Chronic eosinophilic leukemia, NOS

Name
Chronic eosinophilic leukemia, NOS
ICD-O-1 Morphology
9810/3
Effective 1978 - 1991
ICD-O-2 Morphology
9865/3
Effective 1992 - 2000
ICD-O-3 Morphology
9964/3
Effective 2001 and later
Reportable
for cases diagnosed 1978 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
CEL
Chronic eosinophilic leukemia
Chronic eosinophilic leukemia (and the hypereosinophilic syndrome)
HES
Hypereosinophilic (idiopathic) syndrome
Hypereosinophilic syndrome
Definition
Persistent high eosinophil count when all known causes of a raised eosiniphil count (such as parasite infection) have been excluded. Rare variant of acute myeloid leukemia in which blasts and immature eosinphils proliferate. CNS involvement appears to be common.

Autonomous clonal proliferation of eosinophilic precursors results in persistently increased numbers of eosinophils in the blood, bone marrow, and peripheral tissues.
Abstractor Notes
Diagnosis of exclusion when secondary and clonal causes of eosinophilia are excluded. HES is proven by persistent eosinophilia that is associated with damage to multiple organs. CEL is a multisystem disorder. The peripheral blood and bone marrow are always involved. Tissue infiltration by the eosinophils and release of cytokines and tumoral factors from the eosinophil granules lead to tissue damage in a number of organs, but the heart, lungs, CNS, skin and GI tract are commonly involved. The patient must have a sustained absolute eosinophil count greater than 500/pl that persists longer than six months. The physician excludes reactive eosinophilia, eosinophilia secondary to other neoplastic diseases including T-cell neoplasia and other myeloid disorders. If above excludes peripheral blood blasts >2%, bone marrow >5% but <19%. HES is traditionally treated with prednisone. Second-line drugs are interferon or hydroxyurea which induce remission in the majority of patients. If the prednisone. interferon and hydroxurea are not effective, the patient may receive various chemotherapeutic drugs.
Definitive Diagnostic Methods
Bone marrow biopsy
Clinical diagnosis
Peripheral blood smear
Genetics Data
8
8p11 syndromes
i(17q)
t(6;8)(q27;p11)
t(8;13)(p11;q12)
t(8;9)(p11;q32-34)
Immunophenotyping
None
Treatments
Bone marrow transplant
Chemotherapy
Hormone
Immunotherapy
Surgery
Transformations from
None
Corresponding ICD-9 Codes
207.8 Other specified leukemia
Corresponding ICD-10 Codes
C94.7 Other specified leukemias
Corresponding ICD-10-CM Codes (effective October 1, 2015 U.S. only)
C94.8 Other specified leukemias
Signs and Symptoms
None
Diagnostic Exams
None
Recurrence and Metastases
None
Epidemiology and Mortality
None