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Lymphoma - non B cell
Hodgkin lymphoma
Nodular lymphocyte predominant
Editor: Lauren B. Smith, M.D., University of Michigan (see Reviewers page)
Revised: 21 September 2012, last major update July 2010
Copyright: (c) 2002-2012, PathologyOutlines.com, Inc.
Definition
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● Uncommon variant of Hodgkin’s lymphoma (HL) with indolent behavior, characterized by total replacement of nodal architecture by expansive vague nodules of small lymphocytes with sparse, relatively large LP tumor cells; may have follicular B cell origin
Epidemiology
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● More common in young men
● 5% of all Hodgkin lymphoma cases
Sites
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● Peripheral lymph nodes generally
Etiology
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● A distinct entity from classic Hodgkin lymphoma (Clin Lymphoma Myeloma 2009;9:206)
● L & H cells may have follicular B cell origin, as they have identical IgH gene rearrangements and have Vh segments with somatic hypermutation, seen in follicular B cells (J Exp Med 2008;205:2251)
Clinical features
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● Indolent behavior but higher recurrence rate than classical Hodgkin lymphoma
● Usually nodular; existence of diffuse subtype is controversial
● Usually young men with cervical or axillary adenopathy; rarely mediastinal or bone marrow involvement (Am J Surg Pathol 2004;28:489); often long history of localized disease
● Usually stage I/II, excellent prognosis (10 year survival of 80-90%)
● Frequently recurs (Cancer 2010;116:631)
● May be associated with progressive transformation of germinal centers (large germinal centers with infiltration by small lymphocytes, Am J Surg Pathol 1984;8:253)
● 3-5% transform to diffuse large B cell lymphoma, and tumor cells in each may be clonally related (Am J Clin Pathol 2001;116:506)
● IgD+ cases (27% frequency) compared to IgD- cases are associated with a younger median age (21 vs. 44 years), male predominance (M/F of 23:1 vs. 1.5:1), and frequent cervical lymph node involvement (56% vs. 18%, Am J Surg Pathol 2006;30:585)
Poor Prognostic factors
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● Bone marrow involvement is associated with aggressive disease (Am J Surg Pathol 2004;28:489)
● Diffuse T cell rich pattern may be associated with recurrence (Am J Surg Pathol 2003;27:1346)
Case reports
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● With gamma heavy-chain disease (Arch Pathol Lab Med 2001;125:803)
Treatment
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● Recent approaches have included more conservative therapy such as localized radiation therapy, excision or observation
● Treatment-related morbidity and mortality are common with aggressive therapy
● Rituximab may be useful for relapses / refractory cases (Blood 2008;111:109)
Micro description (Histopathology)
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● Usually total replacement of nodal architecture by expansive vague nodules of small lymphocytes with sparse, relatively large tumor cells with multilobulated or round nucleus, thin nuclear membrane, finely granular chromatin and variable small nucleoli
● The large cells are called LP cells (previously known as popcorn cells or L&H (lymphocytic & histiocytic) cells
● LP cells are admixed with numerous small B lymphocytes, epithelioid histiocytes and CD21+ dendritic reticulum cells
● Post-capillary venules may be prominent
● Often T-cell rosettes surrounding LP cells (Am J Surg Pathol 2008;32:1252)
● May have rim of normal lymph node
● Scant eosinophils, plasma cells or fibrosis
● Prominent sclerosis is unusual but may occur in older lesions
● Small germinal centers are rare between the nodules and usually present only at the compressed edge of normal lymph node tissue
● No/rare classic Reed-Sternberg (RS) cells, although LP cells may occasionally resemble R-S cells/variants
● No well-formed granulomas, although epithelioid histiocytes may be prominent
● Diffuse cases have background of reactive T cells
● Syncytial variant is rare (Am J Surg Pathol 2009;33:1725)
Patterns (usually mixed):
● Classic B cell rich nodular, serpiginous/interconnected nodular, nodular with prominent LP cells, T cell rich nodular, diffuse with T cell rich background, diffuse (moth eaten) B cell rich (Am J Surg Path 2003;27:1346)
Bone marrow involvement:
● 3% of cases
● Involvement by large B cells (<10% of all cases) in background of T cells and histiocytes
Micro images
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LP cells
3 LP cells with highly irregular nuclear LP cells resembles RS cell
Contours in a background of small lymphocytes
Large numbers of LP cells Wreath of epithelioid histiocytes
surrounds the nodule
Nodules are larger than Background has small lymphocytes
in follicular lymphoma and epithelioid histiocytes
Diffuse scattering of LP cells
with vague nodular pattern
Low power
RS vs. LP cells Nodular pattern and LP cells
Only rare Hodgkin cells are identified Various images
Most of infiltrate is small round lymphocytes
T cell/histiocyte rich nodules Transition to diffuse large cell lymphoma,
with sheets of LP cells in internodular areas
Nodular LP HL and large cell lymphoma
A/B: darkly stained nodules at top and bottom show a predominance of small lymphocytes, and
some nodules are ringed by epithelioid histiocytes
C: pale staining nodules of A and diffuse areas (not shown) have predominance of large cells
Nodular LP HL and classic interfollicular HL
A: nodule at top has features of NLPHL but interfollicular infiltrate at bottom shows features of
classic HL
B: small lymphocytes and LP cells are CD20+, but classic RS cells are CD20-
C: LP cells are CD15- but classic RS cells are CD15+
CD20+
CD45RB+ CD57+ cells form a wreath around the LP cells
Other images: axillary lymph node #1; #2; #3
Positive stains
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LP cells:
● CD45 (LCA), CD20 (Am J Clin Pathol 2003;119:192)
● bcl6, OCT-2, BOB.1 and PU.1 (Mod Pathol 2006;19:1010)
● Variable CD79a, variable EMA
Background cells:
● CD20+ small B cells within nodules
● CD21+ expanded follicular dendritic cell meshwork
● T-cell rosettes around LP cells contain PD-1, BCL6 and CD57+ (variable) cells (Am J Surg Pathol 2000;24:1068); T cells are often CD4+ CD8+ (Am J Clin Pathol 2006;126:805)
Negative stains
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● LP cells: CD3, CD15, CD30 (very rare cells may be positive), EBV; usually fascin and JunB (Appl Immunohistochem Mol Morphol 2010;18:16)
Cytogenetics
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● t(3;14)(q27;q32)in 20% (J Mol Diagn 2005;7:352)
Differential Diagnosis
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● Classic Hodgkin lymphoma-lymphocyte rich: often extranodal, has RS but not LP cells, no background of small reactive B cells, CD15+, CD20-, fascin+, JunB+
● Diffuse large B cell lymphoma (T-cell rich large B-cell variant): often marrow involvement; diffuse, not nodular; no LP cells, no T cell rosettes surrounding LP cells (table)
● Follicular lymphoma (especially the floral variant): usually age 30+ years, complete effacement of nodal architecture, no LP cells, background is atypical cells, not small regular cells as seen in nodular LP HL
● Progressive transformation of germinal centers: well circumscribed nodules of confluent sheets of CD20+, CD45RA+ small cells with scattered T cells, fewer T cell rosettes than nodular LP HL (Am J Surg Pathol 1999;23:27); no effacement of lymph node, no LP cells
● Nodular paracortical hyperplasia
● SLL/CLL: no LP cells
Additional references
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End of Lymphoma-non B cell > Hodgkin lymphoma > Nodular lymphocyte predominant
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