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

Help me code for dx year :

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. Most patients present with constitutional symptoms or evidence of infection. There is generally marked hepatosplenomegaly. Occasionally spleen size is normal at diagnosis but rapidly increases thereafter. Although JMML rarely transforms into Acute leukemia, it is a rapidly-fatal disease for most children if left untreated. The median survival time without allogeneic stem cell transplant is about one year. 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
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
None
Diagnostic Exams
None
Recurrence and Metastases
None
Epidemiology and Mortality
None