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 Abstractor Notes and Module 4: Rules PH7, PH8

Help me code for diagnosis year :

Grade
Code grade specified by pathologist. If no grade specified, code 9
Module Rule
Module 4: PH7, PH8
Alternate Names
Agranular CD4+ natural killer cell leukemia
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]
Mlalignant lymphoma, lymphoblastic, NOS
Monomorphic NK-cell lymphoma
Precursor cell lymphoblastic lymphoma, NOS
Definition
Until recently, the designation of lymphoblastic lymphoma 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. Most patients are elderly, but it can occur at any age, including childhood. This neoplasm tends to involve multiple sites: skin (almost 100% of cases), bone marrow and peripheral blood (60%-90%), and nodes (40%-50%). Patients usually present with symptomatic solitary or multiple skin lesions that can be nodules, plaques, or bruising. 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
Biopsy skin, nodes
Bone marrow
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
No Transformations
Transformations from
No 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
None
Diagnostic Exams
None
Recurrence and Metastases
None
Epidemiology and Mortality
None