Eosinophilic Disorders Quiz

A condition where there is an abnormally high number of eosinophils in the blood, often causing tissue damage.

Fatigue, muscle aches, fever, itching, swelling, diarrhea, and cough.

The heart is a common target, leading to inflammation and dysfunction. This can cause heart failure, a serious condition.

Nerve problems can arise, affecting both the central and peripheral nervous systems. This can cause weakness, numbness, tingling, and even dementia in severe cases.

The lungs can be affected, leading to inflammation, scarring, and fluid buildup. This can cause shortness of breath and difficulty breathing.

The digestive system can be affected, causing diarrhea, stomach pain, and inflammation. This can lead to malnutrition and other complications.

The skin can be affected, causing itching, swelling, and rashes. This can be uncomfortable and even disfiguring.

A group of disorders characterized by the presence of a certain type of white blood cell called eosinophils in the blood.

Elevated levels of troponin in the blood, abnormal ECG findings, or abnormal echocardiogram suggest cardiac involvement and warrant further investigation with cardiac MRI.

A CT scan of the chest, abdomen, and pelvis is used to assess for enlarged spleen, enlarged lymph nodes, undetected lung involvement, and potential tumors.

A biopsy of affected tissues, if feasible, helps confirm the diagnosis, although patchy involvement might limit its usefulness.

Pulmonary function tests are essential for patients suspected of having lung involvement or showing abnormalities on chest CT.

A bone marrow aspirate and biopsy are crucial for patients with an absolute eosinophil count above 5.0 × 109/L or those exhibiting features suggestive of LHES or MHES, except for those with established diagnoses like EGPA or parasitic infection.

Testing for specific gene rearrangements and mutations, such as BCR::ABL1, FIP1L1::PDGFRA, and those involving PDGFRB, JAK2, FGFR1, and KIT, is guided by initial testing results and bone marrow examination.

NGS myeloid panel, whole-exome or targeted sequencing, and other genetic testing are used for further investigation depending on the initial evaluation.

A PET scan and EBV viral load measurement can be helpful in patients suspected of having LHES.

A type of HES with specific characteristics like male dominance, organ damage, and high serum tryptase. It's often linked to a gene mutation causing increased tyrosine kinase activity, making it responsive to imatinib.

A type of HES characterized by a large population of unusual T-cells that produce specific immune signals (IL-4, IL-5). It tends to affect the skin and can sometimes be asymptomatic.

Chromosome 4q12 deletion leads to a fusion gene (FIP1L1–PDGFRA) in myeloproliferative HES, resulting in enhanced tyrosine kinase activity.

The protein product of the FIP1L1–PDGFRA fusion gene exhibits increased tyrosine kinase activity, making it sensitive to low-dose imatinib therapy.

LHES is often diagnosed based on clinical presentation and identification of specific T-cell populations in the blood. In-depth testing is needed to confirm the diagnosis.

LHES can sometimes progress to a more serious blood cancer, highlighting the importance of ongoing monitoring.

LHES is a diagnosis made after ruling out other potential conditions, as the characteristics of the abnormal T-cells can resemble those seen in other blood cancers.

The surface characteristics of the abnormal T-cells in LHES can be similar to those in some blood cancers, requiring careful differentiation.

A heterogeneous group of rare disorders characterized by persistent high eosinophil counts and clinical manifestations ranging from fatigue to life-threatening organ damage.

An absolute eosinophil count (AEC) greater than or equal to 1.5 x 10^9/L.

A common occurrence, found in 1% to 2% of the general population.

A rare condition, affecting an estimated 0.315 to 6.3 per 100,000 individuals in the United States.

Disorders that cause mild to moderate eosinophilia.

Disorders that can cause a wide range of eosinophilia, from asymptomatic to life-threatening.

The most common cause of marked eosinophilia worldwide, often related to parasites that invade tissues.

Disorders where eosinophilia may or may not be present, and its role in the clinical manifestations is often unclear.

Disorders including leukemia, lymphoma, and solid tumors, particularly adenocarcinoma.

Disorders usually diagnosed in childhood, often associated with recurrent infections.

A diverse group of rare disorders involving hypereosinophilia, including idiopathic HES, lymphocytic variant HES, and familial HES.

Conditions like radiation exposure, hypoadrenalism (low cortisol), cholesterol emboli, and IL-2 administration.

An AEC > 1.5 x 10^9/L for longer than 6 months, with end-organ manifestations attributed to eosinophilia.

An AEC > 1.5 x 10^9/L on two separate occasions at least one month apart, with evidence of tissue eosinophilia and confirmed organ damage.

Includes clinical classifications like idiopathic (unknown cause), primary (clonal/neoplastic), secondary (cytokine driven), and hereditary.

The presence of high levels of eosinophils in the blood, often associated with allergic reactions, parasitic infections, or autoimmune diseases.

A treatment approach that aims to reduce elevated eosinophil levels in HES, often involving corticosteroids, targeted biologics, and other therapies.

A type of steroid medication used to reduce inflammation and suppress the immune system, often the initial treatment for acute HES.

Specialized medications that specifically target and reduce eosinophil levels in the blood and tissues, used in HES management.

The process of determining the most likely underlying cause of HES, important for guiding individual treatment strategies.

A clinical presentation of HES characterized by a rapid response to corticosteroid therapy, but potential long-term issues with toxicity and resistance.

A clinical manifestation of HES that involves severe and/or life-threatening symptoms, requiring immediate and aggressive intervention.

A specific subtype of HES where the primary abnormality lies in the T lymphocytes, a type of white blood cell. This subtype often presents with a specific pattern of CD markers on these cells.

A blood test that checks for the presence and number of different types of blood cells in the blood. It's helpful for diagnosing blood disorders.

A procedure where a sample of bone marrow is taken to examine the blood-forming cells. This helps diagnose various hematological disorders.

A type of medication that suppresses the immune system, often used to treat inflammatory conditions or autoimmune diseases.

A type of medication used to treat certain cancers and autoimmune diseases. It works by reducing the production of new blood cells.

A type of medication used to treat certain parasitic infections, including Strongyloides.

A type of medication used to treat a variety of parasitic infections, including Strongyloides.