Friday, 16 October 2020

A Rare One!

Quite a rare one here... I had to look it up! Pearson’s Syndrome.. it’s a Congenital Sideroblastic anaemia and Pancreatic dysfunction. I was trying to work out why this patient had macrocytic red cells and thrombocytopenia. Reading around this, it seems that these features are consistent with this form of sideroblastic anaemia. I usually associate sideroblastic anaemia with microcytes and dimorphic red cells and hadn’t appreciated that mainly macrocytes could be a feature and how many different types of sideroblastic anaemia there actually were. Reading about this, it seems that a good starting point in searching for the cause of sideroblastic anaemia is to look at the MCV. So MCV is important! Bottomley, S. Leung L. Tirnauer, JS. (2020) Causes and the Pathophysiology of the Sideroblastic Anaemias
I also saw these. Pappenheimer bodies, which are blue iron containing inclusions in the red cell, and often found near the edge , I usually scan for these when I have a patient with an unexplained anaemia and dimorphic red cells. A lot learnt today! I’ll be using this blood film in morphology training to demonstrate the diversity of red cell size in the Sideroblastic Anaemias.

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