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Hematopathology WHO > Nodular Lymphocyte Predominant Hodgkin Lymphoma > Flashcards

Nodular Lymphocyte Predominant Hodgkin Lymphoma Flashcards

1
Q

What are the basic defining features of

Nodular Lymphocyte Predominant

Hodgkin Lymphoma?

A
  • B cell neoplasm
  • nodular or a nodular/diffuse pattern of growth
  • many small lymphocytes with scattered, large, neoplastic B cells
    • LP cells or popcorn cells
    • ringed by T lymphocytes
  • neoplastic cells reside in large nodular meshworks of Follicular Dendritic cells
    • contain histiocytes and non-neoplastic lymphocytes
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2
Q

Which malignancy can have morphologic overlap

with NLPHL?

A
  • T-cell/histiocyte-rich large B cell lymphoma
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3
Q

How frequent is NLPHL and what population

is often affected?

A
  • accounts for 10% of Hodgkin Lymphoma
  • predominantly males
  • 30-50 years old
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4
Q

What is the typical localization of

NLPHL?

A
  • rare in the mediastinum, unlike CHL
  • predominantly in:
    • cervical
    • axillary
    • inguinal lymph nodes
  • Note: mesenteric lymph node involvement can be seen
  • sometimes spleen and bone marrow involved in advanced disease
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5
Q

What are the key clinical features of

NLPLHL?

A
  • most patients have localized peripheral lymphadenopathy (Stage I or II)
  • 20% have advanced stage disease
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6
Q

What is the typical morphology

on H&E of NLPLHL?

A
  • small lymphocytes, histiocytes, epithelioid histiocytes and intermingled LP cells
  • can grow in nodular, nodular diffuse, or diffuse patterns
  • 6 distinct immunoarchitectural patterns
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7
Q

When there is a prominence of extranodular

LP cells, what is that associated with?

A
  • propensity to develop a diffuse growth pattern and look similar to THRBCL
    • this is often seen in patients with a recurrence

Note: if solid/diffuse growth is present, IHC is needed to ID the LP cells

  • even if there is only one area, enough to exclude THRBCL
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8
Q

What is the classic morphology of

LP or popcorn cells?

A
  • .large with a single nucleus and scant cytoplasm
    • can resemble popcorn with cytoplasm (folded or multilobated
  • nucleoli are usually multiple and basophilic
    • smaller than those seen in CHL
    • can be single and prominent though occasionally

Note: histiocytes and polyclonal plasma cells can be at the edge of nodules with LP cells

  • Neutrophils and eosinophils rarely seen
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9
Q

Which precursor or simultaneous lesion

is often associated with NLPHL?

A

Progressive Transformation of

Germinal Centers

Note:

  • vast majority of patients with reactive hyperplasia and progressive transformation of GC do not develop Hodgkin Lymphoma
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10
Q

What is the typical IHC of the LP cells

of NLPHL?

A
  • positive:
    • CD20, PAX5, CD79a, BCL-6
    • CD45
    • OCT2, BOB1
    • CD75
    • Note: remaining mantle zone B cells will be weakly positive for OCT2 and CD75, LP cells will be strong positive
    • J chain and EMA positive (>50%)
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11
Q

What is the staining of LP cells

for CD15 and CD30?

A
  • Negative for both markers
    • CHL will be positive

Note: if there are scattered CD30+ cells these are likely to be reactive immunoblasts, unrelated to the LP cells

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12
Q

What is present in the follicular dendritic

meshwork of the nodules of NLPHL?

A
  • FDC highlighted by CD21 or CD23
  • predominantly filled by by stander B cells and T cells of the follicular helper type
    • PD1 and CD57 positive
    • form rosettes around LP cells
    • T cells also express germinal center markers
      • BCL6, IRF4/MUM1, and CD134
      • don’t produce IL-2 or IL-4
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13
Q

What is an interesting flow finding of

T cells that are found in NLPHL?

A
  • double positive for CD4 and CD8

IMP: on H & E for a diffuse growth pattern the finding of T cells double positive for CD4, CD8, PD1 and CD57 favors NLPHL

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14
Q

What combination of IHC findings in a

diffuse growth pattern would favor a

T cell histiocyte rich B cell lymphoma?

A
  • total absence of small B cells
  • low numbers of CD57 T cells
  • dominant CD8+ cells and TIA+ cells
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15
Q

What is the postulated normal counterpart

of NLPHL?

A
  • germinal center B cell
  • centroblastic stage of development
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16
Q

What are frequent genetic alterations

encountered in NLPHL?

A
  • LP cells harbour clonally rearranged IGHV genes
    • can’t detect in whole tissue
    • must isolate LP cells
    • high load of somatic mutations, with ongoing mutations evident
    • mutations functional = IG mRNA transcripts being produced
  • BCL6 rearrangements in about 50%
  • Aberrant somatic hypermutations in 80% of NLPHL cases, usually PAX5
17
Q

What is the EBV status of

NLPHL?

A
  • detected in LP cells in only 3-5% of cases
    • both adults and children
  • incidence may be higher in Asia
  • EBV can involve some of the bystander B cells occasionally
18
Q

What is the relationship between NLPHL

and THRBCL?

A
  • LP cells similar to CHL and THRBCL by gene expression profiling
    • partial loss of B cell phenotype
    • deregulation of many apoptosis regulators and putative oncogenes
  • NLPHL and THRBCL
    • express BCL6, CD75, EMA, J chain
  • THRBCL may be a variant of NLPHL or just a spectrum
    • difference may lie in cellular environment
19
Q

What is the genetic susceptibility to

NLPHL?

A
  • some familial clusters seen
  • Hermansky-Pudlak Syndrome
    • type 2
    • NK-cell and T cell defects in these patients
  • Patients with autoimmune lymphoproliferative disorders with mutations in FAS
20
Q

What is the prognosis of classic morphology

NLPHL?

A
  • 10 year survival (stage I and II disease)- 80%
  • develops slowly with frequent relapses
    • remains responsive to therapy
    • rarely fatal
21
Q

What variant morphologies have a worse

prognosis in NLPHL?

A
  • LP cells outside of nodules
  • B-cell depletion
  • THRBCL-like architecture/transformation
  • indicates advanced disease with higher relapse rate
  • good to put in report
22
Q

What percentage of NLPHL

progress to DLBCL?

A
  • 3-5% of cases
  • cells can resemble LP cells, immunoblastic, centroblastic
  • keep the LP cell immunophenotype
  • some DLBCL have been found to precede NLPHL diagnosis
  • if localized have a generally good prognosis
23
Q

If you are thinking that NLPHL

is involving the bone what should you

consider?

A
  • although rare, NLPHL can involve the bone marrow
  • BUT must think of THRBCL as well or transformation
  • If it is NLPHL involving the bone marrow then it is clinically aggressive with worse prognosis
24
Q

What is the definition of progressive

transformation of germinal centers?

A
  • Reactive hyperplasia of the follicles:
    • large follicles: 4-5x normal with hyperplastic GC
    • disruption of GCs due to infiltration of mantle zone B cells
    • typically affected follicles are at different stages
  • cause is unknown
    • virus is suspected
25
Q

What age groups are usually affected by Progressive Transformation

of Germinal centers and what locations does it occur in?

A
  • can occur in any age but usually young adult males
    • 20% occur in children
  • usually involves peripheral lymph nodes
    • cervical lymph nodes are the most common
    • axillary and inguinal less common
  • can be the predominant change occuring in the lymph node biopsy
26
Q

What are the symptoms/clinical presentation of patients

with Progressive Transformation of the Germina centers?

A
  • most patients are asymptomatic with localized lymphadenopathy
  • generalized lymphadenopathy seen in:
    • adolescents presenting with viral like illness
    • patients with autoimmune diseases
  • PTGC can be incidental finding in patients with lymphoma
  • Usually spontaneously resolves but can recur in same or different lymph nodes
  • NOT associated with HIV
27
Q

What are the findings on histology of PTGC?

A
  • large follicles, 4-5x normal with secondary reactive follicles
  • usually focal, only involves few follicles in a lymph node
  • process proceeds in stages
    • initial: GC become hyperplastic, starry sky pattern
    • 2-3 GCs per follicle, which fuse together
    • Mantle zone B cells infiltrate and disrupt GC
    • Eventually GC disappears
  • IMP: follicles appear to be in different stages (asynchronous)
  • almost always have interfollucular hyperplasia
  • rarely can show Castlemann-like changes
28
Q

What disease entities can PTGC coexist with?

A
  • NLPHL (most common)
  • Classic Hodgkin lymphoma
  • Plasma cell myeloma
29
Q

What is the cytology of PTGC?

A
  • mixed population of small and large lymphocytes
    • Small, round lymphocytes = Mantle zone B cells and reactive T cells
    • Germinal centrocytes and centroblasts
  • NO LP cells or RS cells
  • No plasma cells, neutrophils or eosinophils
30
Q

What are the immunohistochemical findings of

PTGC?

A
  • GC
    • B cell antigens (+): CD10, BCL6
      • negative BCL2
    • Disruption of the follicular dendritic cells: CD21 and CD23 +
  • Mantle Zone
    • B cell antigens (+): IgD, BCL2
      • Negative: CD10 and BCL6
  • relatively few T cells in PTGC

Hematopathology WHO (29 decks)

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