This is a 25 years old Malay man who has been admitted with lethargy and palpitation for 3weeks duration. Clinically he is pigmented with frontal bossing and prominent maxillary cheek bone. He is also short and has hepatospenomegaly with a liver of 5 cm below R costal margin and spleen of 4 cm below L costal margin. He has one other younger brother who has the same features as him.The peripheral blood film is as above.
Question 1 :
What does the peripheral blood film show ?
Question 2 :
What are the causes of these type of PBF ?
Question 3 :
What is the most likely cause for the above patient ?
Question 4 :
What investigations would you do to confirm your diagnosis ?
Question 5 :
How would you manage this patient ?
2 comments:
1. The PBF shows hypochromic, microcytic red blood cells with anisocytosis and poikilocytosis, nucleated red blood cell(erythroblast)and target cells.
2. (a)iron deficiency anemia
(b)Thalassaemia
(c)anemia of chronic disease
(d)sideroblastic anemia
3. Thalassaemia, because the patient is presented with classic thalassaemic facies(frontal bossing and prominent maxillary cheek bone), hepatosplenomegaly and family history suggestive of an hereditary disease.
4. (a)FBC
(b)Hb electrophoresis
(c)genetic tests of the alpha-globin cluster(PCR)
(d)iron studies TRO iron deficiency anemia
5. (a)Blood transfusion to keep Hb>9g/dl
(b)iron-chelators (desferrioxamine)
(c)splenectomy when transfusion requirements increase(patient should receive immunization against pneumococcal and Hemophilus influenzae B infection as well as penicillin prophylaxis)
(d)Folate supplements
(e)High doses of ascorbic acid
(f)Bone marrow transplant can be considered if there's a suitable donor.
(g)Gene therapy??
ps.have a enjoyable and safe trip:)
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