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Erythocytosis / Eiosinophilia / Thrombocytosis / Leucocytosis / Neutrophilia

  • Neutrophilia
  • EOSINOPHILIA / HYPER-EOSINOPHILIA
    • Definition: (Case 7-2014)
      • Eosinophilia: absolute eosinophil count of more than 500 per cubic millimeter, 
      • Hyper- eosinophilia: absolute eosinophil count of more than 1500 per cubic millimeter. 
    • Pathophysiology of Eosinophilia
      • 2 distinct mechanisms of eosinophilic production
        • Due to cytokine overproduction: (Case 7-2014)
          • GM-CSF, IL-3, IL-5 I(IL-5 is the only eosinophilic specific interleukin)
            • Derived from CD 34 + progenitor cells - Th2 lymphocytes 
              •  IL-4 - stimulates Ig E production and eosinophil recruitment
              • IL - 5 (enhanced eosinophilic production in BM)
        • Clonal Eosinophilic proliferations
      • Mechanism of Tissue Damage
        • Eosinophils primarily reside in tissues (blood count cannot predict extent of organ damage), hence even though tissue damage is seen at high eosinophil count, can also occur without significant increase in eosinophil count 
        • Mature true eosinophils persist in peripheral circulation for 24 hr before migrating to extravascular sites where they reside for days (Case 7-2014)


How I treat hypereosinophilic syndromes Blood 2015
    • DDx of Eosinophilia 
      • Allergic: Asthma, Eczema (can be difficult to distinguish from L-HES); 
      • Drug Hypersensitivity: including non-prescription drugs, herbal  remedies, and dietary supplement
      • Infection:
        • Helminth: Strongyoidiasis (often asymptomatic, and endemic worldwide), Hookworm Infection, Filiariasis
        • Ectoparasistes: Scabies, Myiasis
        • Protozoan: Isosoporiasis, Sacrocystitis Myositis
        • Bacterial:  Chronic TB, Resolving Scarlet Fever
        • Fungal: ABPA, Cocciodiomycocis
        • Viral: HIV, HTLV - 1
        • Ref: 
      • Neoplasm: ALL,  T-cell Lymphoma, Hodgkin's Lymphoma,  MPN including Systemic Mastocytosis, Solid organ adenocarcinoma 
      • Autoimmune and Idiopathic Disorders : CTD, Sacroidiosis, AI-LPD, Eosinophili-GPA, Idiopathic Eosinophilic Synovitis, IBD
      • Others: Hypoaldosteronism, IL -2 Therapy, Cholesterol Embolization, Radiation exposure,  Hypereiosinophilic Syndromes (HES) 


    • Clinical Manifestation of eiosinophilia: 
      • Can be same for primary and secondary eosinophilia 
        • Internal Organ Involvement:
          • Skin (most common; 35 + %)
          • Lungs (25 + %)
          • GI Tract (around 15%)
          • Potentially life threatening
            • Cardiac (5%) ; 3 main clinical manifestations (Case 7-2014)
              • Mild and Transient: Hypersensitive Myocarditis
              • Acute and Severe: Acute Necrotizing eosinophilic myocarditis
              • Chronic : Loeffler's Endocarditis, Davies' Disease, Endomyocardial Fibrosis
            • Neurological Involvement (5 %)
 


Case Description
JAM

55 yo M is seen for N and V. He has prior diagnosis of Atopic Dermatitis, Asthma, Allergy, Chronic Rhinosinusitis, HF, and frequently noted elevated troponin, Psoriasis and Weight loss. CBC with Diff is as below. CMP is normal expect for Alb of 2.6. 

CRP > 10. ESR 58. ANA and ANCA were negative. 

How do we work this up further?

Answer: 
Syndromic Diagnosis of HES: Absolute Eosinophil Count is > 10000. 
DDx for HES to this extent includes the following. 
Source: ACR 2016

Clinical manifest ion: Is present. 

Is it life threatening? No. If it was emperic steroid and Ivermection should be started. Since, it is not, we should proceed with evaluation. 

Given cocern for Leukemia, BM biopsy is done, and reveals the following confirming it is not Leukemia. 

BM Biopsy is done, and reveals the folioing. 

 


At this time, following tests should be done. 
  1. Quantitative IgE: 5128 IU/mL
  2. Serum Troponin: Chronically elevated. > 0.5 BNP is 258. 
  3. ECHO is as follow: 
  4. PFT including Diffusion Study: Normal
  5. Exhaled Nitric OxideL 37 ppb (normal < 25ppb)
  6. Cortisol: Normal
  7. Chest CT, And and Pelvis : Mediasternal LN of 1.3 cm, Basilar atelactasis and consolidation, Bladder thickening, Small pericardial effusion,
  8. Skin Biopsy is done from left medial leg, and reveals features conceding for psoriasis. 
  9. Aspergillus Ab screen: Is negative
  10. ANA and ANCA were tested. Unclear why. But were negative. 
  11. Ig G level and fractionation is normal. 
  12. Hypersensitivity Pneumoitis profile is ordered and reveals the following. 

Following test still needs to be done
  1. FIP1L1-PDGFRA 
  2. Serum tryptase and B12 level
  3. Flow cytometry
  4. Repeat CT chest, abd, pelvis
What are the current, and future treatment options for HES?
ACR 2016
  • ERYTHROCYTOSIS:

a.     Myeloproliferative Disorder: Polycythemia Rubra Vera

b.     Secondary Causes eg. COPD etc

The diagnosis and management of erythrocytosis BMJ 2013   

Erythrocytosis:
Case 23-2010 (Renal lymphangiectasis / Renals Cysts)

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