M. Paetzel (Cleveland/USA)  

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9 Years  

Female  

Head-Bones  

congenital  

9 year old girl, paleness, hepatosplenomegaly  

Autosomal hereditary hemoglobin defect with a resulting hypocromatic, microcytic anemia. Through hemolysis is an iron overload common. Extensive medullary and extramedullary bleeding.  

<- view X-Ray 1

X-Ray 1: Lateral View



<- view X-Ray 2

X-Ray 2: Cranium (extensive): Macrocephaly with widend diploe, thinned tabula externa and subperiostal spiculae. Holes in the tablua externa re possible.




<- view X-Ray 3

X-Ray 3: Hand: Trabelces are affected in the metacarpals. Widening of the bone marrow and thinning of the cortical zone through the hypertophied blood-producing marrow..

Further typical changes of the increased erythropoeisis is seen in the ribs, facial skeleton and in the vertebral bodies are possible not shown.

In the MRI (T2): Signal reduction in bone marrow, in the liver and in the spleen (iron deposition).


 

Genetics  

Other hemolytic anemias (spherocytosis, enzyme defects)  

N/A  

N/A  

N/A  

N/A  

Paleness, hepatosplenomegaly, autosomal, hemoglobin defect, microcytic, anemia, Thalassemia intermedia, child, childhood, pediatric radiology  

M Paetzel. Thalassemia intermedia. PedRad [serial online] vol 2, no. 1.
URL: www.PedRad.info/?search=20020126181655