Prolymphocytic leukemia, NOS

Name
Prolymphocytic leukemia, NOS
ICD-O-3 Morphology
9832/3
Effective 2001 and later
Reportable
for cases diagnosed 2001 and later
Primary Site(s)
C421

Help me code for dx year :

Grade
Code grade specified by pathologist. If no grade specified, code 9
Module Rule
None
Alternate Names
PLL
Definition
General term for non-specific prolymphocytic leukemia (unknown if B-cell or T-cell)
A type of chronic lymphocytic leukemia (CLL), in which too many immature white blood cells (prolymphocytes) are found in the blood and bone marrow
Abstractor Notes
Patients typically have systemic disease at presentation, with bone marrow and peripheral blood involvement as well as hepatosplenomegaly, generalized lymphadenopathy, and skin infiltrates.

Due to the systemic nature of this disease, leukemic cells can be found in peripheral blood, lymph nodes, bone marrow, spleen, liver and skin. A high lymphocyte count (> 100 x 109/L) along with anemia and thrombocytopenia are common findings. HTLV-1 serologies are negative and serum immunoglobins are within normal limits with no paraproteins present
Definitive Diagnostic Methods
Bone marrow biopsy
Genetics testing
Immunophenotyping
Peripheral blood smear
Genetics Data
inv 14(q11,q32)
Immunophenotyping
CD1a-
CD2+
CD3+
CD4+/CD8-
CD7+
TdT-
Treatments
Chemotherapy
Hormone
Transformations to
No Transformations
Transformations from
No Transformations
Same Primaries
Corresponding ICD-9 Codes
204.8 Other lymphoid leukemia
Corresponding ICD-10 Codes
C91.3 Prolymphocytic leukemia
Corresponding ICD-10-CM Codes (effective October 1, 2015 U.S. only)
C91.Z Other lymphoid leukemia
Signs and Symptoms
None
Diagnostic Exams
None
Recurrence and Metastases
None
Epidemiology and Mortality
None