Quiz: Hair-on-End Skull

I always enjoy the Image Challenge Contest at The New England Journal of Medicine, time ago there was a similar picture of this:

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U P D A T E: A nice comment and correct answer by Drmikeunoe

We can see generalized osteopenia, widening of the diploetic space, and thinning of the outer table.

This characteristic “hair on end” appearance of the outer table is compatible with the presence of massive hyperplasia of the bone marrow, as can be seen in the homozygous state of beta-thalassemia (i.e. thalassemia major).

Beta thalassemia syndromes are a group of hereditary disorders characterized by a genetic deficiency in the synthesis of beta-globin chains (adult hemoglobin usually is α2β2) resulting in excess α-chains and severe transfusion-dependent anemia, thus causing bone marrow hyperplasia.

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4 Responses to “Quiz: Hair-on-End Skull”

  1. Drmikeunoe says:

    We can see generalized osteopenia, widening of the diploetic space, and thinning of the outer table. This characteristic “hair on end” appearance of the outer table is compatible with the presence of massive hyperplasia of the bone marrow, as can be seen in the homozygous state of beta-thalassemia (i.e. thalassemia major). Beta thalassemia syndromes are a group of hereditary disorders characterized by a genetic deficiency in the synthesis of beta-globin chains (adult hemoglobin usually is α2β2) resulting in excess α-chains and severe transfusion-dependent anemia, thus causing bone marrow hyperplasia.

  2. Excellent explanation and perfect answer!!

    Thank you for your kind comment.

  3. [...] I know we all want “glowing hair” but this is amazing. Last week’s host, Dr. Jon Mikel, presents a “quiz” that stumped me (easily done, by the way). Check out Hair-On-End Skull at Unbounded Medicine. What is that stuff? And Jon takes it up-to-eleven with this post, where a photo is worth a thousand words. A bit of a stunner, check out Limb-to-Crotch! [...]

  4. Dr sutraj gupte says:

    We have seen “hair-on-end” appearance of skull in chronic iron-deficiency anemia, sickle-cell anemia, thalassemia major and cyantic congenital heart disease.

    – Prof Suraj Gupte
    Head
    Postgr Dept of Pediatrics
    Narayana Medical College
    Nellore, Souith India

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