Blastic plasmacytoid dendritic cell neoplasm

Name
Blastic plasmacytoid dendritic cell neoplasm
ICD-O-2 Morphology
9865/3
Effective 1992 - 2000
ICD-O-3 Morphology
9727/3
Effective 2001 and later
Reportable
for cases diagnosed 1992 and later
Primary Site(s)
See Module 4: Rules PH7, PH8
Most common sites of involvement: skin, bone marrow, peripheral blood, and lymph nodes

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Grade
Code grade specified by pathologist. If no grade specified, code 9
Module Rule
Module 4: PH7, PH8
Alternate Names
Agranular CD4+/CD56+ hematodermic neoplasm/tumor
Blastic natural killer leukemia/lymphoma
Blastic NK-cell lymphoma [OBS]
BPDC
LBL
Lymphoblastoma [OBS]
Malignant lymphoma, convoluted cell [OBS]
Malignant lymphoma, lymphoblastic, NOS
Monomorphic NK-cell lymphoma
Precursor cell lymphoblastic lymphoma, NOS
Definition
Until recently, the designation of lymphoblastic lymphoma (LBL) was used to describe precursor B- and T-cell tumors with predominant lymph node involvement. Most LBL are tumors of precursor T lymphocytes. LBL is composed of medium-sized cells with finely dispersed chromatin and little cytoplasm with inconspicuous nucleoli.
Abstractor Notes
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
Germline T-cell and B-cell receptor genes
Immunophenotyping
BDCA-2
CD4
CD7
CD33
CD43
CD45RA
CD56
CD123
CD303
CLA
Interferon-alpha dependent molecule MxA
TCL1
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 (effective October 1, 2015 U.S. only)
C86.4 Blastic NK-cell lymphoma
C96.Z Other specified malignant neoplasms of lymphoid, hematopoietic and related tissue
Signs and Symptoms
Easy bruising or bleeding
Fatigue
Fever
Petechiae
Shortness of breath
Weakness
Weight loss or loss of appetite
Diagnostic Exams
Recurrence and Metastases
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