Heme - Lymph Node Ap/cp Boards

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Rosai-Dorfman (Sinus histiocytosis with massive lymphadenopathy)

Unknown etiology
Massive painless enlargement cervical LNs
Distension of sinuses by histiocytes, lymphocytes, and plasma cells – diagnostic hallmark of the disease

(pic - intersinusal tissue abundant plasma cells)

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Rosai Dorfman

Many of the histiocytes have intact lymphocytes within their cytoplasm – an important diagnostic feature known as emperipolesis.

Self-limiting

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Asian female painless cervical node

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Kikuchi lymphadenopathy

Unknown etiology, self-limiting, painless
young Asian females
Paracortical well-circumscribed necrotizing lesions

PLASMA CELLS & NEUTROPHILS ARE RARE

DDx: lymphoma w necrosis

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Sarcoidosis

Non-necrotizing granulomas with epithelioid histiocytes, Langhans giant cells

Asteroid bodies (pic) can be seen within giant cells and are composed of aluminum, silicon, Ca, P, lipoproteins

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Amyloid

Congo Red

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Castleman's Disease
90% of solitary lesions are hyaline-vascular type
Large lymphoid follicles and sclerotic bands
Abnormal germinal centers with hyalinization and vasc prolif
*** Concentric layering of lymphocytes resulting in an onion-skin appearance

Asymptomatic

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Castleman's Disease, plasma cell type
Systemic / multicentric
Diffuse plasma cell proliferation interfollicular
** symptomatic & poor px: F, anemia, elevated ESR, hypergammaglobulinemia, and hypoalbuminemia

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19M painless axillary lymphadenopathy

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Toxoplasmosis

Hyperplastic follicles with rx germinal centers with numerous TBMs and clusters of epithelioid histiocytes.
ALSO MONOCYTOID B CELL HYPERPLASIA

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DDx for double+ CD4 & CD8

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T cell lymphoma
PTGC (Progressively transformed germinal ctrs)
Thymoma
NLP-HD

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Necrotizing neutrophilic granulomas seen in?

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- Cat scratch
- LGV
- Yersinia
- tularemia
- fungal

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Marked follicular hyperplasia with giant irregular shaped follicles. Can involute and show depleted follicles with fibrosis.

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HIV related lymphadenopathy

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Multiple germinal centers in 1 follicle
Lollipop germinal centers with blood vessels
Onion skin mantle zone

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Castleman's disease
(associated with HHV8)

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Follicular dendritic cell markers

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CD21
CD23
CD35
D240

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Follicular hyperplasia
Epithelioid hyperplasia
Monocytoid B cells in sinuses

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marginal zone,Toxoplasma gondii

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Paracortical proliferation immunoblasts
Sinus distension monocytoid B cells
Focal necrosis and apoptosis

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Mono

Large atypical cells are CD30+!

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Pale paracortical expansion
Increased Langerhans and IDR cells
Melanin pigment

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Dermatopathic lymphadenitis.

... LN draining a rash.
Can mimic MF

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Large zones of necrosis
Crescentic histiocytes
Karyorrhexis
(No neutrophils or eos or plasma cells)

Dx?
DDx?

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Kikuchis
-young Asian females

SLE (plasma cells)
Cat scratch

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Suppurative granulomas
Stellate abscess
NEUTROPHILS

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Bartonella henselae

(Warthin-Starry or Brown Hopps stain)

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Distended sinuses with foamy histiocytes
Bilateral cervical LN
Teenager with fever

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Rosai-Dorfman disease

(emperipoiesis)

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Erythrophagocytosis DDx:

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"Hemophagocytic syndrome"
- Virus
- TCL
- X-linked syndrome

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Immunodeficient patients
Vascular nodular proliferation
Plump endothelium

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Bacillary angiomatosis

(Also caused by Bartonella henselae!)

(Looks nodular; looks like soft tissue tumor: DDx is Kaposi! Do warthin-starry stain)

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Dim CD20
CD5
CD23

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CLL/SLL

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Poor prognostic markers in CLL

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CD38
ZAP70

trisomy 12
del(11q)
del(17p)

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Bright
CD11c
CD25
CD103
Annexin A1
TRAP
DBA.44

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Hairy cell leukemia

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HCL in spleen?
Bone marrow?
Peripheral blood?

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RED PULP LAKES

Fried egg & reticulin fibrosis

Pancytopenia & MONOCYTOPENIA

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What is unique about treatment for HCL?

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NO CHOP

good response to purine analog

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What do we call HCL with a an atypical feature (eg. leukocytosis

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monos, lacking a classic marker),HCL variant.

POOR PROGNOSIS & resistant to therapy

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CD10
bcl2
bcl6

genetics?

when is bcl2 negative?

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FISH t(14; 18)
MBC, mcr

bcl2 is negative in cutaneous FL

Also, as FL grade increases, bcl2 expression decreases

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CD20
CD5
CD43
bcl2
bcl1

genetics?

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Mantle cell lymphoma
(bcl1 = cyclin D1)

t(11;14)

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Mantle cell lymphoma involving GI tract?

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lymphomatous polyposis

Front 31

Follicular colonization by marginal zone
Monocytoid cells
Plasmacytoid cells
DUTCHER BODIES

gene? In what %? px implication?
when is the classic translocation not seen?

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Marginal zone lymphoma

t(11;18) in 25-50% MALT & resistant to abx therapy!
MLT and AP12 genes
Not seen in DLBCL from MZL
Not seen in nodal MZL

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MZL precursors

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Hashimoto
Sjogrens
HCV
Lyme Disease (skin)
Chlamydia (eye)
Campylobacter (sm int)
H pylori (gastric)

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IgM gammopathy + LPL + bone marrow involvement

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Waldenstrom macroglobulinemia

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Cryoglobulinemia
Hyperviscosity
Dutcher bodies

Dx?
IHC?

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LPL

CD5- / CD10- / CD23-

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Starry sky
Bubbly cytoplasm
Tingible body macrophages

genetics?

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Burkitt lymphoma

t(8;14) myc/IgH
t(2;8) kappa/myc
t(8;22) myc/lambda

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Butt cell

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Follicular lymphoma

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Flower cell

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Adult T cell Leuk/Lymphoma

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Hallmark cell

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Anaplastic large cell lymphoma

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Popcorn cell

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NLP-HL

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CD20
CD10
bcl6
MIB1 100%
bcl2 in 20%

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Burkitt

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3 forms of Burkitt

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1. Endemic - Africa, kids, jaw, EBV
2. Sporadic - 50% childhood lymphoma. ave 30y, GI, breast.
3. Immunosuppressed

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2 main types of DLBCL and prognosis?

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Germinal center type (better px)

Activated B cell type

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Leg lesion old lady
Strong bcl2
bcl6
MUM1

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Primary cutaneous DLBCL of leg

Aggressive and poor px

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<10% large B cells
CD20
+- CD30
bcl6+

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T cell rich DLBCL

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30F with SVC syndrome and mediastinal mass

histology?

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Mediastinal / thymic DLBCL

Lots of fibrosis
lots of cytoplasm = fried egg appearance

*lack surface Ig by flow. Can do PCR IgH rearr

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Angiocentric and angiodestructive lesion in the lung or brain

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Lymphomatoid granulomatosis

EBV+ B cells
Lots of reactive T cells

CD79a around blood vessels

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CD20
CD45
EMA
OCT2
BOB1

classic cell?

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NLP-HL

POPCORN CELL or L&H cell

Progressive transformation of germinal centers

NO EOS or NEUTROPHILS

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CD15
CD30
50% LPM1 / EBER
PAX5

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Classic HL

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Thick capsule
Fibrous bands
EOS
NEUTROPHILS
RS CELLS

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NS-HL

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HL with highest level of EBV
and
Least involvement of mediastinum

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Mixed cellularity HL
(interfollicular pattern, less fibrosis)

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Regressed germinal centers
No eos

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PMNs
RS in mantle zones,Lymphocyte rich HL

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LN with cells with increased cytoplasm

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background eos and histiocytes

ITK/ SYK translocation,Peripheral Tcell lymphoma, NOS

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Lymphoepithelioid variant of TCL

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Lennert lymphoma

(looks like large histiocytes but they are T cells)

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Rash
Hepatosplenomegaly
Hypergammaglobulinemia

CD23+ around vessels

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Angioimmunoblastic T cell lymphoma

Arborizing vessels
Effaced architecture
Polymorphous infiltrate

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CXCL1
PD1

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AITCL

also 2/3/4/5/10

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CD45-
EMA+
CD30+
ALK1+

cell?

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Anaplastic large cell lymphoma

Hallmark cell (kidney shape, wreath nucleus)

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t(2;5)

Back 57

ALCL

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Is ALK1 nuclear or cytoplasmic?

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both!
Nuclear in ALK-NPM1 translocation t(2;5)
Cytoplasmic in others

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Lytic bone lesions
Hepatosplenomegaly
Skin lesions
Hypercalcemia

cell?

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Adult TCell Leuk/Lymphoma

Flower cell

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HTLV1+ in Japan

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Caribbean, Africa,Adult Tcell Leuk/Lymphoma

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TCLIA translocation

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T prolymphocytic lymphoma

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T cell lymphoma with main ddx of MCL & Burkitt?

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T-lymphoblastic lymphoma
TdT+
CD5-
CyclinD1-

MIB<80%

Front 63

HTLV1
FOXP3
CD25

Back 63

Adult T cell leuk/lymphoma

(also associated with lots of osteoclasts)

Front 64

Jejunal perforation
Celiac sprue

px?

Back 64

Enteropathy-type TCL

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9q34

CD5-
CD8+
CD103+
2/3/7/56+

Back 65

Enteropathy-type TCL

Front 66

Young men
Aggressive
Hepatosplenomegaly
No lymphadenopathy

Back 66

Hepatosplenic TCL

Subtle - sinusoids have T cells

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gamma-delta cells
CD4-
CD8-

iso7q
trisomy 8

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Hepatosplenic TCL

Front 68

Young Asian
Large midline facial tumor
Angiocentric
Necrosis

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Extranodal NK-T lymphoma nasal type

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EBV+
sCD3-
cCD3e+
CD56/67+

Back 69

Extranodal NK-T lymphoma nasal type

Front 70

Lymphocytes surrounding fat lobules in sub q
fat necrosis
karyorrhexis

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Subcutaneous panniculitis-like TCL

Front 71

alpha-beta cells or gamma-delta in SPLTCL?

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alpha-beta

gamma-delta in gamma-delta cutaneous TCL

Front 72

Scaly red rash becomes tumor
Epidermotropism
Pautrier microabscesses
CD4+

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Mycosis fungoides

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Cerebriform nuclei in peripheral blood & LAD

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Sezary syndrome

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Reactive follicular hyperplasia

Front 75

Dx?
IHC?
Genetics?

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Follicular lymphoma

+ CD19/20/22/79a
+ CD10, bcl2, bcl6
- CD5, CD43

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What is CD43?

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Expressed in normal and neoplastic T cells

Anomalous expression in MCL, CLL, some MZL
NEGATIVE in FL

(More sensitive than CD5 but less specific: 95% CLL stains CD5+ but 100% with CD43; 90% MCL CD5+, 100% CD43; MZL 10% CD5+, 50% CD43+)

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Asteroid body of sarcoid

The radiating filamentous arms contain complex lipoproteins, calcium, phosphorus, silicon, and aluminum.

Front 78

Which form of Castelmans has poor px?

What syndrome is it associated with?

What virus?

Back 78

Multicentric (usually plasmacytic)

POEMS syndrome
(polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin abnormalities)

HHV8

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Castlemans buzzwords / clues

Back 79

Onion skinning of mantle zone
Lollipop (germinal center w blood vessel)
Multiple germinal centers in one follicle
SMALL follicles (vs HIV - LARGE!)
HHV8

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Stages of HIV-related lymphadenopathy

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Follicular hyperplasia (giant irregularly shaped) --> involution --> depletion / fibrotic

Front 81

subcutaneous nodules revealed hyperplasia of germinal centers and a prominent perinodal eosinophilic infiltrate.

Back 81

Kimura's disease

an inflammatory disorder of unknown origin endemic in the far east.

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Pattern of toxoplasmosis: follicular, paracortical, or sinus?

Mono?

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Follicular (follicular hyperplasia, epithelioid histiocytes, monocytoid B cells in sinuses)

Mono is paracortical pattern: Paracortical proliferation of immunoblasts, sinus distension of monocytoid B cells, focal necrosis / apoptosis, Large atypical cells (CD30+, EBER)

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Major DDx of infectious mono?

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T-cell rich DLBCL

Front 84

Markers of acute EBV?

chronic?

Back 84

IgM & IgG VCA (viral capsid antigen)
Positive heterophil ab

Chronic markers:
IgG EBNA, IgG VCA
(EBV nuclear antigen)

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EBV associations...

Back 85

Nasopharyngeal carcinoma
Oral hairy leukoplakia
Aplastic anemia
...

Front 86

What are the atypical lymphocytes circulating in mono?

Back 86

Reactive CD8+ T cells, responding to B-cell infection with the virus

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Back 87

Dermatopathic lymphadenopathy

Stains Langerhans cells and IDRCs

Front 88

Conditions associated with follicular pattern?

Back 88

RLH
FL
Castleman
HIV
Toxo
RA
Syphilis

Front 89

Conditions associated with a paracortical pattern?

Back 89

EBV
Dermatopathic
Kikuchi
Cat Scratch
Drug
Lupus

Front 90

Conditions associated with a sinusoidal pattern?

Back 90

Rosai-Dorfman
Hemophagocytic syndrome
Bacillary angiomatosis
LCH
Whipple

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Major DDx of Kikuchi?

Back 91

SLE (plasma cells!)

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Back 92

CLL

DIM CD20, 23, 11c

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Back 93

HCL

BRIGHT CD20, 23, 11c

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What is bcl-1?

Back 94

cyclin D1

Front 95

Leg of an old lady

Back 95

Primary cutaneous DLBCL
AGRESSIVE

Immunoblasts - prominent nucleolus

Unique IHC:
bcl2+ / bcl6+ / MUM1+ / CD10-

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30y female with SVC syndrome

Back 96

Mediastinal / thymic large B cell lymphoma

Lots of fibrosis
Increased cytoplasm (~fried egg of HCL)

**Lack surface Ig by flow - must do IgH rearr by PCR

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Large mediastinal mass think of 2 heme things?

Back 97

Mediastinal / thymic large B cell lymphoma

Hodgkin

Front 98

Lung lesion

Back 98

Lymphomatoid granulomatosis

Lung & brain

Angiocentric & Angiodestructive!

EBV+
Lots of rx T cells

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Progressive transformation of germinal centers

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Reactive process
- 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 NLPHL

Associated with NLPHL
also pediatric nodal marginal zone lymphomas (boys, localized, etc)

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IHC of popcorn cell / LH cell?

Back 100

(NLPHL)

CD20
CD45
EMA
J chain
OCT2 AND BOB1 (cHL will be neg for one of these)
T cells ring around LH cell and are CD3 & 57+

-CD15, CD30

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Dx?
IHC?

Back 101

Nodular sclerosis HL
Thick capsule
Classic reed sternberg cells

CD15/30+
CD20/45-
50% LMP1, EBER
PAX5+
fascin+

Front 102

HL with less fibrosis, no thickening of capsule, and interfollicular pattern?

Back 102

Mixed cellularity HL

Front 103

HL with highest frequency of EBV+?

Back 103

MCHL, 75%

Front 104

Hl with least involvement of mediastinum?

Back 104

MCHL

Front 105

Regressed germinal centers
Nodular growth pattern
RS cells in mantle zone
No eos or PMNs in background

(what other disease has regressed germinal centers?)

Back 105

Lymphocyte-rich HL



(Castleman's!)

Front 106

What 3 T-cell lymphomas classically present in the lymph nodes?

Back 106

Peripheral T-cell lymphoma, NOS
Angioimmunoblastic T-cell lymphoma
Anaplastic large cell lymphoma

Front 107

What T-cell lymphomas present in the blood / bone marrow?

Back 107

Adult T-cell leukemia/lymphoma
T pLL (prolymphocytic leuk)
T LGL
T LbL (lymphoblastic lymphoma)
Aggressive NK-cell leukemia

Front 108

What T-cell lymphomas present in extranodal sites?

Back 108

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

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Peripheral T cell lymphoma NOS

T cells have increased clear cytoplasm
Background of EOS & HISTIOCYTES

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Back 110

Lennert lymphoma
(lymphoepithelial variant pTCL NOS)

Looks like histiocytes but they are T cells

Front 111

Rash
Hepatosplenomegaly
Hypergammaglobulinemia

Back 111

Angioimmunoblastic TCL

Front 112

IHC of AITCL?

Back 112

CD3
*****CD10*****
(From TReg cells = germinal center!)

EBER+
CXCL13+
PD1+

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Back 113

Hallmark cell of ALCL

Front 114

Dx?

IHC?

Back 114

Sinusoidal growth pattern of ALCL

**CD45-
EMA+
CD30+

Front 115

Does ALK+ ALCL have better or worse px than ALK-?

Back 115

Better
Usually younger patients (<30)

Front 116

Molecular of ALCL & how does IHC assist in identifying the cytogenetics?

Back 116

t(2;5)
also t(1;2), t(2;3), inv(2)... all involve ALK gene

IHC for ALK can help as the classic translocation t(2;5) is ALK-NPM, which shuttles to & from the nucleus, so there is ALK nuclear positivity in t(2;5); cytoplasmic in others

Front 117

What type of ALK+ ALCL does NOT have a good prognosis?

Back 117

small cell variant

Front 118

Which lymphoma is caused by HTLV1, and what patient population is classic?

Back 118

Adult T cell leuk/lymphoma

Japan, Caribbean, African

Front 119

Lytic bone lesions
Hypercalcemia
Skin lesions

Back 119

Adult TCL/L

Front 120

Back 120

Flower cell of Adult TCL/L

Front 121

Which lymphoma?

Back 121

Adult TCL/L

LYTIC BONE LESIONS
LOTS OF OSTEOCLASTS!

Front 122

Classic stains for adult TCL/L

Back 122

HTLV1
FoxP3 (usu nl in paracortical)
CD25

CD4+/5+

EBV-

Front 123

Which lymphoma occurs in celiac, presents with perforated jejunum, and has a very bad px?

Back 123

Enteropathy-associated TCL

Front 124

What T cell marker is classically NOT expressed in enteropathy associated TCL?

Back 124

CD5-

Front 125

young male with hepatosplenomegaly, without lymphadenopathy. What is the aberrant T cell marker?

Back 125

Hepatosplenic TCL

GAMMA DELTA T CELLS!

Usually CD4/-8-

+ CD2, 3, 7, 8, 56, 103

Front 126

Iso(7q) or tri(8)

Back 126

Hepatosplenic TCL

Front 127

Back 127

Hepatosplenic TCL

INVOLVES SINUSOIDS of liver and spleen;
SPARES WHITE PULP

CLASSICALLY INVOLVES BONE MARROW TOO!

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Young Asian patient with large midline facial mass

Back 128

Extranodal NK/T lymphoma nasal type

ANGIOCENTRIC
NECROSIS
hemophagocytosis

T(CD3+) or NK (CD56); EBER+

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Back 129

Subcutaneous panniculitis-like TCL

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

Front 130

CD4+

Back 130

Mycosis fungoides

Long natural history

Epidermotropism
Cerebriform nuclei
PAUTRIER MICROABSCESSES

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Cerebriform nuclei of MF

Front 132

Sezary syndrome

Back 132

Exfoliative erythroderma, generalized lymphadeopathy and malignant (Sezary) T cells in the peripheral blood

Spares bm

Front 133

ITK/SYK translocations

Back 133

PTCL

Front 134

TCL1A translocations

Back 134

T-PLL

Front 135

9q34 amplifications

Back 135

Enteropathy associated TCL

Front 136

Isochromosome 7q

Back 136

Hepatosplenic TCL

Front 137

Trisomies

Back 137

Angioimmunoblastic TCL