Hypersplenism

Condition in which spleen removes circulating RBCs, granulocytes and platelets in excess quantity.

Diagnosis:

1. Pancytopenia
2. Normal or hypercellular bone marrow
3. Splenomegaly
4. Correction of cytopenias after splenectomy

Classification

1. Primary – when no aetiology for enlarged spleen is found.
2. Secondary –
1. Portal hypertension
2. Infiltrative disease, lymphoma, myelofibrosis
3. Hemolyitc anaemias, hematological disorders
4. Rheumatoid arthritis (Felty’s syndrome)
5. Tropical splenomegaly syndrome

Pathogenesis

When splenic size increases, there is increases pooling of blood in an environment with relatively reduced availability of nutrients but full of phagocytes.

This leads to exaggerated sequestration and destruction of cells leading to

1. Pancytopenia
2. Hemolysis
3. Increased plasma volume

Treatment

Therapy needed when cytopenias become severe and symptomatic

Treatment of underlying causes

Splenectomy if underlying cause cannot be corrected or treated

Type 1 Hypersensitivity

Type 1 Hypersensitivity

1. Eczema
2. Hay Fever
3. Asthma ( atopy )
4. Theobald Smith phenomenon
5. Anaphylactic shock
6. Acute dermatitis
7. Urticaria
8. Prusnitz kustner reaction
   

Behcet's Syndrome

It is a type of vasculitis

Symptoms:

1. Oral and genital ulcers 
   
2. Uveitis 
3. Optic atrophy

Pickwickian Syndrome:

It is a symptom complex presenting with the following symptoms:

1.Obesity 

2.Hypoventilation 

3.Somnolence 

4.Erythrocytosis

This comdition has been associated with sleep apnoea syndrome which has now been established as a separate entity.

Thoracic Aortic Aneurysm

Description:

Aabnormal dilatation of blood vessel

Pathogenesis:

Intimal tears progress to longitudinal intraluminal tears forming a lumen in the media.

There is collection of basophilic mucoid material in media and elastic tissue.

Most important factors promoting continued propagation of dissection are hypertension and velocity of left ventricular ejection.

Complications:

• Aortic rupture causing cardiac tamponade
  
• Leakage may cause pericarditis
  
• Dysphagia
  

Clinical Features:

• hypertension on initial presentation
  
• pericardial friction rub or AR murmur
  
• aortic insufficiency
  
• pulse deficit
  
• focal neurologic deficits may be present
  

Treatment:

1.Medical management for uncomplicated distal dissection

• sodium nitroprusside
• beta blockers
  

2.Surgery for acute proximal aortic dissection

• Operative repair to prevent rupture
  
• Emergency surgical repair for dissection of ascending aorta
  
• Cerebrospinal fluid drainage during and after surgery may reduce risk of neurological injury .