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Medicine

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Hematology

Question
5 out of 11
 

Commonest acute presentation of sickle cell anaemia is: (AIIMS Nov 2009)



A Priapism
B Bone pain

C Fever
D Splenomegaly.

Ans. B

Bone pain

The clinical feature

1. Acute :

a. Infarctive or painful crisis: Severe skeletal pain but no change in Hb%

b. Sequestration crisis: Sudden massive pooling of red cells in spleen with an acute fall in Hb concentration.

c. Hemolytic crisis (uncommon): Fall in hemoglobin concentration with marked increase in jaundice.

Extra Edge: Commonest acute presentation of sickle cell anaemia is bone pain

2. Chronic:

a. Hemolysis: i. Anemia ii. Cardiomegaly iii. Hepatomegaly iv. Bone marrow hyperplasia v. Leg ulcer vi. Jaundice vii. Pigment gall stone

b. Increase viscosity:

Table -Clinical Features of Sickle Hemoglobinopathies (Ref. Hari-18th ed., Pg. 855, table 104.2)

Condition

Clinical Abnormalities

Hb Level g/L (g/dL)

MCV, fL

Hb Electrophoresis

Sickle cell trait

None; rare painless hematuria

Normal

Normal

Hb S/A:40/60

Sickle cell anemia

Vaso-occlusive crises with infarction of spleen, brain, marrow, kidney, lung; aseptic necrosis of bone; gallstones; priapism; ankle ulcers

7–10

80–100

Hb S/A:100/0

Hb F:2–25%

S/β° thalassemia

Vaso-occlusive crises; aseptic necrosis of bone

7–10

60–80

Hb S/A:100/0

Hb F:1–10%

S/β+ thalassemia

Rare crises and aseptic necrosis

10–14

70–80

Hb S/A:60/40

Hemoglobin SC

Rare crises and aseptic necrosis; painless hematuria

10–14

80–100

Hb S/A:50/0

Hb C:50%

Complications

1. Thrombotic crisis/infarction crisis:

2. Aplastic crisis: This is usually due to parvovirus infection and is characterized by a low reticulocyte count.

3. Sequestration crisis: Due to RBC trapping, the spleen and liver enlarge. Anemia becomes very severe which can be an acute manifestation and cause death in infants. Later, repeated infarction and fibrosis of spleen leads to ‘autosplenectomy’. Functional asplenia occurs much earlier (early childhood).

4. Hemolytic crisis –

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