Search Database ICD-O-3 Code Lists

Name

Blastic plasmacytoid dendritic cell neoplasm (BPDC)

ICD-O-3 Morphology

9727/3: Blastic plasmacytoid dendritic cell lymphoma
Effective 2001 and later

Reportable

for cases diagnosed 1978 and later

Primary Site(s)

See Module 7
Most common sites of involvement: skin, bone marrow, peripheral blood, and lymph nodes

See also abstractor notes

Coding Manual: Hematopoietic Coding Manual (PDF)

Abstractor Notes

Blastic plasmacytoid dendritic cell neoplasm (BPDC) is part of the Plasmacytoid dendritic cell neoplasms lineage table in the WHO 5th edition of Hematolymphoid Tumors. (See Appendix B in the Hematopoietic Manual, Table B10)

Skin is the most common site of involvement, with about 60-95% of patients presenting with skin lesions. Most common parts of skin include the upper thorax, head and neck, and upper extremities.

Some patients have a leukemic presentation (bone marrow involvement) without extramedullary disease.

CNS involvement may be detected at diagnosis (as metastatic disease), is common at relapse. 

In addition, patients may present with antecedent (before) or concurrent (same time) with myelodysplastic or myeloproliferative myelodysplastic neoplasm.

Diagnostic confirmation of this neoplasm based on immunophenotyping is expression of CD123 and one other pDC marking in addition to CD4 and/or CD56
OR Expression of any three pDC markers and absent expression of all expected negative markers (see immunophenotyping fields).

Diagnostic Confirmation

This histology can be determined by positive histology (including peripheral blood) with or without genetics and/or immunophenotyping. Review the Definitive Diagnostic Methods, Immunophenotyping and Genetics Data sections below, and the instructions in the Hematopoietic Manual for further guidance on assigning Diagnostic confirmation.

Module Rule

Alternate Names

Lymphoblastoma [OBS] (see 9811/3 for 2010+)
Malignant lymphoma, convoluted cell [OBS] (see 9811/3 for 2010+)
Malignant lymphoma, lymphoblastic, NOS (see 9811/3 for 2010+)
Precursor cell lymphoblastic leukemia (see 9811/3 for 2010+)

Definition

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a haematological neoplasm consisting of immature cells with plasmacytoid dendritic cell (pDC) differentiation, characterized by a high frequency of cutaneous involvement and systemic dissemination. (WHO 5th edition)

Definitive Diagnostic Methods

Bone marrow biopsy
Genetic testing
Histologic confirmation
Immunophenotyping

Genetics Data

Germline T-cell and B-cell receptor genes
G1/S transition (CDKN2A, CDKN2b, CDKNIB, RBI, NR3C1)
Monoallelic and biallelic 12p13/ETV6 deletions

Immunophenotyping

CD4+ (expression/positive)
CD34- (no expression/negative)
CD56+ (expression/positive)
CD303+ (expression/positive)
TCF4+ (expression/positive)
TCL1A+ (expression/positive)
CD304+ (expression/positive)
CD3- (no expression/negative)
CD14- (no expression/negative)
CD19- (no expression/negative)

Treatments

Chemotherapy

Transformations to

There are no known transformations

Transformations from

There are no known transformations

Same Primaries

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

C85.7 Other specified types of non-Hodgkin lymphoma

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

C86.4 Blastic NK-cell lymphoma (effective October 01, 2015 - September 30, 2024)
C86.40 Blastic NK-cell lymphoma, not having achieved remission (effective October 01, 2024)
C86.41 Blastic NK-cell lymphoma, in remission (effective October 01, 2015)

Signs and Symptoms

Easy bruising or bleeding
Fatigue
Fever
Petechiae
Shortness of breath
Weakness
Weight loss or loss of appetite

Diagnostic Exams

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

Progression and Transformation

80-90% of patients show positive response to chemotherapy, but many releapse with subsequent resistance to drugs
Long lasting remission seen in sporadic cases

Epidemiology and Mortality

Age: 61-67 years median age (occurs in children and adults)
Incidence: rare
Sex: male predominance
Survival: 8.7 to 24 months median survival

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: Histiocytic/dendritic cell neoplasms
Pages: Part A: 226-229

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