Myelodysplastic syndrome associated with isolated del(5q)

Name
Myelodysplastic syndrome associated with isolated del(5q)
ICD-O-3 Morphology
9986/3
Effective 2001 and later
Reportable
for cases diagnosed 2001 and later
Primary Site(s)
C421

Help me code for diagnosis year :

Grade
9 - Grade/differentiation unknown, not stated, or not applicable
Module Rule
None
Alternate Names
5q- syndrome
MDS with 5q deletion
Myelodysplastic syndrome with 5q deletion (5q-syndrome)
Definition
Bone marrow dysplasia showing refractory macrocytic anemia with oval macrocytes and nonlobulated megakaryocytes. 75% of patients have increased cellularity of marrow. One third of patients have chromosomal abnormality that consists of deletion of the long arm of chromosome 5 (5q). Marrow blasts <5%.
Platelet count normal to elevated
No Auer rods
Normal to increased megakaryocytes
Abstractor Notes
MDS with associated del(5q) is an MDS involves that blood and bone marrow. The most common symptoms are usually related to anemia which is often severe and usually macrocytic. Thrombocytosis is present in up to one half of patients while thrombocytopenia is uncommon. This disease is associated with a median survival of 145 months with transformation to acute myeloid leukemia occurring in less than ten percent of patients. Recently the thalidomide analog lenalidomide has been shown to benefit MDS patients. Transfusion independence was achieved in two-thirds of patients.

MDS is a group name for a number of specific diseases. As the MDS progresses, it may manifest as several subtypes. This is a part of the disease process. Abstracting each of the subtypes would result in over-counting these diseases. Code the first subtype that is diagnosed. Do not change the histology code or create a new abstract for any subsequent specific MDS subtypes.
Definitive Diagnostic Methods
Bone marrow biopsy
Genetic testing
Genetics Data
Deletion between bands q31 and 33 on chromosome 5
Immunophenotyping
None
Treatments
Chemotherapy
Other treatment
Transformations from
None
Corresponding ICD-9 Codes
238.74 Myelodysplastic syndrome with 5q deletion
Corresponding ICD-10 Codes
D46.7 Other myelodysplastic syndromes
Corresponding ICD-10-CM Codes (effective October 1, 2015 U.S. only)
D46.C Myelodysplastic syndrome with isolated del(5q) chromosomal abnormality
Signs and Symptoms
None
Diagnostic Exams
None
Recurrence and Metastases
None
Epidemiology and Mortality
None