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FD9 > 42 - Mastocytosis > Flashcards

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
Q

Darier sign is readily demonstrated in _____, but often less pronounced in adult skin lesions

A

Childhood UP and mastocytomas

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26
Q

Seen almost exclusively jn infants

A

Diffuse cutaneous mastocytosis

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27
Q

Dermographism with formation of hemorrhagic blisters can occur in the first months to years of life in children with

A

Diffuse cutaneous mastocytosis

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28
Q

Seen almost exclusively in adults; appears as telangiectatic macules and patches without hyperpigmentation

A

Telangiectasia macularis eruptiva perstans

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29
Q

Complaints of fever, night sweats, malaise, weight loss, bone pain, epigastric distress, and problems in mentation (cognitive disorganization) often signal the presence of

A

Systemic mastocytosis

30
Q

Extracutaneous disease is extremely uncommon in children, but when present _____ are the most frequent. _____ is the most common symptom

A

GI symptoms

Diarrhea

31
Q

Systemic mastocytosis with an associated clonal hematologic non-mast-cell-lineage disease occurs almost uniquely in

A

Adults

32
Q

Preformed mast cell mediators

A

Histamine
Heparin
Tryptase
Chymase

33
Q

Newly synthesized mast cell mediators

A

Leukotrienes

Prostaglandins

34
Q

Special stains for mast cells

A

Toluidine
Giemsa
Antitryptase
CD117

35
Q

_____-tryptase is elevated in patients with systemic mastocytosis

A

Alpha

36
Q

_____-tryptase can be detected in both mastocytosis patients and in allergic patients experiencing anaphylactic reactions

A

Beta

37
Q

_____-tryptase levels are most commonly measured and correlate with the extent of mast cell disease

A

Total alpha and beta serum

38
Q

Total serum tryptase levels of > _____ ng/mL are currently considered abnormal

A

20

39
Q

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

A

6-12 months

40
Q

Major urinary metabolite of histamine

A

1,4-methylimidazole acetic acid

41
Q

Next most common urinary histamine metabolite

A

Methylhistamine

42
Q

Foods high in histamine content

A

Spinach
Eggplant
Cheeses
Red wines

43
Q

Bones most commonly affected in SM

A 
Proximal long bones
Skull
Spine
Ribs
Pelvis
44
Q

More sensitive but less specific than routine radiographs

A

Skeletal scintigraphy (bone scan)

45
Q

Reasonable preliminary test for detecting bone involvement in mastocytosis

A

Radiographs of the skull, spine and pelvis

46
Q

Y/N: The bone marrow is often involved in patients with systemic mastocytosis

A

Yes

47
Q

A bone marrow biopsy in childhood-onset disease is not recommended unless there is

A

Evidence of systemic involvement and/or

Unexplained peripheral blood abnormalities

48
Q

Roentgenographic abnormalities of the GI tract fall into 3 major categories

A
  1. Peptic ulcers
  2. Abnormal mucosal patterns such as mucosal edema, multiple nodular lesions, coarsened mucosal folds, or multiple polyps
  3. Motility disturbances
49
Q

Initial evaluation of a prepubertal child with mastocytosis

A

Does not require extensive testing

50
Q

Presence of noncutaneous signs and symptoms or disease onset in adolescents or adults necessitates

A 
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
Q

Characterized by symptoms of mast cell mediator release but lack the evidence of unregulated mast cell proliferation

A

Nonclonal mast cell activation syndrome

52
Q

In patients with flushing, the diagnosis of _____ should be considered

A

Carcinoid tumor or pheochromocytoma

53
Q

Carcinoid tumor or pheochromocytoma excrete increased amounts of

A

5-hydroxyindoleacetic acid

54
Q

Patients with ISM who have evidence of hepatosplenomegaly, lymphadenopathy, and/or serum tryptase levels of > 200 ng/mL

A

Smoldering SM

55
Q

First line topical treatment for CM

A

Topical glucocorticoids

56
Q

Second line topical treatment for CM

A

Avoidance of triggering factors such as heat, friction, or drugs
H1 with or without H2 antihistamines

57
Q

Second-generation H1 antihistamines

A

Cetirizine
Loratadine
Fexofenadine

58
Q

Advantages of second-generation vs first-generation H1 antihistamines

A

Longer half-lives

More specific activity on the H1 receptor

59
Q

Tricyclic antidepressant with potent H1 activity

A

Doxepin

60
Q

Doxepin should be used with caution in older patients because it can cause

A

Cardiac QT interval prolongation

61
Q

Antihistamines with potential mast-cell stabilizing properties

A

Ketotifen

Azelastine

62
Q

H2 antihistamines

A

Cimetidine
Ranitidine
Famotidine
Nizatidine

63
Q

Appears to be the treatment of choice in patients with advanced mastocytosis

A

2-chlorodeoxyadenosine

64
Q

May limit the use of 2-chlorodeoxyadenosine

A

Myelosuppression

65
Q

First-generation tyrosine kinase inhibitor

A

Imatinib

66
Q

Second-generation tyrosine kinase inhibitors

A

Dasatinib

Nilotinib

67
Q

Multikinase inhibitor

A

Midostaurin

68
Q

Reported to cure a mastocytosis patient who expressed only normal (wild-type) c-kit; ineffective in patients expressing KIT D816

A

Imatinib

69
Q

Inhibit the in vitro growth of D816V-expressing mast cell lines

A

Dasatinib and nilotinib

70
Q

Inhibits both wild-type and mutated c-kit

A

Midostaurin

FD9 (108 decks)

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