Name

Myelodysplastic syndrome with multilineage dysplasia

ICD-O-3 Morphology

9985/3: Refractory cytopenia with multilineage dysplasia
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

MDS-MLD
RCMD
Refractory cytopenia of childhood
Refractory cytopenia with multilineage dysplasia

Definition

Myelodysplastic syndrome (MDS) with multilineage dysplasia (MDS-MLD), is an MDS characterized by one or more cytopenias and dysplastic changes in two or more of the myeloid lineage (erythroid, granulocytic, and megakaryocytic).

Abstractor Notes

Blood and bone marrow are always involved. At least 2 types of blood counts are low and have an abnormal appearance under the microscope (dysplasia). The number of blasts is less than 5%.

This histology code also includes childhod MDS. MDS is very rare in children. Both the peripheral blood and bone marrow are involved.

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.

Definitive Diagnostic Methods

Bone marrow biopsy
Genetic testing

Genetics Data

del (5q)
del (7q)
del (20q)
Monosomy 5

Immunophenotyping

None

Treatments

Chemotherapy
Hematologic Transplant and/or Endocrine Procedures
Immunotherapy

Transformations from

None

Corresponding ICD-9 Codes

238.72 Low grade myelodysplastic syndrome lesions

Corresponding ICD-10 Codes

D46.7 Other myelodysplastic syndromes

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

D46.A Refractory cytopenia with multilineage dysplasia (effective October 01, 2015)
D46.B Refractory cytopenia with multilineage dysplasia and ring sideroblasts (effective October 01, 2015)

Signs and Symptoms

Cytopenia (of two or more myeloid lines)
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

~10% of cases evolve to AML in 2 years

Epidemiology and Mortality

Age (Adult): 70 years median age
Age (Childhood): all age groups
Incidence (Adult): accounts for ~30% of MDS cases
Incidence (Childhood): most common MDS of childhood, ~50%
Sex (Adult): slight male predominance
Sex (Childhood): no male or female predominance
Survival: 30 month median survival (~1/2 of patients died within 2 years)

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: 111-113, 116-120

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