Dr.Ch. Venkat Kumar raju, Dr. Sajitha Nair, Dr. Bhanu Vikraman Pillai, Dr.C. Jayakumar
A 10-year-old girl presented with non-bilious vomiting of 2 weeks and progressive abdominal distention for 1-week duration.
No history of fever, cough, or weight loss
At admission child presented with a grossly distended abdomen
Per abdomen- umbilicus was everted with no organomegaly
No signs of liver cell failure or organomegaly
At this point our D/Ds were
1. Chronic Liver disease
2. Budd Chiari syndrome
3. Nephrotic syndrome
4. Pancreatitis
5. Right-sided heart failure
6Protein loosing enteropathy
Labs leukocytosis (28K) (N 10, L 16,E74%) with an AEC of 27,552 cells/mm3. ESR 2mm Peripheral smear showed eosinophilia with normal morphology.
USG and CT abdomen were normal.
Serum IgE was 674Iu/ml.
Stool for parasites was negative.
No history of contact with pets.
Food and inhalational tests were negative. Ascitic tap showed 42,000 cells/mm3 (90% eosinophils)
Exudative(LDH ratio- 1.63, Protein ratio of 0.85), no malignant cells, Negative for TB, Initial upper GI and colonoscopy with biopsy was negative. Ml
The child was discharged on diuretic and DECDiethyl carbamazine , and AEC came down to 208
Our D/D at this point was eosinophilic ascites under evaluation
Her Immunoglobulin profile, flow cytometry and DHR were negative
On follow up she continued to have eosinophilia. BMA and biopsy showed Increased eosinophils with normal morphology.
FISH for eosinophilic mutation BCR/ABL, PDGFR (platelet derived growth factor receptor )was negative.
USG Abdomen repeat showed bladder wall thickening and gastric pylorus wall thickening.
Repeat Upper GI with colonoscopy was done which showed erythema in the antrum which was suggestive of eosinophilic gastritis.
HPE of the esophagus showed intraepithelial eosinophils (>20/HPF) associated with basal hyperplasia.
She was given a trial of oral budesonide, as she didn’t tolerate it,child was started on an Oral Steroid dose.
The Child initially showed a dramatic response (AEC 6707 -> 718).
USG abdomen showed persistent pyloric wall thickening.
As AEC was increasing (1886) on tapering of steroids child had evidence of end-organ involvement, Imatinib was added as a cytoreductive therapy. Currently child is 11-year-old school going , Last AEC is 1886
Erythema at antrum
Eosinophilia in biopsy
AEC trends
Hypereosinophilic SyndromeHypereosinophilic syndrome (HES) was first described in 1968 Hardy and Anderson.
• According to WHO, Idiopathic Hypereosinophila syndrome(IHES)is characterized eosinophilia greater than 1.5 x 109/L for at least 6 months and the presence of end-organ damage
•Incidence in the general population is 4.2 per 100,000 in the USA
• Prednisone remains the mainstay of therapy in the acute setting for severe and/or life-threatening manifestations of HES
• Biologics were approved for the treatment of HES like Mepolizumab (IL-5),
• For mutation positive (PDGFR) Imatinib is approved
• For mutation-negative and steroid-refractory cases, Studies are yet to come
•
Take home message- When confronted with persistent eosinophilia, diligent screening for malignancies and hypereosinophillic syndrome is imperative. Timely identification of underlying causes is essential for effective intervention and improved prognosis .