eMedicine Specialties > Pediatrics: General Medicine > Allergy & Immunology
Hypereosinophilic Syndrome: Treatment & Medication
Updated: Aug 15, 2008
- Overview
- Differential Diagnoses & Workup
- Treatment & Medication
- Follow-up
Treatment
Medical Care
- No therapy is indicated in absence of organ damage.
- Angioedema and urticaria suggest a benign course.
- Mucosal ulcers do not respond to corticosteroids.
- Rapid intervention for cardiac disease is essential.
- Reducing the eosinophil load is the major goal of treatment. Although seemingly important, aggressive control of eosinophilia is hypothetical because all reports about treatment approaches are essentially anecdotal for this rare disorder. In the absence of organ disease, however, any indication for treatment is unclear, except with respect to thrombosis risk.
- Therapy to prevent the risk of thrombosis may be reasonable for all patients.
Surgical Care
- Cardiac surgery is indicated for annuloplasty, valve replacement, thrombectomy, and aortic prostheses.
- Because patients with mechanical valve replacements are especially prone to thrombosis, bioprostheses are recommended.
- Splenectomy may ameliorate platelet sequestration and is indicated for splenic infarction and pain due to splenic distention.
Consultations
- Obtaining subspecialty input from a rheumatologist and hematologist is essential.
Medication
No therapy is indicated in the absence of organ damage. Treatment is directed at organ system involvement and at reducing the eosinophil load and perhaps the eosinophil effect.
Corticosteroids
These agents interfere with eosinophilopoiesis by antagonizing IL-5, IL-3, and granulocyte/monocyte cell–stimulating factor. They also suppress eosinophilia; however, discontinue corticosteroids if eosinophilia is not suppressed. Response to steroids is considered a good prognostic indicator.
Prednisone (Deltasone, Meticorten, Orasone, Sterapred)
Immunosuppressant for treatment of autoimmune disorders; may decrease inflammation by reversing increased capillary permeability and suppressing PMN activity. Stabilizes lysosomal membranes and suppresses lymphocytes and antibody production.
Adult
60 mg/d PO; eventually taper or change to alternate-day regimen
Pediatric
1 mg/kg PO qd; eventually taper or change to alternate-day regimen
Coadministration with estrogens may decrease clearance; concurrent use with digoxin may cause digitalis toxicity secondary to hypokalemia; phenobarbital, phenytoin, and rifampin may increase glucocorticoid metabolism (consider increasing maintenance dose); monitor for hypokalemia with coadministration of diuretics
Documented hypersensitivity; active infection; ocular herpes simplex; chickenpox, measles, or live virus exposure
Pregnancy
B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals
Precautions
May mask infections; abrupt discontinuation of glucocorticoids when used >2 wk may cause adrenal crisis; may cause hyperglycemia, edema, osteonecrosis, myopathy, peptic ulcer disease, hypokalemia, osteoporosis, euphoria, psychosis, myasthenia gravis, growth suppression, and infections
Immunosuppressant and immunomodulator agents
These drugs are used to inhibit DNA synthesis, but only case reports of their effectiveness are available.
Hydroxyurea (Hydrea)
Interferes with DNA synthesis. Used to reduce total leukocyte count to <10,000/µL. Requires 7-14 d for effectiveness.
Adult
1-3 g/d PO, continued as long as no significant reduction in platelet count occurs
Pediatric
20-30 mg/kg/d PO
Potentiates pancreatitis with antiretroviral medications; coadministration with fluorouracil can increase neurotoxicity
Documented hypersensitivity; bone marrow depression, leukopenia <2500/µL, or thrombocytopenia <100,000/µL; pancreatitis
Pregnancy
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
Closely monitor blood counts at least weekly; severe anemia requires resolution before initiating therapy; renal failure requires dose adjustment; erythema occurs if individual has received radiation therapy in past; produces anemia, which often requires blood transfusion, and thrombocytopenia, which occasionally requires platelet transfusion
Vincristine (Oncovin)
Used to reduce total leukocyte count to <10,000/µL. Effective in 1-3 d and spares bone marrow toxicity but may cause paresthesias.
Adult
1.5-2 mg IV as a single dose at 2-wk intervals
Pediatric
<10 kg: 0.05 mg/kg IV
>10 kg: 1.5-2 mg/m2 IV
Not to exceed 2 mg/dose
Acute pulmonary reaction may occur when taken concurrently with mitomycin-C; asparaginase, CYP3A4 inhibitors (eg, itraconazole, quinupristin/dalfopristin, sertraline, ritonavir), GM-CSF (eg, sargramostim, filgrastim), or nifedipine increase toxicity; CYP3A4 inducers (eg, carbamazepine, phenytoin, phenobarbital, rifampin) may decrease effects
Documented hypersensitivity; IT administration (may be fatal); bone marrow depression; cytopenia; demyelinating form of Charcot-Marie-Tooth syndrome
Pregnancy
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
Ensure vascular access intact because extravasation produces severe tissue damage; if severe tissue damage occurs, injection of hyaluronidase and application of heat helps disperse drug and reduce damage; do not inject directly but only through IV access line established as nonleaking; obtain CBC count before each dose; does not cross blood-brain barrier, but do not administer intrathecally; avoid eye contamination; hydrate patient to avoid uric acid nephropathy; if uric acid elevations are severe, consider allopurinol prophylaxis; if bilirubin >3 mg/dL, reduce dose by 50%; may cause paresthesias
Cyclophosphamide (Cytoxan)
Used to reduce total leukocyte count to <10,000/µL.
Adult
50-125 mg/d PO
Pediatric
10-15 mg/kg IV q7-10d; alternatively, 3-5 mg/kg IV twice weekly or 1-5 mg/kg/d PO
Allopurinol may increase risk of bleeding or infection and enhance myelosuppressive effects; may potentiate doxorubicin-induced cardiotoxicity; may reduce digoxin serum levels and antimicrobial effects of quinolones
Chloramphenicol may increase half-life while decreasing metabolite concentrations; may increase effect of anticoagulants; coadministration with high doses of phenobarbital may increase rate of metabolism and leukopenic activity; thiazide diuretics may prolong cyclophosphamide-induced leukopenia and neuromuscular blockade by inhibiting cholinesterase activity
Bone marrow depression, cytopenia
Pregnancy
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
Monitor blood counts and perform urinalyses weekly; complicates general anesthesia; toxicity increased in adrenalectomized (possibly also steroid-treated) patients; may compromise wound healing; treatment >90 d increases likelihood of sterility induction
Busulfan (Myleran)
Used to reduce total leukocyte count to <10,000/µL.
Adult
4-8 mg/d PO
Pediatric
60 mcg/kg/d PO or 1.8 mg/m2/d PO
Thioguanine increases toxicity; acetaminophen, phenytoin, or itraconazole may decrease clearance; high doses of cyclophosphamide and busulfan may cause cardiac tamponade
Bone marrow depression, cytopenia
Pregnancy
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
Requires extreme vigilance in monitoring; requires weekly blood counts because WBC count may actually increase in first 10-15 d of treatment, do not alter dose for apparent inadequate response; patient must promptly report fever, sore throat, local infection, bleeding, any anemia-indicative symptom, breathing difficulties, or weakness
Methotrexate (Rheumatrex)
Used to reduce total leukocyte count to <10,000/µL.
Adult
30 mg PO 2-3 times/wk
Pediatric
Not established; perhaps, 30 mg/m2 PO qwk
Intestinal absorption reduced by tetracycline, chlorambucil, nonabsorbable broad-spectrum antibiotics; hepatotoxicity increased by etretinate or other retinoids; may decrease theophylline clearance; charcoal lowers levels; coadministration with etretinate may increase hepatotoxicity; response may be decreased by folic acid or its derivatives contained in some vitamins
Probenecid, NSAIDs, salicylates, procarbazine, and sulfonamides, including TMP-SMZ, can increase plasma levels; may decrease phenytoin plasma levels; may increase plasma levels of thiopurines; fatal reactions reported when administered concurrently with NSAIDs
Documented hypersensitivity; alcoholism; hepatic insufficiency; documented immunodeficiency syndromes; preexisting blood dyscrasias (eg, bone marrow hypoplasia, leukopenia, thrombocytopenia, significant anemia); renal insufficiency
Pregnancy
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
Monitor blood counts closely; elimination reduced in impaired renal function, ascites, or pleural effusion; liver disease may preclude use; decisions about folic acid replacement must be individualized
Chlorambucil (Leukeran)
Used to reduce total leukocyte count to <10,000/µL.
Adult
4-10 mg/m2/d PO for 4 consecutive days every other mo
Pediatric
0.1-0.2 mg/kg/d PO; use for minimal time because of risk of secondary malignancies
None reported
Blood dyscrasias, thrombocytopenia, leukopenia, severe anemia
Pregnancy
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
Requires weekly blood counts; may cause chromosome damage and sterility; do not administer within 4 wk of radiation therapy; induces secondary malignancies; caution in history of seizure disorders or current bone marrow suppression; total doses >6.5 mg/kg increases risk of irreversible bone marrow damage
Etoposide (VP16-213, VePesid)
Podophyllotoxin derivative that acts as topoisomerase II inhibitor to cause DNA damage.
Adult
50-100 mg/m2/d PO on days 1, 3, and 5
Pediatric
Administer as in adults
May prolong the effects of warfarin and increase methotrexate clearance; cyclosporine and etoposide have additive effects in the cytotoxicity of tumor cells
Documented hypersensitivity; IT administration may cause death; blood dyscrasias, thrombocytopenia, leukopenia, severe anemia
Pregnancy
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
Closely monitor blood counts before each cycle; withhold therapy platelet count <50,000/µL or absolute neutrophil count <500/µL; administer as slow infusions over 30-60 min; anaphylactic reactions can occur; reduce dose in presence of renal insufficiency; do not use acrylic or acrylonitrile/butadiene/styrene (ABS) plastics in the infusions
Interferon alfa 2a or 2b (Roferon-A, Intron-A)
Empirically applied to many diseases as immunomodulator. Acts at biologically active sites in eosinophil action.
Adult
8 million U/d IM/SC initially, then 2 million U/d or 5-7 million U 3 times per wk
Pediatric
Not established; consider 2.5-5 million U/m2/d IM/SC; deaths reported with doses of 30 million U/m2/d
Reduces theophylline clearance
Documented hypersensitivity
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Assess CBC counts before therapy; check preservative and biologic source to ensure patient is not allergic; brands not interchangeable; avoid tasks requiring mental alertness after high dose therapy; warn patient that depression and suicidal ideation are adverse effects; can cause flulike symptoms; caution in severe hepatic or renal insufficiencies, seizure disorders, multiple sclerosis, or compromised CNS
Cyclosporine (Sandimmune, Neoral)
Inhibits T-cell clonal release of eosinophilopoietin cytokines.
Adult
2.5 mg/kg/d PO; not to exceed 10 mg/kg/d; toxicity problematic at high dose
Pediatric
Not established; possibly 2.5 mg/kg/d PO; not to exceed 10 mg/kg/d; toxicity problematic at high dose
NSAIDs or grapefruit juice increase levels; octreotide may decrease levels by interfering with bioavailability; increases digoxin, methotrexate, and diclofenac levels; coadministration with other nephrotoxic drugs (eg, amphotericin B, ketoconazole, tacrolimus, cimetidine, ranitidine, gentamicin, tobramycin, vancomycin, trimethoprim, sulfamethoxazole, NSAIDs) may potentiate renal dysfunction; CYP3A4 inhibitors (eg, diltiazem, nicardipine, verapamil, fluconazole, itraconazole, ketoconazole, clarithromycin, erythromycin, methylprednisolone, allopurinol, bromocriptine, danazol, metoclopramide, indinavir, nelfinavir, ritonavir, saquinavir) may increase levels; CYP3A4 inducers (eg, rifampin, carbamazepine, phenobarbital, phenytoin, ticlopidine) may decrease levels
Live attenuated virus vaccines may not result in protective immunization when administered with cyclosporine or within 3-12 mo following discontinuation of cyclosporine
Documented hypersensitivity; uncontrolled hypertension or malignancies; do not administer concomitantly with PUVA or UVB radiation in psoriasis because may increase risk of cancer
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Monitor BUN, creatinine, and serum bilirubin levels, BP, and LFT results q2wk for first 3 mo and monthly thereafter; reduce dose by 25% if hypertension occurs; may increase risk of infection and lymphoma; reserve IV use for only patients who cannot take drug PO
Sorafenib (Nexavar)
Multikinase inhibitor that targets serine/threonine and tyrosine receptor kinases in both the tumor cell and the tumor vasculature. Targets kinases involved in tumor cell proliferation and angiogenesis, thereby decreasing tumor cell proliferation. These kinases include RAF kinase, VEGFR-2, VEGFR-3, PDGFR-beta, KIT, and FLT-3.
Adult
400 mg PO bid 1 h ac or 2 h pc
Pediatric
Not established
CYP450 2B6 and 2C8 inhibitor; predominantly eliminated by UGT1A1 pathway (caution when coadministered with other drugs eliminated by UGT1A1 [eg, irinotecan]); coadministration with warfarin may increase INR or bleeding
Documented hypersensitivity
Pregnancy
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
Common adverse reactions include hand or foot skin reaction and rash (modify dose); may increase risk of hemorrhage, cardiac ischemia and/or infarction, alopecia, pruritus, or diarrhea; caution with severe hepatic impairment (ie, Child-Pugh C)
Imatinib mesylate (Gleevec)
Specifically designed to inhibit tyrosine kinase activity of the bcr-abl kinase in Ph+ leukemic chronic myeloid leukemia (CML) cell lines. Well absorbed after PO administration, with maximum concentrations achieved within 2-4 hours. Elimination is primarily in feces in form of metabolites. FDA-approved for chronic hypereosinophilic syndrome in adults. Also indicated to treat pediatric patients with Ph+ chronic phase CML whose disease has recurred after stem cell transplant, or have demonstrated interferon alpha resistance.
Adult
100-400 mg PO qd with food
Pediatric
Up to 260 mg/m2/d PO with food
CYP3A4 inhibitors (eg, ketoconazole, itraconazole, erythromycin, clarithromycin) increase imatinib distribution; CYP3A4 substrates (eg, simvastatin) increases imatinib maximum concentration by a factor of 2-3.5-fold; CYP3A4 inducers (eg, phenytoin, dexamethasone, carbamazepine, rifampin, phenobarbital, St. John's Wort) decrease imatinib AUC by approximately one-fifth of typical AUC; likely to increase blood levels of drugs that are substrates of CYP2C9, CYP2D6, and CYP3A4/5 (eg, benzodiazepines, dihydropyridine calcium channel blockers, HMG-CoA reductase inhibitors, warfarin)
Documented hypersensitivity
Pregnancy
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
Dose must be reduced or interrupted if edema or anemia occur, transaminases or bilirubin become elevated, or grade 3-4 neutropenia or thrombocytopenia develop; Stevens Johnson syndrome has been reported; pediatric patient commonly experience musculoskeletal pain; may cause/exacerbate CHF or left ventricular dysfunction, monitor patients with preexisting cardiac disease); complete blood counts and liver function testing are advised weekly for 1 month, biweekly for a month and then every 2-3 months; do not breast feed
Anti-inflammatory agents
Dapsone is a sulfone antimicrobial. Its anti-inflammatory action, which enables its use for dermatologic conditions, is not fully understood but does not appear to be associated with its antibacterial action.
Dapsone (Avlosulfon)
Sulfone specifically useful for skin involvement.
Adult
50-300 mg/d PO
Pediatric
Not established; consider 0.8-4 mg/kg/d PO; not to exceed 100 mg/d
May inhibit anti-inflammatory effects of clofazimine; hematologic reactions may increase with folic acid antagonists (eg, pyrimethamine); monitor for agranulocytosis during the second and third months of therapy; probenecid increases toxicity; coadministration with trimethoprim may increase toxicity of both drugs; because of increased in renal clearance, levels may significantly decrease when administered concurrently with rifampin
Documented hypersensitivity; G-6-PD or methemoglobin reductase deficiencies; hemoglobin M or hemolysis-inducing conditions
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Perform weekly CBC counts for first month; then perform WBC counts monthly for 6 mo; then semiannually; discontinue if significant reduction in platelets, leukocytes, or hematopoiesis observed
Caution in methemoglobin reductase deficiency, G-6-PD deficiency (patients receiving >200 mg/d), or hemoglobin M because of high risk for hemolysis and Heinz body formation; caution in patients exposed to other agents or conditions (eg, infection, diabetic ketosis) capable of producing hemolysis; peripheral neuropathy can occur (rare); phototoxicity may occur when exposed to UV light
Anticoagulant and antiplatelet agents
These agents are used in an effort to reduce frequency of thrombotic events. Warfarin and aspirin have well-established roles in preventing thrombosis. Warfarin acts as an anticoagulant by antagonizing vitamin K in its role as a cofactor in the carboxylation process of coagulation factors II, VII, IX, and X. Aspirin possess antiplatelet ability by permanently inactivating cyclooxygenase (COX) activity of prostaglandin synthase-1 and prostaglandin synthase-2 (ie, COX-1 and COX-2).
Thromboxane A2 (TXA2) induces platelet aggregation and vasoconstriction. Nonsteroidal anti-inflammatory drugs (NSAIDs) inhibit TXA2 by reversible inhibition of COX-1. The level of reversible inhibition provided by NSAIDS may be inadequate to effectively block platelet aggregation in vivo. However, the NSAID indobufen, which is not available in the United States, is a potent inhibitor and has biochemical activity comparable to aspirin. Further investigation of effective antiplatelet drugs is essential to overcome obstacles associated with aspirin (eg, toxicity, resistance).
Warfarin (Coumadin)
Used to achieve sufficient anticoagulation to prevent thrombotic events. Interferes with hepatic synthesis of vitamin K–dependent coagulation factors. Used for prophylaxis and treatment of venous thrombosis, pulmonary embolism, and thromboembolic disorders. Adjust dose to maintain INR of 2-3 in absence of associated anticardiolipin syndrome.
Adult
5-15 mg/d PO qd for 2-5 d initially adjusted according to desired INR (ie, 2-3); maintenance dose must be monitored periodically by INR; if recurrent emboli occur, maintain INR of 3-3.5
Pediatric
0.1-0.2 mg/kg/d PO, adjust doses over initial 5 d according to target INR; then monitor maintenance dose with periodic INR and adjust accordingly
Drugs that may decrease anticoagulant effects include griseofulvin, carbamazepine, glutethimide, estrogens, nafcillin, phenytoin, rifampin, barbiturates, cholestyramine, colestipol, vitamin K, spironolactone, PO contraceptives, and sucralfate
Medications that may increase anticoagulant effects include PO antibiotics, phenylbutazone, salicylates, sulfonamides, chloral hydrate, clofibrate, diazoxide, anabolic steroids, ketoconazole, ethacrynic acid, miconazole, nalidixic acid, sulfonylureas, allopurinol, chloramphenicol, cimetidine, disulfiram, metronidazole, phenylbutazone, phenytoin, propoxyphene, sulfonamides, gemfibrozil, acetaminophen, and sulindac
Documented hypersensitivity; severe liver or kidney disease; open wounds or GI ulcers; recent or contemplated CNS, eye, or major trauma surgery; GI, genitourinary or respiratory bleeding; dissecting aorta; infectious endocarditis or pericarditis
Pregnancy
X - Contraindicated; benefit does not outweigh risk
Precautions
PT must be monitored weekly until stable at desired INR and then monthly or with changes in concomitant medication or diet; do not switch brands after achieving therapeutic response; caution in active tuberculosis or diabetes mellitus; patients with protein C or S deficiency are at risk of developing skin necrosis
Aspirin (Anacin, Ascriptin, Bayer)
Inhibits prostaglandin synthesis, preventing formation of platelet-aggregating TXA2. May be used in low dose to inhibit platelet aggregation and improve complications of venous stases and thrombosis.
Adult
81 mg/d PO or 1 mg/kg/d PO; some patients need as much as 12 times typical dose
Pediatric
Administer as in adults
Increases toxicity of lithium, methotrexate, and possibly cyclosporine (especially renal with methotrexate, cyclosporine); may impair diuretic effectiveness
Effects may decrease with antacids and urinary alkalinizers; corticosteroids decrease salicylate serum levels; additive hypoprothrombinemic effects and increased bleeding time may occur with coadministration of anticoagulants; may antagonize uricosuric effects of probenecid and increase toxicity of phenytoin and valproic acid; doses >2 g/d may potentiate glucose lowering effect of sulfonylurea drugs
Documented hypersensitivity; liver damage, hypoprothrombinemia, vitamin K deficiency, bleeding disorders, asthma, porphyria; do not use in children (<16 y) with flu because of association with Reye syndrome
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
Assess ADP-induced and collagen-induced platelet aggregation to titer the aspirin; may cause transient decrease in renal function and aggravate chronic kidney disease; avoid in severe anemia, history of blood coagulation defects, or current anticoagulant use
Interleukin inhibitor
Results from a Phase III study demonstrate significantly more patients who received mepolizumab for treatment of hypereosinophilic syndrome were able to maintain disease control with reduced corticosteroid use (84% vs 43%, p<0.001).7
Mepolizumab (Bosatria)
Humanized anti-interleukin-5 monoclonal immunoglobulin G1 antibody. Orphan drug status in the United States and the European Union.
Adult
750 mg IV q4wk
Pediatric
Unknown. Could find no reports on pediatric usage for this disorder.
Limited data available; none reported
Documented hypersensitivity; bacterial infection, parasitic infestations, collagen vascular disease, vasculitis, graft-versus host disease, malignancy
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Limited data available
More on Hypereosinophilic Syndrome |
| Overview: Hypereosinophilic Syndrome |
| Differential Diagnoses & Workup: Hypereosinophilic Syndrome |
 Treatment & Medication: Hypereosinophilic Syndrome |
| Follow-up: Hypereosinophilic Syndrome |
| References |
| « Previous Page | Next Page » |
References
Martinelli G, Rondoni M, Ottaviani E, Paolini S, Baccarani M. Hypereosinophilic syndrome and molecularly targeted therapy. Semin Hematol. 2007;44(Suppl 2):S4-S16.
James J. Pediatric hypereosinophilic syndrome (HES) differs from adult HES. Pediatrics. 2006;118:S49-S50. [Full Text].
Carey JP, Burke AC. Transient hypereosinophilia in the infant of a mother with hypereosinophilic syndrome. Arch Intern Med. Sep 1982;142(9):1754-5. [Medline].
Roche-Lestienne C, Lepers S, Soenen-Cornu V, et al. Molecular characterization of the idiopathic hypereosinophilic syndrome (HES) in 35 French patients with normal conventional cytogenetics. Leukemia. May 2005;19(5):792-8. [Medline].
Schoch C, Reiter A, Bursch S, et al. Chromosome binding analysis, FISH and RT-PCR performed in parallel in hyperesosinophilic syndrome establishes the diagnosis of chronic eosinophilic leukemia in 22% of cases: A study of 40 patients. Blood. 2004;104:2444.
Miyazawa K, Kakazu N, Ohyashiki K. Clinical features of hypereosinophilic syndrome: FIP1L1-PDGFRA fusion gene-positive disease is a distinct clinical entity with myeloproliferative features and a poor response to corticosteroid. Int J Hematol. Jan 2007;85(1):5-10. [Medline].
[Best Evidence] Rothenberg ME, Klion AD, Roufosse FE, et al. Treatment of patients with the hypereosinophilic syndrome with mepolizumab. N Engl J Med. Mar 20 2008;358(12):1215-28. [Medline].
Adame J, Cohen PR. Eosinophilic panniculitis: diagnostic considerations and evaluation. J Am Acad Dermatol. Feb 1996;34(2 Pt 1):229-34. [Medline].
Adams HW, Mainz DL. Eosinophilic ascites. A case report and review of the literature. Am J Dig Dis. Jan 1977;22(1):40-2. [Medline].
Alfaham MA, Ferguson SD, Sihra B, Davies J. The idiopathic hypereosinophilic syndrome. Arch Dis Child. Jun 1987;62(6):601-13. [Medline].
Anders HJ, Schattenkirchner M. Destructive joint lesions and bursitis in idiopathic hypereosinophilic syndrome. Rheumatology (Oxford). Feb 1999;38(2):185-6. [Medline].
Bain BJ. Eosinophilic leukaemias and the idiopathic hypereosinophilic syndrome. Br J Haematol. Oct 1996;95(1):2-9. [Medline].
Chusid MJ, Dale DC, West BC, Wolff SM. The hypereosinophilic syndrome: analysis of fourteen cases with review of the literature. Medicine (Baltimore). Jan 1975;54(1):1-27. [Medline].
Cryer PE, Kissane J. Hypereosinophilic syndrome with pulmonary hypertension. 1976;60:239-247.
Davies J, Spry CJ, Sapsford R, et al. Cardiovascular features of 11 patients with eosinophilic endomyocardial disease. Q J Med. Winter 1983;52(205):23-39. [Medline].
Flaum MA, Schooley RT, Fauci AS, Gralnick HR. A clinicopathologic correlation of the idiopathic hypereosinophilic syndrome. I. Hematologic manifestations. Blood. Nov 1981;58(5):1012-20. [Medline].
Kang EY, Shim JJ, Kim JS, Kim KI. Pulmonary involvement of idiopathic hypereosinophilic syndrome: CT findings in five patients. J Comput Assist Tomogr. Jul-Aug 1997;21(4):612-5. [Medline].
Katz HT, Haque SJ, Hsieh FH. Pediatric hypereosinophilic syndrome (HES) differs from adult HES. J Pediatr. Jan 2005;146(1):134-6. [Medline].
Layzer RB, Shearn MA, Satya-Murti S. Eosinophilic polymyositis. Ann Neurol. Jan 1977;1(1):65-71. [Medline].
Lierman E, Folens C, Stover EH, et al. Sorafenib is a potent inhibitor of FIP1L1-PDGFRalpha and the imatinib-resistant FIP1L1-PDGFRalpha T674I mutant. Blood. Aug 15 2006;108(4):1374-6. [Medline].
Parrillo JE, Borer JS, Henry WL, et al. The cardiovascular manifestations of the hypereosinophilic syndrome. Prospective study of 26 patients, with review of the literature. Am J Med. Oct 1979;67(4):572-82. [Medline].
Postovsky S, Daitzchman M, Dale A, Elhasid R, Ben Arush MW. Unusual presentation of mastoid eosinophilic granuloma in a young patient. Pediatr Hematol Oncol. Jun 2001;18(4):283-9. [Medline].
Spark RP, Gleason DM, DeBenedetti CD, Gigax JH. Is eosinophilic ureteritis an entity? 2 case reports and review. J Urol. Jun 1991;145(6):1256-60. [Medline].
Tefferi A. Blood eosinophilia: a new paradigm in disease classification, diagnosis, and treatment. Mayo Clin Proc. Jan 2005;80(1):75-83. [Medline].
van den Hoogenband HM. Skin lesions as the first manifestation of the hypereosinophilic syndrome. Clin Exp Dermatol. May 1982;7(3):267-71. [Medline].
Wardlaw AJ, Moqbel R, Kay AB. Eosinophils: biology and role in disease. Adv Immunol. 1995;60:151-266. [Medline].
Weller PF, Bubley GJ. The idiopathic hypereosinophilic syndrome. Blood. May 15 1994;83(10):2759-79. [Medline].
White WL, Wahner HW, Brown ML, James EM. Sequential liver imaging in the hypereosinophilic syndrome: discordant images with scintigraphy, ultrasound, and computed tomography. Clin Nucl Med. Feb 1981;6(2):75-7. [Medline].
Further Reading
Keywords
disseminated eosinophilic collagen disease, endomyocardial disease, eosinophilia, eosinophilic leukocytosis, myeloproliferative, lymphocytic, idiopathic, hypereosinophilic syndrome, endomyocardial fibrosis, platelet-derived growth factor receptor alpha, PDGFRA, secondary endocarditis, anemia, thrombocytopenia, ascites, hepatic thrombosis, Budd-Chiari syndrome, Raynaud phenomenon, thrombotic phenomenon, mastitis, dementia, endomyocardial fibrosis, myocarditis, arrhythmia, congestive heart failure, valvular incompetence, mitral regurgitation
tricuspid regurgitation, aortic valve disease, vesiculobullous rash, papulonodular rash, livido reticularis, angioedema, cellulitis, erythroderma, erythema annulare, subungual petechiae, digital necrosis, multifocal bursitis, pauciarticular arthritis, small-bowel necrosis, sclerosing cholangitis, chronic active hepatitis, enterocolitis, pancreatitis, hepatosplenomegaly, pleuritis, pulmonary hypertension, encephalopathy, pupillotonia, keratoconjunctivitis sicca, episcleritis, retinal vein thrombosis
