Eosinophilic Disorders Quiz

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Questions and Answers

Which of the following is NOT a constitutional symptom associated with the clinical manifestation discussed?

Diarrhoea (correct)

Muscle aches

Fever

Fatigue

What is the primary cause of mortality in patients with the condition described?

Acute renal failure

Septic shock

Pulmonary infiltrates

Dilated cardiomyopathy (correct)

Which of the following complications can arise from the involvement of the central and peripheral nervous systems?

Dysphagia

Myopathy

Paraparesis (correct)

Hypertension

Identifying the tissue damage, which mediators are primarily responsible for local damage caused by eosinophils?

Eosinophil cationic protein and major basic protein Signup and view all the answers

Which type of HE/HES involves clinically defined eosinophilic syndromes that overlap with idiopathic forms?

Overlap HES Signup and view all the answers

In which subtype of HE/HES is a clonal or phenotypically aberrant T-cell population involved?

Lymphocytic variant HE/HES Signup and view all the answers

Which gastrointestinal manifestation is NOT typically associated with eosinophilia?

Dyspepsia Signup and view all the answers

What characterizes familial HE/HES?

Occurrence in more than one family member Signup and view all the answers

What characterizes the myeloproliferative variant of hypereosinophilic syndrome (HES)?

Presence of interstitial deletion on chromosome 4q12 Signup and view all the answers

Which statement about the lymphocytic variant of hypereosinophilic syndrome (LHES) is true?

It presents equally in males and females Signup and view all the answers

What is a gold standard for the diagnosis of LHES?

Demonstration of a clonal and/or aberrant population of T-cells Signup and view all the answers

What is a potential outcome for patients with LHES over time?

Progression to a lymphoid malignancy Signup and view all the answers

What is the typical response of myeloproliferative variant HES to steroid therapy?

No response at all Signup and view all the answers

Which cytokines are primarily produced by T-cells in LHES?

IL-4 and IL-5 Signup and view all the answers

What additional finding may assist in diagnosing LHES?

Elevated serum and thymus activation-regulated chemokines Signup and view all the answers

What type of malignancies must LHES be distinguished from?

Angioimmunoblastic T-cell lymphoma Signup and view all the answers

What is the primary reason for considering cardiac MRI in selected patients?

To identify potential cardiac tissue involvement Signup and view all the answers

Which patient group is specifically recommended to undergo bone marrow aspirate and biopsy?

Patients with absolute eosinophil count (AEC) greater than 5.0 × 10^9/L Signup and view all the answers

In the context of elevated serum tryptase and/or B12 levels, which genetic testing should be prioritized?

FIP1L1::PDGFRA testing Signup and view all the answers

What is the role of pulmonary function tests in the evaluation process?

To evaluate for pulmonary involvement in suspected cases Signup and view all the answers

Why might cardiac biopsy have limited utility in diagnosing cardiac tissue involvement?

Cardiac tissue involvement can be patchy Signup and view all the answers

For patients with suspected LHES, which additional tests may be warranted?

PET scan and EBV viral load assessment Signup and view all the answers

What should guide the testing for BCR::ABL1 and other genetic translocations or mutations?

Initial testing results and bone marrow examination findings Signup and view all the answers

What additional causes should be tested for if secondary causes are suspected?

As indicated by clinical history and physical examination Signup and view all the answers

What is the primary therapeutic approach for life-threatening manifestations of hypereosinophilia syndrome (HES)?

Prednisone Signup and view all the answers

In which scenario should additional therapy be considered within 24 to 48 hours of treatment?

When there are signs of a myeloid neoplasm Signup and view all the answers

What is the role of eosinophil-targeting biologics in acute cases of HES?

Their use is generally discouraged. Signup and view all the answers

Which secondary cause of eosinophilia should be considered early in the diagnostic process?

Helminth infection Signup and view all the answers

What should further evaluation focus on after a patient's status stabilizes?

Identifying the clinical subtype of hypereosinophilia Signup and view all the answers

When considering therapy for symptomatic HES, which factor most limits the long-term use of corticosteroids?

The development of treatment resistance Signup and view all the answers

Which laboratory finding was revealed after the complete blood count in the clinical case discussed?

Eosinophilia, anemia, thrombocytopenia Signup and view all the answers

What was the initial ineffective treatment approach given for the patient's pruritus and swelling?

Antibiotics for presumed folliculitis Signup and view all the answers

What condition was the patient initially treated for with prednisone?

Idiopathic thrombocytopenic purpura Signup and view all the answers

What was the outcome of the serologic test for Strongyloides?

Indeterminate Signup and view all the answers

Which of the following therapies was initiated after hydroxyurea therapy was ineffective?

Interferon α Signup and view all the answers

What laboratory finding indicated an atypical T-cell population in the patient?

Clonal pattern of T-cell receptor testing Signup and view all the answers

What was the peak absolute eosinophil count (AEC) recorded during the patient's treatment?

26.0 x 10^9/L Signup and view all the answers

What notable finding was observed on the CT scan during the patient's referral?

Borderline splenomegaly Signup and view all the answers

What was the patient's platelet count at the time of referral?

114,000 Signup and view all the answers

What symptom did the patient report that was primarily concerning at the time of referral?

Extreme pruritus Signup and view all the answers

What is the minimum absolute eosinophil count (AEC) that distinguishes hypereosinophilia from normal levels?

1.5 × 10^9 / L Signup and view all the answers

Which of the following disorders is most commonly associated with marked eosinophilia?

Asthma Signup and view all the answers

In the context of hypereosinophilic syndrome, which of the following would not typically be classified as an end-organ manifestation?

Asymptomatic eosinophilia Signup and view all the answers

What is the general incidence rate of hypereosinophilia in the general population?

0.315 to 6.3 per 100,000 Signup and view all the answers

Which type of eosinophilia would be more challenging to diagnose due to its overlapping symptoms with other disorders?

Idiopathic hypereosinophilia Signup and view all the answers

Which condition is associated with eosinophilia but typically does not result in marked eosinophilia?

Atopic dermatitis Signup and view all the answers

Which type of examination is required as part of the WHO definition for diagnosing hypereosinophilic syndrome?

Tissue examination Signup and view all the answers

What aspect does the consensus definition of hypereosinophilic syndrome emphasize in its criteria?

Organ damage and exclusion of other conditions Signup and view all the answers

Which of the following conditions is least likely to cause hypereosinophilia?

Bacterial pneumonia Signup and view all the answers

Which category of disorders includes eosinophilia due to infections, notable for its worldwide occurrence?

Infection and infestation Signup and view all the answers

The definition of hypereosinophilic syndrome states that eosinophilia must persist for how long?

At least 6 months Signup and view all the answers

Which of the following is considered an example of an inborn error of immunity?

Omenn syndrome Signup and view all the answers

What is the primary underlying cause of drug hypersensitivity-induced eosinophilia?

Immune system activation Signup and view all the answers

Which of the following drug reactions is least commonly associated with eosinophilia?

Photosensitivity reaction Signup and view all the answers

Study Notes

Approach to the Patient with Suspected Hypereosinophilic Syndrome

Hypereosinophilic syndromes (HES) are a diverse group of rare disorders with various clinical presentations, ranging from fatigue to life-threatening conditions like endomyocardial fibrosis and thromboembolic events.

Hypereosinophilic Syndromes

HES are a heterogeneous group of rare disorders characterized by an abnormally high number of eosinophils in the blood.
Clinical manifestations can range from mild fatigue to serious conditions like life-threatening endomyocardial fibrosis and thromboembolic events.

Eosinophilia

Eosinophilia, a higher-than-normal absolute eosinophil count (AEC), is relatively common, occurring in approximately 1% to 2% of the general population.
Hypereosinophilia (HE), a significantly higher AEC (≥ 1.5 x 10⁹/L), is rare, with an estimated incidence of 0.315 to 6.3 per 100,000 in the United States.

Disorders Associated with Marked Eosinophilia

A range of conditions can be associated with significant eosinophilia, including atopic disorders (e.g., asthma), drug hypersensitivity reactions, infections (e.g., helminth infections, fungal infections, viral infections like HIV and COVID-19, ectoparasites, and protozoal infections), autoimmune and immunodysregulatory disorders (e.g., inflammatory bowel disease, sarcoidosis, IgG4-related disease), and neoplasms (e.g., leukemia, lymphoma).

Classification of HES

Myeloid HE/HES: Involves suspected or confirmed eosinophilic myeloid neoplasms, often associated with gene rearrangements (e.g., PDGFRA).
Lymphocytic variant HE/HES: Characterized by a clonal or phenotypically aberrant T-cell population producing cytokines that drive eosinophilia.
Overlap HES: Single-organ restricted eosinophilic disorders that clinically overlap with idiopathic HES, featuring conditions like eosinophilic gastrointestinal disorders and eosinophilic granulomatosis with polyangiitis.
Associated HE/HES: Eosinophilia occurring in the context of a defined disorder, such as helminth infection, neoplasm, immunodeficiency, or hypersensitivity.
Familial HE/HES: Conditions related to a genetic predisposition occurring in multiple family members, excluding associated HE/HES.
Idiopathic HE/HES: These syndromes have no known cause, excluding other subtypes.

Clinical Manifestations

Patients with HES can present with constitutional symptoms (fatigue, muscle aches, fever).
Skin involvement can include pruritus, angioedema, papules, or plaques; and rarely, kidneys or bones, may be affected.
Gastrointestinal involvement may manifest as diarrhea, gastritis, colitis, and/or hepatitis, or Budd-Chiari syndrome.
The nervous system can be affected, leading to mononeuritis multiplex, paraparesis, encephalopathy, and potentially dementia.
Pulmonary involvement features pulmonary infiltrates, fibrosis or pleural disease; and cardiac manifestations can lead to endomyocardial fibrosis, myocarditis, pericarditis, and possible death due to dilated cardiomyopathy.

Tissue Damage in Eosinophilia

Tissue damage in eosinophilia is believed to be secondary to eosinophil degranulation and release of mediators, such as eosinophil cationic protein and major basic protein.
These mediators primarily act locally in eosinophil-infiltrated tissues to cause damage.
Increased serum tryptase may suggest a role for other cells (mast cells) in some instances of clonal eosinophilia.

Diagnosis of HES

Comprehensive evaluation: A complete medical history, physical exam, complete blood count, routine chemistries, and quantitative serum immunoglobulin levels are conducted.
Serum tryptase and B12 levels: Evaluating these levels can provide further information depending on the context.
Further specialized testing: T-and B-cell receptor rearrangement studies, flow cytometry, serum troponin, electrocardiogram, echocardiogram, and chest/abdomen/pelvis CT scans help to assess for various conditions.
Bone marrow aspirate and biopsy: Useful in suspected cases to identify certain conditions.
Pulmonary function tests, and testing for BCR, ABL1, FIP1L1:PDGFRA, potentially NGS myeloid panel, genetic testing, and other related tests depending on the suspected condition.
Assessment of additional criteria: Detailed evaluation that includes consideration for family history of eosinophilia, preliminary evaluations, and clinical classifications for a more precise diagnosis.

Approach to Therapy

The correct therapeutic approach for suspected or confirmed HES depends heavily on the suspected underlying cause. Secondary conditions like infections often require different therapies and should be identified early in the diagnostic process. Severity and nature of associated symptoms should also inform the urgency and specifics of any treatment interventions.
Prednisone is commonly used for severe or life-threatening manifestations.
Additional therapies may include specific targeted treatments based on suspected underlying causes (i.e., imatinib for myeloid neoplasm) and/or conventional second-line agents depending on initial evaluation and suspected etiology.

Eosinophil-Targeting Biologics

Eosinophil-targeting biologics are specialized therapies designed to reduce eosinophil levels in the blood and tissues, but their use in an acute setting can be controversial depending on the criteria for use.

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Description

Test your knowledge on eosinophilic disorders, including constitutional symptoms, complications, and associated syndromes. This quiz covers key aspects of health conditions related to eosinophilia and their impact on gastrointestinal and nervous systems. Dive into the specifics of HE/HES and the underlying mechanisms involved.