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Bologna 80-100 > 91 Histiocytoses > Flashcards

91 Histiocytoses Flashcards

1
Q

List 5 primary cutaneous generally self-healing histiocytoses?

A 
Jason BIGGie
JXG
Benign cephalic histiocytosis
Intermediate cell histiocytosis
Generalized eruptive histiocytoMA
Giant cell RETICULOhistioCYTOMA
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2
Q

Clin for generalized eruptive histiocytoMA?

A

recurrent crops (100s) of small red-brown papules on trunk/proximal extremities/face &raquo_space; MM

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3
Q

tx for generalized eruptive histiocytoMA?

A

self-limiting (part of self-healing histiocytoses)
few months
PUVA, isotretinoin, cryo

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4
Q

2 conditions generalized eruptive histiocytoma a/w?

A

acute/chronic leukemia

tx w/retinoids - trigger memory for malignancy

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5
Q

Clinical presentation of giant cell reticulohistiocytoma?

A

solitary lesion <3 head

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6
Q

Treatment for giant cell reticulohistiocytoma?

A

spontaneous resolution
can excise
on spectrum w/ munticentric reticulohistiocytosis

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7
Q

Age group for giant cell reticulohistiocytoma? Langerhan or non-langerhan?

A

adults

non-LCH

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8
Q

H&E hallmark of giant cell reticulohistiocytoma?

A

ground glass multinucleated giant cells (in name)

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9
Q

clinical of Intermediate Cell Histiocytosis?

A

solitary - soft erythematous papl -> yellow w/ age

generalized - firm red-brown papules < 1 cm -> yellow w/age

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10
Q

Histo stains in intermediate cell histiocytosis?

A

+ S100 and CD1a (intermediate, so has similarities with LCH)

CD68+

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11
Q

systemic involvement in intermediate cell histiocytosis?

A 
classic systems:
ocular - conjunctiva/cornea
rare bone lesions
rare visceral
BCL, AML, etc
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12
Q

tx for Intermediate Cell Histiocytosis?

A

wil regress
can surgically excise
can do isotretin, MTX, thalidomide
F/U b/c of visceral invovlment risk and hemeCa risk

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13
Q

clin for benign cephalic histiocytosis?

A

head and neck> trunk; <1yo

red-brown macules/papules

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14
Q

ultrastructural finding in benign celpahlic histiocytosis

A

worm-like bodies

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15
Q

systemic involvement in benign cephalic histio?

A 
DI reported (head!)
no organ involvement
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16
Q

3 histo patterns in benign cephalic histiocytosis (likely low yield)

A

papillary dermal
diffuse
lichenoid

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17
Q

tx for benign cephalic histiocytosis

A

self-limited

regular exam for exacerbation and DI

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18
Q

age for JXG? distribution?

A

young child <2
clasically head > upper trunk?extremities
few» numerous

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19
Q

list 3 associated organs in JXG?

A 
ocular
heme
lung
lytic bone
viscera
(aka similar to  
 LCH w/Ls)
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20
Q

when would you refer to optho in JXG?

A

if 3+ lesions or close to eye, or presentation < 2 yo

<0.5% blindness 2’ glaucoma or hyphema

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21
Q

tx for JXG?

A 
monitoring - regress around 3-6 yo
excision
>>>>>
XRT
CsA
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22
Q

What triad is JXG part of ?

A

JXG + NF1 + JMML

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23
Q

Classic cells on histo of JXG?

A

Touton giant cells

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24
Q

MC histiocytosis?

A

JXG

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25
Q

2 forms of JXG?

A 
small nodular (<5 mm)
large nodular: 1-2 cm
26
Q

JXG variants?

A 
GIANT COCKUPS
Giant (small/large nodular are <2 cm)
Clustered
Oral
Keratotic
Pedunculated
Plaque-like
SubQ
27
Q

MC extracutaneous organ in JXG?

A

eye

28
Q

histo for JXG?

A

• Path: dense infiltrate of histiocytes in superficial dermis -> subQ, loss of rete ridges, ulceration
o early: histiocytes with eosinophilic cytoplasm
o mature lesions: histiocytes have lipid in their cytoplasm, becoming foamy and “xanthomatous”
 Touton giant cells are characteristics
o positive HAM56, CD68 and factor XIIIa, some S100+
o negative CD1a and Langerin (CD207)

29
Q

clin of necrobiotic xanthogranuloma

A

PERIORBITAL - look like xanthomas
> face/trunk/periartiuclar
indurated papules/nodules w/ yellow xanthomatous hue

30
Q

NXG - systemic manifestations - give 2 organs and 3 conditions?

A

50% opthalmic (think about distribution)
orbital masses, ectropion, ptosis, keratitis, proptosis

IgG monoclonal gammo
HSM
leukopenia
plasma cell dyscrasias

31
Q

NXG histo?

A

in the name:
xanthogranulomas (palisaded) in mid dermis
+ histiocytes, foam cells, plasmas, giant cells with zones of necrobiosis
cholesterol clefts

32
Q

Tx for NXG?

A 
CCCC around eye
chlorambucil
cyclophosphamide
Cs
CO2
plasmapharesis
XRT
33
Q

cutaneous sx of multicentric reticulohistiocytosis?

A

“coral bead” like papules along proximal and lateral nail fold
articular regions of limbs
head, fingers and mucosa
leonine facies possible

34
Q

extracutaneous of multicentric reticulohistiocytosis?

A

multicentric: arthritis (symmetric, erosive, multiple) -> arthritis mutilans
heme Ca
solid cancer Ca
(about 30%)

35
Q

H&E for multicentric reticulohistiocytosis?

A

lymphs, histiocytes, plasmas and eos
histiocytes multinuclear, angulated, ++ eosinophilic finely granular cytoplasm -> “ground glass effect” like in giant cell reticulohistiocytosis

36
Q

Tx for multicentric reticulohistiocytosis?

A 
remission in 5-10 yrs
rarely fatal
cancer screen
surgical excision curative
reports of NSAIDS, PO CS, MTX, CsPh, etc
37
Q

clinical presentation of Rosai-Dorfman dz?

A

cyclical coures
eyelids and malar area papules
painless bilateral cervical LAD
+- fever, anemia, polyclonal hypergamma,

38
Q

systemic in Rosai-Dorfman?

A

NHL, HL associated

skin, soft tissue, eyes, salivary, CNS, bone involved

39
Q

unique thing on H&E for Rosai-Dorfman?

A

emperipolesis - histiocytes eat lymphocytes/plasma cells

40
Q

Tx for Rosai-Dorfmann?

A 
regresses over the years
XRT
excision
steroids
thalidomide
41
Q

clin of xanthoma disseminatum?

A

yellow, red or brown papules in 100s
symmetric,flexural and intertriginous

triad:
DI
skin xanthomas
MM xanthomas

42
Q

systemic involvement in xanthoma disseminatum?

A

DI
corneal and conjunctival involvement can threaten vision
rare monno gammos, thyroid disorders

43
Q

Tx for Xanthoma disseminatum?

A 
no good tx
surgery
radiotherapy
cryo and laser
CsA
44
Q

List cutaneous histiocytoses w. frequent systemic involvement?

A

NaRX

NXG
Rosai Dorfman
Multicentric reticulohistiocytosis
xanthoma disseminatum

45
Q

What histiocytic d/o can manifest with leonine facies?

A

multicentric reticulohistiocytosis

46
Q

what histiocytic d/o is linked to petechiae/purpura?

A

LCH

47
Q

what histiocytic d/o is linked ot molluscum-like lesions?

A

LCH

48
Q

what histiocytic d/o has “coral beads “around nail folds?

A

multicentric reticulohistiocytosis

49
Q

what histiocytic d/o has highest rate of solid organ and heme malignancies

A

multicentric reticulohistiocytosis - 30%

50
Q

Which histiocytic disorder has groundglass on histology?

A

multicentric reticulohistiocytosis and giant cell reticulohistiocytoma (both related)

51
Q

which histiocytic disorder has bilateral painless cervical LAD?

A

Rosai-Dorfman

52
Q

you got this?

A

yes!

53
Q

histiocytic disorders with eye inv.

A 
hans schuller Christian
JXG
ICH
XNG
xanthoma disseminatum
54
Q

histiocytic d/os with gammopathies?

A

NXG
Rosai-Dorfmann
Xanthoma disseminatum

(basically all NLCH with frequent systemic involvements except multicentric reticulohist, which is +++ heme Ca)

55
Q

List 7 skin directed therapies in LCH?

hint: lymphoma-like tx

A

• examine heme, pulmonary, hepatosplenic, renal, skeletal systems to assess involvement
• mild, single system: topical agents incl. TCS, antimicrobials, mechlorethamine (nitrogen mustard), imiquimod, phototherapy (nbUVB, PUVA)
• extensive cutaneous: thalidomide, AZA, MTX
• reports of BRAFi vemurafenib
• systemic therapy if: multisystem LCH
o single-system LCH w/multifocal bone lesions
o single-system LCH w/ “special site lesions” like craniofacial bone w/ soft tissue extension
o single-system LCH w/ at risk CNS

56
Q

Workup for LCH?

A 
Skin biopsy ± V600e analysis (also in blood or CSF)
CBC + diff, INR/PTT, ESR, CRP, lytes, LFTs
Glucose, TSH
SPEP + immunofixation
Flow cytometry (T-cell analysis)
UA + urine osmolality
CXR, abdo U/S ,CT – chest/abdo/pelvis
Skeletal survey, bone scan
Bone marrow biopsy
57
Q

Common mutation in LCH?

A

BRAF V600E

58
Q

systems invovled in LCH?

A

Ls - liver, lytic bone, LN,
lung (smokers)
DI
otitis media

59
Q

risk organs in LCH?

A

heme, liver, lungs, spleen

single system or no risk organs -> mortality <5%

60
Q

LCH: S100, CD1a, Langerin, Factor XIIIa, CD68, HAM56?

A

positive: S100, CD1a, Langerin
negative: factor XIIIa, macrophage factors such as CD68, HAM56

Bologna 80-100 (3 decks)

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