rahman

AU - Sharifah, N. A.

AU - Ainoon, O.

AU - Hussin, Noor Hamidah

PY - 2009/12

Y1 - 2009/12

N2 - Juvenile myelomonocytic leukaemia (JMML), previously known as juvenile chronic myeloid leukaemia (JCML) is a rare, myelodysplastic - myeloproliferative disease typically presenting in early childhood. This disorder is difficult to distinguish from other myeloproliferative syndrome such as chronic myeloid leukaemia (CML) because of the similarities in their clinical and bone marrow findings. However, because of its unique biological characteristics such as absolute monocytosis with dysplasia, absence of Philadelphia chromosome or BCR-ABL fusion protein, hypergammaglobulinaemia and raised fetal haemoglobin level, this disorder does not satisfy the criteria for inclusion in the CML or chronic myelomonocytic leukaemia (CMML) group, as seen in adult patients. We describe a series of three patients with JMML, who had almost similar clinical and laboratory findings, and discuss the difficulty in the classification and treatment of the disease.

AB - Juvenile myelomonocytic leukaemia (JMML), previously known as juvenile chronic myeloid leukaemia (JCML) is a rare, myelodysplastic - myeloproliferative disease typically presenting in early childhood. This disorder is difficult to distinguish from other myeloproliferative syndrome such as chronic myeloid leukaemia (CML) because of the similarities in their clinical and bone marrow findings. However, because of its unique biological characteristics such as absolute monocytosis with dysplasia, absence of Philadelphia chromosome or BCR-ABL fusion protein, hypergammaglobulinaemia and raised fetal haemoglobin level, this disorder does not satisfy the criteria for inclusion in the CML or chronic myelomonocytic leukaemia (CMML) group, as seen in adult patients. We describe a series of three patients with JMML, who had almost similar clinical and laboratory findings, and discuss the difficulty in the classification and treatment of the disease.

KW - Juvenile myelomonocytic leukaemia

KW - Myelodysplastic - myeloproliferative disease

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