Name

ICD-O-1 Morphology

9602/3: Malignant lymphoma, convuluted cell types, NOS

ICD-O-2 Morphology

9865/3: FAB M2, NOS; Acute myelocytic leukemia with maturation

ICD-O-3 Morphology

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

Reportable

for cases diagnosed 2001 and later

Primary Site(s)

See Module 4: Rules PH7, PH8
Most common sites of involvement: skin, bone marrow, peripheral blood, and lymph nodes

Grade

Not Applicable

Module Rule

Module 4: PH7, PH8

Alternate Names

Agranular CD4+ NK leukemia [OBS]
Agranular CD4+ CD56+ hematodermic neoplasm/tumor
Blastic natural killer leukemia/lymphoma
Blastic NK-cell lymphoma [OBS]
Blastic NK leukemia/lymphoma [OBS]
BPDC
LBL
Lymphoblastoma [OBS]
Malignant lymphoma, convoluted cell [OBS]
Monomorphic NK-cell lymphoma
Precursor cell lymphoblastic lymphoma, NOS

Definition

Blastic plasmacytoid dendritic cell neoplasm is a clinically aggressive tumor derived from the precursors of plasmacytoid dendritic cells (PDCs, also called professional type 1 interferon-producing cells or plasmacytoid monocytes), with a high frequency of cutaneous and bone marrow involvement and leukemic dissemination.

Abstractor Notes

2018 Update: For cases diagnosed 1/1/2018 and forward, this neoplasm is now abstracted as a leukemia only. Primary site must be C421.

Rare type of hematopoietic neoplasm.

Bone marrow involvement can be minimal at presentation but invariably develops with progression of disease.

Following initial response to chemotherapy, relapses invariably occur involving skin alone or associated with other sites including soft tissue and the central nervous system.

Definitive Diagnostic Methods

Bone marrow biopsy
Genetic testing
Histologic confirmation
Immunophenotyping

Genetics Data

BPDCN gene expression
CDKN2A deletions
Germline T-cell and B-cell receptor genes
Loss of chromosome 9
5q21
6q23-qter
5q34
12p13
13q13-21
15q

Immunophenotyping

CD2AP expression
CD4 expression
CD7
CD33
CD43 expression
CD45RA expression
CD56 expression
CD123 (IL3) expression
CD303 expression
SPIB expression
TCF4 expression
TCL1A expression
Type I interferon-dependent molecule MX1 expression

Treatments

Chemotherapy

Transformations to

There are no known transformations

Transformations from

There are no known transformations

Corresponding ICD-9 Codes

202.9 Other and unspecified malignant neoplasms

Corresponding ICD-10 Codes

C96.7 Other specified malignant neoplasms of lymphoid, hematopoietic

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

C86.4 Blastic NK-cell lymphoma (effective October 01, 2015)

Signs and Symptoms

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

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: 12-14 months median survival

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: Blastic plasmacytoid dendritic cell neoplasm
Pages: 174-177

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
Glossary