Dermatologic Manifestations of Hypereosinophilic Syndrome Treatment & Management

Updated: Aug 21, 2019
  • Author: Felix Urman, MD; Chief Editor: Dirk M Elston, MD  more...
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Treatment

Medical Care

For FIP1L1-PDGFRA fusion gene patients, imatinib is first-line therapy. [48] Imatinib's adverse effects and expense warrant consideration before using it. Specifically, imatinib, which blocks the effects of platelet-derived growth factor, has transformed the care of a large subset of patients with hypereosinophilic syndrome (HES) and can be helpful. It can lead to a sustained drop in the eosinophil count. However, because the causes of hypereosinophilic syndrome are variable, some patients might need other therapies.

Patients with increased IL-5 production due to clonally expanded T-cell population (lymphocytic variant) initially receive corticosteroids, followed by agents including hydroxycarbamide, interferon-alfa, and imatinib. [49, 50] Mepolizumab, an anti-IL-5 antibody, may be an effective corticosteroid-sparing agent for patients with lymphocytic hypereosinophilic syndrome. [2, 51] Other agents used to treat hypereosinophilic syndrome include alemtuzumab and reslizumumab. [52, 53, 54, 55]

The goal of therapy is to control organ damage, which, in many cases, especially those involving the heart, does not correlate with the level of hypereosinophilia. Thus, no therapy is necessary in asymptomatic disease without any evidence of organ damage. However, because cardiac damage can develop insidiously, patients need close clinical and echocardiographic follow-up care.

Previously, corticosteroids were the first line of therapy for hypereosinophilic syndrome. With the development of imatinib, while corticosteroids might be the optimal initial therapy, imatinib can be seen as the most potent and durable treatment for hypereosinophilic syndrome. The steroids should rapidly (usually within 4 h) decrease the eosinophil count. If steroids fail to reduce the eosinophil count, they may be discontinued. (Some patients have symptomatic improvement without changes in eosinophil counts.) Patients who respond to steroids (usually those with urticaria/angioedema and high IgE levels) usually have a good prognosis. A short trial of corticosteroids in patients who are asymptomatic may help predict the future response to therapy.

Chemotherapeutic agents (ie, hydroxyurea, vincristine, etoposide [VP16-213], chlorambucil) have been used with variable success in patients whose conditions were unresponsive to steroids. [56, 57, 58]

Experimental treatment with anti–IL-5 antibody SCH55700 and alemtuzumab has been reported to be effective. [59] Biologic response modifiers, such as interferon alfa and cyclosporine, have been used. [60]

In 2007, Taverna et al [61] noted that infliximab is a therapy for idiopathic hypereosinophilic syndrome.

Leukapheresis is sometimes used. It removes eosinophils from the blood. Leukapheresis results in short-lived reductions in eosinophil counts and is largely unsuccessful as a therapy modality. It can be used in emergency situations in patients with extremely high eosinophil counts.

Anticoagulants and antiplatelet agents are used in patients with evidence of thrombosis or thromboembolism because thromboembolic manifestations are often a part of hypereosinophilic syndrome and cause many of its worst symptoms. No data exist that show whether anticoagulation treatment has any benefit. The effectiveness of anticoagulation treatment is anecdotal because some patients continue to have thrombotic complications despite therapy. Many patients still form clots despite anticoagulation therapy.

Phototherapy with psoralen and UV-A (PUVA), dapsone (papulonodular lesions), and sodium chromoglycate have been used with anecdotal success for patients with pruritus. Narrow band UV-B phototherapy might be effective as well, but its use has not been described.

Antihistamines can be used, but they are only add-on therapies and not primary treatments. Antihistamines that have a good effect include cetirizine, hydroxyzine, and doxepin. Because doxepin can affect the heart, it should be used with caution in patients with hypereosinophilic syndrome. Sedating antihistamines, such as hydroxyzine and doxepin, can provide symptomatic relief.

Complications, such as CHF, should be treated aggressively.

Bone marrow transplantation after chemotherapy has rarely been used in severe cases of hypereosinophilic syndrome, but, because of the morbidity involved with this treatment, it should be used sparingly.

Long-term remission of hypereosinophilic syndrome has been reported following allogeneic stem cell transplantation in spite of transient eosinophilia posttransplant.

Prompt hospitalization and treatment of disease and therapy complications in hypereosinophilic syndrome are essential. If the patient has experienced cardiac or other systemic collapse, the patient must be transferred to the ICU. Because hypereosinophilic syndrome is usually a slowly progressing disease, transfer is not often necessary.

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Surgical Care

Splenectomy can reduce the pain due to splenic enlargement and is also beneficial in cases of thrombocytopenia secondary to hypersplenism. However, leukocyte and eosinophil counts can increase following splenectomy.

Cardiac surgery for annuloplasty, thrombectomy, and valve replacement has a definite role in treating heart disease due to hypereosinophilic syndrome. Bioprosthetic valves should be used because mechanical valves are more prone to thrombosis.

Rarely, in neurologic dysfunction, underlying edema of the brain and an increase in CSF pressure may be present. If these result, the patient must immediately undergo surgery by a neurosurgeon to insert a shunt or other means for normalizing CSF pressure to prevent herniation.

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Consultations

Hypereosinophilic syndrome is a multisystem disorder. It is often hard to diagnosis because its symptoms are not specific. Consultation from all medical specialties can be helpful in making the diagnosis. In particular, consultation with a cardiologist, a hematologist, and a dermatologist can be helpful.

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Prevention

At this time, no prevention of hypereosinophilic syndrome is known.

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Long-Term Monitoring

Careful clinical, laboratory, and imaging follow-up care is necessary to ensure that new disease symptoms and signs are identified and that appropriate therapy is promptly instituted.

Multidisciplinary interaction between a hematologist, a dermatologist, a cardiologist, a surgeon, and other subspecialists should be readily available when necessary.

Patients can have waxing and waning disease; thus, long-term treatment of this condition might be necessary.

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