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The child born on 4/12/92.

Refered to hematology service on 3/1/94 because of

hypochromic-microcytic anemia.She is being followed by cardiology

service for very small CIV.

The child was without complains,and the physical examination whitout

alterations whit normal growth.

The past history and family are irrelevant and without consanguineous

couples.

She had anirratic {*filter*}iron therapy.

The laboratory analisys showed:         hgb=6.3 g/dl

                                        mcv=55,8 ft

                                        mch=12.9 pg

                                        mchc=26.7g/l

                                        rdw=20.4%            

                                        leucocytes and platelets =normal

                               iron=7.1 micromol/l  tibc=66

micromol/l    ferritin=23,6microg/ml

Despite the normal value of the ferritin,the hematologic picture was

interpreted as iron deficiency anemia and

treated during 20 days whit {*filter*}iron at the dose of 5mg/kg/day during

hospitalization to be well controled therapy.

After exclusion of beta-talassemic,sideroblastic anemia and lead

intoxication and the iron deposits present but scarcity in the bone

marrow aspiration,determined the parenteric therapy.

For calculated dose of 200 mg(for correction of hemoglobin and

reposition of stocks)a total dose of 130 mg was given and the response

was the followed:     hgb=8,5    mcv=55   mch=13.7   mchc=24.6  

retics=2%

                                             iron=7,4   tibc=65  

ferritin=61

Evolution:

From june 94 to february 95the hemoglobin was down to 6.5,the mcv was

mantained alwais 60,the serum iron was alwais very low,the total

iron-binding capacity was around 60 and the ferritin was down from 61 to

24.

In february 95the child was treated whit intravenous iron at the dose of

60 mg(calculated according to hgb results).The response was incresed

hgb,in two months,from 6,5 to 8,5 the mcv was mantained in 60 and the

ferritin increased from 24 to 65.

From march 95 to july 96 the child was without therapy,the hgb decreased

from 8,5 to 7 the mcv from 60 to 55

and the ferritin from 65 to 11.

In july 96 was treated with 237 mg i.v. iron (for a total to refill the

stocks to 366mg).The total dose was not given because of elevation of

temperature as the result of a abscess at the elbow fold and dificult

vein access.The ferritin was up to 150(during the previous therapy was

not above 65)the hgb up in 1,5 gr and the mcv to 65.

Other laboratory exams done:    ocult {*filter*} investigation    alwais neg.

                                cintigrafy to investigate Meckels

diverticulum   neg.

                                nuclear magnetic ressonance to evaluate

the levels of deposit of iron in parenchymatous organs   neg.

                               investigation of hgb H neg

                               hgb F,hgb A2 electrophoresis of hgb  

normal

                               acute phase reactants(including

sedimentation velocituy)normal

                               lactate dehydrogenase,alkaline

phosphatase   normal

                               hepatic and renal chimestry   normal

                               imunoglobulins   normal

                               hiv1,hiv2 neg.

                               pulmonar xray and abdominal eco    normal

                               cd4/cd8=3,3

                               free erythrocyte protoporphyrin=17,8

microg/g hgb (1,2-3,3)

                               marrow normal celularity whit normal

relative proportion of erytroid to mieloid cells.Hemosiderin present but

scarcy.

 Interpretation:

4,5 year young child was found to have hypocromic-microcitic anemia.

was interpreted as iron deficiency anemia and after various tentatives

of corretion with {*filter*}and parenteral iron had parcial response.

Does not have cronic disease because clinicaly was not obvious with good

growth for the age and the acute phase reactants were alwais negative.

The parameters related to the iron status were always contraditive.The

normal or elevated ferritin was always contrary to the very low iron in

the serum the tibc and the imunologic dosage of transferrin were always

normals.The low transferrin saturation were always at the cost of very

low iron in the serum.The dosage of FEP is 5-6 times superior to normal

which suggest erithropoieses severly depleted in iron.

We have tried the i.v. iron therapy always with fear of its accumulation

in parenchymatous organs.At the moment we do not have any evidence of

any accumulation.

Is this a new entity?

what to do?

continue the treatments whit total doses or even superior doses?

other interpretation?

TNHANK VERY MUCH FOR YOUR ATTENTION.