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Bologna 80-100 > 91 Histiocytoses > Flashcards

91 Histiocytoses Flashcards

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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
2 forms of JXG?
``` small nodular (<5 mm) large nodular: 1-2 cm ```
26
JXG variants?
``` GIANT COCKUPS Giant (small/large nodular are <2 cm) Clustered Oral Keratotic Pedunculated Plaque-like SubQ ```
27
MC extracutaneous organ in JXG?
eye
28
histo for JXG?
• 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
clin of necrobiotic xanthogranuloma
PERIORBITAL - look like xanthomas > face/trunk/periartiuclar indurated papules/nodules w/ yellow xanthomatous hue
30
NXG - systemic manifestations - give 2 organs and 3 conditions?
50% opthalmic (think about distribution) orbital masses, ectropion, ptosis, keratitis, proptosis IgG monoclonal gammo HSM leukopenia plasma cell dyscrasias
31
NXG histo?
in the name: xanthogranulomas (palisaded) in mid dermis + histiocytes, foam cells, plasmas, giant cells with zones of necrobiosis cholesterol clefts
32
Tx for NXG?
``` CCCC around eye chlorambucil cyclophosphamide Cs CO2 plasmapharesis XRT ```
33
cutaneous sx of multicentric reticulohistiocytosis?
"coral bead" like papules along proximal and lateral nail fold articular regions of limbs head, fingers and mucosa leonine facies possible
34
extracutaneous of multicentric reticulohistiocytosis?
multicentric: arthritis (symmetric, erosive, multiple) -> arthritis mutilans heme Ca solid cancer Ca (about 30%)
35
H&E for multicentric reticulohistiocytosis?
lymphs, histiocytes, plasmas and eos histiocytes multinuclear, angulated, ++ eosinophilic finely granular cytoplasm -> "ground glass effect" like in giant cell reticulohistiocytosis
36
Tx for multicentric reticulohistiocytosis?
``` remission in 5-10 yrs rarely fatal cancer screen surgical excision curative reports of NSAIDS, PO CS, MTX, CsPh, etc ```
37
clinical presentation of Rosai-Dorfman dz?
cyclical coures eyelids and malar area papules painless bilateral cervical LAD +- fever, anemia, polyclonal hypergamma,
38
systemic in Rosai-Dorfman?
NHL, HL associated | skin, soft tissue, eyes, salivary, CNS, bone involved
39
unique thing on H&E for Rosai-Dorfman?
emperipolesis - histiocytes eat lymphocytes/plasma cells
40
Tx for Rosai-Dorfmann?
``` regresses over the years XRT excision steroids thalidomide ```
41
clin of xanthoma disseminatum?
yellow, red or brown papules in 100s symmetric,flexural and intertriginous triad: DI skin xanthomas MM xanthomas
42
systemic involvement in xanthoma disseminatum?
DI corneal and conjunctival involvement can threaten vision rare monno gammos, thyroid disorders
43
Tx for Xanthoma disseminatum?
``` no good tx surgery radiotherapy cryo and laser CsA ```
44
List cutaneous histiocytoses w. frequent systemic involvement?
NaRX NXG Rosai Dorfman Multicentric reticulohistiocytosis xanthoma disseminatum
45
What histiocytic d/o can manifest with leonine facies?
multicentric reticulohistiocytosis
46
what histiocytic d/o is linked to petechiae/purpura?
LCH
47
what histiocytic d/o is linked ot molluscum-like lesions?
LCH
48
what histiocytic d/o has "coral beads "around nail folds?
multicentric reticulohistiocytosis
49
what histiocytic d/o has highest rate of solid organ and heme malignancies
multicentric reticulohistiocytosis - 30%
50
Which histiocytic disorder has groundglass on histology?
multicentric reticulohistiocytosis and giant cell reticulohistiocytoma (both related)
51
which histiocytic disorder has bilateral painless cervical LAD?
Rosai-Dorfman
52
you got this?
yes!
53
histiocytic disorders with eye inv.
``` hans schuller Christian JXG ICH XNG xanthoma disseminatum ```
54
histiocytic d/os with gammopathies?
NXG Rosai-Dorfmann Xanthoma disseminatum (basically all NLCH with frequent systemic involvements except multicentric reticulohist, which is +++ heme Ca)
55
List 7 skin directed therapies in LCH? | hint: lymphoma-like tx
• 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
Workup for LCH?
``` 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
Common mutation in LCH?
BRAF V600E
58
systems invovled in LCH?
Ls - liver, lytic bone, LN, lung (smokers) DI otitis media
59
risk organs in LCH?
heme, liver, lungs, spleen | single system or no risk organs -> mortality <5%
60
LCH: S100, CD1a, Langerin, Factor XIIIa, CD68, HAM56?
positive: S100, CD1a, Langerin negative: factor XIIIa, macrophage factors such as CD68, HAM56

Bologna 80-100 (3 decks)

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