Skip to main content

Sickle Cell Anemia


Sickle cell anemia is hereditary hemolytic anemia. This disease is characterized by presence of sickle and crescent shaped erythrocytes. The normal HbA that is adult hemoglobin is replaced by HbS that is abnormal sickle hemoglobin.

HbS defers from normal adult hemoglobin in the substitution of valine for glutamic acid in the sixth position of the beta chain. Presence of HbS imparts the sickle shape to deoxygenated red cells which is responsible for this disease. Under reduced oxygen tension, HbS molecules undergo crystallization within the red cell, which elongates and distorts the cells. The sickle cells themselves contribute to increased blood viscosity and circulatory stasis. The consequence is thrombosis which may lead to ischemia. 
Symptoms-
 1. Anemia.
 2. In majority of patients this disease remains asymptomatic. Manifestation of this disease may be acute or episodic and chronic or progressive.
  3. Severe abdominal pain and cramps. 
4. Bone and joint pain, haematuria, neurological manifestations or ulcers over the malleoli. 
5. In early stage splenomegaly occurs. But later on this spleen undergoes infarction and marked contraction with eventual autosplenectomy. 
6. Pain. 
7. Fever. 
8. Retarded growth. 
9. Development of bone and joint disease, pulmonary, hepatobiliary manifestation. 
10. Pigment gallstones appear later in life. 
11. Majority of patients with this disease die in the first decade of life, though a few may survive up to fifth decade. Death may result from intercurrent infections, cardiac or renal failure. 

Investigations- 
1. Blood smear- shows presence of characteristic sickle cells. 
2. Hemoglobin electrophoresis- shows predominately HbS and variable amounts of HbF and no HbA. 
3. Leucocytosis. 
4. Increased number of platelets. 
5. Elevation of serum bilirubin.
Do you know about- Idiopathic Thrombocytopenic Purpura

Popular posts from this blog

Bazin’s Ulcer

These ulcers are associated with ‘erythrocyanosis frigida’, which is an exclusive disease of young women. These patients have thick ankles with abnormal amount of subcutaneous fat, combined with an abnormally poor arterial supply to the ankle skin.

Health Zone of Rice Bran Oil

"Rice bran oil" phrase, words are used very much these days. But, do you know why these words are used so much these days? These words, rice bran oil suddenly became popular because of health benefits which rice bran oil has. Yes, rice bran oil is a nutritious gift from oil category of nature.

8 Diseases that may affect you during Monsoon Season

Monsoon season is house of diseases. The reason being appropriate moisture of balance and warmth. This appropriate balance let’s development of disease causing micro-organisms to flourish. They are transmitted to humans through infected water and food. They are also transmitted to human blood through bite of disease causing vectors, like mosquitoes. Chikungunya, diarrhoea, cholera are diseases that affect people during monsoon season. Hepatitis A, Leptospirosis and influenza also affect people during monsoon. Like that there are around total 8 diseases that affect people during monsoon. Lets have a look on all of them in detail. Image Source - https://pixabay.com/en/virus-microscope-infection-illness-1812092/