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Lymphoma - B cell neoplasms

B cell lymphoma subtypes

Small lymphocytic lymphoma


Reviewer: Nikhil Sangle, M.D., University of Utah and ARUP Laboratories (see Reviewers page)
Revised: 6 February 2012, last major update February 2011
Copyright: (c) 2001-2012, PathologyOutlines.com, Inc.

See also CLL (chronic lymphocytic leukemia)

Definition
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● Common low grade B cell lymphoma with pseudofollicles composed of mature lymphocytes resembling soccer balls in peripheral blood; cells are CD5+, CD23+

Clinical features
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● Usually older patients (median age 60 years), 2/3 male, with disease in bone marrow, lymph nodes, spleen, liver
● Often presents with leukemia, although patients may be asymptomatic
● SLL may progress to blood (leukemic) involvement, but if so, there is usually less leukocytosis than cases with initial diagnosis of CLL
● Almost all cases are B cell origin
● Associated with hypogammaglobulinemia, monoclonal immunoglobulin spikes in some, infections; also autoantibodies to red blood cells and platelets causing hemolytic anemia and thrombocytopenia
● Median survival 4-6 years; indolent unless it transforms

Poor prognostic factors
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● 17p deletions, 11q22-23 deletion, non-mutated immunoglobulin genes, aberrant expression of CD2, CD7, CD10, CD13, CD33 or CD34 (Am J Clin Pathol 2003;119:824)

Gross Description
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● Cases identified at prostatectomy have enlarged lymph nodes (mean 3.2 cm, Arch Pathol Lab Med 2003;127:567)

Gross images
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Liver #1 , #2

Micro description
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● Effacement of nodal architecture by pale staining pseudofollicles or proliferation centers with ill-defined borders, containing small round mature lymphocytes, prolymphocytes (larger than small lymphocytes, abundant basophilic cytoplasm, prominent nucleoli), paraimmunoblasts (larger cells with distinct nucleoli) and many smudge cells
● Pseudofollicular centers are highlighted by decreasing light through the condenser at low power; cells have pale cytoplasm but resemble soccer balls or smudge cells on peripheral smear (cytoplasm is bubbly in mantle cell lymphoma); may have plasmacytoid features
● May have marginal zone, perifollicular or interfollicular patterns, but these cases also have proliferation centers (Mod Pathol 2000;13:1161)
● Interfollicular pattern: large, reactive germinal centers; resembles follicular lymphoma but germinal centers are bcl2 negative and tumor cells resemble SLL by morphology and immunostains (Am J Clin Path 2000;114:41)
● Paraimmunoblastic variant: diffuse proliferation of paraimmunoblasts (normally just in pseudoproliferation centers); rare, <30 reported cases; usually multiple lymphadenopathies and rapid disease progression; case report in 69 year old man (Hum Pathol 2002;33:1145); consider as mantile cell lymphoma if t(11;14)(q13;q32) is present; may also represent CD5+ diffuse large B cell lymphoma
Bone marrow: small focal aggregates of variable size with irregular, poorly circumscribed outlines; lymphocytes are well differentiated, small, round with minimal atypia; may have foci of transformation; rarely has granulomas (J Clin Pathol 2005;58:815)
● Marrow infiltrative patterns are also described as diffuse (unmutated IgH genes, ZAP-70+, more aggressive), nodular (associated with IgH hypermutation, ZAP-70 negative) or mixed (variable mutation of IgH, variable ZAP-70, Hum Pathol 2006;37:1153)

Micro images
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Prolymphocytic and Hodgkin transformation #1; #2 (CD15, CD30, CD45RO)

Marginal zone growth

       

Interfollicular growth #1; #2 (CD5+); #3 (CD23+)


Incidental findings at prostatectomy

Virtual slides
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Lymph node, bone marrow, liver and kidney

Lymph nodes

Positive stains
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● CD5, CD19, CD20 (dim), CD23, surface Ig light chain, surface IgM (dim)
● Also CD43, CD79a, CD79b (dim in 20%, Arch Pathol Lab Med 2003;127:561), bcl2
● Variable CD11c, FMC7 (42%)

Negative stains
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● CD10, cyclin D1

Molecular
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● Trisomy 12 (30%, associated with atypical CLL and CD79b), deletion 13q14 (25-50%), deletion of 11q23 (worse prognosis, 10-20%)

Molecular images
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IgH gene rearrangement

Differential diagnosis
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● Other low grade lymphomas with leukemic phase (mantle cell lymphoma, follicular lymphoma, rarely lymphoblastic lymphoma, Mod Pathol 2002;15:1111), Hodgkin’s lymphoma, peripheral T cell lymphoma, reactive hyperplasia, persistent polyclonal B cell lymphocytosis (Mod Pathol 2004;17:1087)

End of Lymphoma - B cell neoplasms > Small lymphocytic lymphoma / Chronic lymphocytic leukemia


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