This neoplasm is not reportable.

Name

Transient abnormal myelopoiesis (TAM)

ICD-O-3 Morphology

9898/1: Transient abnormal myelopiesis
Effective 2010 and later

Reportability

This neoplasm is not reportable

Primary Site(s)

No primary site specified
Primary site bone marrow (C421)
This hematopoietic disease is not reportable for the U.S.
Canada, refer to your specific province for reportability requirements.

Abstractor Notes

Transient abnormal myelopoiesis (TAM) is part of the Secondary myeloid lineage table in the WHO 5th edition of Hematolymphoid Tumors. (See Appendix B in the Hematopoietic Manual, Table B7)

TAM manifests mainly in the peripheral blood. Severe cases affect the liver, spleen, pleura, and pericardium. Bone marrow involvement is usually mild.

TAM can be a life-threatening disease to an asymptomatic incidental finding.

Alternate Names

Transient abnormal myelopoiesis associated with Down syndrome

Definition

Transient abnormal myelopoiesis (TAM) associated with Down syndrome is a unique disorder of newborns with Down syndrome that presents with clinical and morphological findings indistinguishable from those of acute myeloid leukemia. The blasts have morphological and immunological features of megakaryocytic lineage.

Definitive Diagnostic Methods

This data item does not apply

Genetics Data

This data item does not apply

Immunophenotyping

This data item does not apply

Corresponding ICD-10 Codes (Cause of Death codes only)

D47.7 Other specified neoplasms of uncertain or unknown behavior of lymphoid, hematopoietic, and related tissue

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

D47.Z9 Other specified neoplasms of uncertain behavior of lymphoid, hematopoietic and related tissue (effective October 01, 2015)

Signs and Symptoms

Cholestatic jaundice
Hepatomegaly (severe disease only)
Leukocytosis
Pleural/pericardial effusion (several disease only)
Splenomegaly (severe disease only)
Vesiculopapular skin rash (severe disease only)

Diagnostic Exams

Bone marrow aspiration and biopsy
CT (CAT) scan
Cytogenetic analysis
Flow cytometry
Immunophenotyping
Lumbar puncture
Molecular analysis
Peripheral blood smear
Physical exam and history

Epidemiology and Mortality

25-30% of newborns with Down Syndrome have TAM
TAM is the most common leukemia in neonates

Sources

WHO Classification of Tumours Editorial Board. Haematolymphoid tumours. Lyon (France): International Agency for Research on Cancer; 2024. (WHO classification of tumours series, 5th ed.; vol. 11). https://publications.iarc.who.int/637.
Section: Myeloid proliferations associated with Down syndrome
Pages: Part A: 182-183

International Classification of Diseases for Oncology, 3rd edition (including revisions). Geneva: World Health Organization, 2001, 2011, 2020.
Section: ICD-O-3.2 (2020) Morphological Codes
Pages: http://www.iacr.com.fr/index.php?option=com_content&view=category&layout=blog&id=100&Itemid=577
Glossary