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42 - Mastocytosis Flashcards

1
Q

Most cases of mastocytosis arise in

A

Children

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2
Q

Mast cells arise from the bone marrow as granular _____+ pluripotent progenitor cells

A

CD34

KIT (CD117)

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3
Q

_____, a type III tyrosine kinase is the product of the protooncogene c-kit located in chromosome 4q12

A

KIT

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4
Q

KIT is expressed on mast cells, as well as

A

Melanocytes
Primitive hematopoietic stem cells
Primordial germ cells
Interstitial cells of Cajal

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5
Q

Ligand of KIT

A

Stem cell factor

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6
Q

Crosslinking of KIT by SCF is essential for

A

Mast cell maturation

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7
Q

Other cytokines that are jmportant in regulating mast cell growth and differentiation

A 
IL-3
IL-4
IL-6
IL-9
IFN-gamma
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8
Q

Mutations in _____ appear to play a central role in mastocytosis

A

c-kit

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9
Q

Mutations in this codon are most common in adults with mastocytosis

A

816

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10
Q

Classification of mastocytosis

A 
Cutaneous mastocytosis 
Indolent systemic mastocytosis 
Systemic mastocytosis with an associated clonal hematologic non-mast-cell-lineage
Aggressive systemic mastocytosis
Mast cell leukemia
Mast cell sarcoma
Extracutaneous mastocytoma
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11
Q

Represent the majority of patients with mastocytosis

A

Cutaneous mastocytosis

Indolent systemic mastocytosis

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12
Q

Most children have cutaneous mastocytosis, which is manifested as

A

Urticaria pigmentosa

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13
Q

Systemic mastocytosis occurs mostly in adults, and _____ is the most common form

A

Indolent systemic mastocytosis

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14
Q

Frequently have evidence of impaired liver function, hypersplenism, and/or malabsorption, but do not have a distinctive hematologic disorder or mast cell leukemia; have rapidly increasing mast cell numbers and are difficult to manage medically

A

Aggressive systemic mastocytosis

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15
Q

Characterized by multiorgan failure and bone marrow smears demonstrating a greater than 20% mast cell population

A

Mast cell leukemia

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16
Q

Tumors with highly atypical and immature mast cells; have localized destructive growth

A

Mast cell sarcomas

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17
Q

Usually localized to the lung and consist of mature mast cells

A

Extracutaneous mastocytomas

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18
Q

Urticaria pigmentosa frequently appear on the

A

Trunk

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19
Q

Urticaria pigmentosa lesions in children vs adults

A

Children - 1-2.5 cm

Adults - 5 mm or less (much smaller)

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20
Q

Adult UP skin lesions are most numerous on the

A

Trunk and proximal extremities

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21
Q

Y/N: Skin involvement is more common in patients with more advanced forms of systemic mastocytosis (SM-AHNMD, ASM, or MCL)

A

No - less common

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22
Q

Solitary mastocytomas frequently appear on the

A

Distal extremities

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23
Q

Onset of solitary mastocytomas generally before

A

6 months of age

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24
Q

Scratching or rubbing skin lesions leads to urtication and erythema

A

Darier sign

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25
Darier sign is readily demonstrated in _____, but often less pronounced in adult skin lesions
Childhood UP and mastocytomas
26
Seen almost exclusively jn infants
Diffuse cutaneous mastocytosis
27
Dermographism with formation of hemorrhagic blisters can occur in the first months to years of life in children with
Diffuse cutaneous mastocytosis
28
Seen almost exclusively in adults; appears as telangiectatic macules and patches without hyperpigmentation
Telangiectasia macularis eruptiva perstans
29
Complaints of fever, night sweats, malaise, weight loss, bone pain, epigastric distress, and problems in mentation (cognitive disorganization) often signal the presence of
Systemic mastocytosis
30
Extracutaneous disease is extremely uncommon in children, but when present _____ are the most frequent. _____ is the most common symptom
GI symptoms | Diarrhea
31
Systemic mastocytosis with an associated clonal hematologic non-mast-cell-lineage disease occurs almost uniquely in
Adults
32
Preformed mast cell mediators
Histamine Heparin Tryptase Chymase
33
Newly synthesized mast cell mediators
Leukotrienes | Prostaglandins
34
Special stains for mast cells
Toluidine Giemsa Antitryptase CD117
35
_____-tryptase is elevated in patients with systemic mastocytosis
Alpha
36
_____-tryptase can be detected in both mastocytosis patients and in allergic patients experiencing anaphylactic reactions
Beta
37
_____-tryptase levels are most commonly measured and correlate with the extent of mast cell disease
Total alpha and beta serum
38
Total serum tryptase levels of > _____ ng/mL are currently considered abnormal
20
39
Total serum tryptase levels also can provide an estimate of a patient’s overall mast cel burden, and thus serial measurements in adults every _____ may prove useful in following disease progression
6-12 months
40
Major urinary metabolite of histamine
1,4-methylimidazole acetic acid
41
Next most common urinary histamine metabolite
Methylhistamine
42
Foods high in histamine content
Spinach Eggplant Cheeses Red wines
43
Bones most commonly affected in SM
``` Proximal long bones Skull Spine Ribs Pelvis ```
44
More sensitive but less specific than routine radiographs
Skeletal scintigraphy (bone scan)
45
Reasonable preliminary test for detecting bone involvement in mastocytosis
Radiographs of the skull, spine and pelvis
46
Y/N: The bone marrow is often involved in patients with systemic mastocytosis
Yes
47
A bone marrow biopsy in childhood-onset disease is not recommended unless there is
Evidence of systemic involvement and/or | Unexplained peripheral blood abnormalities
48
Roentgenographic abnormalities of the GI tract fall into 3 major categories
1. Peptic ulcers 2. Abnormal mucosal patterns such as mucosal edema, multiple nodular lesions, coarsened mucosal folds, or multiple polyps 3. Motility disturbances
49
Initial evaluation of a prepubertal child with mastocytosis
Does not require extensive testing
50
Presence of noncutaneous signs and symptoms or disease onset in adolescents or adults necessitates
``` CBC with differential Liver function test Total serum tryptase level Skeletal radiographs Bone marrow biopsies (recommended by some clinicians for all adult mastocytosis patients) ```
51
Characterized by symptoms of mast cell mediator release but lack the evidence of unregulated mast cell proliferation
Nonclonal mast cell activation syndrome
52
In patients with flushing, the diagnosis of _____ should be considered
Carcinoid tumor or pheochromocytoma
53
Carcinoid tumor or pheochromocytoma excrete increased amounts of
5-hydroxyindoleacetic acid
54
Patients with ISM who have evidence of hepatosplenomegaly, lymphadenopathy, and/or serum tryptase levels of > 200 ng/mL
Smoldering SM
55
First line topical treatment for CM
Topical glucocorticoids
56
Second line topical treatment for CM
Avoidance of triggering factors such as heat, friction, or drugs H1 with or without H2 antihistamines
57
Second-generation H1 antihistamines
Cetirizine Loratadine Fexofenadine
58
Advantages of second-generation vs first-generation H1 antihistamines
Longer half-lives | More specific activity on the H1 receptor
59
Tricyclic antidepressant with potent H1 activity
Doxepin
60
Doxepin should be used with caution in older patients because it can cause
Cardiac QT interval prolongation
61
Antihistamines with potential mast-cell stabilizing properties
Ketotifen | Azelastine
62
H2 antihistamines
Cimetidine Ranitidine Famotidine Nizatidine
63
Appears to be the treatment of choice in patients with advanced mastocytosis
2-chlorodeoxyadenosine
64
May limit the use of 2-chlorodeoxyadenosine
Myelosuppression
65
First-generation tyrosine kinase inhibitor
Imatinib
66
Second-generation tyrosine kinase inhibitors
Dasatinib | Nilotinib
67
Multikinase inhibitor
Midostaurin
68
Reported to cure a mastocytosis patient who expressed only normal (wild-type) c-kit; ineffective in patients expressing KIT D816
Imatinib
69
Inhibit the in vitro growth of D816V-expressing mast cell lines
Dasatinib and nilotinib
70
Inhibits both wild-type and mutated c-kit
Midostaurin

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