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Histiocytic Diseases Flashcards

(48 cards)

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1
Q

What are Langerhans cells

A

Interstitial DCs in the epidermis

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2
Q

What are interdigitating DCs

A

DCs that occur in the T cell domains in the LNs and spleen

Can be resident DCs or migratory DCs in LNs
- migratory arrive in lymphatics from tissues

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3
Q

What cytokines and GFs influence DC development

A

FLT3 ligand
GM-CSF
TNF-alpha
IL-4
TGF-beta
M-CSF (macrophage CSF)

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4
Q

Blood monocytes can differentiate into what and what controls which way they go

A

Macrophages - under influence of M-CSF

DCs - under influence of GM-CSF and IL-4

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5
Q

Where are dermal interstitial DCs found

A

In the dermis, especially adjacent to postcapillary venules

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6
Q

Canine DCs abundantly express what 3 things

A

CD1a
MHC I
MHC II

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7
Q

CD11/CD18 are also known as

A

Beta-2 integrins

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8
Q

What do Langerhans cells express

A

CD11c
E-cadherin

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9
Q

What do macrophages express

A

CD11b

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10
Q

Macrophages in what locations express CD11d

A

Splenic red pulp and bone marrow

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11
Q

Dermal interstitial DCs express what

A

CD11c
CD11b
CD90/Thy-1

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12
Q

What costimulatory molecules are on DCs and what are their ligands

A

CD80/86 (B7 family)

Ligands: CD28 and CTLA-4

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13
Q

In situ DCs have high/low expression of MHC class II and costimulatory molecules

A

Low

They are more receptive to antigen uptake

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14
Q

Migratory DCs have high/low expression of MHC II and costimulatory molecules

A

High

better at antigen presentation to T cells

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15
Q

Which CDs are only detectable in fresh smears or snap frozen tissues

A

CD1a
CD11b
CD11c
CD80
CD86

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16
Q

What CDs can be used on formalin fixed paraffin embedded tissues to presumptively identify histiocytes

A

CD18
CD11d

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17
Q

What other markers can be used on FFPE tissue

A

Iba-1 - identifies macrophages and DCs

CD163 and CD204 - class A scavenger receptors; largely expressed by macrophages in normal tissues but also expressed by DC subsets in some species

E-cadherin

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18
Q

IHC cutaneous histiocytoma

A

E-cadherin+

**This is unique to them! Can differentiate them from reactive histiocytoses (cutaneous histioticytosis and systemic histoicytosis)

Cutaneous Langerhans cell histiocytosis is identical IHC to histiocytomas

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19
Q

Regression of histiocytomas is mediated by what

A

CD8+ alpha/beta T lymphocytes

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20
Q

Cutaneous Langerhans cell histiocytosis more common in what breed

21
Q

Cutaneous histiocytosis IHC

Study These Flashcards
A

E-cadherin NEGATIVE (different from LHC/histiocytoma)

CD1a +
CD1b+
CD1c+
CD11c+
MHC II +
Thy-1+
CD4+

22
Q

Systemic histiocytosis IHC

Study These Flashcards
A

Identical to cutaneous histiocytosis

23
Q

What breeds are predisposed to histiocytic sarcoma

Study These Flashcards
A

Flat coated retrievers
Rotties
Mini schnauzers
Corgis in Japan

24
Q

What genetic changes have been identified in Berners and flat coats with histiocytic sarcoma

Study These Flashcards
A

Deletions of TSGs CDKN2A/B, RB1, and PTEN

25
A genome wide association study found a haplotype where in 96% of Berners
Haplotype spanning MTAP and part of CDKN2A
26
A gain of function mutation was recently identified in what gene in 37% of Berners with histiocytic sarcoma (and 9% of other breeds with histiocytic sarcoma)
PTPN11
27
Risk of periarticular histiocytic sarcoma in Berners has been linked to what other condition
Prior joint disease
28
Flat coats are more likely to have histiocytic sarcoma of the __ and Berners more likely __
Flat coats = limbs Berners = diffuse visceral disease
29
Localized histiocytic sarcoma is __x more frequent in flat coats vs Berners while disseminated HS is __x more frequent in Berners than flat coats
7x 2x
30
What percent of primary brain tumors are histiocytic sarcoma? Secondary?
Primary = 5% Secondary = 4.5%
31
How do you differentiate periarticular histiocytic sarcoma from synovial cell sarcoma using IHC? What about synovial myxoma?
Periarticular histiocytic sarcoma: CD18+ Synovial cell sarcoma: cytokeratin + Synovial myxoma: vimentin+, HSP25+, cadherin 11+; cytokeratin -, S-100-; +/- CD18+ (20-40%)
32
What serum test may serve as a potential marker of histiocytic sarcoma in Berners
Serum ferritin - it was high in most dogs with clinical HS and 2 of 5 dogs with early HS
33
What lung lobe is the most common pulmonary location for HS?
Right middle lung lobe
34
Internal air bronchograms are common/uncommon on rads with pulmonary HS
Common
35
MST for periarticular HS with amputation? Met rate?
6m Met rate = 91%
36
What location is associated with better MST with HS?
Periarticular - MST 391d vs. 128d for other locations Even with Mets they do better 13 of 19 the periarticular cases in that study had suspected Mets
37
Is metastasis prognostic with periarticular histiocytic sarcoma
YES - while they can do well with Mets, they do even better without No mets MST 980d With mets MST 253d
38
Response rate for CCNU for HS in the gross disease setting, median duration of response, and MST of responders in Skorupski 2007 study?
Response rate 46% Med duration of response 85d MST responders 172d (vs 60d in no responders)
39
What bloodwork findings are associated with hemophagocytic histiocytic sarcoma
Anemia - 94% Thrombocytopenia - 88% Hypoalbuminemia - 94% Hypocholesterolemia - 69%
40
Do steroids improve response to therapy?
Debatable - they did NOT in the Korupski CCNU 2007 study
41
Response rat and med response duration in Rassnick 2010 study
RR 29% Med response duration 96d
42
What was the MST of dogs with localized HS treated with local therapy (sx or RT) + CCNU?
19m
43
Response rate for alternating dox + CCNU q2wks? Med time to progression?
RR 58% Med TTP 185d
44
Response rate of dacarbazine as rescue for HS? Event free ST of responders?
RR 18% Event free ST of responders = 70d
45
Epirubicin response rate in HS
29%
46
MST for periarticular treated with RT? Did they live longer than those that weren’t treated with RT?
MST 182d Yes they did better (duh)
47
MST for dogs treated with palliative RT + CCNU
208d
48
MST for hemophagocytic sarcoma regardless of therapy
1-2m

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