IDIOPATHIC HYPEREOSINOPHILIC SYNDROME
Dr. M. M. Karva, M.D. (Ped), Solapur*, Dr. Sunil M, Mehta M.D., (Med), DNB, MNAMS**
Department of Pediatrics*, Department of Pediatrics**
Abstract: A case of Idiopathic Hypereosinophilic Syndrome (HES) who presented with cardiac complications and responded well to oral corticosteroids.
Keywords: Hypereosinophilic Syndrome (SES), Absolute Eosinophilic Count (AES)
Case Report: A 4½ old male child came with c/o fever, cough - 3 days, oedema over body and dyspnea - 1 day. No H/o arthritis, skin rash, paroxysmal nocturnal dyspnea, hematuria/oliguria, jaundice, worms infestation, allergic diathesis, contact with pet animals, TB drugs.
O/E: Wt. - 17 kg, Temperature - Normal, B.P. - 90/60 mm of Hg; Pulse 104/min, regular; Mild dyspnea +, R.R. 40/min, No cyanosis, No clubbing, JVP raised, No icterus, No lymphadenopathy; Mild anasarca +, Pallor + CVS - Heart sounds normal, systolic murmur in pulmonary area - Grade II, P2 Normal.
RS - Bilateral generalized crepitations and rhonchi heard
P/A - Liver 8 cms., firm, non-tender; spleen not palpable; ascites present
Fundus: Normal CNS - No abnormal finding detected.
Investigations

Date


TLC/cu. mm

Eosinophils

AEC

Platelet lac/cu. mm


ESR mm

Hb gm

28/8/06

46900

60%

28140

3.59

50

9.4 gms

8/9/06

21400

39%

5508

3.30

70

9.0 gms

3/10/06

10200

14%

1428

3.0

27

11.3 gms


Urine exam.: NAD; Sr. Creatinine - 0.8 mg %, Stool exam - NAD; SGPT - 20 IU/L, Serum IgE - above 2000 IU/ml. (Normal = 0.4 to 350 IU/ml) X-ray chest - No cardiomegaly; bilateral paracardiac haze, no pleural effusion.

ECG - Normal

USG Abdomen: Moderate hepatomegaly with increased parenchymal reflectivity and coarse echo pattern, mild splenomegaly, mild ascites, dilated thickened echogenic bowel wall loops seen.
Colour Doppler: Large mass filling right ventricular apex (thrombus) with mild tricuspid regurgitation; no pericardial thickening or effusion, no pulmonary hypertension - suggestive of fibro plastic cardiomyopathy - (due to HES).
Treatment: IV, Lasix, Decadron, Cefotaxime, and then oral prednisone
At discharge - oral prednisone 1 mg/kg/day, T. Warfarin sodium 1.25 mg OD; and oral hematinics.

Present findings - Wt-14 kg, No oedema or ascites, pulse 84/min, CVS No murmur, P/A - Liver 3 cm firm, RS - clear

Discussion: Hypereosinophilic syndrome (HES) is leucoproliferative disease of unknown etiology - characterized by overproduction of eosinophils. Tissues infiltration with eosinophils results in multiple organs damage including heart, skin, brain, lungs, liver, spleen, intestines, and peripheral nerves. Chusid et al have defined 3 features of diagnosis of HES:
  1. Sustain (AEC) eosinophilia more than 1500/cu. mm - for at least 6 months
  2. Eosinophilia causing organs damage
  3. No obvious etiology found for eosinophilia. Male/Female ratio (9:1), general HES is seen between age group of 20 to 50 years.

The 3 hallmarks of cardiac pathologic findings of HES are (1) Endocardial fibrosis, (2) Myocardial inflammation with eosinophils, (3) Mural thrombi in right and left ventricle. Heart failure can occur due to hypersensitivity or toxic necrotizing myocarditis due to HES and also due to development of restricted cardiomyopathy or severe mitral/tricuspid regurgitation. ECG - may be normal in 35% of cases - or may show T-wave inversion; LAH and LV hypertrophy.

Levels of IGE, CPK - MB, or troponin may be elevated. Medical Treatment - Prednisone 1 mg/kg/day for 3-12 months. If poor or partial response - Hydroxyurea 20 mg/kg/day; Vincristine, Cyclosporine, imatinib mesylate, interferon alpha, Cladibrine and etoposide can be used. For heart failure - ACE inhibitors, diuretics and betablockers are used. Anticoagulants are used if intracardiac mural thrombi or thromboembolic phenomenon occur.
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