B-cell prolymphocytic leukemia

Name
B-cell prolymphocytic leukemia
ICD-O-3 Morphology
9833/3
Effective 2001 and later
Reportable
for cases diagnosed 2001 and later
Primary Site(s)
C421

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Grade
6 - B-cell
Module Rule
None
Alternate Names
B-PLL
Prolymphocytic leukemia, B-cell type
Definition
More aggressive, but still treatable form of chronic lymphocytic leukemia. The malignant B cells are larger than average. The name is commonly abbreviated B-PLL. It can involve deletions from chromosome 11 and chromosome 13. It has a relatively poor prognosis.

Malignancy of B-prolymphocytes medium-sized, round lymphoid cells with prominent nucleoli, moderately condensed nuclear chromatin, relatively small amount of faintly basophilic cytoplasm, affecting blood, bone marrow and spleen. Prolymphocytes must exceed 55% of lymphoid cells in blood. Extremely rare, 1% of lymphocytic leukemias.
Abstractor Notes
This neoplasm affects the peripheral blood (PB), bone marrow (BM) and spleen. Prolymphocytes must exceed 55% of lymphoid cells in PB. B-PLL is extremely rare, comprising approximately 1% of lymphocytic leukemias. The leukemic cells are found in the PB, BM and spleen. Patients usually present with massive splenomegaly with absent or minimal peripheral lymphadenopathy and a rapidly rising lymphocyte count.
Definitive Diagnostic Methods
Bone Marrow biopsy
Immunophenotyping
Peripheral blood smear
Genetics Data
Ig genes clonally rearranged, t(11;14)(q13;q32) in 20%, abnormalities of p53 in 53%, del at 11q23, del at 13q14
Immunophenotyping
CD5
CD19
CD20
CD22
CD23
CD79a
CD79b
FMC7
Strong surface IgM +/- IgD
Treatments
Chemotherapy
Hormone
Radiation
Transformations to
None
Transformations from
None
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)
C91.3 Prolymphocytic leukemia of B-cell type
Signs and Symptoms
None
Diagnostic Exams
None
Recurrence and Metastases
None
Epidemiology and Mortality
None