Hypereosinophilic Syndrome
Values
Eosinophil > 200 ...........................Esinopenia
Eosinophil (200 – 600 ) ................. Normal
Eosinophil (600 – 1500 ) ............... Mild esinophilia
Eosinophil (1500 – 5000 ) ............. Moderat esinophilia
Eosinophil < 5000 ......................... Severe esinophilia
Def.: Leukoprolifrative disorders characterized by an over production of esinophiles that results in organ damage.
Incidence: Sex:- Male : Female (9 : 1 ) , Age:-HES is most commonely diagnosed in patients aged 20–50 Years
Types:
1-Tropical hyperesinophilic syndrome: This is due to micro fliriasis which collect & cause impaction in the
alveoli cause irritation of the alveoli = Pulmonary esinophilic syndrome.
NB:-Oral Q:-What will u found,If Blood culture done? We will found Eosinophilia(Not Microfilaria As it died)
2-Idiopathic hyperesinophilic syndrome: It is eosinopllia more than 150,000 cell/cmm for more than 6 months
for unknown cause after exclusion of other causes of eosinophilia
Pathogenesis
-Dysregulation of eisinopoiesis.
-Overproduction of the mediators that control eisinopoiesis (IL3 , IL5 , GM CSF ).
-Lack of negative feedback suppression of eisinopoiesis.
-Increased sensitivity of receptors of eisinopoiesis.
C/P: Multi organ affection + Eosinophilia
1-Cardiac findings: 4-Hematologic symptoms:
---Damage typically occurred in 3 stages:- - Thrombotic episode, splenomegaly, anemia that is
-Acute necrosis. observed with HES.
-Thrombotic phase.
- endomyocardial fibrosis. 5-Rheumatologic symptoms:
---Common symptoms in these phases include chest -Arthralgias and myalgias are frequent complaints.
pain, dyspnea, or orthopnea. -Raynaud phenomenon occurs but is infrequent.
2-Neurologic symptoms: 6-Gastrointestinal symptoms:
-Strokes or transient ischemic episodes. -Diarrhea.
-Encephalopathy. -Nausea and abdominal pain .
-Blurred vision and slurred speech. -Small bowel necrosis due to microthrombi can occur.
3-Pulmonary symptoms: 7-Dermatologic symptoms:
-A chronic, persistent cough, usually nonproductive. -Skin involvement is common and nonspecific.
-Patients often have recurrent angioedema. -The most common symptom is pruritus.
-Dyspnea may occur due to CHF or pleural effusions. -Angioedema.
-Less frequently, pulmonary fibrosis occurs after
prolonged disease and often accompanies cardiac 8-Constitutional symptoms:
fibrosis. -fever and night sweats.
-Bronchospasm and asthmatic symptoms r infrequent -anorexia and weight loss.
-Rhinitis is sometimes a presenting symptom.
-Eosinophilic infiltrates in the bases and periphery of
the lungs.
Investagtion:- Treatment:-
1-exclude other causes of esinophllia 1-symtomatic 2-corticosteroids:-60mg\day
2-CBC:-esinophllia, leucocytosis 3- Tyrosine kinase inhibitors. 4- Alpha interferone.
3-X-ray for pleural effusion, cardiomyopathy 5- Anti IL5 . 6-immunosuppresive drugs
4-CT for ICT, STROKE 7-Azathioprine, cyclosporine, and methotrexate
8-splenectomy 9-PUVA:-for pruritis & psoriasis
10-DEC= hitrazan for Pulmonary esinophilic syndrome