JUVENILE MYELOMONOCYTIC LEUKEMIA

Description:

Serious chronic leukemia- affecting children aged 4 & below

Symptoms:

  • General fatigue or weakness
  • Recurrent infections
  • Bone and joint pain
  • Abdominal pain
  • Swollen lymph nodes (painless)
  • Pallor (abnormal paleness of the skin)
  • Abnormally enlarged liver and/or spleen
  • Shortness of breath with minimal exercise
  • Looking pale
  • Sore mouth due to mouth ulcers
  • Purpura – a rash of small red dots seen often on the lower limbs
  • Easy bruising
  • Tendency to bleeding from the nose and gum
  • Infections (bronchitis and tonsillitis)
  • Pain or a feeling of fullness below the ribs
  • Skin rashes
  • Small yellowish skin tumours
  • Abnormal bone marrow function
  • Decreased appetite, poor weight gain, irritability
  • Maculopapular rash

Causes:

  • Genetic mutations (acquired mutation)
  • Abnormal change-white blood cells affected
  • Number of blood cells- usually lower than normal
  • Abnormalities in DNA- cellular malignant transformation

Risk factors:

  • Rare: accounts for 2-3% of all childhood leukemias
  • Children with neurofibromatosis-500 times more chances of having JMML
  • Sex: Males are affected more often than females
  • Congenital condition in infants (below 3 months)
  • Mutations in one of the RAS family oncogenes-25% chances of having JMML

Diagnostic tests:

  • Blood test
  • Bone marrow aspiration and biopsy
  • Imaging studies
  • Lumbar puncture
  • Flow cytometry tests
  • Chromosomal tests
  • Tissue typing or HLA (human leukocyte antigen) typing
  • GM-CSF hypersensitivity assay
  • Evaluation of liver and kidney functions
  • Genetic studies.

Differential diagnostics:

  • Hematolymphoid disorders with neutrophilia and or mastocytosis
  • Hypereosinophilia
  • Loeffler syndrome
  • Collagen vascular diseases
  • Kimura disease
  • Chronic myelomonocytic leukemia (CMML)
  • Acute myeloid leukemia
  • Chronic myeloid leukemia
  • leukemoid reaction
  • Pharyngo-tonsillitis or bronchitis
  • Hepatomegaly and lymphadenopathy
  • Enlarged spleen

Treatments:

  • Bone marrow transplant
  • A) Stem cell transplant.
  • High doses of chemotherapy,
  • Radiation therapy (some cases)
  • Aggressive therapy

Medications:

  • Targeted therapies
  • DNA-hypomethylating agents
  • Nonintensive treatment (6-mercaptopurine, 6-thioguanine)
  • Tipifarnib- stop the growth of JMML Cell

Lifestyle Management:

  • Prompt medical attention
  • Genetic counseling
  • Opportunities to speak with other childhood cancer survivors