Difference

Abbas,You are welcome

Unfortunately Carao is not available in Pakistan ,You can order it from www.tropicalhealthfoods.com ,It is kinda expensive for us as it is in dollars but it worked for my daughter and it has worked for lot of intermedias by helping them maintain their hb and prolong their transfusion intervals.

Zaini.

hi,

i had been at home so long, sorry for my absence..........

here is the CBC and hb report of Najaf:

Hb 7.1
hematocrit 26.6
RBC 3.22
MCV 82.2
MCH 22.2
MCHC 26.8

HB A2 1.5
hB F 98.5
WBC 14.7
NRBC 9/100WBC
Platelets 193


so we got Najaf checked, and doctor says it does not look to be the case of thalassemia instead it looks to spherocytosis, the doctor is very professional, experienced and is the president of Thalassemia Society of Pakistan. she asked for reticulocyte count and osmotic fragility for confirmation.

spherocytosis means that rbc are not of normal shape and are sphero type, because of this spleen takes them as abnormal cells and they are destroyed soon. her spleen is also a bit enlarged which is common for spherocytosis..it is caused by defects in proteins of cell membrane of RBC

now i have questions in my mind.......
can sphero cytosis cause regular hb drop as we have transfused Najaf 3 times in 3 months and hb was pretty low at around 7 every time?
can spherocytosis justify 98.5 hb F???
is it a combination of thalassemia and spherocytosis?

it is also to be noted that Najaf's body temperature especially of palms and forehead is high every time even in winter it looks as though she has just come from the heater.  in september she had very svere fever for almost 10 days and then she got 2nd ever (first in 5,6 after birth) transfusion, even after this tx she got fever again ( i wrote this may be its of some help)

Abbas

Abbas,

I am sorry i don't know much about spherocytosis,but why would her HB F be so high if she doesn't have thalassemia?  May be Andy would be able to shed some more light on that.

Zaini.

Abbas

We had a post here about spherocytosis, i will try to find it and send you the link. 

All i know is that it is recommended for those patients to do splenctomy after the age of 5 because this stops the destruction of red blood cells and eliminates transfusion

manal

I found this one post about spherocytosis,this is not much,

http://www.thalassemiapatientsandfriends.com/index.php?topic=780.msg5911;highlight=spherocytosis#msg5911

The thing is you'll get the clear picture after you do those tests Dr Javeria suggested,so keep us updated with the results.

Zaini.

This latest electrophoresis report has a major difference from the previous report.

http://www.thalassemiapatientsandfriends.com/index.php?topic=3181.msg32057#msg32057

previously a doctor in multan took some tests and i think electrophreseis too
i m stating some of her results if u could understand
Hb A2 2%
Hb F 55.6%
Hb A 42.34
global chain variant = N/C
possible genotype B+/B+
VDLC/ TLC=18400
Poly 37%
lympho 56%
retics 18.8%
platelets 140000
normoblasts 16

below is the quotation by the testing lab

" severe hypocloromia, microsytosis. moderate macrocytosis, anisocytosis, poikilacytosis, many spherocytes, pessay cells, target cells and normoblasts.

http://www.thalassemiapatientsandfriends.com/index.php?topic=3206.msg32872#msg32872

here is the CBC and hb report of Najaf:

Hb 7.1
hematocrit 26.6
RBC 3.22
MCV 82.2
MCH 22.2
MCHC 26.8

HB A2 1.5
hB F 98.5
WBC 14.7
NRBC 9/100WBC
Platelets 193

Spherocytosis would not cause a fetal hemoglobin level this high. It is normally a few per cent and at the most 15-20%. The difference in the two reports concerning HbA and HbF are inexplicable, so someone has made a mistake somewhere. 42% HbA wouldn't suddenly disappear. There have been rare cases reported where thal major and spherocytosis co-existed (  http://www.turktel.net/cgi-bin/medshow.pl?makale_no=56327 ). Beta thal major is the only explanation for zero HbA and over 90% HbF. The previous report looked more like thal intermedia (B+, B+), with possible spherocytosis. This new report makes little sense, so I would suggest that they have the electrophoresis test done at another lab for comparison sake.

I would say there is a strong chance the child will need regular transfusions. If it is spherocytosis alone or combined with thal, a splenectomy may be considered. Splenectomy might eliminate the need for transfusions if it is spherocytosis. Both thal and spherocytosis call for folic acid supplements, so make sure the parents are giving a daily dose of folic acid.

Andy,

i was also thinking on the same lines, only spherocytosis cant cause such a high HbF, either it is a combination of thalassemia and spherocytosis, or some mistake has been made in reports.
but the 2nd report is from the most authentic lab of Pakistan ( the Aga Khan University Hospital, anyhow we will do electropheresis again
yes, she is taking folic acid right from the beginning
Pray that Najaf has only spherocytosis.....

Abbas

Abbas,

If the second test was done by Aga Khan,i'll go with that,and high Hb F indicates thal major,but do repeat electrophoresis along with the tests dr Javeria suggested if you can,this will give a clear picture.

Good luck and praying for Najaf,

Zaini.

Andy,

But how can Najaf be a thal major and she only tansfused 3 times during her past 4 years???

manal

Manal,

This is why I think the earlier test was accurate and this current test cannot possibly be correct. Zero HbA results in regular transfusions. This looks more like the thal intermedia that was originally diagnosed, with the possibility of co-existing spherocytosis. Whoever did this second test, made a mistake. It can't be 90%+ HbF and not be regularly transfusing unless there is also co-existing hereditary persistence of fetal hemoglobin (highly unlikely). I don't think we'll know anything for certain until further results are obtained. If it is intermedia with spherocytosis, a splenectomy may remove all need for transfusions.

Hi,

Andy and Manal thanks for having a close look on reports.....
May second report is incorrect...
We are going to have tests very soon.

Abbas

Thanks Andy for your reply.

Abbas,
I wish her all the best

manal

Hi,

Najaf's Osmotic Fragility is going to be done today, and appointment with doctor is also due today.....
till today's evening everything will be decided....

keep ur fingers crossed

Abbas

Good luck Abbas,we are praying for Najaf .

Zaini.

an update,
Najaf's retic count is 12. will some one shed some light on that???

Abbas