Mast cell leukemia

ICD-O-1 Morphology
9900/3
Effective 1978 - 1991
ICD-O-2 Morphology
9900/3
Effective 1992 - 2000
ICD-O-3 Morphology
9742/3
Effective 2001 and later
Reportable
for cases diagnosed 1978 and later
Primary Site(s)
C421
Primary site must be bone marrow (C421)

Help me code for diagnosis year :

Grade
9 - Grade/differentiation unknown, not stated, or not applicable
Module Rule
None
Alternate Names
MCL
Definition
Criteria for systemic mastocytosis met, and bone marrow aspirate shows 20% or more of cells are mast cells or 10% or more mast cells seen in circulating blood, shape of mast cells and nuclei have malignant features. No associated clonal hematologic non-mast cell disease. Aleukemic mast cell leukemia is a rare variant where <10% of circulating nucleated cells are mast cells.
Abstractor Notes
Acute mast cell leukemia is an aggressive disorder with leukemic mast cells in blood and in large numbers in marrow. Mast cells must equal or exceed 20% of all nucleated cells in bone marrow aspirate smears. Bone marrow will show a diffuse, compact infiltrate of mast cells with a marked reduction of normal precursor blood cells and fat cells.

If the leukemia occurs before or simultaneously with Mast Cell Sarcoma (9740/3), see M3 and Module 5:PH 9.
Definitive Diagnostic Methods
Bone marrow biopsy
Genetic testing
Immunophenotyping
Genetics Data
Somatic point mutation at codon 816 in KIT receptor gene (most common mutation is D816V)
Immunophenotyping
CD2+
C9+
CD14-
CD15-
CD16-
CD25+
CD33+
CD45+
CD68+
CD117+
Immunohistochemical markers anti-tryptasechymas and anti-CD117
Treatments
Blood thinners; anti-coagulant medications
Chemotherapy
Other RX
Transformations to
There are no known transformations
Transformations from
There are no known transformations
Corresponding ICD-9 Codes
207.8 Other specified leukemia
Corresponding ICD-10 Codes
C94.3 Mast cell leukemia
Corresponding ICD-10-CM Codes (effective October 1, 2015 U.S. only)
C94.3 Mast cell leukemia
Signs and Symptoms
Abdominal pain
Bleeding (hemorrhage)
Bone pain
Dermatographism
Diaphoresis
Flushing
Fractures
Gastrointestinal distress
Headache
Osteopenia/osteoporosis
Respiratory problems
Weight loss
Diagnostic Exams
Progression and Transformation
None
Epidemiology and Mortality
Survival: ~ few months