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Dermatology > Sec 25 Skin Manifestations of Bone Marrow or Blood Chemistry Disorders > Flashcards

Sec 25 Skin Manifestations of Bone Marrow or Blood Chemistry Disorders Flashcards

1
Q

Skin lesions that bear a clinical and/or histopathologic resemblance to lymphoma

A

Cutaneous pseudolymphoma

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2
Q

Relatively dense lymphoid infiltrate in the reticular dermis usually B-cell rich resembling lymphoma

A

Cutaneous lymphoid hyperplasia

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3
Q

Medications that may induce CLH

A 
Phenytoin
Carbamazepine
Phenobarbital
B blockers
Calcium channel blockers
ACE inhibitors
Allopurinol
D-penicillamine
Penicillin
Mexiletine chloride
Cyclosporine
Histamine inhibitors
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4
Q

B and T cell

reticular dermis

A

Cutaneous lymphoid hyperplasia

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5
Q

B and T cell
subcutis
lymphadenopathy

A

Kimura disease

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6
Q

B and T cell
reticular dermis
eosinophilia

A

Angiolymphoid hyperplasia with eosinophilia

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7
Q

B and T cell
subcutis
POEMS syndrome, lymphadenopathy

A

Castleman disease

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8
Q

T cell

papillary dermis and epidermis

A

Pseudomycosis fungoides

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9
Q

T cell
papillary dermis and epidermis
contact allergens

A

Lymphomatoid contact dermatitis

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10
Q

T cell

perivascular and periadnexal dermis

A

Lymphocytic infiltration of the skin (Jessner’s)

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11
Q

Lymphoma B symptoms

A 
fever of unknown origin
unexplained weight loss
night sweats
fatigue
malaise
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12
Q

Presents most commonly as a solitary nodule or as a localized array of nodules, plaques or papules on the head, neck, extremities, breasts and genitalia with doughy to firm consistency

A

Cutaneous lymphoid hyperplasia

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13
Q

Syndrome caused by anticonvulsants such as phenytoin which presents with fever, lymphadenopathy, hepatosplenomegaly, arthralgia, eosinophilia and generalized macules and papules or nodules

A

Hydantoin-associated pseudolymphoma syndrome

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14
Q

Presents as a unilateral eruption of angiomatous papules on the extremities with dense lymphoid infiltrate associated with histiocytes, plasma cells and prominent thickened capillaries

A

Acral pseudolymphomatous angiokeratoma of children

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15
Q

Cutaneous pseudolymphoma of mixed cell or large forms of primary B-cell lymphoma

A

Large cell lymphocytoma

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16
Q

Reveals a dense, nodular or diffuse lymphoid infiltrate in the reticular dermis tends to be top heavy and taper to the lower dermis; epidermis is normal and separated from infiltrate with narrow grenz zone

A

Cutaneous lymphoid hyperplasia

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17
Q

Infiltrate consists of predominantly small lymphocytes with an admixture of large lymphoid cells, histiocytes and eosinophils

A

Cutaneous lymphoid hyperplasia

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18
Q

Present in germinal centers that contain phagocytized debris from apoptotic lymphoid cells

A

Tingible-body macrophages

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19
Q

Defining immunophenotypic feature of CLH

A

Small, nongerminal center B cells and plasma cells are polytypic

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20
Q

CLH with presence of a dominant B-cell clone

A

Clonal CLH

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21
Q

Disorders that can contain dense cutaneous infiltrates of both T cells and B cells

A
  1. Lymphoid keratosis
  2. Pseudolymphomatous folliculitis
  3. Systemic immunoglobulin IgG4-related plasmacytic syndrome
  4. CD4+ small/medium pleomorphic T cell lymphoma
  5. Cutaneous lymphoid hyperplasia
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22
Q

Progonis: CLH

A

Resolve sponataneously or persist indefinitely

Regress with biopsy

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23
Q

First line treatment: CLH

A

Excision
Topical corticosteroids (mid to high potent twice daily)
Intralesional corticosteroids (5-40 mg/ml, 1ml monthly)
Systemic antibiotics (minocyline, cephalexin)
Topical tacrolimus

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24
Q

Second line treatment: CLH

A 
Cryotherapy
G-Aminolevulinic acid PDT
Laser therapy (PDL)
Hydroxychloroquine
Systemic corticosteroids
Local radiation therapy
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25
Q

Presents as a solitary or multiple nodules in the subcutis, represents a florid, subcutaneously deep seated form of disease like CLH which is more common in Asian men with peripheral eosinophilia and regional lymphadenopathy

A

Kimura disease

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26
Q

Presents with multiple, smaller, more superficial intradermal papulonodules that are usually unilateral which are malformation of blood vessels caused by and underlying arteriovenous shunt which is more common in women

A

Angiolymphoid hyperplasia with eosinophilia

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27
Q

Histopath: Hyperplasia of small blood vessels lined by plump endothelial cells protruding into the lumen with vascular hyperplasia with thickened vascular walls, lymphocytic infiltrate and prominent eosinophilia

A

Angiolymphoid hyperplasia with eosinophilia

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28
Q

Complication of Kimura disease

A

Lichen amyloidosis

Nephrotic syndrome

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29
Q

First line treatment of Kimura disease and Angiolymphoid hyperplasia

A 
Excision
Topical corticosteroids (high potency 2x/day)
Intralesional corticosteroids (5-40 mg/ml, 1 ml monthly)
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30
Q

Also known as Angiofollicular lymphoid hyperplasia or Giant lymph node hyperplasia

A

Castleman disease

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31
Q

4 Subtypes of Castleman disease

A
  1. Hyaline-vascular
  2. Plasma cell
  3. HHV-8 associated
  4. Multicentric, NOS
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32
Q

POEMS Syndrome

A 
Polyneuropathy
Organomegaly
Endocrinopathy
M protein
Skin changes
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33
Q

Presents as an isolated mediastinal mass can be nodal or extranodal associated with polyneuropathy, organomegaly, endocrinopathy, presence of M protein, hyperpigmentation and hypertrichosis

A

Castleman disease

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34
Q

Histopathology: Exhibits small, concentrically whorled, lymphoid follicles surrounded by small lymphocytes arranged in a concentric, onionskin pattern with extensive proliferation of capillaries in between follicles

A

Castleman disease - Hyaline-vascular

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35
Q

Histopathology: Exhibits large, hyperplastic secondary lymphoid follicles associated with highly vascular interfollicular zone rich in plasma cells

A

Castleman disease - Plasma cell

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36
Q

Complications: Castleman disease

A 
Paraneoplastic pemphigus
Plane xanthoma
Vasculitis
Peliosis hapitis
Lymphoma
Follicular dendritic cell sarcoma
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37
Q

Prognosis: Castleman disease

A

Favorable for localized, extranodal

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38
Q

Treatment: Castleman disease

A 
Excision (first line)
Second line:
Radiation therapy
Chemotherapy
Rituximab +/- chemotherapy or thalidomide
Anti-IL-6 or anti-IL-6 antibody
Bortezomib
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39
Q

Present as one or few plaques on trunk or extremities with history of associated disease or medication

A

Pseudomycosis fungoides

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40
Q

Medications associated with Pseudomycosis fungoides

A 
Hydantoin
Carbamazepine
Propyl valerate
Imatinib mesylate
Dexchlorpheniramine maleate
Antihistamines
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41
Q

Histopathology: Papillary dermal, band-like infiltrate containing mostly small and medium-sized atypical lymphocytes with clefted and cerebriform nuclei, no epidermal-dermal junction obscured, no grenz zone, minimal epidermotropism

A

Pseudomycosis fungoides

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42
Q

Treatment: Pseudomycosis fungoides

A 
First line:
Drug discontinuation
Treatment of underlying disorder
Topical corticosteroids
UVB and narrowband UVB phototherapy
Psoralen plus UVA phototherapy
Second line:
Systemic corticosteroids (60/40/20 mg PO taper)
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43
Q

Presents as generalized red scaly papules and plaques that may become confluent with resultant exfoliative erythroderma associated with pruritus in adults of both genders

A

Lymphomatoid contact dermatitis

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44
Q

Allergens responsible in Lymphomatoid contact dermatitis

A 
Phosphorus sesquisulfide
Para-tertiary butyl phenol formaldehyde resin
Ethylenediamine dihydrochloride
N-isopropyl-N-phenyl-p-phenylenediamine
Benzydamine hydrochloride
Teak wood
Cobalt naphthalene
Gold
Nickel
Para-phenylenediamine
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45
Q

Histopathology: Exhibits superficial lymphocytic dermatitis that contains foci of spongiosis simulating the appearance of cutaneous T-cell lymphomas with edema in the papillary dermis

A

Lymphomatoid contact dermatitis

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46
Q

Treatment: Lymphomatoid contact dermatitis

A 
First line:
Elimination of responsible allergen
Topical corticosteroids
Second line:
Topical tacrolimus and pimecrolimus
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47
Q

Presents as one or more erythematous plaques or nodules generally localized to one or few sites on the face, neck and upper trunk or arms in middle-aged adults some with photoexacerbation

A

Lymphocytic infiltration of the skin

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48
Q

Histopathology: Reveals a superficial and deep perivascular and variably periadnexal infiltrate of small mature lymphocytes with histiocytes, plasma cells and plasmacytoid macrophages

A

Lymphocytic infiltration of the skin

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49
Q

Prognosis: Lymphocytic infiltration of the skin

A

Chronic coursee

Spontaneous remission and eventual resolution

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50
Q

Treatment: Lymphocytic infiltration of the skin (first line)

A

Topical corticosteroids
Topical tacrolimus and pimecrolimus
Photoprotection
Intralesional steroid injection (5-40mg/ml, 1 ml monthly)

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51
Q

Treatment: Lymphocytic infiltration of the skin (second line)

A

Hydroxycholoroquine (100-200 mg PO daily)
Systemic corticosteroids (60/40/20 taper 5 days each)
Auranofin (3 mg PO daily)
Acitretin (25-50 mg PO daily)
Thalidomide (100mg PO daily)
Pulse dye laser (595 nm)

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52
Q

They are antigen presenting cells that interact with T cells

A

Dendritic cells

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53
Q

Has a long cytoplasmic projections and large almost kidney-shaped nucleus with characterisic Birbeck granule, a tennis racket-shaped structure involved in pinocytosis and receptor-mediated endocytosis

A

Langerhan cell

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54
Q

Receptor that permits internalization of antigen into Birbeck granules and presentation of antigen at the cell surface and most specific marker for Langerhan cells

A

Langerin (CD207)

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55
Q

LCH prototype of acute disseminated multisystemic form in infants or newborns and if untreated, fatal

A

Letterer-Siwe disease

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56
Q

Chronic progressive multifocal form of LCH beginning in childhood

A

Hand-Schuller-Christian disease

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57
Q

Localized benign form of LCH

A

Eosinophilic granuloma

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58
Q

Benign self-healing variant of LCH

A

Hashimoto-Pritzker disease

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59
Q

Presumptive diagnosis of LCH

A

Light morphologic characteristics

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60
Q

Designated diagnosis of LCH

A

Light morphologic characteristics + 2 or more supplemental positive results to stains for adenosine triphosphatase, S100 protein, a-D-mannosidase, and peanut lectin

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61
Q

Definitive diagnosis of LCH

A

Light morphologic characteristics + Birbeck granules in the lesional cell visible with EM and/or positive results on staining for CD1a antigen on the lesional cell

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62
Q

Classification of LCH

A

Single-system Disease - Localized
Single-system Disease - Multiple site
Multisystem Disease - Low risk group
Multisystem Disease - High risk group

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63
Q

Monostotic bone involvement
Isolated skin involvement
Solitary lymph node involvement

A

Single-system Disease - Localized

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64
Q

Polyostotic bone involvement
Multifocal bone disease
Multiple lymph node involvement

A

Single-system Disease - Multiple site

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65
Q

Disseminated disease with involvement of skin, bone, lymph node, or pituitary

A

Multisystem Disease - Low risk group

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66
Q

Disseminated disease with involvement of hematopoietic system, lungs, liver and/or spleen

A

Multisystem Disease - High risk group

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67
Q

Presents with small translucent papule 1-2 mm in diameter slightly raised rose-yellow usually on the trunk or scalp which may show scaling and can become ulcerated and crusted

A

Langerhans-Cell Histiocytosis

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68
Q

Characterized by the eruption of multiple or solitary elevated form red-brown nodules or flesh-red lesions similar to infantile angiomas which ulcerate easily growing in size then forming crusts then shed leaving whitish atrophic scars

A

Hashimoto-Pritzker disease

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69
Q

T or F: Oral manifestations may be the first sign of LCH

A

True

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70
Q

T or F: Nail changes are unfavorable prognostic sign

A

True

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71
Q

Nail changes in LCH

A 
Fragile lamina
Paronychia
Subungual pustules
Nail fold destruction
Onycholysis
Subungual hyperkeratosis
Longitudinal grooving
Pigmented and purpuric striae of nail bed
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72
Q

T or F: Bone lesions are the most frequent manifestation of LCH

A

True

80%, seen alone in 50-60% of cases

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73
Q

Part of the skull most often involved in LCH with lead to limited osteolytic foci that merge to form typical “map” lesions

A

Temporoparietal region

74
Q

Involvement of this bone is responsible for exophthalmos

A

Retro-ocular bone involvement

75
Q

Chest radiograph finding in LCH

A

Classic “honeycomb” appearance or micronodular pattern

76
Q

Most common lymph nodes affected in LCH

A

Cervical lymph nodes

77
Q

Present in more than 50% of cases and occurs more often in patients with involvement of the skull and orbits

A

Diabetes insipidus

78
Q

First sign of CNS involvement in LCH

A

Cerebellar manifestations

79
Q

Most common intracranial manifestation in LCH

A

Hypothalamic pituitary region infiltration (10-15% - associated with diabetes insipidus and anterior pituitary hormone deficiency)

80
Q

Second most common intracranial manifestation in LCH

A

Neurodegenerative LCH

81
Q

Type of reaction where extensive epidermotropism, lichenoid infiltration of LCH cells in the upper dermis.

A

Proliferative reaction

82
Q

Type of reaction which consists of aggregatio of LCH cells with multinucleated histiocytes and a varying number of eosinophils

A

Granulomatous reaction

83
Q

Type of reaction which consists of mainly HSC and numerous foam cells intermingled with LCH cells and eosinophils

A

Xanthomatous reaction

84
Q

Expression of this is an indicator of good prognosis and limited disease in LCH

A

E-cadherin

85
Q

Most important predictor of poor outcome in LCH

A

Organ dysfunction

86
Q

Treatment for Single-system Disease (skin)

A 
Observation (children)
Nitrogen mustard (adult)
87
Q

Treatment for Single-system Disease (bone)

A

Excision - accessible

88
Q

Represents a broad group of different disorders by proliferation of histiocytes other than Langerhans cells

A

Non-Langerhans cell histiocytosis

89
Q

Laboratory investigations for LCH

A 
CBC
Coagulation profile
Serum protein electrophoresis
ESR
CRP level
Serum glucose level
LFT
Electrolyte levls
T and B cell counts
T cell subset analysis
Urinalysis (urine osmalality test)
90
Q

Treatment for Single-system Disease (bone) in children

A

Indomethacin

91
Q

Treatment for Multisystem Disease LCH

A

Vinblastine (0.1-0.2 mg/kg) +/- Glucocorticoids

92
Q

Treatment for Multisystem Disease LCH if monotherapy is not effective

A

Vincristine
Cyclophosphamide
Doxorubicin
Chlorambucil

93
Q

Treatment for refractory and advanced Multisystem Disease LCH

A

Cyclosporin
Interferon-a2
2-chlorodeoxyadenosine (Cladribine)

94
Q

Accounts for 80-90% of Non-Langerhans cell histiocytosis

A

Juvenile xanthogranuloma

95
Q

NLCH that appears within the first year of life which is a benign self-healing disorder characterized by asymptomatic yellowish papulonodular lesions of the skin without metabolic disorders

A

Juvenile xanthogranuloma

96
Q

Variant of JXG rare with less than 20 cases onset on first year of life more common in males with lesions ranging from 2-12mm in diameter with generalized distribution no tendecy to merge into plaques rapidly become xanthomatous

A

Papular xanthoma

97
Q

Rare NLCH with approximately 40 cases may start at any age with asymptomatic eruption of round or oval papules that are firm, or dark red and range from 3 to 10mm appearing in successive crops usually in hundreds and symmetrically distributed involving mucous membranes

A

Generalized eruptive histiocytosis

98
Q

Rare NLCH with 30 cases onset is 5-34 months considered a limited form of GEH with asymptomatic slightly raised round or oval orange-red or red-brown lesions 2-8mm in diameter on upper face (eyelids, forehead, cheeks)

A

Benign cephalic histiocytosis

99
Q

Exceptional form of NLCH in few children and adults which is a progressive disease with no tendency to involute spontaneously which is a proliferation of mature spindle-shaped cells

A

Progressive nodular histiocytosis

100
Q

Rare benign NLCH with 100 cases more frequent in males onset is before 25 years associated with diabetes insipidus with cutaneous manifestations consisting of hundreds of papules that are red-brown then become yellowish symmetrically involve the eyelids, trunk, face and proximal extremities and in flexures and folds

A

Xanthoma disseminatum

101
Q

Rare MLCH 60 cases variant of XD with visceral manifestations and progressive

A

Erdheim-Chester disease

102
Q

Uncommon NLCH more than 100 cases purely cutaneous Caucasian women 40 years of age more commonly affected which may represent an abnormal histiocytic reaction like trauma (solitary forms) or internal malignancy and autoimmune disease (diffuse forms)

A

Multicentric reticulohistiocytosis

103
Q

NLCH described in 60 cases with no gender predilection onset from 17 to 85 years with lesions consisting of indurated papulonodules varying in color from red-orange to violaceous or yellow slowly enlarging into plaques with well-demarcared edges with central atrophy and telangiectasia and linked to paraproteinemia (90%)

A

Necrobiotic xanthogranuloma

104
Q

Relatively rare benign self-limited NLCH with approximately 365 cases with worldwide distribution increased in blacks no gender predilection and any age group confined mainly to cervical lymph nodes

A

Sinus histiocytosis with massive lymphadenopathy

105
Q

JXG form which is characterized by numerous firm hemispheric lesions 2-5 mm in diameter irregularly scattered throughout the skin but are located mainly on upper body

A

Papular JXG

106
Q

JXG form which is less frequent occurs as few nodules generally round 10-20mm in diameter, translucent and show telangiectasia

A

Nodular JXG

107
Q

Lesions of JXG greater than 2cm

A

Giant juvenile xanthogranuloma

108
Q

Group of JXG lesions with a tendency to coalesce into a plaque

A

JXG en plaque

109
Q

2 new variants of JXG

A

Blueberry muffin baby - bluish papules and nodules

Multiple lichenoid JXG - hundreds of discrete papules

110
Q

Most typical extracutaneous finding in JXG

A

Ocular involvement

111
Q

Extremely rare extracutaneous manifestation of papular form with CNS involvement and related to systemic lesions

A

Juvenile chronic myelogenous leukemia

112
Q

Histopathology: Nonepidermotropic histiocytic infiltrate lacking Langerhans granules with monomorphic nonlipid-containing histiocytic infiltrate occupying upper half (early) and foam cells, FB giant cells, and Touton giant cell (mature)

A

Juvenile xanthogranuloma

113
Q

Complication: Juvenile xanthogranuloma

A

Severe secondary glaucoma

114
Q

Prognosis: Juvenile xanthogranuloma

A

Flatten with time
Evolves separately - different stages
Good prognosis

115
Q

Histopathology: Dense, monomorphous nonxanthomatous histiocytic infiltrate (containing nucleus with scanty chromatin and abundant light poorly limited cytoplasm) in the papillary and midportion of the dermis with few lymphocytes

A

Generalized eruptive histiocytosis

Benign cephalic histiocytosis

116
Q

Electron microscopy: Tumor cells tend to contain a large number of dense and regularly laminated bodies clustered together with occasional worm-like bodies

A

Generalized eruptive histiocytosis

117
Q

Electron microscopy: Many coated vesicles with diameter ranging from 500-1500 nm with clusters of comma-shaped bodies

A

Benign cephalic histiocytosis

118
Q

Prognosis: GEH/BCH

A

Regress spontaneously

119
Q

Presents with yellow to orange papules 2-10mm in diameter that are randomly scattered over the body without localization in the flexural areas

A

Progressive nodular histiocytosis - superficial papules

120
Q

Presents with deep dermal nodules ranging from 1 to 5 cm without overlying telangiectasia mainly on the trunk

A

Progressive nodular histiocytosis - deep nodules

121
Q

Histopathology: Xanthogranuloma with predominance of storiform spindle-shaped histiocytes that are positive for macrophage/dendritic cell line markers (CD68) and negative for CD1a and S100

A

Progressive nodular histiocytosis

122
Q

Prognosis: Progressive nodular histiocytosis

A

Progresses but good health

123
Q

Most common sign in Erdheim-Chester disease

A

Chronic bone pain

124
Q

Associated with multiple myeloma, Waldenstrom macroglobulinemia and monoclonal gammopathy

A

Xanthoma disseminatum

125
Q

Histopathology: Mixture of histiocytes, foam cells, inflammatory cells and later, foam cells and Touton giant cells with siderosis observed

A

Xanthoma disseminatum

126
Q

Histopathology: Shows an infiltrate of lipid-laden histiocytes intermingled with Touton giant cells and eosinophils surrounded by fibrosis

A

Erdheim-Chester disease

127
Q

Complications: Xanthoma disseminatum

A 
Conjunctival inflammation
Respiratory obstruction
Siezures
Diabetes insipidus
Growth retardation
128
Q

Clinical variants of Xanthoma disseminatum

A
  1. Self-healing form
  2. Persistent form
  3. Progressive form
129
Q

Associated with cutaneous and mucosal eruption with severe arthropathy and other visceral symptoms with papulonodular lesions (few mm to 2 cm) round, translucent and yellow-rose or yellow-brown grouping into plaques with cobblestone appearance that do not ulcerate preferential to fingers, palms and back of fingers arranged on the nail folds (coral beads)

A

Multicentric reticulohistiocytosis

130
Q

MRH that is characterized by a single firm rapidly growing nodule varying in color from yellow-brown to dark red most commonly on the head which may be preceded by trauma

A

Solitary cutaneous reticulohistiocytosis

131
Q

MRH that is purely cutaneous with eruption of form, smooth asymptomatic pinkish yellowish (early) to red-brown (older) papulonodular lesions 3-10 mm in diameter scattered diffusely

A

Diffuse cutaneous reticulohistiocytosis

132
Q

Initial sign of Multicentric reticulohistiocytosis

A

Severe chronic diffuse polyarthritis with arthralgia

133
Q

MRH characterized by familial occurrence and typical ocular involvement (glaucoma, cataract, uveitis)

A

Familial histiocytic dermatoarthritis

134
Q

Histopathology: Composed of histiocytes and lymphocytes then older lesions showing presence of numerous large mononucleated histiocytes with abundance of eosinophilic homogenous cytoplasm containing fine granules that has a ground-glass appearance

A

Multicentric reticulohistiocytosis

135
Q

Prognosis: Multicentric reticulohistiocytosis

A

Favorable for purely cutaneous forms (involute spontaneously)

136
Q

Associated with myeloma, arthropathy, hypertension, neuropathy, neoplastic syndrome, primary biliary cirrhosis and Graves’ disease

A

Necrobiotic xanthogranuloma

137
Q

Histopathology: Granulomatous infiltrate involving the whole dermis and subcutis composed of mixture of lymphocytes epithelioid cells, foam cells, and Touton giant cells with areas of severe clearly defined necrosis

A

Necrobiotic xanthogranuloma

138
Q

Complications: Necrobiotic xanthogranuloma

A 
Lagophthalmos
Conjunctivitis
Keratitis
Scleritis
Uveitis
Corneal ulceration
Loss of ocular function
139
Q

Prognosis: Necrobiotic xanthogranuloma

A

Chronic progressive course difficult to predict

140
Q

Presents with yellowish macules and patches reddish-borwn papules and plaques and nodules that may become ulcerated or eroded accompanied by fever

A

Sinus histiocytosis with massive lymphadenopathy

141
Q

Histopathology: Diffuse dermal infiltrate composed predominantly of histiocytes with large vesicular nuclei and abundant pale cytoplasm with emperipolesis (phagocytosis of leukocytes, lymphocytes)

A

Sinus histiocytosis with massive lymphadenopathy

142
Q

Prognosis: Sinus histiocytosis with massive lymphadenopathy

A

Benign course with spontaneous regression

143
Q

NLCH which require treatment

A

Xanthoma disseminatum
Erdheim-Chester disease
Multicentric reticulohistiocytosis
Necrobiotic xanthogranuloma

144
Q

Group of disorders characterized by an abnormal accumulation of mast cells

A

Mastocytosis

145
Q

Type III tyrosine kinase receptor product of proto-oncogene located on chromosome 4q12 expressed on mast cells and others

A

KIT (CD117)

146
Q

Cytokine that shares a number of signal transduction pathways wth SCF but minimal direct effect in human mast cell proliferation

147
Q

Cytokine that enhances mast cell function when added to matures cultures

148
Q

Cytokine that has been shown to increase mast cell mediator concentration

149
Q

Cytokine that increase the number of mast cell in culture

150
Q

Cytokine that inhibits mast cell proliferation and influcence mast cell phenotype and function

151
Q

Criteria: Systemic Mastocytosis

A

Major
-Multifocal dense infiltrates of mast cells in BM and/or other extracutaneous organs
Major
-More than 25% of the mast cells in BM aspirate smears or tissue biopsy sections are spindle shaped or display atypical morphology
-Detection of a codon 816 c-kit point mutation in blood, BM or lesional tissue
-Mast cell in BM, blood or other lesional tissue expressing CD25 or CD2
-Baseline total tryptase level greater than 20 ng/ml

152
Q

Mastocytosis unique to adults

A

Systemic mastocytosis with an associated clonal hematologic nonmast cell lineage disease

153
Q

Hematologic disorders associated with SM-AHNMD

A

Myeloproliferative disorders

Myelodysplastic disorders

154
Q

Patients with this type of Mastocytosis have impaired liver function, hypersplenism and malabsorption, have increasing mast cell numbers and are difficult to manage clinically

A

Aggressive systemic mastocytosis

155
Q

Mastocytosis that is characterized by multiorgan failure and BM smears mast cells represent greater than 20% of the nucleated cell population

A

Mast cell leukemia

156
Q

Mastocytosis that is rare, with locally destructive growth and with distant spread with mast cells that are highly atypical and immature

A

Mast cell sarcoma

157
Q

Most common skin manifestation of Cutaneous mastocytosis

A

Urticaria pigmentosa

158
Q

Appear as tan to brown papules or macules, from 1.0-2.5 that present at birth or arise during infancy on trunk sparing central facem scalp, palms and soles

A

Urticaria pigmentosa (children)

159
Q

Appear as reddish-brown macules and papules with variable hyperpigmentation and fine telangiectasias on trunk and proximal extremities

A

Urticaria pigmentosa (adults)

160
Q

Tan-brown nodules that occur in 10-35% of children on distal extremities with onset before 6 months of age with associated flushing and hypotension if with trauma

A

Mastocytoma

161
Q

Scratching or rubbing lesions of Cutaneous mastocytoma leading to urtication and erythema

A

Darier’s sign

162
Q

Mastocytosis that is seen almost exclusively in infants although it may persist into adult life

A

Diffuse cutaneous mastocytoma

163
Q

Rare and seen almost exclusively in adults appearing as telangeictatic macules with irregular borders

A

Telangiectasis macularis eruptiva perstans

164
Q

Mast cell mediators

A 
Preformed
-Histamine
-Heparin
-Tryptase
-Chymase
Newly synthesized
-Leukotrienes
-Prostagladins
Cytokines
-Stem cell factor
-TNF-a
-TGF-b
-IL-5
-IL-6
-GM-CSF
165
Q

T or F: Symptoms of mastocytosis can be exacerbated by exercise, heat or local trauma`

166
Q

Stains for detecting mast cells

A

Toluidine
Giemsa
CD117 (KIT)

167
Q

Elevated in patient with SM

A

a-tryptase

168
Q

Can be detected in both mastocytosis and anaphylactic reactions

A

b-tryptase

169
Q

Total (a and b) serum typtase levels

A

> 20 ng/ml - abnormal
20-75 ng/ml - evidence of SM
75 ng/ml - systemic involvement

170
Q

Major urinary metabolite of histamine

A

1,4-methylimidazole acetic acid

171
Q

Next most common urinary metabolite

A

Methyhistamine

172
Q

Treatment: Cutaneous mastocytosis (first line)

A

Emollients

H1 +/- H2 antihistamines

173
Q

Treatment: Cutaneous mastocytosis (second line)

A 
Topical glucocorticoids
Calcineurin inhibitors
Psoralen + UVA
PDL (TMEP)
Leukotriene antagonists
Oral cromolyn
174
Q

Treatment: Cutaneous mastocytosis (third line)

A

Intralesional corticosteroids
Surgical excision (mastocytoma)
Glucocorticoids

175
Q

Treatment: Noncutaneous mastocytosis - Gastrointestinal

A 
1st
H2 antihistamines
Cromolyn
2nd
PPI
Leukotriene antagonist
Anticholinergics
3rd
Glucocorticoids
176
Q

Treatment: Noncutaneous mastocytosis - Cardiovascular

A 
1st
H1 and H2 antihistamines
Subcutaneous epinephrine (anaphylaxis)
2nd
Glucocorticoids (prophylaxis)
177
Q

Treatment: Noncutaneous mastocytosis - Musculoskeletal

A 
1st
Calcium supplements
Vitamin D
2nd
Bisphosphonates
NSAIDs (with caution)
3rd
Local radiation
178
Q

Treatment: Noncutaneous mastocytosis - Hematologic

A

Systemic chemotherapy (appropriate)

179
Q

Characterized by vascular contraction (leading to slowing of blood flow), platelet adhesion, and activation and subsequent development of plug at site of injury

A

Primary hemostasis

180
Q

Promotes vascular integrity when primary hemostasis is not sufficient and when larger vessels are involved.

A

Secondary hemostasis

181
Q

Maintains by vasoconstriction in secondary hemostatis

A

Prostaglandins
Serotonin
Thromboxane

Dermatology (26 decks)

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