Name

Myelodysplastic syndrome with excess blasts

ICD-O-2 Morphology

9983/1: Refractory anemia with excess of blasts

ICD-O-3 Morphology

9983/3: Refractory anemia with excess blasts
Effective 2001 and later

Reportable

for cases diagnosed 2001 and later

Primary Site(s)

C421
Primary site must be bone marrow (C421)

Grade

Not Applicable

Module Rule

None

Alternate Names

RAEB
RAEB I
RAEB II
RAEB-T
Refractory anemia with excess blasts
Refractory anemia with excess blasts in transformation

Definition

RAEB-1 (Type 1) and RAEB-2 (Type 2) are defined based on the number of blasts:
1. RAEB-1: 5-9% blasts in bone marrow, < 5% blasts in blood
2. RAEB-2: 10-19% blasts in bone marrow
(note if 5-19% blasts in blood and < 10% blasts in marrow, should be RAEB-2 OR RAEB with Auer rods). aka RAEB-T (RAEB in transformation which has ICD-O-3 code of 9984/3)

Abstractor Notes

The principle sites of involvement are the peripheral blood and bone marrow. Any one of the cell types can be low and look abnormal under the microscope.

For MDS diseases (9980, 9982, 9983, 9985, 9986, 9989, 9991, 9992), abstracting each of the subtypes would result in over-counting of the diseases.
1. Code only the first subtype that is diagnosed.
2. Do not change the histology code or create a new abstract for any subsequent specific MDS subtypes

Code 9984/3 in ICD-O-3 is for: RAEB-T (Refractory anemia with excess blast in transformation). This code became obsolete effective 1/1/2010. Any cases noted to be RAEB-T for diagnosis dates 1/1/2010 and forward should be coded to 9983/3.

Hematologic Transplant and/or Endocrine Procedures treatments include bone marrow and stem cell transplants.

Definitive Diagnostic Methods

Bone marrow biopsy
Genetic testing
Immunophenotyping

Genetics Data

-5
-7
8
del (5q)
del (7q)
del (20q)

Immunophenotyping

CD13
CD33
KIT (CD117)

Treatments

Chemotherapy
Hematologic Transplant and/or Endocrine Procedures
Immunotherapy

Transformations from

None

Corresponding ICD-9 Codes

238.73 High grade myelodysplastic syndrome

Corresponding ICD-10 Codes

D46.2 Refractory anemia with excess of blasts

Corresponding ICD-10-CM Codes (U.S. only)

D46.20 Refractory anemia with excess of blasts, unspecified (effective October 01, 2015)
D46.21 Refractory anemia with excess of blasts 1 (effective October 01, 2015)
D46.22 Refractory anemia with excess of blasts 2 (effective October 01, 2015)

Signs and Symptoms

Easy bruising or bleeding
Petechiae (flat, pinpoint spots under the skin caused by bleeding)
Shortness of breath
Skin paler than usual
Weakness or feeling tired

Progression and Transformation

~25% of RAEB-1 and 33% of RAEB-2 transform to AML

Epidemiology and Mortality

Age: > 50 years of age
Incidence: ~40% of all MDS
Sex: no male or female predominance
Survival: ~16 months for RAEB-1 and ~3-12 months for RAEB-2

Sources

Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J (Eds):
WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues (Revised 4th edition)
IARC: Lyon 2017
Section: Myelodysplastic syndromes
Pages: 113-114

International Classification of Diseases for Oncology, Third Edition, First Revision. Geneva: World Health Organization, 2013.
Section: ICD-O-3.1 (2011) Morphological Codes
Pages: http://codes.iarc.fr/codegroup/2

National Cancer Institute
Section: General Information About Myelodysplastic Syndromes
Pages: https://www.cancer.gov/types/myeloproliferative/patient/myelodysplastic-treatment-pdq
Glossary