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Hypereosinophilic SyndromeHypereosinophilic Syndrome – General InformationThe Hypereosinophilic Syndrome is categorized as a leukoproliferative disorder that manifests by an over-secretion of eosinophils cells, leading to damage of certain organs. The most common form of the disease is the peripheral eosinophilia. Usually, patients experience signs and symptoms depending on the organ that has been affected by the condition. The disease presents a form, also known as benign eosinophilia, that can cause no signs and symptoms at all, and the patient can carry the disease without knowing it. The disease appears rather as a collection of certain similar entities which tend to disseminate and damage the part of the body where they have the origin. There are cases in which the eosinophilia blood is associated with other diseases, including parasitic infections, eosinophilia-myalgia syndrome and Churg-Strauss syndrome. The difference between the ways of eosinophil secretion in Hypereosinophilic Syndrome and in the other cases is still unknown. There were some cases which have been diagnosed with the condition, which involved malignant transformation of the eosinophils. Unfortunately, most patients who suffer from this kind of disease do not present a subsequently identified neoplastic association that might help diagnosing the condition. Due to the similarities that exist between the condition’s cells and eosinophilic leukemia cells, doctors tend to treat the leukemia by administrating medicines that worsen the patient’s medical status. If the diseases are mistaken, the condition can progress, eventually to death. It is very important to treat the condition in an early stage to prevent any future complications.Hypereosinophilic Syndrome – SymptomsThe signs and symptoms of Hypereosinophilic Syndrome can appear simultaneously or individually, and can be sudden and dramatic. If the symptoms are left untreated they can progress to cardiac, neurologic and thrombotic complications, which eventually lead to death. The manifestations depend on the part of the patient’s body that has been affected and can appear as cardiac symptoms, developed in three stages: acute necrosis, thrombotic phase and endomyocardial fibrosis, and can appear as chest pain, dyspnea, or orthopnea; hematological symptoms which are not specific to the condition and can appear as fatigue, anemia, left upper quadrant pain, which if are left untreated can lead to the appearance of hyper-coagulability; neurologic symptoms which can appear as embolic and thrombotic strokes, encephalopathy, caused by the central nervous system dysfunction, blurred vision and slurred speech; pulmonary symptoms which can appear as eosinophilic infiltrates in the bases and periphery of the patient’s lungs, angioedema, a chronic, persistent cough, usually nonproductive, dyspnea, pulmonary fibrosis, bronchospasms and rhinitis; rheumatologic symptoms that can appear are arthalgias and myalgias or raynaud phenomenon; dermatologic symptoms can appear as skin rashes, pruritus, dermatographism and angioedema; gastrointestinal symptoms can appear as diarrhea, nausea, abdominal pain and small bowel necrosis due to microthrombi; constitutional symptoms can appear as fever, night sweats, anorexia, weight loss, which appear only if the cardiac tissue has been affected. It is very important to treat Hypereosinophilic Syndrome in an early stage to prevent any more severe adverse effects that appear in the future.Hypereosinophilic Syndrome – TreatmentThe main goal of any treatment against this medical disorder is to suppress the organ damage and eradicate Hypereosinophilic Syndrome. In most cases, the condition appears in a chronic form, with rare cases of resolution. Usually, the periods in which the condition manifests are followed by periods of partial improvement. There are cases in which the disease can also affect the cardiac muscle. When the condition causes cardiac damage, and appears in mild form, the patients are monitored closely without applying a treatment. The treat Hypereosinophilic Syndrome, doctors can prescribe certain medical therapies, which can consist in corticosteroids or other chemotherapeutic agent and biologic agents. There are cases in which the condition is present but there is no sign that may indicate the affections of any organ, and the first treatment option is a short term treatment trial with steroids, which can also be administered to maximize the effects of other therapies. Anticoagulation can also be used in some patients who present thrombotic and embolic complications, another cause of morbidity. This kind of procedure consists in administering anticoagulant and antiplatelet agents. However, in most cases it is ineffective, as some patients even continued to form clots. There are cases in which doctors use antihistamines such as hydroxyzine or doxepin, that are also used to control pruritus. Another way to treat Hypereosinophilic Syndrome is leukapheresis, a therapy that uses apheresis to remove the condition from the peripheral blood. This procedure is effective only for short term and in some cases, unsuccessful. If the patient presents prominent skin eruption and prutitus, the best way to have control over these symptoms is the use of psoralen and ultraviolet-A therapies. If the treatment is not effective, the best way to treat Hypereosinophilic Syndrome is the valve replacement or endocardectomy. Depending on the part of the body that has been affected by the disease, doctors can perform a splenectomy, also used to treat patients who experience severe pain or thrombocytopenia caused by hypersplenism. There are also some rare cases in which the patient can require bone marrow transplantation, but the toxicity of the transplantation procedure is risky because it can cause side effects. After the treatments are applied, no further diet or special modification in activity is needed. The main and most effective treatment option for Hypereosinophilic Syndrome is the use of corticosteroids. The most common medicine that is administered is prednisone, which is a prototypical medicine. The aim of the steroidal treatment is to slow the suppression of the eosinophils cells. The patients who respond to the treatment have increased curing rates. The way of responding to the treatment is the significant reduction in the number of eosinophils in the first 24 hours after administration. A high dose of corticosteroids can be toxic causing the disease to become chronic and hard to treat; most patients present an advanced form of the disease, which leads eventually to organ impairment. If the patient does not respond the steroidal treatment trial, doctors can recommend them to use certain chemotherapeutic or biologic agents. |
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