Chronic myelomonocytic leukemia

Name
Chronic myelomonocytic leukemia
ICD-O-2 Morphology
9868/3
Effective 1992 - 2000
ICD-O-3 Morphology
9945/3
Effective 2001 and later
Reportable
for cases diagnosed 1992 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
Chronic myelomonocytic leukemia in transformation
Chronic myelomonocytic leukemia, NOS
Chronic myelomonocytic leukemia, Type I
Chronic myelomonocytic leukemia, Type II
Chronic myelomonocytic syndrome
CMML
CMML with eosinophilia
CMML-1
CMML-2
Subacute myelomonocytic leukemia
Definition
CMML is a clonal disorder of a bone marrow stem cell, in which monocytosis is a major defining feature. The clinical, hematologic and morphologic features of CMML are heterogeneous, and vary along a spectrum from predominantly myelodysplastic to mainly myeloproliferative in nature.

A slowly progressing type of myelodysplastic/myeloproliferative disease in which too many myelomonocytes (a type of white blood cell) are in the bone marrow, crowding out other normal blood cells, such as white blood cells, red blood cells and platelets. Also called chronic myelomonocytic leukemia.
Abstractor Notes
The peripheral blood and bone marrow are always involved. The spleen, liver, skin and lymph nodes are the most common sites of extramedullary infiltration. The peripheral blood has persistent monocytosis. There are fewer than 20% blasts in the peripheral blood and bone marrow.

The physician will run tests to exclude the BCR-ABL1 fusion gene, the rearrangement of PDGFRA or PDGFRB. The physician uses the information from these tests to do a diagnosis of exclusion (clinical diagnosis) of Chronic myelomonocytic leukemia.
Definitive Diagnostic Methods
Bone marrow biopsy
Clinical diagnosis
Genetic testing
Immunophenotyping
Peripheral blood smear
Genetics Data
-7/del(7q)
+8
Structural abnormalities of 12p
Immunophenotyping
CD13+
CD33+
Treatments
Bone marrow transplant
Chemotherapy
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)
C93.1 Chronic myelomonocytic leukemia
Signs and Symptoms
Fatigue
Fever
Hepatomegaly
Increased white blood count (WBC) (in some cases)
Night sweats
Splenomegaly
Weight loss
Progression and Transformation
Transformation to AML occurs in ~15-30% of cases
Epidemiology and Mortality
Age: 65-75 years median age
Incidence: 12.8 cases per 100,000 persons per year
Sex: male predominance
Survival: 20-40 months median survival