Histiocytoses Flashcards

1
Q

Langerhans cells are antigen presenting cells in the epidermis, bronchi and mucosa

A

True

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2
Q

Skin biopsy of LCH demonstrates an inflammatory infiltrate of eosinphils, histiocytes and lymphocytes

A

True

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3
Q

Stains of LCH demonstrate positivity to CD1a,CD117

A

False = not CD117, which is for mast cells, but CD207 +

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4
Q

Hand-Schuller-Christian is self healing

A

False. This is Hashimoto-Pritzker .

- Hand Schuller Christian has exophthalmos, diabetes insipidus and lytic bone lesions and is low-risk multisystem LCH

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5
Q

Benign cephalic histiocytosis occurs in infants >1 year of age most commonly

A

False - <1year of age

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6
Q

Benign cephalic histiocytosis mostly occurs by age 1 and always within the first 3 years

A

T

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7
Q

What are the 3 histologic patterns for benign cephalic histiocytosis

A

PDL

  • papillary dermal
  • diffuse
  • lichenoid
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8
Q

Generalised eruptive histiocytoma affects adults more than children

A

True

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9
Q

Treatment is generally required for generalised eruptive histiocytoma

A

False - generally NOT required

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10
Q

Indeterminate cell histiocytosis is positive for s100 and CD1a which are LCH markers, and non LCH markers CD68+ and CD163+

A

True

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11
Q

Indeterminate cell histiocytosis has two variants : solitary and generalised

A

True

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12
Q

Indeterminate cell histiocytosis is Langerin positive (CD207)

A

False - Langerin negative

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13
Q

Touton giant cells present in indeterminate cell histiocytosis

A

True

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14
Q

Juvenile xanthogranuloma is the most common histiocytosis

A

True

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15
Q

JXG associated with hyperlipidaemia

A

False - reason for progressive lipidation of histiocytes in absence of hyperlipidaemia is unclear

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16
Q

JXG Fatalities have been reported with liver involvement and CNS involvement

A

True

17
Q

Ocular JXG is usually bilateral

A

False- unilateral and develops in <0.5% of patients

18
Q

Oral JXG is common

A

False - rare; usually lateral tongue or midline of the hard palate

19
Q

JXG have a firm and rubbery consistency

A

True

20
Q

JXG stains

A
— CD68+
— CD163+
— Factor XIIIa 
— HAM56
— Fascin
— sometimes s100
21
Q

JXG histo

A

Sheet like nodular aggregates of histiocytes, lymphocytes, eosinophils with admixed Touton -like giant cells in the dermis

22
Q

JXG can exhibit needle shaped clefts

A

False - unlikely sclerema neonatorum, SC fat necrosis, post steroid panniculus and cholesterol emboli

23
Q

NXG average age of onset is the eighth decade

A

False - sixth decade

24
Q

NXG- males to female ratio 1:1

A

True

25
Q

NXG associated with IgA monoclonal gammopathy

A

False- IgG

26
Q

NXG have ocular involvement in 5%

A

False - 50% have ocular involvement with orbital masses, ectropion , ptosis, conjunctival lesions, keratitis and scleritis , anterior uveitis, proptosis

27
Q

SC nodules of NXG need to be distinguished from nodules of RA and SC granuloma annulare

A

True

28
Q

Reticulohistiocytosis can be a single lesion or multicentric with systemic involvement

A

True

29
Q

Reticulohistiocytosis pathology shows…

A

Histiocytes characteristic with abundant Eosinophilic cytoplasm, which is homogenous and finely granular, creating a “ground glass” effect

30
Q

Rosai-Dorfman affects the elderly and not children and young adults

A

FALSE - affects children and young adults most commonly

— except skin limited form affects adult women most commonly

31
Q

In Rosai-Dorfman, fever and polyclonal hypergammaglobulinaemia are often seen

A

True

32
Q

Emperipolesis is a common histologic finding of Rosai Dorfman in the skin but not the lymph nodes

A

False - both

33
Q

Rosai Dorfman and H syndrome have mutations in the same gene SLC29A3

A

True

34
Q

Males are overall more affected by Rosai Dorfman than females

A

True

35
Q

Emperipolesis refers to..

A

Taking up of intact lymphocyte sand plasma cells by histiocytes

36
Q

Rosai Dorfman stains..

A

CD68, CD163, CD11c, CD14, laminin 5 and lysozyme

37
Q

Xanthoma disseminatum triad.. ?

A

Cutaneous xanthoma, diabetes insipidus, mucous membrane xanthomas

38
Q

In xanthoma disseminatum, most patients have abnormal lipid levels

A

False - normal

39
Q

Xanthoma disseminatum can be treated with radiotherapy

A

True for airway obstructive disease