Biology of Disease

THROMBOCYTOPENIA AND THROMBOCYTOSIS

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Figure 13.29 Family tree to show the inheritance of the hemophila A gene in descendants of Queen Victoria.

CASE STUDY 13.1

Daniel, a four-year-old child of Jamaican origin, was

brought into the hospital because of frequent severe

headaches and abdominal pain. He had also been tired

and sleepy for several months. His sclerae were yellow

and abdomen distended. Palpation revealed an enlarged

spleen. His blood samples give the following data

(references ranges in parentheses):

Erythrocyte 2.2 s 1012 dm–3 (4.4 

5.9 s 1012 dm–3

count (men))

Hemoglobin 47 g dm–3 (140–180 g dm–3)

Serum bilirubin +++ (±)

(unconjugated)

A fresh smear of blood showed a few crescent-shaped

cells and so an electrophoresis strip was run of hemolyzed

erythrocytes from Daniel and both his parents, with the

following result:

OriginOrigin

+

Father Mother Daniel

Juan Carlos

King of Spain

Female

Unaffected male

Affected male

Carrier female

Queen

Victoria

Prince

Albert

Princess King Edward VII

Alice

Louis,

Grand Duke

of Hesse

Nicholas II Alexandra

Tsar of Russia

Alfonso XIII

King of Spain

Queen

Ena

British

Royal family

German

Royal family

Princess

Victoria

Frederick

III

Princess

Beatrice

Prince Henry

of Battenburg