Hyper eosinophilia

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Approach to a child with idiopathic hypereosinophilia

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Hyper eosinophilia

  1. 1. Admission Rounds13 / 09/2011Dr Saptharishi L GPediatric Allergy- Immunology
  2. 2. Patient details  Name :S  Age : 3 yrs  Sex : female  R/o : KARNAL , Haryana  Father  Daily wage employee  Mother  HousewifeAdmitted in Pediatric Allergy Immunology
  3. 3. Chief complaintsSkin lesions  5 monthsFever  5 months
  4. 4. Skin Lesions
  5. 5. History of presenting illness Fever Most bothersome to the parents  Moderate grade  Intermittent ; multiple spikes per day  Documented up to 102 F  Intermittently a/w chills and rigors  No diurnal variation  Responsive to anti-pyretics ; transiently  Maximum fever free interval  10 days  a/w decreasing activity / poor feeding  a/w loss of weight / bed bound / stopped walking
  6. 6. History of presenting illness Loose stools  During 3rd month of illness  Duration – 14 days  Hospital admission – 6 days  Increased frequency / altered consistency  No blood / mucus  Responded to IV dehydration correction and IV antibiotics  Subsequently stool frequency / consistency – WNL
  7. 7. Relevant negative history No h/o rapid breathing / burning mictuirition / vomiting No h/s/o any focus of sepsis No h/o photosensitivity / malar rash No h/o any mucosal involvement No h/o any skin nodules / joint involvement No h/o similar illness/ skin lesions in the past No h/o any musculoskeletal / abdominal pain No recent travel / contact with animals No h/o clinical repsonse to antibiotics /anti-helminthics
  8. 8. PAST HISTORY No h/o skin rash / atopy-like illness in the first yr of life No known drug / other allergies No h/o similar illness in the past No h/o any other major illnesses / hospitalizations H/o one blood transfusion 15 days back
  9. 9. FAMILY HISTORY 30 yr old 33 yr oldAbortion 6 yr old 3 yr old
  10. 10. PERINATAL HISTORY Not contributory Born by Full term Normal vaginal delivery at home Birth weight  Not known No adverse antenatal / perinatal events
  11. 11. Other relevant history Developmentally Normal Immunized appropriate for age Poor socio-economic status ENVIRONMENTAL HISTORY :  No h/o contact with dogs/ cats  No h/o poultry / cattle near home  No h/o consumption of unpasteurized milk  No definite allergen could be identified on history  No h/o similar rash in other family members / neighbors
  12. 12. Treatment History Treatment on OPD basis from multiple private practitioners Received topical and indigenous oral preparations h/o improvement in skin rash after topical application No h/o addition or withdrawal of drugs in the last 2 wks
  13. 13. Summary of the history
  14. 14. General &SystemicExamination
  15. 15. General examination Alert , active and interacting well with mother HEAD to TOE examination Examination of the skin
  16. 16. PALMS & SOLES – InvolvedDesquamation present but not asprominent as the rest of the body
  17. 17. Dermatological findings Eczematous lesion with oozing Areas showing secondary skin changes including crusting, lichenification Associated with redness of skin – underlying / surrounding Seborrheic dermatitis of scalp Total body surface area involved  90 % ERYTHRODERMA CAUSE  ? Atopic eczema
  18. 18. Anthropometry WEIGHT – 9.3 kg ( 66% of expected) Height - 86.5 cm (91% of expected) OFC - 47 cm (WNL)
  19. 19. Weight-for-age GIRLS Birth to 5 years (z-scores) 30 30 3 28 28 2.5 26 26 2 24 24 22 22 20 20Weight (kg) 18 0 18 16 16 14 -2 14 -2.5 12 -3 12 10 10 8 8 6 6 4 4 2 2 Months 2 4 6 8 10 2 4 6 8 10 2 4 6 8 10 2 4 6 8 10 2 4 6 8 10 Birth 1 year 2 years 3 years 4 years 5 years Age (Completed months and years) WHO Child Growth Standards
  20. 20. Systemic examination CVS  S1 S2 Normal / No murmurs RS  NVBS + ; No added sounds Abdominal  Liver – 4 cm below RCM Soft, non tender, Span – 11 cm Spleen not palpable , No LN mass CNS  Unremarkable
  21. 21. Database
  22. 22. Other possibilities (less likely) Nutritional deficiencies  Acrodermatitis enteropathica  Essential fatty acid deficiency Icthyosis + Erythroderma  Netherton syndrome  Conradi –Hunerman syndrome DRESS syndrome  ? Drug induced
  23. 23. Biochemistry 20-8-11 23-8-11 27-8-11 01-9-11Na 133 137 130 137K 4.9 4.5 4.0 4.2Cl 99 100 103Urea 12 15 10 16Creat 0.3 0.3 0.3 0.4Ca / P 8.2 / 3.8 8.8/2.9 8.1/4.1TP/ alb 5.9/2.1 5.9/2.0 5.4/1.8AST/ALT 102/41 75/37 107/38ALP 157 161 138Bil 0.7 0.7 0.7 0.7Mg 2.3CRP 49.7 51.73 35.44
  24. 24. Hemograms 20-8-11 23-8-11 24-8-11 1-9-11 2-9-11 8-9-11Hb 9.9 10 9.6 8.1 8.6 7.2TLC 16000 27700 17100 10200 29000DLC 50 28 66 68 10 12 6 21 6 5 3 4 34 55 25 7Plts 552,000 462,000 261000 852000ESR 52 48 38 58 QNSAEC 9418 9405 2550 2030
  25. 25. DatabaseHYPER -EOSINOPHILIA INFLAMMATION
  26. 26. Work-up to rule out underlying infections Urine R/E (two samples)  WNL Stool R/E (two samples)  No ova / cyst Blood c/s & Urine c/s  Sterile Malaria card test and smears  Negative Filarial serology  Negative USG Abdomen  No collections Mantoux / CXR / GA (AFB)  Negative for TB
  27. 27. No evidence of lyticlesions on skull X ray
  28. 28. To Rule out an underlying Immunodeficiency NBT reduction test  Normal Immunoglobulin profile  Ig G  1064 (490 – 1610)  Ig A  59 (40 – 200)  Ig M  176 (50 – 200)  Ig E  1400 (<60) Retro test  Negative
  29. 29. Initial Treatment IV antibiotics ( Ceftriaxone / Amika / Clox) Trial of Albendazole Supportive care Nutritional rehabilitation & Supplements PERSISTENT FEVER SPIKES NO CLINICAL IMPROVEMENT WORSENING ERYTHRODERMA
  30. 30. Possibility of Idiopathic Hyper- Eosinophilia Dermatology consultation Skin biopsy Bone marrow biopsy No evidence of underlying malignancy Routine Parasitology work up negativeSerology ( Trichinella / Toxocara / Toxopl ) awaited
  31. 31. Approach toEosinophilia
  32. 32. Eosinophil Biology•Role of IL 5 in Eosinophilopoeisis •Most eosinophil specific cytokine •Production / release / tissue accumulation•Variety of cytokines involved •Eotaxin 1,2 & 3  most important •CCR 3 receptor•Eosin staining granules •MBP/ECP / EDN /EPO • Lipid mediators  LT C4 D4 E4 • Chemoattractants and pro-fibrotic molecules
  33. 33. How do we define Eosinophilia ? Normal frequency  1-3 % AEC > 500/cmm (or 450/cmm)  eosinophilia AEC > 1500/cmm  Hyper-eosinophilia
  34. 34. How do we classify Eosinophilia?  Arbitrary classification  MILD  AEC 500/cmm to 1500/cmm  MODERATE  AEC 1500/cmm to 5000/cmm  SEVERE  AEC > 5000/cmmReference : WHO defined eosinophilic disorders –2011 update on diagnosis, risk-stratification andmanagement
  35. 35. Whenever you face eosinophilia, Always consider  Degree of eosinophilia  Location of eosinophilia ( blood / tissue / both)  Clinical presentation Careful history reg  Symptoms of atopy / gastro-intestinal disease  Helminth endemicity information  Drug ingestion  Systemic symptoms suggesting malignancy
  36. 36. WORKING DIAGNOSIS in index caseIdiopathic hyper-Eosinophilia syndrome Does it meet the criteria?  Old criteria – Chusid et al (1975)  Newer criteria – WHO Types  Myeloproliferative (m-HES)  Lymphocytic (L-HES)  Familial ( f- HES) Lymphocytic variant of HES   only cutaneous involvement / skin plus one other organ system  Elevated Ig E levels  Risk of subsequent malignancy – T cell lymphomas
  37. 37. Double negative T cells (CD 3+ CD4- CD 8-) A clone of lymphocytes described in lymphocytic variant of HES
  38. 38. Therapeutic decision making
  39. 39. Treatment strategies Etiology specific therapy Hyper-eosinophilic states  Corticosteroids  PREDNISOLONE  1 mg/kg  slow taper  Hydroxyurea  Imatinib ( m-HES)  Interferon alfa  Mepolizumab
  40. 40. Index Case Started on Oral steroids at 1 mg/kg/day Significant clinical improvement ; activity better Erythroderma improving No fever PLAN  Steroids for 2 wks  Taper over 2-3 months Routine follow up ; Decide need for step-up
  41. 41. TAKE HOME MESSAGE Try to approach eosinophilia systematically Always rule out secondary causes MOST IMPORTANT parasitic infections BUT not every eosinophilia should be blindly given Albendazole and sent home Keep malignancies / immunodeficiency states in consideration if no other secondary cause found Lastly, consider HES and the tissue damage that Eosinophils may be producing Decide regarding appropriate therapy
  42. 42. THANK YOU
  43. 43. QUERIES ?

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