OB-GYN-L Messages for August, 2007: Re: anemia and neuro pt

Re: anemia and neuro pt
From: Terrence.Jones@kp.org
Tue Aug 28 21:27:25 2007

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It has been wild here as well, so I really never had much chance to participate in this thread (or any thread, for that matter). The "microcytosis" (chk spelling below) is concerning. We had a Patient with a 3-gene alpha globin deletion that behaved like this with pregnancy. There is an accumulation of beta globin tetramers (Hemoglobin H on cresyl blue), which result in oxidative RBC membrane damage. With further oxidative stress due to pregnancy, and the addition of pro-oxidant ferrous ions (given to correct the presumed iron deficiency anemia attributed to the low MCV), there results a hemolytic crisis. So I'd hesitate to say "not a hemoglobinopathy", just because of the HgE4 (electrophoresis).
We see alot of alpha thal (most common mutation in the population, worldwide; as early hemolysis, when infected with Plasmodium, may be protective [reduced replication]). SF has a robust Asian population. It is also present in Our Afro-American Patients, but the deletion is inherited as trans (so no risk of Bart's [4-gene deletion] to baby). The Asian deletion is cis, so two carriers have a potential problem. A single alpha globin gene deletion might go undetected by HgE4 and MCV. So a "normal" Parent might contribute a single gene deletion (silent), while the other contributes a 2 gene cis deletion. This leaves the offspring with only 1 functioning alpha globin allele (HgbH).
With 2 or 3 gene deletion the MCV will be low, with nml ferritin. The ratio of MCV/RBC (Mentzer index) is useful, as alpha thal usually has adequate numbers of RBC's, they are just small. A ratio < 13 would raise concern of alpha thal. I don't know that We have been given the RBC and MCV as yet in this thread? The most useful indices would be those prior to transfusion.
Not all carriers are Asian or Afro-American, the congenital deletion has also been identified in European ethnicity (case report from New Orleans, around 2003-4). And defects in X-chromosome activation of alpha globin gene may be acquired in certain myelodysplastic syndromes. Her improving Hgb postpartum might argue against this latter, and would raise the question of other, transient, acquired marrow related contributors. The one that comes to mind would be Parvo B-19. Might want to check IgM. Now back to work; first have'ta find Dante, and check which circle I was in... /tj
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"R. Daniel Braun" <rd.braun@gmail.com> Sent by: ob-gyn-l@OBGYN.net 08/28/2007 12:41 Please respond to ob-gyn-l@OBGYN.net
To Multiple recipients of list OB-GYN-L <ob-gyn-l@dns.obgyn.net> cc
Subject Re: anemia and neuro pt
Certainly not a Hemoglobinopathy.
On 8/27/07, Babycatchers@aol.com <Babycatchers@aol.com> wrote: It has been a wild time here, so I just couldn't get back on this patient. I finally talked to one of the referral canter docs that we have worked with before and then I got one of the neurologists. They said "we have no idea what is happening with her, but you will never get the heme/oc guys to admit it".
Her Hemoglobinopathy Heb soluability- neg hgb A-98.6 hgb S- 0 hgb C- 0 hgb A2-1.4 hgb F- 0
anisocytosis- slight poikilocytos 1+ microsytosis 3+ polychromasia 1+
She has not had any more unconscious 'spells' in the past 2 weeks and her hgb is actually up to 8.6. They are continuing the FE Iv for now.
Baby is fine. So all is well that ends well for us. I hope she does ok now.
Vicki Smith, CNM, MSN West Virginia
Vicki Smith, CNM, MSN West Virginia Midwives- changing the world one baby at a time.
Get a sneak peek of the all-new AOL.com.

-- R. Daniel Braun, MD FACOG(L) CMT Professor Emeritus Dept. of Obstetrics and Gynecology Indiana U. School of Medicine
R. Daniel Braun
"Science without Religion is LAME; Religion without Science is BLIND" Einstein 1941
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         It has been wild here as well, so I really never had much chance to participate in this thread (or any thread, for that matter). The "microcytosis" (chk spelling below) is concerning. We had a Patient with a 3-gene alpha globin deletion that behaved like this with pregnancy. There is an accumulation of beta globin tetramers (Hemoglobin H on cresyl blue), which result in oxidative RBC membrane damage. With further oxidative stress due to pregnancy, and the addition of pro-oxidant ferrous ions (given to correct the presumed iron deficiency anemia attributed to the low MCV), there results a hemolytic crisis. So I'd hesitate to say "not a hemoglobinopathy", just because of the HgE4 (electrophoresis).         We see alot of alpha thal (most common mutation in the population, worldwide; as early hemolysis, when infected with Plasmodium, may be protective [reduced replication]). SF has a robust Asian population. It is also present in Our Afro-American Patients, but the deletion is inherited as trans (so no risk of Bart's [4-gene deletion] to baby). The Asian deletion is cis, so two carriers have a potential problem. A single alpha globin gene deletion might go undetected by HgE4 and MCV. So a "normal" Parent might contribute a single gene deletion (silent), while the other contributes a 2 gene cis deletion. This leaves the offspring with only 1 functioning alpha globin allele (HgbH).         With 2 or 3 gene deletion the MCV will be low, with nml ferritin. The ratio of MCV/RBC (Mentzer index) is useful, as alpha thal usually has adequate numbers of RBC's, they are just small. A ratio < 13 would raise concern of alpha thal. I don't know that We have been given the RBC and MCV as yet in this thread? The most useful indices would be those prior to transfusion.         Not all carriers are Asian or Afro-American, the congenital deletion has also been identified in European ethnicity (case report from New Orleans, around 2003-4). And defects in X-chromosome activation of alpha globin gene may be acquired in certain myelodysplastic syndromes. Her improving Hgb postpartum might argue against this latter, and would raise the question of other, transient, acquired marrow related contributors. The one that comes to mind  would be Parvo B-19. Might want to check IgM. Now back to work; first have'ta find Dante, and check which circle I was in...  /tj   NOTICE TO RECIPIENT:  If you are not the intended recipient of this e-mail, you are prohibited from sharing, copying, or otherwise using or disclosing its contents.  If you have received this e-mail in error, please notify the sender immediately by reply e-mail and permanently delete this e-mail and any attachments without reading, forwarding or saving them.  Thank you. <table width0%> <tr valign=top> <td width@%>"R. Daniel Braun" <rd.braun@gmail.com> Sent by: ob-gyn-l@OBGYN.net 08/28/2007 12:41 <table border> <tr valign=top> <td bgcolor=white> <div alignÎnter>Please respond to ob-gyn-l@OBGYN.net</div></table> <td widthY%> <table width0%> <tr> <td> <div align=right>To</div> <td valign=top>Multiple recipients of list OB-GYN-L <ob-gyn-l@dns.obgyn.net> <tr> <td> <div align=right>cc</div> <td valign=top> <tr> <td> <div align=right>Subject</div> <td valign=top>Re: anemia and neuro pt</table> <table> <tr valign=top> <td> <td></table> </table> Certainly not a Hemoglobinopathy. On 8/27/07, <a href=mailto:Babycatchers@aol.com>Babycatchers@aol.com</a> <<a href=mailto:Babycatchers@aol.com>Babycatchers@aol.com</a>> wrote: It has been a wild time here, so I just couldn't get back on this patient. I finally talked to one of the referral canter docs that we have worked with before and then I got one of the neurologists. They said "we have no idea what is happening with her, but you will never get the heme/oc guys to admit it". Her Hemoglobinopathy Heb soluability- neg hgb A-98.6 hgb S- 0 hgb C- 0 hgb A2-1.4 hgb F- 0 anisocytosis- slight poikilocytos 1+ microsytosis 3+ polychromasia 1+ She has not had any more unconscious 'spells' in the past 2 weeks and her hgb is actually up to 8.6. They are continuing the FE Iv for now. Baby is fine. So all is well that ends well for us. I hope she does ok now. Vicki Smith, CNM, MSN West Virginia   Vicki Smith, CNM, MSN West Virginia Midwives- changing the world one baby at a time. <hr>Get a sneak peek of the all-new <a href="http://discover.aol.com/memed/aolcom30tour/?ncid=AOLAOF00020000000982" target=_blank>AOL.com</a>. -- R. Daniel Braun, MD  FACOG(L)  CMT Professor Emeritus Dept. of Obstetrics and Gynecology Indiana U. School of Medicine R. Daniel Braun       "Science without Religion is LAME; Religion without Science is BLIND"                        Einstein 1941 

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