Name

Pure erythroid leukemia

ICD-O-1 Morphology

9840/3: Acute erythroid leukemia
9841/3: Acute erythremia
Effective 1978 - 1991

ICD-O-2 Morphology

9840/3: Acute erythroid leukemia
9841/3: Acute erythremia
Effective 1992 - 2000

ICD-O-3 Morphology

9840/3: Acute erythroid leukemia
Effective 2001 and later

Reportable

for cases diagnosed 1978 and later

Primary Site(s)

C421
Primary site must be bone marrow (C421)

Grade

Not Applicable

Module Rule

See abstractor notes

Alternate Names

Acute erythremia
Acute erythremic myelosis
Acute erythroid leukemia
AML M6
Di Guglielmo disease
Erythremic myelosis, NOS
FAB M6
M6A
M6B
Pure erythroid leukemia, M6b

Definition

Pure erthyroid leukemia is a neoplastic proliferation of immature cells (undifferentiated or proerythroblastic in appearance) committed exclusively to the erythroid lineage (> 80% of the bone marrow cells are erythroid, with greater than or equal to 30% proerythroblasts), with no evidence of a significant myeloblastic component.

Abstractor Notes

If the leukemia occurs before or simultaneously with Myeloid Sarcoma (9930/3), see M3 and Module 5:PH10.

Definitive Diagnostic Methods

Bone marrow biopsy
Immunophenotyping
Peripheral blood smear

Genetics Data

None

Immunophenotyping

CD71 expression
CD34 negative
CD36 positive
CD41 negative
CD61 negative
E-cadherin positive
HLA-DR negative
KIT (CD117) positive

Treatments

Chemotherapy
Hematologic Transplant and/or Endocrine Procedures

Transformations to

None

Corresponding ICD-9 Codes

207.0 Acute erythremia and erythroleukemia

Corresponding ICD-10 Codes

C94.0 Acute erythremia and erythroleukemia

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

C94.0 Acute erythroid leukemia (effective October 01, 2015)

Signs and Symptoms

Circulating erythroblasts
Easy bruising or bleeding
Shortness of breath
Weakness
Weight loss or loss of appetite

Progression and Transformation

None

Epidemiology and Mortality

Age: predominantly adults
Incidence: <5% of all AML cases
Survival: poor, aggressive clinical course

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: Acute myeloid leukemia and related precursor neoplasms
Pages: 161-162

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 Acute Myeloid Leukemia
Pages: https://www.cancer.gov/types/leukemia/hp/adult-aml-treatment-pdq
Glossary