Lymph Noes 2 Flashcards

1
Q

Burkitt Lymphoma - 3 Clinical variants

A

Endemic, sporadic, immunosupressed

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2
Q

ENDEMIC BURKITT LYMPHOMA

A

Africa

Children 4-7 y/o

Male

Malaria

Jaw/facial bones

95% EBV +

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3
Q

Sporadic Burkitt lymphoma

A
  • adult median 30 y.o.
  • 30-50% of childhood lymphomas
  • EBV positive cells in 30% of cases
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4
Q

Burkitt - Immunophenotype, molecular

A
• Immuno
-Positive for CD20, CD10 & bcl-6
-Ki-67 100% of cells
-Neg for bcl-2, or weakly+ in 20%
• Molecular
-T(8;14) IgH/myc
-T(2;8) myc/kappa
-T(8;22) myc/lambda
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5
Q

Burkitt - Factsoids

A

• Translocation involving MYC is highly characteristic but
not specific for Burkitt lymphoma
• No single parameter alone (ie morphology, genetics, IHC)
can be used as the gold standard for the diagnosis of
Burkitt lymphoma
• A combination of several techniques is required for dx.
• The 2008 WHO does not recognize atypical Burkitt
lymphoma as a distinct entity.
• Instead, it acknowledges that there are cases of Burkitt
lymphoma with nuclear pleomorphism, which previously
would have been called atypical Burkitt

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6
Q

DLBCL - Diffuse Large B-Cell Lymphoma

A
• Common morphologic variant
-Centroblastic
-Immunoblastic
-Anaplastic
• Molecular subgroups
-Germinal centre B cell like
-Activated B cell like
• IHC subgroups
-CD5+ DLBCL
-Germinal centre B cell like
-Non germinal centre B cell like
• Subtypes
-T cell/histiocyte-rich large B ‘ cell lymphoma
-Primary DLBCL of the CNS
-Primary cutaneous DLBCL, leg type
-EBV + DLBCL of the elderly
• Other lymphomas of large B cells
-Primary mediastinal (thymic) large B cell lymphoma
-Intravascular large B cell lymphoma
-DLBCL associated w chronic inflammation
-Lymphomatoid granulomatosis
-ALK-positive LBCL
-Plasmablastic lymphoma
-LBCL arising in HHV8+ `multicentric Castlemans
-Primary effusion lymphoma
• Borderline cases
Discussed later
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7
Q

Subtypes of DLBCL

A

– T cell/histiocyte-rich large B cell lymphoma
– Primary DLBCL of the CNS
– Primary cutaneous DLBCL, leg type
– EBV + DLBCL of the elderly

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8
Q

T cell/histiocyte-rich large B cell lymphoma

A

Large cells
<10% of total cellularity
Can look like centroblasts, immunoblasts, L&H cells, RS cells
CD20+, LCA+, CD30+/-, rare CD15+, bcl-6+, bcl2+/-

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9
Q

DLBCL, leg type

A
• Old diagnosis for E.O.R.T.C.
• New diagnosis from WHO
• elderly females
• Aggressive
• Tx, combination chemotherapy
• 5 year survival 55% (Blood, 2005)
• Histology
– Immunoblastic
– Round cell morphology

Immunos

  • Strong bcl-2+, unlike cutaneous follicular lymphoma
  • MUM1/IRF+, FOXP1, CD10 usually -, BCL-6+,
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10
Q

Other lymphomas of large B cells

A
  • Primary mediastinal (thymic) LBC lymphoma
  • Intravascular large B cell lymphoma
  • DLBCL associated w chronic inflammation
  • Lymphomatoid granulomatosis
  • ALK-positive LBCL
  • Plasmablastic lymphoma
  • LBCL arising in HHV8+ multicentric Castlemans
  • Primary effusion lymphoma
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11
Q

Mediastinal (thymic) Large B-cell Lymphoma

A

Clinical
B cell lymphoma in mediastinum
May have superior vena cava syndrome
20-40 year old women

Immunos/molecular
Lack of surface immunoglobulin by flow
But IgH rearrangement by PCR
B-cell markers +
Bcl-2 and bcl-6 can be +
CD30 + (80%), but weak
CD15 only rarely +
MAL protein +

compartmentalizing fibrosis

B cells w clear cytoplasm

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12
Q

Lymphomatoid granulomatosis

A
  • Angiocentric and angiodiestructive lymphoproliferative dz of extranodal sites, composed of EBV+ B cells and reactive T cells.
  • Used to be thought of as a T cell lymphoma
  • Lung, brain, liver, kidney skin
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13
Q

Hodgkin lymphoma

A
• WHO classification:
–Nodular lymphocyte predominant
– Classical
• Nodular sclerosis
• Mixed cellularity
• Lymphocyte-rich
• Lymphocyte-depleted
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14
Q

Nodular Lymphocyte Predominant Hodgkin lymphoma

A

L & H cells with 1 large folded nucleus, and scant cytoplasm (popcorn cells)
Absence of PMNs and eosinophils
Progressive transformation of germinal centers (PTGC) is associated

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15
Q

Nodular Lymphocyte Predominant Hodgkin lymphoma - Immunophenotype

A

– CD45+, CD20+, J-chain and EMA+
– CD3+ and CD57+ T-cells in background ring malignant cells
– Nearly all cases are CD15 & CD30 neg
– Oct2 and BOB.1 consistently present whereas in
classic HL one or both will be absent

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16
Q

Hodgkins- PROGRESSIVE TRANSFORMATION OF GERMINAL CENTERS

A
  • Large follicles (3-4 times normal)
  • inward migration of perifollicular small B cells and
    activated T cells into the germinal centers
  • Absent L and H cells
  • May proceed, follow or accompany NLPHD
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17
Q

Classic Hodgkin lymphoma

A
    • 95% of HL with bimodal age with 15-35 year peak and 2nd peak late life
    • Mediastinal
18
Q

Nodular Sclerosis Hodgkin lymphoma

A

CD30+, CD15+ (75-85%);
CD45 neg, LMP-1 and EBER variable,
BSAP(PAX5)+
Reed Sternburg cells in up to 40% of cases

19
Q

Mixed cellularity Hodgkin lymphoma (MCHL)

A
  • Interfollicular growth pattern may be seen
  • May have interstitial fibrosis but no capsular thickening or
    broad bands of collagen fibrosis
  • EBV-LMP- highest frequently-75%
  • Mediastinal involvement uncommon
20
Q

Lymphocyte rich Hodgkin lymphoma

A

nodular growth pattern
Regressed germinal centers
RS cells in mantle zone
No eos + PMNs in background

21
Q

T CELL LYMPHOMA BY LOCATION

A

Lymph node:
Peripheral T-cell lymphoma, NOS
Angioimmunoblastic T-cell lymphoma
Anaplastic large cell lymphoma

Leukemia/bone marrow:
Adult T-cell leukemia/lymphoma
T-cell prolymphocytic leukemia
T-cell large granular lymphocytic leukemia
Aggressive NK-cell leukemia
T cell lymphoblastic lymphoma
Extranodal
Enteropathy-type T cell lymphoma
Hepatosplenic T-cell lymphoma
Extranodal NK/T-cell lymphoma, Nasal type
Subcutaneous panniculitis-like T cell lymphoma
Γδ cutaneous T cell lymphoma
Mycosis fungoides
Primary cutaneous CD30+ T cell LPD
22
Q

PERIPHERAL T-CELL LYMPHOMA, NOS

A

• T cell lymphoma in which cases do not better fit into a different category
• H&E
– clear cytoplasm
– Inflammatory background of eosinophils and epithelioid histiocytes
– Lymphoepithelioid variant (Lennert lymphoma)

23
Q

Angioimmunoblastic T-cell lymphoma

A
Signs and Symptoms
B symptoms 68-85
Generalized lymphadenopathy 94-97
Splenomegaly 70-73
Hepatomegaly 52-72
Rash 48-58
Arthritis 18
Ascites/effusions 23-37
Lab Results:
Anemia 40-47
Other cytopenias 20
Eosinophilia 39
Hypergammaglobulinemia 50-83
Hypogammaglobulinemia 9-27
Autoantibodies 66-77
LDH 70
ESR 45
24
Q

AILT - Histology

A

Arborizing blood vessels w PAS material

Follicular dendritic cells CD23+ surround vessels

25
Q

Anaplastic large cell lymphoma

A
  • Large cells with marked atypia
  • Hallmark cells are large kidney shaped nucleus with eosinophilic paranuclear area
  • Reed-Sternberg like cells, wreath-like
  • Multinucleate
  • Sinusoidal growth pattern
  • Show perivascular rosetting, but does not destroy blood vessels.
26
Q

Translocations and fusion proteins involving ALK

A

slide 2110 - this is an image slide that I need to transcribe

27
Q

ALCL - response to therapy

A

ALCL is one of the T cell lymphomas that has the best responses to therapy.

Alk+ has better px.

•Except with the small cell variant. In small cell variant, ALK positivity does not lead to a better prognosis.

28
Q

ATLL Clinical syndrome

A

• Caused by HTLV1
• Japan, Caribbean, central Africa,
• Lymphocytosis (62%), lymphadenopathy (61%), lytic bone lesions (36%) HSM (61%), skin lesions (61%) hypercalcemia (73%),
• Large polylobated “flower” cells
• Skin, epidermotropism w Pautrier-like microabscesses
• Bone marrow, tumor cells + osteoclasts
• Dilated lymph node sinuses with tumor cells.
• Variants
1. Acute–leukemic
2. Lymphomatous variant—lymphadenopathy
3. Chronic variant—skin lesions
4. Smoldering variant–WBC normal

29
Q

ATLL Phenotype

A
  • T cell markers, CD4+, CD5+
  • HTLV1+, FoxP3+, CD25
  • EBV-
  • Cytotoxic markers -
30
Q

Enteropathy - type T- cell lymphoma

A

Associated with gluten-sensitive enteropathy
(celiac disease); but occurs less commonly
without gluten-sensitive enteropathy
Jejunal involvement; perforation common
Stomach and colon not commonly affected
Aggressive, death from multiple perforations.

31
Q

ETTCL-Morphology

A
1. T cell lymphoma part
Mixture of small and large lymphocytes
A mass of lymphocytes
2. Gluten sensitive enteropathy part
Intraepithelial T-cells in adjacent mucosa
Villous atrophy
32
Q

ETTCL - Phenotype

A
  • T cell markers
  • CD8+, but can also be negative
  • CD103+
  • CD5-
  • CD30+/-, CD56+/-
  • Cytotoxic markers
33
Q

Hepatosplenic T cell lymphoma

A
  • Young men
  • Hepatosplenomegaly
  • Often no lymphadenopathy
  • Aggressive
  • T cells in sinusoids of liver and spleen.
  • Spares portal triads & white pulp
  • Most commonly Γδ cells– TCRδ1+, βF1-, Usually CD4-/CD8-, but can be CD4-/CD8+, CD5-, Tia+, but perforin –
  • Iso 7q, trisomy 8
34
Q

Extranodal NK/T-cell lymphoma, Nasal type

A
  • AKA polymorphic reticulosis, lethal midline granuloma, angiocentric lymphoma.
  • Young/Asian
  • Big midline facial tumor, but can be seen in other locations
  • Angiocentric with necrosis
  • Plasma cells, eos, macs
  • Hemophagocytosis
  • T cell markers, Surface CD3-, cytoplasmic CD3έ+
  • CD56+, CD57, cytotoxic markers, EBV+
35
Q

Subcutaneous panniculitis-like T cell lymphoma

A
  • Aggressive T cell lymphoma
  • WHO distinguishes between αβ and γδ
  • Αβ are included in SPLTCL
  • Γδ are called cutaneous γδ T cell lymphoma
  • Lesions are confined to subcutis
  • Lymphocytes encircle fat lobules
  • Septum involvement is mild
  • Karyorrhexis and fat necrosis almost always present
36
Q

Mycosis Fungoides

A
  • Clinical-Scaly erythematous rash that progresses from patches/plaques and may form a solid skin tumor
  • Disease has a long natural history, so patients may have nonspecific infiltrates in the skin and rashes for years before diagnostic histology develops.
  • Morphology-lymphocytes w cerebriform nuclei in dermis with extension to epidermis (epidermotropism)
  • Pautrier abscesses are collections of malignant T cells in the epidermis
  • Halos
  • Dermal infiltrate—non specific
  • Usually CD4+, rarely CD8+
  • USUALLY NO CYTOTOXIC FEATURES
37
Q

Sezary Sydrome

A
  • Exfoliative erythroderma, generalized lymphadeopathy and malignant (Sezary cells in the peripheral blood
  • Spares bm
38
Q

T cell lymphoma molecular abnormalities

A
PTCL - ITK/SYK translocations
AILT - Trisomies
ALCL - ALK translocations
T-PLL - TCL1A translocations
ETTCL - 9q34 amplifications
HSTCL - Isochromosome 7q
39
Q

LYMPHOBLASTIC LYMPHOMA

VS. MANTLE CELL LYMPHOMA

A

slide 2147 image slide to transcribe

40
Q

LBL VS BURKITT

A

slide 2148 - slide has tables need to transcribe

41
Q

TCRLB vs. Nodular LP Hodgkins

A

slide 2149 - slide has tables need to be transcribed