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Lymphoma - B cell neoplasms

B cell lymphoma subtypes

Prolymphocytic leukemia


Reviewer: Nikhil Sangle, M.D., University of Utah and ARUP Laboratories (see Reviewers page)
Revised: 7 February 2012, last major update March 2011
Copyright: (c) 2001-2011, PathologyOutlines.com, Inc.

Definition
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● Rare (2% of chronic lymphoid leukemias) disorder with prolymphocytes representing >55% of leukemic infiltrate

Clinical features
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● Elderly men with peripheral blood lymphocytosis > 100,000, marked splenomegaly
● 80% are B cell, 20% are T cell
● B-PLL has better prognosis than T-PLL patients, but still considered incurable

Case reports
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● 88 year old woman (Case of the Week #120)

Treatment
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● Symptomatic patients may receive rituximab or other immunotherapy (Ann Hematol 2004;83:319)
● Also high dose chemotherapy followed by stem cell transplantation or fludarabine-like drugs (Med Sci Monit 2007;13:RA69)

Micro description
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● Involves red and white pulp of spleen and peripheral blood
● Prolymphocytes in peripheral blood are large cells with scant to moderate pale blue agranular cytoplasm, round to polygonal nuclei and prominent central nucleoli; may have occasional nuclear grooves
● T-prolymphocytes have very irregular nuclear outlines, and cytoplasmic protrusions or blebs

Flow cytometry
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● Monomorphic B cell population that expresses CD19, CD20, CD79a, CD22, FMC7 with monotypic light chains; CD5 and CD23 in about 20% of cases (Semin Oncol 2006;33:257)
● T prolymphocytes express CD2, CD3, CD7, CD52, TCL1 (oncogene); can co-express CD4 & CD8; are negative for Tdt and CD1a

Flow cytometry images
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Peripheral smear (blood) images:
               

Flow cytometry images: #1; #2; #3; #4; #5; #6

Positive stains
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● Bright surface immunoglobulin, CD5, CD19, CD20 (bright), CD22, FMC7

Molecular description
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● 14q+ is most common
● Cytogenetic abnormalities are complex, suggesting clonal evolution and genomic instability

Differential diagnosis
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Prolymphocytic transformation of CLL: prior history of CLL or presence of CLL cells
Blastic variant of mantle cell lymphoma: positive for t(11;14) (Am J Clin Pathol 2001;115:571, Am J Clin Pathol 2002;117:246)

End of Lymphoma - B cell neoplasms > B cell lymphoma subtypes > Prolymphocytic leukemia


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