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WILLIAM'S HEMATOLOGY > 65 Eosinophils and Their Disorders > Flashcards

65 Eosinophils and Their Disorders Flashcards

1
Q

Most specific eosinophilopoietic growth factor

A

IL-5

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2
Q

Eosinophils persist in the circulation for ______ hours

A

25 hours

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3
Q

Granular proteins uniquely expressed by eosinophils

A

EPX and MBP-2

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4
Q

Majority of mature eosinophilic granules are

A

Secondary (specific) large granules

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5
Q

The smaller primary granules of eosinophils contain

A

Charcot-Leyden Crystal protein (Galectin-10)

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6
Q

Cell surface markers of eosinophils using flow cytometry

A

EMR-1, Siglec-8, and CCR3 (CD193)

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7
Q

In tissues, eosinophils can be detected by immunohistochemistry using

A

MBP, ECP, and EPX stains

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8
Q

Eosinophilia is defined as

A

Exceeding the upper limit of normal and an absolute eosinophil count (AEC) of 0.35–0.5 ×10 9 /L

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8
Q

Severity of eosinophilia

A

Mild (AEC <1.5 ×10 9 /L)
Moderate or marked (AEC 1.5–5 × 10 9 /L)
Severe or massive (AEC >5 × 10 9 /L)

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9
Q

Hypereosinophilia (HE) is defined as

A

Persistent eosinophilia greater than 1.5 × 10 9 /L

Divided into primary (clonal or neoplastic), secondary (reactive), hereditary (familial), or HE of undetermined significance

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10
Q

Hypereosinophilic syndrome (HES)

A

(a) a persistent eosinophilia of** greater than 1.5× 10 9 /L **for longer than **6 months **or death before 6 months associated with the signs and symptoms of hypereosinophilic disease

(b) a lack of evidence for parasitic, allergic, or other known causes of eosinophilia; and

(c) presumptive signs and symptoms of organ involvement

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11
Q

Idiopathic HE or HE of undetermined significance

A

Patients with an absolute eosinophil** greater than 1.5 × 10 9 /L **are asymptomatic with no end-organ damage

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12
Q

Gene rearrangement associated with myeloid/lymphoid neoplasms with eosinophilia

A

PDGFRA, PDGFRB, and fibroblast growth factor receptor (FGFR) 1, PCM1-JAK2

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13
Q

Incidence of FIP1L1–PDGFRA fusion with idiopathic HE or HES

A

10%

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14
Q

Most common clinical manifestations at presentation of HES

A

Skin (37%)
Lungs (25%)
GI tract (14%)

At table: Hematopoetic, heart, skin, nervous system

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15
Q

Rare HES-like hereditary immune deficiencies characterized by an elevated IgE level, eczema-like skin rashes, characteristic facial and dental abnormalities, and recurrent infections

A

Hyper IgE syndromes

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16
Q

Mutations in Hyper IgE syndromes

A

Autosomal dominant mutations- STAT3 (Job syndrome)

Autosomal recessive mutations- DOCK8, PGM3, SPINK5, or TYK2

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17
Q

An elevated serum vitamin B12 level is commonly seen in primary HES/CEL, NOS, caused by __________

A

Increased production of the haptocorrins

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18
Q

Useful screening test in patients with HE

A

Tryptase

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19
Q

Useful in the diagnosis of lymphocyte-variant HE/HES

A

Testing for clonal T lymphocytes by flow cytometry and polymerase chain reaction (PCR)/next-generation sequencing (NGS)

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20
Q

TRUE OR FALSE

Given its dire consequences, all patients with HE and HES should be screened for cardiac involvement with troponin (T or I) looking for myocarditis and an echocardiogram (+/- N-terminal pro B-type natriuretic peptide [NT-BNP]) to evaluate the presence of cardiomyopathy.

A

TRUE

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21
Q

In using FISH in the diagnosis of primary HES, this is a surrogate for FIP1L1-PDGFRA fusion gene

A

CHIC2 deletion

Interstitial submicroscopic microdeletion on the long arm of chromosome 4

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22
Q

Immunohistochemical staining to identify increased numbers of neoplastic mast cells

A

CD117, tryptase, and CD25

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23
Q

Translocations that warrants FISH testing for PDGFRA, PDGFRB, FGFR1, JAK2, or FLT3

A

4q12, 5q31~33, 8p11~12, 9p24, or 13q12

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24
Q

In patients with elevated tryptase, testing should be undertaken for________

A

KIT D816V mutation

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25
Q

Most common gene partner of:

PDGFRA: _________
PDGFRB: _________
FGFR1: __________
JAK2: _________
ABL1: _________
FLT3: _________

A

PDGFRA: FIP1L1
PDGFRB: ETV6
FGFR1: ZMYM2
JAK2: PCM1
ABL1: ETV6
FLT3: ETV6

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26
Q

There is a remarkable ________ predominance in patients with FIP1L1PDGFRA, PDGFRB, and JAK2 fusions for unknown reason

A

male predominance

27
Q

Treatment of choice for patients with PDGFRA and PDGFRB rearrangements

A

Imatinib

PDGFRA: starting dose of 100 mg/day with dose increase to 400 mg
PDGFRB: initially with 400 mg/day, and the dose is then lowered to 100 mg/day

28
Q

TRUE OR FALSE

Higher standard doses of imatinib being required for patients with PDGFRA and PDGFRB rearrangements than patients with CML

A

FALSE

LOWER standard doses of imatinib being required for patients with PDGFRA and PDGFRB rearrangements than patients with CML who present with chronic phase disease since in vitro, PDGFRA and PDGFRB rearrangements are MORE SENSITIVE to imatinib compared with BCR-ABL1

29
Q

Drug added to Imatinib to dampen the immune response and lessen the risk of myocardial injury from degranulation of eosinophils

A

Prophylactic glucocorticoids during the initial 1 to 2 weeks of imatinib therapy

30
Q

Most common mutation in patients with primary or secondary resistance to imatinib in patients with PDGFRA/PDGFRB rearrangements

A

T674I (most commonly) and D842V

31
Q

TKI with partial activity against the FGFR1 tyrosine kinase

A

Ponatinib

32
Q

FGFR1 inhibitor

A

Pemigatinib

33
Q

TRUE OR FALSE

AHSCT is recommended following TKI inhibition in PDGFRA and PDGFRB

A

FALSE

34
Q

This fusion gene is cryptic and not detected by routine cytogenetics unless alternatve gene partners present

A

FIP1L1

35
Q

Preferred or Potential TKI in JAK2

A

Ruxolitinib

36
Q

Preferred or Potential TKI in ABL1

A

Imatinib
Nilotinib
Dasatinib

37
Q

Preferred or Potential TKI in FLT3

A

Sorafenib
Sunitinib
Midostaurin
Second generation FLT3 inhibitors

38
Q

HE with concomitant evidence of a clonal cytogenetic or molecular abnormality

OR

An increase in myeloblast percentage in the marrow or blood (≥5% and 2%, respectively but <20% to exclude acute leukemia)

A

CHRONIC EOSINOPHILIC LEUKEMIA, NOT OTHERWISE SPECIFIED

39
Q

Treatment options for CEL, NOS

A

Hydroxyurea
Interferon (INF)-α
Imatinib
Allogeneic HSCT

40
Q

Flow immunophenotypic abnormalities in lymphocyte variant HE or HES

A

Absence of CD3 from the T-cell receptor complex (CD3 CD4+)

Double-negative immature T cells (CD3+ CD4– CD8–)

CD3+ CD4+ CD7–

41
Q

Patients with lymphocyte variant HE or HES are at a slightly increased risk of _______________

A

Lymphomas, mainly T-cell lymphoma and Sézary syndrome

42
Q

Treatment for lymphocyte variant HE or HES

A

Glucocorticoids

Glucocorticoid-sparing agent such as INF-α, cyclosporine, or anti–IL-5–directed therapy

43
Q

A humanized monoclonal IgG1 antibody that binds free IL-5

A

Mepolizumab

44
Q

A humanized afucosylated monoclonal antibody against the IL-5Rα chain

A

Benralizumab

45
Q

Mainstay of treatment for idiopathic HES

A

Glucocorticoids

46
Q

Mainstay of treatment for organ-restricted HES

A

Glucocorticoids

46
Q

Treatment for eosinophilic asthma

A

Mepolizumab (100 mg subcutaneously every 4 weeks)
Reslizumab (anti–IL-5 antibody dosed at 3 mg/kg intravenously every 4 weeks)
Benralizumab (30 mg subcutaneously every 4 weeks)

47
Q

Treatment for eosinophilic esophagitis

A

Reslizumab

48
Q

TRUE OR FALSE

Eosinophilia can be seen in patients with several WHO-defined myeloid and lymphoid neoplasms, but it is common to have eosinophil-mediated organ damage in these patients.

A

FALSE

Eosinophilia can be seen in patients with several WHO-defined myeloid and lymphoid neoplasms, but it is UNCOMMON to have eosinophil-mediated organ damage in these patients.

49
Q

Mutation in AML with increased marrow eosinophils

A

Core binding factor (CBF)

50
Q

In MDS patients, eosinophilia greater than ___________ predicts significantly reduced survival and marrow eosinophilia is associated with complex karyotype

A

0.35 × 10 9 /L

51
Q

TRUE OR FALSE

The presence of eosinophilia in MDS and SM carries prognostic significance

A

TRUE

The presence of eosinophilia in MDS and SM carries prognostic significance

52
Q

Rare disease characterized by cyclic eosinophilia, angioedema, fever, weight gain, polyclonal IgM, and aberrant CD3 - clonal T cells that occur every 3–4 weeks and self-resolves with spontaneous diuresis

A

Gleich syndrome

53
Q

Rare benign chronic inflammatory disease characterized by lymphadenopathy, elevated IgE levels, and eosinophilia

A

Kimura disease

54
Q

Toxic oil:________
Eosinophilia–myalgia syndromes: __________

A

Toxic oil:contaminated rapeseed oil
Eosinophilia–myalgia syndromes: tryptophan supplements

55
Q

Mature eosinophils are normally present in the gastrointestinal (GI) tract except in the

A

Esophagus

56
Q

Eosinophil’s granular contents

A

Proteins (eg, major basic proteins [MBP], eosinophil peroxidase [EPX], eosinophil cationic protein [ECP], and eosinophil derived neurotoxin)
Enzymes (eg, nonspecific esterase and catalase)
Inflammatory mediators/cytokines (eg, eotaxin, ILs, and leukotriene C4)

57
Q

TRUE OR FALSE

The workup of patients presenting with HE starts with excluding secondary (reactive) causes and screening for end-organ involvement

A

TRUE

The workup of patients presenting with HE starts with excluding secondary (reactive) causes and screening for end-organ involvement

58
Q

TRUE OR FALSE

Elevated immunoglobulin (Ig) E levels is a nonspecific finding that is mostly seen in reactive conditions (infectious, allergic, vasculitis, and lymphocytevariant HES)

A

TRUE

Elevated immunoglobulin (Ig) E levels is a nonspecific finding that is mostly seen in reactive conditions (infectious, allergic, vasculitis, and lymphocytevariant HES)

But its elevation is variable in patients with primary HES/CEL, NOS

59
Q

Patients with neurologic symptoms should undergo brain imaging (with or without cerebral angiography), with preference for

A

Magnetic resonance imaging

60
Q

Mixed lineage (eg, undifferentiated or biphenotypic leukemia) presentation is more common with ____________ fusions

A

FGFR1 fusions

61
Q

TRUE OR FALSE

The presence of T-cell receptor rearrangements without phenotypic abnormality on flow cytometry is not considered sufficient for the diagnosis of lymphocyte-variant HES and should be interpreted with caution

A

TRUE

The presence of T-cell receptor rearrangements without phenotypic abnormality on flow cytometry is not considered sufficient for the diagnosis of lymphocyte-variant HES and should be interpreted with caution

62
Q

TRUE OR FALSE

Patients with idiopathic HES who have marrow findings similar to CEL, NOS (discussed earlier) but do not meet its diagnostic criteria have a worse prognosis than those without such marrow abnormalities

A

TRUE

Patients with idiopathic HES who have marrow findings similar to CEL, NOS (discussed earlier) but do not meet its diagnostic criteria have a worse prognosis than those without such marrow abnormalities

63
Q

In patients presenting with eosinophilic fasciitis, evaluation of underlying_______ is recommended.

A

MDS

64
Q

TRUE OR FALSE

Acute and chronic graft-versus-host disease can present with eosinophilia and is probably a poor prognostic biomarker in these patients

A

FALSE

Acute and chronic graft-versus-host disease can present with eosinophilia and is probably a good prognostic biomarker in these patients

WILLIAM'S HEMATOLOGY (89 decks)

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