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Diagnosed with hereditary spherocytosis, kid has gushing nosebleeds, severe headaches, fevers. Same symptoms ?

Hello. I was diagnosed with hereditary spherocytosis in my late 30s following a 3rd pregnancy that brought the HS from mild & undetected to severe. I was splenectomized and have not received a transfusion in two years. I had all 3 kids tested last year - all 3 negative. However, unless they had spherical cells present, wouldn t they test negative? I ask because I see similar early signs in my older children - very active, otherwise healthy 14 yo son has massive, gushing nosebleeds and severe headaches , and my daughter age 11 in April is very small (50lbs), gets many unexplained fevers, and tires quite easily. Should I have them checked again or no? At last check, both were slightly anemic but MCV low.
Asked On : Fri, 22 Feb 2013
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Pathologist and Microbiologist 's  Response
Hello and welcome to HCM,
Hereditary spherocytosis is a hereditary disorder which is transmitted as autosomal dominant trait or autosomal recessive trait.
It will not manifest in children when transmitted as autosomal recessive trait.
A peripheral blood smear examination shows more than 50% spherocytes.
Hereditary spherocytosis is confirmed by osmotic fragility test.
Anemia with low MCV could be due to iron deficiency anemia.
What is the status of MCH, MCHC, RDW and reticulocyte count?
The result of all these parameters will determine whether there is any evidence of spherocytes in your children since MCV is low in spherocytosis also.
Osmotic fragility test should also be done to confirm or rule out spherocytes.

Thanks and take care
Dr Shailja P Wahal

Answered: Wed, 21 Aug 2013
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