Juvenile myelomonocytic leukemia

Name
Juvenile myelomonocytic leukemia
ICD-O-3 Morphology
9946/3
Effective 2001 and later
Reportable
for cases diagnosed 2001 and later
Primary Site(s)
C421
Primary site must be bone marrow (C421)

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Grade
9 - Grade/differentiation unknown, not stated, or not applicable
Module Rule
None
Alternate Names
JMML
Juvenile chronic myelomonocytic leukemia
Definition
A rare form of childhood leukemia in which cancer cells often spread into tissues such as the skin, lung and intestines.

JMML is a clonal hematopoietic disorder of childhood that is characterized by proliferation principally of the granulocytic and monocytic lineages. Erythroid and megakaryocytic abnormalities frequently present, in keeping with evidence that JMML arises from a BM stem cell with multilineage potential in the myeloid series.
Abstractor Notes
The peripheral blood and bone marrow are always involved; proliferation of the granulocytic and monocytic lineages. Blasts plus promonocytes account for <20% of PB and BM. Leukemic infiltrates are common in the skin.

The physician will test to confirm that the Philadelphia chromosome and the BCR-ABL1 fusion gene are absent.

Although JMML rarely transforms into acute leukemia, it is a rapidly-fatal disease for most children if left untreated.

In the absence of effective treatment most children die from organ failure, such as respiratory failure, due to leukemic infiltration. Stem cell transplant can cure about half of the patients.
Definitive Diagnostic Methods
Bone marrow biopsy
Genetic testing
Peripheral blood smear
Genetics Data
Monosomy 7
Immunophenotyping
None
Treatments
Chemotherapy
Radiation
Stem cell transplant
Surgery
Transformations from
None
Corresponding ICD-9 Codes
205.1 Chronic myeloid leukemia
Corresponding ICD-10 Codes
C92.1 Chronic myeloid leukemia
Corresponding ICD-10-CM Codes (effective October 1, 2015 U.S. only)
C93.3 Juvenile myelomonocytic leukemia
Signs and Symptoms
Easy bruising or bleeding
Enlarged tonsils
Fatigue
Fever
Frequent infections
Hepatosplenomegaly
Infection
Lymphadenopathy
Pain or a feeling of fullness below the ribs
Pale skin
Petechiae
Shortness of breath
Skin rashes
Recurrence and Metastases
Rarely transforms to acute myeloid leukemia
Epidemiology and Mortality
Age: 0-14 years (75% of cases <3 years of age)
Incidence: 1.3 per million children 0-14 years of age per year
Sex: male predominance
Survival: 1 year (without stem cell transplant). Cure is possible for patients who have a stem cell transplant