We report a child with hypereosinophilic syndrome who presented with cardiogenic shock. In addition, she had skin and joint involvement. The clinical condition improved and eosinophil counts normalized with steroid therapy. However, the skin lesions and hypereosinophilia relapsed on stopping the steroids. The child was subsequently maintained in remission on low dose prednisolone.

Keywords: Cardiogenic shock, Child, Hypereosinophilic syndrome.

Hypereosinophilic syndrome (HES) is a rare disorder characterized by unexplained, persistent eosinophilia and end organ damage due to eosinophilic infiltration. We report a girl with HES who presented acutely with cardiogenic shock along with skin and joint involvement.

On examination, her pulse rate was 136/min, respiratory rate 34/min and blood pressure 84/60 mm Hg. She had raised jugular venous pressure, bilateral pedal edema and arthritis of bilateral knee and wrist joints. There were erythematous papules with scaling on the extremities and angioedema of the perioral and periorbital regions. There was no pallor, icterus, cyanosis or clubbing. The anthropometry was normal. Examination of the chest revealed bilaterally decreased air-entry in the infrascapular areas. Cardiac apex was at the left 5th intercostal space in the mid clavicular line. Heart sounds were muffled. There were no murmurs. She had tender hepatomegaly 4 cm below the right costal margin (span 11 cm). Spleen was just palpable. Rest of the systemic examination was normal.

Investigations revealed hemoglobin 12.4 g/dL, total leucocyte count of 29,000/mm3 (polymorphs 68.9%, lymphocytes 6%, eosinophils 22.2%, absolute eosinophil count: 6438/mm3), platelet count 647 × 103/mm3 and erythrocyte sedimentation rate of 8 mm in first hour. Peripheral blood smear showed marked eosinophilia, few eosinophils showed multilobation of nuclei and hypogranulation (Fig. 1). Other cells had normal morphology.

Fig.1 Peripheral smear showing eosinophils with multilobation of nuclei and hypogranularity (Leishman stain X 100).

Child was treated with oral prednisolone (1 mg/kg/day) for 2 weeks. Thereafter, the steroids were gradually tapered and stopped over 8 weeks. At 8 weeks, the WBC count was 12000/mm3 with 0.2% eosinophils. The pericardial effusion resolved after a week. One month after stopping the steroids, she developed fever, erythema and angioedema over the face. The absolute eosinophil count was 2470 cells/mm3. Echocardiography was normal. She was started on prednisolone 1 mg/kg. Her skin lesions disappeared and eosinophil counts became normal after 4weeks. She has been maintained on low dose alternate day prednisolone (0.25 mg/kg) and is asymptomatic without peripheral eosinophilia.

Hyperosinophilic syndrome (HES) is a rare disease in children, the exact prevalence is not known(1). The diagnostic criteria for "idiopathic HES" proposed in 1975 are blood eosinophilia > 1500 cells/mm3 for at least 6 months, absence of an underlying cause of eosinophilia despite extensive evaluation, and presence of end organ damage or dysfunction due to eosinophil infiltration(2). Heart, skin and nervous system are most frequently involved. Eosinophils release many cytotoxic substances like eosinophil-derived neurotoxin, eosinophil cationic protein, major basic protein, reactive oxygen species and proinflammatory cytokines, producing end organ damage and fibrosis(2). HES must be distinguished from eosinophilic leukemia, which is characterized by increased blood and bone marrow blasts, eosinophilic clonality and specific cytogenetic abnormalities(2).

Recently three subtypes of HES have been identified(3). The myeloproliferative variant (m-HES) is characterized by an interstitial deletion in chromosome 4q12 in cells of the myeloid lineage and FIP1L1-PDGFRA gene fusion. Supportive evidence of m-HES includes splenomegaly, anemia, throm-bocytopenia, increased circulating myeloid pre-cursors, dysplastic eosinophils, elevated serum vitamin B12 and altered leucocyte alkaline phosphatase levels. Leukemic transformation can occur. The lymphoproliferative variant (l-HES) is associated with clonal proliferation of phenotypically abnormal T cells. Idiopathic HES is a subtype of the disease that is not associated with a specific chromosomal or clonal abnormality. Our patient had overlapping characteristics of myeloproliferative and lympho-proliferative variants.