Eosinophilic gastrointestinal disorder (EGID)

Author: S. Randhawa, M.D., Allergist/Immunologist and Assistant Professor at LSU (Shreveport) Department of Allergy and Immunology
Reviewer: V. Dimov, M.D., Allergist/Immunologist and Assistant Professor at University of Chicago

A 3-year old Caucasian boy with h/o severe anemia (hemoglobin 6 mg/dL) and hemoccult positive stools was admitted to the hospital. He was previously admitted 6 months ago with hemoglobin (Hb) 1.9 mg/dL, transfused, and a GI endoscopy showed a colonic polyp. The patient was started on iron therapy and the Hb improved but was never normal. According to the mother, he had no symptoms of bleeding. The patient’s stool has been black since he was started on iron.

Medications

Iron syrup.

Family and social history

Non contributory.

Birth History: he was born at 40 week gestation without any problems. Immunizations are up-to-date. Diet: Table food. Eats everything. The patient is developmentally appropriate for age.

Physical examination

Vital signs: Temp. 98.1, HR 112 bpm, RR 6, BP 92/58 mm Hg.
Wt 13.8 Kg.
HEENT: External ears normal. Canals clear. TM's normal. Nares normal. Septum midline. Oropharynx clear. Neck: supple, no adenopathy.
CVS: RRR, normal S1/S2, no m/r/g.
Chest: CTA (B).
Abdomen: Soft, Non tender BS +ive.
Extremities: no c/c/e.
Skin: color, texture, turgor normal. No rashes or lesions.

What laboratory tests would you suggest at this point?

CBCD
CMP
ESR
Stool O & P
Stool occult blood
TSH
UA
Blood cultures

Laboratory results

CBCD
WBC – 10.8
Hb – 7.3
Hct – 26.1
MCV- 68
Platelets- 194
Neutrophils - 30%
Lymphocytes - 49%
Monocytes – 4.3%
Eosinophils – 15.9% (1730)
Basophils - 1.6% (170)

CMP
Na - 138
K - 4.2
HCO3 24
BUN 26
Cr 1.1
Bilirubin Normal
AST 22
ALT 18
Ca Normal
Glucose 114
Total Protein 4.5 (low)
Albumin 2.1 (Low)

UA
Specific gravity – 1.020
pH - 5
Protein - None
Glucose - None
Ketone - None
Bilirubin - None
Blood - None
Nitrite - Negative
Leukocyte - Negative
Urobilinogen - None

TSH – 3.6
Stool occult blood - positive
Blood cultures negative
Ova & Parasites - negative
Stool Ova & Parasite
ESR – Within normal limits

The treatment team performed additional tests listed below:

Sweat Chloride test - negative
HIV: ELISA - negative, Western Blot - negative, PCR - no RNA copies identified.
Flow cytometry – Within normal limits

Radiology tests:

CXR - Within normal limits
CT Chest - Within normal limits.
CT abdomen - Within normal limits except for 1.7 cm intraluminal defect seen in the proximal jejunum without bowl dilatation.

Diagnostic procedures performed:

Sputum cytology - Within normal limits.
Pulmonary function testing - Within normal limits.
Upper Endoscopy – Showed signs of inflammation in esophagus, stomach and proximal deudenum. Biopsies were taken which showed an abundance of eosinophils (more than 50 per hpf) in the submucosa.
Colonoscopy - Showed patchy inflammation in the colon. Biopsies showed an infiltration of eosinophils in the submucosa.

Serological testing:

ANA – Negative
RA - Negative

RAST to food allergens:

Chicken - Class 0/1
Egg Class - 2
Fish Class - 0
Milk Class - 2
Soybean Class - 0
Wheat Class - 2

Thyroid antibodies
Anti thyroglobulin Ab – Negative
Anti peroxidase Ab – Negative
Anti microsomal Ab - Negative

Immunoglobulins

Total IgG - Within normal limits
IgG Subclasses (IgG1-4) – Within normal limits
Total IgA - Within normal limits
Total IgM – Within normal limits
Total IgE - 73

Food allergen skin testing - negative to Almond, Apple, Banana, Beef, Celery, Coconut, Corn, Cottonseed, Egg, Fish, Lobster, Milk, Pecan, Pea nut, Soybean, Shrimp, Walnut, Wheat.

DTH
Tetanus – Normal Response
PPD - Normal Response
Trichophyton - Normal Response
Candida - Normal Response

Delayed-type hypersensitivity (DTH) response

The standardized DTH test includes Candida, tetanus, mumps, and TB. Trichophyton is also commonly used. However, the only FDA-approved reagents for DTH are PPD, Candida and mumps.

Final Diagnosis

Eosinophilic gastrointestinal disorder (EGID).

What happened next?

The patient was advised to avoid wheat, eggs and cow's milk. He was started on Prevacid 30 mg po daily, prednisone syrup 1mg/ml, 5ml po daily for 4 weeks and advised to continue the iron syrup.

At follow-up, the patient's CBC showed Hb 12.3 mg/dL, Hct 38.9, eosinophils 2% (186). Steroids were tapered off and oral fluticasone was started. A follow-up EGD was scheduled in 6 months.

Summary

Primary eosinophilic gastrointestinal disorders are defined as disorders that selectively affect the gastrointestinal tract with eosinophil-rich inflammation in the absence of known causes for eosinophilia (eg, drug reactions, parasitic infections, and malignancy).

These disorders include eosinophilic esophagitis, eosinophilic gastritis, eosinophilic gastroenteritis, eosinophilic enteritis, and eosinophilic colitis and are occurring with increasing frequency.

References

Eosinophilic gastrointestinal disorders (EGID). Rothenberg ME. Eosinophilic gastrointestinal disorders (EGID).
Eosinophilic Gastroenteritis. eMedicine Specialties > Gastroenterology > Intestine. eMedicine, 2009.

Published: 12/07/2009
Updated: 09/07/2010

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