40 - Eosinophilic Diseases Flashcards
1
Q
Eosinophils develop in the bone marrow from multipotential, stem cell-derived _____+ myeloid progenitor cells
A
CD34
2
Q
Stimulatory cytokines and growth factors for eosinophils
A
IL-3
GM-CSF
IL-5
3
Q
Most selective eosinophil-active cytokine, but is relatively late-acting
A
IL-5
4
Q
Mature eosinophils are slight (smaller/larger) than neutrophils
A
Larger
5
Q
Five highly basic proteins found within the eosinophil’s cystoplasmic granules
A
Major basic protien-1
Major basic protien-2
Eosinophil-derived neurotoxin/Ribonuclease 2
Eosinophil cationix protien/Ribonuclease 3
Eosinophil peroxidase
6
Q
Other types of the eosinophil’s cystoplasmic granules
A
Primary granules
Small granules
Secretory vesicles
7
Q
Characteristically found in asthmatic sputum and in feces from patients with helminth infections or eosinophilic gastroenteritis
A
Charcot-Leyden crystal protein or galectin-10
8
Q
Charcot-Leyden crystal protein can also be found in
A
Neutrophils
9
Q
Charcot-Leyden crystal protein is contained in
A
Primary granules
10
Q
More common in immature eosinophilic promyelocyes
A
Primary granules
11
Q
Contain acid phosphatase and arylsulfatase
A
Small granules
12
Q
Also referred to as tubulovesicular structures or microgranules, are characterized by their small, dumbbell-shaped appearance and their albumin content
A
Secretory vesicles
13
Q
Most abundant granules in number
A
Secretory vesicles
14
Q
Principal stores of arachidonic acid; aso contain the enzymes cyclooxygenase, 5- and 15-lipoxygenase
A
Lipid bodies
15
Q
Y/N: Eosinophils are present in blood permanently.
A
No - transiently, ranging from 8-18 hours
16
Q
Eosinophils comprise _____% or less of circulating leukocytes
A
6
17
Q
Eosinophils are primarily tissue-dwelling cells, with an average life span of
A
2-5 days; may reach up to 14 days
18
Q
Only tissues in which eosinophils normally reside
A
Bone marrow Spleen Lymph node Thymus Gastrointestinal tract from the stomach to the colon, sparing the esophagus
19
Q
Only organ other than the bine marrow in which extracellular eosinophil granule protein deposition is observed even under homeostatic conditions
A
Gastrointestinal tract
20
Q
Eosinophils and their granule proteins are found in the _____ in normal gastrointestinal tract and are not found in _____
A
Lamina propria
Peyer patches or epithelium
21
Q
The recruitment of eosinophils to the gastrointestinal, thymic, uterine, and mammary tissues is under the control of
A
CCL11
22
Q
Eosinophils can migrate to areas of tissue injury or necrosis through the _____ released from necrotic cells
A
High-mobility group box-1 protein (HGMB1)
23
Q
Y/N: Once eosinophils enter tissues, most do not recirculate
A
Yes
24
Q
Among the products of eosinophils that are most damaging to the host are the
A
Cationic proteins (EPO, ECO, EDN, MBP-1)
25
All 4 of the cationic proteins (EPO, ECP, EDN, MBP-1) likely contribute to the edema observed in skin diseases because of their
Vasodilatory effect
26
Eosiniphil granule proteins induces direct tissue damage to
Host cellls and microbes
27
Comprises the crystalloid core of the specific eosinophil granule
Major basic protein
28
_____, but none of the other eosinophil granule proteins, stimulate histamine and LTC4 release from human basophils
MBP-1 and MBP-2
29
Key participant in generating reactive oxidants and free radical species in activated eosinophils
Eosinophil peroxidase
30
Protein that is present in the highest molar concentration in eosinophil granules
MBP
31
By weight, is the most abundant protein constituting approximatelt 25% of the specific eosinophil granule’s total protein mass
EPO
32
Play a role in viral host defense to RNA viruses
ECP and EDN
33
Functions as an alarmin, alerting the adaptive immune system to preferentially enhance antigen-specific Th2 responses
EDN
34
Y/N: No single surface protein is uniquely expressed in eosinophils
Yes
35
Most important selectin pair in eosinophil migration into tissues
P-selectin and PSGL-1
36
Among the nonselective drugs for eosinophil reduction, _____ generally are very effective
Glucocorticoids
37
Y/N: Glucocorticoids cause an immediate reduction in circulating eosinophils.
Yes - within 3 hours
38
Mutation in myeloproliferative hypereosinophilic syndrome (chronic eosinophilic leukemia)
FIP1L1-PDGFRA
39
Approved for the treatment of chronic myelogenous leukemia and hypereosinophilic syndrome
Imatinib mesylate
40
Monoclonal antibody to CD52; used in the treatment of chronic (B-cell) lymphocytic leukemia and T-cell lymphoma
Alemtuzumab
41
Eosinophils, but not _____, also express CD52
Neutrophils
42
First humanized monoclonal antibody against IL-5
Mepolizumab
43
Anti-IL-5 receptor alpha humanized monoclonal antibody
Benralizumab
44
Y/N: Pathogenic influence of eosinophils may be unrelated to their numbers in tissues
Yes
45
Revised diagnostic criteria for hypereosinophilic syndromes: blood eosinophilia > _____ eosinophils/mm3 on at least 2 separate determinations or evidence of prominent tissue eosinophilia associated with symptoms and marked blood eosinophilia
1500
46
Patients with myeloproliferative hypereosinophilic syndrome frequently present with clinical features resembling those of
Chronic myelogenous leukemia
47
Although chromosomal abnormalities characterize this subtype of HES, the disease may evolve into definite leukemia
Myeloproliferative HES
48
Patients with _____ HES abnormal T-cell clones with unusual surface phenotypes
Lymphocytic
49
An 800-kilobase deletion on chromosome band 4q12 that codes for a tyrosine kinase has been found in _____ HES
Myeloproliferative
50
The FIP1L1-PDGFRA gene is detected in
Mast cells
Eosinophils
Neutrophils
Mononuclear cells
51
Myeloproliferative HES with documented mutations is known as
Chronic eosinophilic leukemia
52
Increased serum levels of IgE are often present in _____ HES
Lymphocytic
53
Levels of vitamin B12 and tryptase may be increased in _____ HES
Myeloproliferative
54
Other than in _____, vasculitis only rarely is associated with HES
Eosinophilic granulomatosis with polyangitis
55
Along with eosinophilia, _____ commonly are found in helminthic infections
Total serum IgE levels higher than 500 IU/mL
56
In patients with isolated urticarial plaques with or without angioedema, the differential diagnosis includes common and persistent urticaria, but demonstration of _____ supports HES
Multiorgan involvement
57
HES with episodic angioedema may resemble hereditary angioedema clinically, although patients with hereditary angioedema have a family history of the disease and rarely have the _____ that characterize HES
Markedly elevated eosinophil counts
58
Major cause of death in HES
Congestive heart failure from restrictive cardiomyopathy
| Followed by sepsis
59
Corticostetoids are considered the first-line therapy in HES patients without the gene mutation, once _____ has been excluded
Strongyloides infection
60
Myeloproliferative HES is responsive to
Imatinib
61
Adverse effect of imatinib
Endomyocardial disease may worsen during the first several days of treatment
62
Should be monitored before and during imatinib therapy
Tropinin levels
63
To improve cardiac function, _____ should be given before and with initiation of imatinib therapy
Glucocorticoids
64
Individual lesions gradually change from bright red to brown-red and then blue-gray or greenish-gray, resembling morphea
Wells syndrome
65
Most frequent systemic complain in Wells syndrome
Malaise
66
A nonspecific hypersensitivity reaction in response to exogenous and/or endogenous stimuli is regarded as the most important pathomechanism in
Wells syndrome
67
Characterized by diffuse dermal infiltration with eosinophils, histiocytes, and foci of amorphous and/or granular material associated with connective tissue fibers
Wells syndrome
68
Y/N: Flame figured is diagnostic of Wells syndrome
No - characteristic for, but not diagnostic of
69
Hallmark of Wells syndrome
Flame figures
70
Prognosis of Wells syndrome
Resolves without scarring, usually within weeks to months, but multiple recurrences are common
71
Angiolymphoid hyperplasia with eosinophilia shows a predilection for the
Head and neck area, including the ears
72
ALHE has been reported to occur in _____, which implies thst sex hormones may be a factor in its development
Pregnancy
73
Also has developed in patients with T-cell clonality, which suggets that it may be an early or low-grade T-cell lymphoma
ALHE
74
Well-defined area in the dermis and/or subcutis of prominent vascular proliferation with large epithelioid or histiocytoid endothelial cells that contain abundant eosinophilic cytoplasm, often with cytoplasmic vacuoles
ALHE
75
The stroma of ALHE is typically
Myxoid
76
KD vs ALHE: lesions
ALHE - smaller, more superficial, more numerous, often asymptomatic
77
Peripheral blood eosinophilia and increase IgE levels in (KD/ALHE)
KD
78
Dominant histologic feature is lymphoid proliferation, often with germinal centers with prominent infiltration of eosinophils. Fibrosis is characteristic, and eosinophil abscesses may occur
KD
79
Represent the dominant characteristic of KD
Lymphoid follicles
80
KD vs ALHE: nephropathy
KD - present in up to 20% of patients
82
ALHE course
Chronic and nonremitting
83
KD treatment of choice
Surgical excision
84
Variants of eosinophilic pustular folliculitis
1. Classical EPF
2. Immunosuppresion-associated EPF
3. Infantile EPF
85
Immunosuppression EPF may be subdivided into
1. HIV-associated type
| 2. Malignancy-associated type
86
Presents as recurrent crops or clusters of follicular papules and pustules, which form an annular pattern and usually resolve in 7-10 days; predominantly on the face and trunk
Classical EPF
87
Infantile type of EPF typically are located on the
Scalp
88
Extremely pruritic discrete follicular papules, typically involving thr head and neck and often the proximal extremities
HIV-associated EPF
89
Although patients with classical EPF usually have eosinophilia with leukocytosis, HIV-positive patients often exhibit
Eosinophilia with lymphopenia
90
Elevated serum IgE is uncommon in _____ EPF
Infantile
91
Infiltration of eosinophils into hair follicles and perifollicular areas, sometimes with follicular damage
EPF
92
EPF prognosis based on type
Infantile - good
| Classical and HIV-associated - recurrences
93
First approach to the treatment of all types of EPF
Topical glucocorticoids
Topical calcineurin inhibitors
Also NSAIDs, particularly indomethacin
94
Papuloerythroderma of Ofuji frequently occur in
Elderly males, especially in the eighth or ninth decade
95
Itchy erythroderma-like eruptions formed by the coalescence of flat-topped, red-to-brown papules with a cobblestone appearance
Papuloerythroderma of Ofuji
96
Papuloerythroderma of Ofuji affects the
Limbs and trunk
97
Sparing of the abdominal skin folds
Deck-chair sign
98
Y/N: Papuloerythroderma of Ofuji affects the mucous membranes, hair, and nails
No - Mucous membranes, hair, and nails are always spared
99
Approximately 20% of cases of papuloerythroderma of Ofuji are associated with
Hematologic neoplasms or
| Visceral malignancies
100
Deck-chair sign is observed in
Papuloerythroderma of Ofuji
| Other erythrodermas, such as psoriasis and atopic dermatitis
101
Histopathology of papuloerythroderma of Ofuji
Nonspecific spongiotic dermatitis-like patterns
102
Papuloerythroderma of Ofuji course
Chronic and non-self-limiting
103
Y/N: Papuloerythroderma of Ofuji is frequently refractory to treatment
Yes
104
Asymptomatic red, brown, or violaceous plaques that are soft, smooth, and well circumscribed, often showing follicular s accentuation and telangiectasia
Granuloma faciale
105
Can be considered a localized chronic fibrosing vasculitis
Granuloma faciale
106
Normal-appearing epidermis, narrow grenz zone, dense and diffuse infiltrate of lymphocytes, plasma cells, eosinophils, and neutrophils with evidence of leukocytoclasis
Granuloma faciale
107
Primary histologic differential diagnosis of granuloma faciale
Erythema elevatum diutinum
108
Granuloma faciale vs EED: histology
Grenz zone is not typical of EED
Eosinophils and plasma cells are more prominent in granuloma faciale
Neutrophils are more frequently found in EED
109
Granuloma faciale course
Chronic and rarely resolves spontaneously
110
Depression along the course of the superficial vein that is more marked upon the elevation of the affected limb
Groove sign
111
Groove sign is a characteristic finding of
Eosinophilic fasciitis
112
Historically related to ingestion of certain lots of L-tryptophan
Eosinophilia-myalgia syndrome
113
Dermoscopic finding of ALHE
Polymorphous vascular pattern
