Eosinophilia Clinical Manifestations Quiz

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Questions and Answers

Which clinical manifestation is primarily associated with cardiac disease mortality?

Dilated cardiomyopathy (correct)

Mononeuritis multiplex

Pulmonary infiltrates

Gastrointestinal colitis

What is the role of eosinophil mediators in tissue damage?

They facilitate the spread of infection to other tissues.

They are responsible for improving tissue repair.

They act systemically to affect multiple organs.

They cause local tissue damage in areas infiltrated by eosinophils. (correct)

Which classification is associated with an unknown cause and exclusion of other subtypes?

Lymphocytic variant HE/HES

Idiopathic HE/HES (correct)

Familial HE/HES

Myeloid HE/HES

Which gastrointestinal manifestation may result from eosinophilia?

Hepatitis Signup and view all the answers

What is a common neurologic complication associated with eosinophilia?

Encephalopathy Signup and view all the answers

Which of the following best describes the Lymphocytic variant HE/HES?

Presence of a clonal or aberrant T-cell population Signup and view all the answers

What condition is most likely to present with diarrhea as a symptom of gastrointestinal involvement?

Budd-Chiari syndrome Signup and view all the answers

What additional condition can lead to eosinophilia as an associated factor?

Helminth infection Signup and view all the answers

What is the primary reason for assessing patients with LHES for occult lymphoma at diagnosis?

To identify clonal T-cell populations Signup and view all the answers

Which test is indicative of potential myeloid neoplasms in patients undergoing diagnostic workup?

Serum tryptase levels Signup and view all the answers

What does a complete blood count with differential analyze in the context of HE?

Dysplastic eosinophils and myeloid precursors Signup and view all the answers

Why are IgE levels typically elevated in various conditions?

They are involved in the body's allergic response Signup and view all the answers

What laboratory finding can indicate end organ involvement in patients with HE?

Routine chemistries and liver function tests Signup and view all the answers

What is a characteristic finding of LHES in lymphocyte phenotyping?

Clonal and/or aberrant T-cell populations Signup and view all the answers

In which scenario should quantitative serum immunoglobulin levels be particularly evaluated?

In patients suspected of having eosinophilia Signup and view all the answers

What suggests a diagnosis of lymphoid neoplasm during the diagnostic workup?

Clonal B cells found in testing Signup and view all the answers

Which imaging test should be considered if abnormalities are found in electrocardiogram or echocardiogram?

Cardiac MRI Signup and view all the answers

What is the purpose of performing a chest/abdomen/pelvis CT?

Assess for splenomegaly and asymptomatic pulmonary involvement Signup and view all the answers

Which patients are required to undergo a bone marrow aspirate and biopsy?

Patients with AEC >5.0 × 10 /L or suggestive features Signup and view all the answers

Which characteristic is NOT associated with the myeloproliferative variant of hypereosinophilic syndrome (HES)?

Increased T-cell populations Signup and view all the answers

In which scenario would testing for FIP1L1::PDGFRA translocation be primarily indicated?

For all patients with elevated serum tryptase and/or B12 levels Signup and view all the answers

What could limit the utility of a cardiac biopsy in certain patients?

Patchy involvement of cardiac tissue Signup and view all the answers

What genetic alteration is most commonly associated with the myeloproliferative variant of HES?

FIP1L1–PDGFRA fusion gene Signup and view all the answers

Which tests are suggested based on the initial evaluation of patients suspected of having LHES?

Combination of NGS myeloid panel and EBV viral load Signup and view all the answers

How is the lymphocytic variant of HES primarily diagnosed?

Identification of a clonal T-cell population Signup and view all the answers

What percentage of patients with lymphocytic variant HES progresses to lymphoid malignancy?

10% Signup and view all the answers

Which of the following genetic testing methods is not typically associated with the evaluation of eosinophilia?

Standard chromosomal karyotyping Signup and view all the answers

What condition is indicated if a patient has an AEC greater than 5.0 × 10 /L but presents with clear historical illness?

Suggests chronic eosinophilic leukemia Signup and view all the answers

Which cytokines are primarily produced by T-cell populations in lymphocytic variant HES?

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

What clinical feature is most typical for lymphocytic variant HES?

Dermatologic manifestations Signup and view all the answers

What is a major challenge in the diagnosis of lymphocytic variant HES?

Indistinguishable surface phenotype from T-cell malignancies Signup and view all the answers

Which test is commonly used to confirm the diagnosis of lymphocytic variant HES?

Intracellular flow cytometry Signup and view all the answers

What was the initial treatment given to the patient for presumed idiopathic thrombocytopenic purpura?

Prednisone Signup and view all the answers

Which of the following laboratory findings was notably elevated during the evaluation of the patient?

IgE Signup and view all the answers

What was the result of FIP1L1::PDGFRA testing in this patient?

Negative Signup and view all the answers

What type of therapy was initiated to manage the patient's eosinophilia after referral to the National Institutes of Health?

Interferon α Signup and view all the answers

Which examination revealed symmetric nonpitting edema of the thighs and excoriations on the lower legs?

Physical examination Signup and view all the answers

What abnormal T-cell population was identified through flow cytometry?

CD3–CD4+CD10+ T cells Signup and view all the answers

Which of the following was NOT a noted symptom at the time of the patient's referral?

Fever Signup and view all the answers

What did the computed tomography (CT) scan reveal about the patient's condition?

Borderline splenomegaly and minimal diffuse lymphadenopathy Signup and view all the answers

What is the primary therapeutic approach for severe and/or life-threatening manifestations of Hypereosinophilia Syndrome (HES)?

Corticosteroid administration Signup and view all the answers

In patients with eosinophilia, what factor should be assessed to determine the urgency of therapeutic intervention?

The presence and severity of clinical manifestations Signup and view all the answers

Which second-line agent is considered for treating eosinophilia when corticosteroid therapy is inadequate?

Cyclophosphamide Signup and view all the answers

What underlying condition is suspected in a patient with eosinophilia who is treated with imatinib?

Myeloid neoplasm Signup and view all the answers

What is a notable limitation of corticosteroid therapy for most patients with symptomatic HES?

Long-term toxicity and resistance Signup and view all the answers

Which of the following symptoms would likely require immediate reconsideration of treatment options for a patient with eosinophilia?

Persistent pruritus or angioedema Signup and view all the answers

What therapy remains controversial for eosinophilia in the acute setting?

Eosinophil-targeting biologics Signup and view all the answers

Which diagnostic screening is critical in evaluating a patient after a year of unexplained eosinophilia symptoms?

Complete blood count Signup and view all the answers

Study Notes

Approach to the Patient with Suspected Hypereosinophilic Syndrome

Hypereosinophilic syndromes (HES) are a heterogeneous group of rare disorders.
Clinical manifestations range from fatigue to life-threatening endomyocardial fibrosis and thromboembolic events.
Eosinophilia, defined as an absolute eosinophil count (AEC) >0.45 x 10⁹/L, is relatively common, occurring in 1% to 2% of the general population.
Hypereosinophilia (HE; 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

Atopic disorders: Asthma, atopic dermatitis, chronic rhinosinusitis, and others.
Drug hypersensitivity: Various drug reactions, eosinophilia-myalgia syndrome.
Infection and infestation: Helminth infections (especially tissue invasive), fungal infections, viral infections (HIV, COVID-19), ectoparasite infestations, and others.
Autoimmune and immunodysregulatory disorders: Inflammatory bowel disease, sarcoidosis, IgG4-related disease, and others.

Classification of Hypereosinophilic Syndromes

Myeloid HE/HES: Suspected or proven eosinophilic myloid neoplasm including those associated with rearrangements of PDGFRA and other recurrent molecular abnormalities.
Lymphocytic variant HE/HES: Presence of a clonal or phenotypically aberrant T-cell population that produces cytokines that drive the eosinophilia.
Overlap HES: Single-organ-restricted eosinophilic disorders and clinically defined eosinophilic syndromes overlapping with idiopathic HES, eosinophilic gastrointestinal disorders, and eosinophilic granulomatosis with polyangiitis.
Associated HE/HES: Occurs in the context of a defined disorder such as helminth infection, neoplasm, immunodeficiency, or hypersensitivity reaction.
Familial HE/HES: Occurrence in more than one family member, excluding associated HE/HES.
Idiopathic HE/HES: Unknown cause and exclusion of other subtypes.

Clinical Manifestations

Constitutional symptoms such as fatigue, muscle aches, fevers, pruritus, angioedema, diarrhea, and cough.
Cardiac diseases are a major cause of mortality in HES, possibly involving endomyocardial fibrosis, pericarditis, myocarditis, and intramural thrombus formation.
CNS involvement can lead to mononeuritis multiplex, paraparesis, encephalopathy, and dementia.
Pulmonary involvement can manifest as pulmonary infiltrates, fibrosis, or pleural disease with effusions.
Gastrointestinal issues such as diarrhea, gastritis, colitis, hepatitis, and Budd-Chiari syndrome are possible.
Skin manifestations such as pruritus, angioedema, papules, or plaques.
Kidney and bone involvement is rare.
Tissue damage in eosinophilia is believed to be secondary to eosinophil degranulation & release of mediators (e.g., eosinophil cationic protein, major basic protein).

Myeloproliferative variant HES

Characterized by male predominance.
Associated with organ damage, elevated serum tryptase, splenomegaly, anemia, thrombocytopenia, bone marrow myeloproliferation, and reticulin fibrosis.
Unresponsive to steroid therapy.
Often associated with an interstitial deletion on chromosome 4q12, causing a FIP1L1-PDGFRA fusion gene leading to a tyrosine kinase with enhanced activity and sensitivity to low-dose imatinib therapy.

Lymphocytic variant HES (LHES)

Characterized by a large CD2⁺ CD3⁻ CD4⁺ T-cell population that produces interleukin (IL)-4 and IL-5 in response to stimulation.
Equally frequent in males and females.
Often presents with dermatologic manifestations.
Any organ system is potentially involved.
Some asymptomatic patients have clonal aberrant T-cell populations indistinguishable from those with symptomatic LHES.
Serum IgM, IgE, and serum and thymus and activation-regulated chemokine levels are elevated.
Diagnosis is based on identification of a clonal and/or aberrant T-cell population.

Diagnostic Workup and Testing

Essential tests for all suspected hypereosinophilia cases, including a detailed history and physical exam to rule out other causes & to identify any potential travel or environmental exposures.
Initial tests include complete blood counts (CBC with differential), routine chemistries (including liver function tests), and quantitative serum immunoglobulin levels.
Further tests may include serum tryptase and B12 levels, & T- and B-cell receptor rearrangement studies.
Flow cytometry is important for lymphocyte phenotyping.
Serum troponin, ECG, and echocardiogram are useful.
Consideration for chest/abdomen/pelvis CT scans.
Possible tissue biopsy.
Consideration of bone marrow aspirate and biopsy.
Pulmonary function tests are needed in patients with suspected pulmonary involvement.

Approach to therapy and clinical case examples

Secondary causes (e.g., helminthic infection) need specific therapies.
Corticosteroids are the first-line treatment for acute severe HES, but additional therapies might be required if the initial response isn't adequate.
Conventional second-line therapies include hydroxyurea and interferon α.
Eosinophil-targeting biologics (e.g. mepolizumab, reslizumab) may be used, but their use in acute settings is controversial.
Prednisone is a primary treatment in acute and severe cases, and for life-threatening situations.

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Description

Test your knowledge on the clinical manifestations associated with eosinophilia and its implications in various diseases. This quiz covers key concepts regarding cardiac disease mortality, gastrointestinal symptoms, and the relationship of eosinophils with tissue damage. Perfect for students and professionals in medicine and immunology.