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FITZPATRICK'S DERMATOLOGY 9TH EDITION (SHAMMY) > 20 - 117: HISTIOCYTOSIS > Flashcards

20 - 117: HISTIOCYTOSIS Flashcards

1
Q

Most sensitive marker since this is associated with Birbeck granules

A

CD 207

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2
Q

Most common presentation of this disease is neck lymphadenopathy observed after an infectious disease.Biopsy was done revealing lymphocytes or neutrophils found in the cytoplasm of histiocytes with a background of mature lymphocytes and plasma cells

A

Sinus histiocytosis with massive lymphadenopathy

Answer: D. Rosai Dorfman Disease p. 2031

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3
Q

Mature lesions of juvenile xanthogranuloma are characterized by the presence of foam cells, touton giant cells and foreign body giant cells. Histopath stains for Stabilin -1, S100 and CD1a would show:

A

(+) Stabilin – 1, (-) S100, (-) CD1a

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4
Q

Characterized by ultrastructural presence of worm-like bodies or histiocytosis with intracytoplasmic worm-like bodies

A

Benign cephalic histiocytosis

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5
Q

Most common non-cutaneous organ involved in LCH

A

Bone

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6
Q

Most common endocrinopathy encountered in LCH

A

Diabetes insipidus

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7
Q

This variant of NLCH preferentially affects male children presenting as sudden appearance of discrete, generalized, small yellow-red to brown papules and nodules with a predilection of flexural and intertriginous areas, as well as mucous membranes

a. Generalized eruptive histiocytoma
b. Xanthoma disseminatum
c. Multicentric histiocytosis
d. Necrobiotic xanthogranuloma

A

Answer: B. p. 2035

  • A. – affects adults
  • C. – appears around the age of 50 years, twice as common in females, yellow-brown papules over wrist and fingers
  • D. yellowish plaques that ulcerate
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8
Q

This variant of NLCH is characterized by persistent fever, splenomegaly with cytopenia, hypertriglyceridemia, and hypofibrogenemia

a. Rosai-Dorfman Disease
b. Hemophagocytic lymphohistiocytosis
c. Juvenile Xanthogranuloma
d. Benign cephalic histiocytosis

A

Answer: B. p. 2032 overall mortality is 50%. Treatment options: high-dose corticosteroids, cyclosporine, etoposide, MTX, vincristine

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9
Q

Long bone involvement is almost universal in this type of NLCH

a. Rosai-Dorfman Disease
b. Hemophagocytic lymphohistiocytosis
c. Multicentric histiocytosis
d. Erdheim Chester disease

A

Answer: D. p. 2038

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10
Q
A
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11
Q

Langerhans cells are positive with what immunohistochemical stains

A

CD1a/ S100B/CD207

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12
Q

most aggressive acute LCH multisystem variant with apparent systemic symptoms such as fever, hepatosplenomegaly, polylymphadenopathy, anemia, arthralgia, malaise, and weight loss

A

Letterer-Siwe disease

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13
Q

organs commonly affected in Letterer-Siwe disease

A

lung, the bone marrow, and the brain

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14
Q

this is a poor prognostic sign in Letterer-Siwe disease

A

purpura

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15
Q

60% of LCH biopsy specimens bear this mutation

A

BRAF-V600E somatic point mutation

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16
Q

these variants of LCH have no systemic involvement

A
  • Hashimoto-Pritzker Disease
  • Eosinophilic Granuloma (Unifocal LCH)
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17
Q

benign clinical variant typically presenting with multiple firm red-brown nodules with an elevated border or papulovesicular and papulocrusted lesions mostly on the scalp and face in the **first few months and years of life. **

These lesions usually heal within 2 to 3 months and occasionally leave whitish atrophic scars.

A

Hashimoto-Pritzker disease

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18
Q

mainly benign isolated or multifocal osteolytic bone LCH, which sometimes affects the skin and mucous membranes

A

Eosinophilic granuloma

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19
Q

these variant of LCH may present with nodulo-ulcerative lesions in the mouth

A
  • Eosinophilic Granuloma
  • Hand-Schüller-Christian disease
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20
Q

Triad of Hand-Schüller-Christian disease

A
  1. Diabetes Insipidus
  2. Bone Lesions
  3. Exophthalmos
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21
Q

this variant of LCH is lethal if untreated

A

Letterer-Siwe disease

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22
Q

affected areas in Single-system LCH from most to least common

A
  • Bone
  • Skin
  • Lymph nodes
  • Lungs
  • CNS
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23
Q

what are the high risk organs in LCH?

A
  • Hematopoietic system
  • Liver
  • Spleen
24
Q

second most common organ involved after bone lesions and can be the earliest sign of disease in LCH

A

cutaneous findings

25
Q

skin manifestations of LCH

A
  • most typical ones are small, translucent rose-yellowish crusted papules or papulovesicles on the trunk, in the intertriginous areas and the scalp, associated with eczematous scaling which resembles “candida intertrigo” or “seborrheic dermatitis”
  • Lesions can also present as hemorrhagic papules and nodules associated with petechiae reminiscent of vascular lesions or “varicella-like eruptions”
  • Vesicles, pustules, and nail involvement have been described
26
Q
A
27
Q
A
28
Q

These findings seem to be associated with a higher risk for multisystem LCH

A

Mucosal lesions and external otitis media

29
Q

These findings might be a poor prognostic sign in LCH

A

Purpura with nail involvement

30
Q

most common noncutaneous organ involved in LCH

A

BONE

  • bone (77% of cases),
  • lymph nodes (19%),
  • liver (16%),
  • spleen (13%),
  • lung (10%),
  • CNS (6%).
31
Q

In children with LCH, what is the most commonly involved bone ?

A

cervical vertebrae

32
Q

In adults with LCH, what is the most common manifestation of bone involvement?

A

Asymmetric Vertebral collapse

can be one of the most prominent diagnostic hints for LCH - vertebral collapse

33
Q

most commonly involved lymph node in LCH

A

Cervical LN

34
Q

These are among the most serious complications of LCH.

A

Cholestasis and sclerosing cholangitis induced by hepatic involvement of LCH

35
Q

The most common manifestations of CNS involvement in LCH

A

diabetes insipidus, and neurodegenerative symptoms

Endocrine abnormalities resulting from large pituitary tumors, most frequently diabetes insipidus, and neurodegenerative symptoms such as ataxia, dysarthria, cognitive dysfunction, and behavior changes.

36
Q

most common endocrinopathy encountered in LCH

A

Diabetes insipidus

Patients present with polyuria, polydipsia, and nocturia.

37
Q

Histology of Langerhans cell histiocytosis (LCH)

A

Histopathologically, typical findings in a skin biopsy show a dense and band-like infiltration of the papillary dermis with LCH cells (Fig. 117-7). These cells are oval shaped with an eosinophilic cytoplasm and typically display an irregular, vesicular, and infolded (kidney-shaped) nucleus

38
Q

Langerhans cell histiocytosis is postitive to what immunohistochemical stains?

A

Positive: CD1a, CD2, CD11b, CD11c, CD13, CD66c, CD68, CD207 (Langerin), CD300LF, S100B, fascin

39
Q

Langerhans cell histiocytosis is negative to what immunohistochemical stain?

A

Negative: stabilin-1

40
Q

in Non-Langerhans cell histiocytosis, how can you differentiate Systemic vs cutaneous?

A
41
Q

This immunohistochemical staining is the most sensitive marker in detecting bircebk granules of langerhans cells

A

CD207, an antibody that recognizes a C-type lectin associated with Birbeck granules

42
Q

In LCH, one of the most important predictors of outcome in patients with multiorgan involvement is how the disease responds to systemic treatment in the first how many weeks?

A

6 weeks

43
Q
A
44
Q

treatment protocol for LCH

A

**6-week **induction chemotherapy phase with:
1. vinblastine (6 mg/m2 weekly intravenous bolus) +
2. prednisolone (40 mg/m2 /day orally for 4 weeks and then tapered over 2 weeks)

and a subsequent therapy with vinblastine/prednisolone with or without mercaptopurine, depending on the treatment response after 6 weeks and the involvement of at-risk organs

45
Q

induction treatment for LCH is done how many weeks?

A

6 weeks

46
Q

represents a group of different disorders characterized by the proliferation of histiocytes that do not meet criteria to be diagnosed as Langerhans cells

A

NON-LANGERHANS CELL HISTIOCYTOSIS

47
Q

N-LCH are immunohistochemically positive for?

A

(+) CD68
(-)S100B and CD1a

48
Q

This can discriminate N-LCH from LCH and other granulomatous diseases

A

Stabilin-1 (formerly MS-1 antigen or MS-1-HMWP)

49
Q

What are the subclassifications of cutaneous N-LCH?

A
  1. Juvenile Xanthogranuloma
  2. Adult xanthogranuloma
  3. Necrobiotic xanthogranuloma
  4. Spidle Cell N-LCH
50
Q

Systemic forms of N-LCH are regarded as an accumulation of?

A

classically activated macrophages (Mφ1)

Mφ1 are known to develop in response to proinflammatory stimuli such as **T-helper (Th) 1 cytokines **(interferon-γ or bacterial products [lipopolysaccharides]).

They are characterized by secretion of proinflammatory cytokines, such as **interleukin (IL)-1, IL-6, and tumor necrosis factor-α, **and possess a strong oxidative burst and a profound antimicrobial activity.

51
Q

Cutaneous N-LCH are characterized by the presence of

A

alternatively activated macrophages (Mφ2)

Mφ2 are induced by Th2 cytokines, including IL-4, IL-10, IL-13, and transforming growth factor-β, or by antiinflammatory mediators such as glucocorticoids.

They express antiinflammatory cytokines such as IL-1R antagonist and IL-10,chemokine receptor antagonists such as AMAC-1, broad-spectrum receptors of innate immunity, such as macrophage mannose receptor and the haptoglobin receptor CD163.

52
Q

main clinical manifestation of Rosai-Dorfman disease (RDD)

A

Lymphadenopathy

The neck lymph nodes are the most common place of histiocyte accumulation, although accumulation outside of lymph nodes may occur

53
Q

an be used as a serum marker to monitor disease progression or therapy response in Rosai-Dorfman disease (RDD)

A

S100B

The RDD cells are also positive for CD68, CD163, α1 -antitrypsin, α1 -antichymotrypsin, fascin, HAM-56 (human alveolar macrophage 56), and stabilin-1

54
Q

hallmark of RDD

A

The hallmark of RDD is emperipolesis, in which different types of bone marrow cells, such as lymphocytes or neutrophils, are found in the cytoplasm of histiocytes with a background of mature lymphocytes and plasma cells.

55
Q

characterized by persistent fever, splenomegaly with cytopenia, hypertriglyceridemia, and hypofibrinogenemia

A

Hemophagocytic lymphohistiocytosis (HLH)

Increased levels of various cytokines and soluble IL-2 receptor are biologic markers of HLH.

56
Q
A

FITZPATRICK'S DERMATOLOGY 9TH EDITION (SHAMMY) (122 decks)

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