(Biermer anemia)

Megaloblastic anemia associated with neurologic and cutaneous disorders. In addition to the congenital causes (see cobalamin deficiency and Ismerlund disease), the acquired causes are:

-        absorption deficit of vitamin B12 caused by:

*         insufficient gastric acidity: one of the main causes of megaloblastic anemia in old patients; beware in case of prolonged intake of a proton pump inhibitor (omeprazole)

*        an autoimmune disease: Grave's disease, thyroiditis, vitiligo... where autoantibodies directed against the intrinsic factor can be found

*        a chronic intestinal condition: Crohn's diease, ulcerohemorrhagic colitis, gluten intolerance before a poor gluten diet is followed, malabsorption consecutive to chronic pancreatitis

*        digestive surgery: gastric surgery (including some bariatric procedures) or large resections of the small bowell

*        metformin intake: it increases the risk of deficiency

-        food-borne vitamin B12 deficiency: in case of strict vegetarianism (veganism), especially in countries where vitamin B12 supplements are not available

Clinical signs:

-        progressive anemia

-        neurologic disorders: walking disorders, numbness of the lower limbs (deep sensibility and proprioception disorders), pain

-        cutaneomucous disorders: angular cheilitis (perleche), glossitis, skin hyperpigmentation (joints, flexion creases)

-        mental disorders: mood changes, depression, sometimes dementia

Treatment: I.M. vitamin B12

NB: folic acid deficiency causes the same hematological picture but without neurologic signs.

Anesthetic implications:

contraindication to the use of the N2O; check the blood count; management according to the neurologic symptomatology

References : 

-         Apporva PS, Shubba STK, Rajakumar PS. 
Infantile tremor syndrome. 
Int J Contemp Pediatr 2017 ; 4 : 2228-30.

-        Sharawat IK, Kasinathan A, Sankhyan N. 
Infantile tremor syndrome: response to B12 therapy. 
J Pediatr 2018; 196:323

-        Agarwal A, Saini AG, Attri S.
Reversible hyperpigmentation and paraparesis: a simple remedy !
J Pediatr 2018; 201: 294.

Updated: January 2019