Splenomegaly in 3rd Trimester Pregnancy

Hello all!

My name is Courtenay, and I am currently 35 weeks pregnant.  I have B-thal minor, and when I was 30 weeks I was suddenly struck with very bad fatigue, vomiting blood, and bleeding under the skin.  I had a CBC done, and my hemoglobin and hematocrit were so low they did three transfusions...During this time, I kept telling them my left shoulder and abdomen hurt, but they didn't think to check my spleen until last.  It had swollen to 19 cm.

My OB and I are currently in process of talking with hemotologist to find out if I should even be allowed to go into labor, due to the splenomegaly.  I guess a couple women died of splenic rupture in pregnancy due to this, but they had b-thal-minor plus hbC-trait.  I've never been tested for hbC-trait before.  Their blood reports were almost identical to mine.

Does anyone here have experience with a swollen spleen during pregnancy, and if so, what did your OB/hemotologist suggest to be done?

Hi from down the thruway in Rochester, Courtenay,

Have you now been checked for HbC trait? If you have, please make sure the doctors have seen this article.

From http://arpa.allenpress.com/arpaonline/?request=get-document&doi=10.1043%2F1543-2165(2006)130%5B1231:FSRIAP%5D2.0.CO%3B2

Double heterozygosis HbC/p-thal is a rare condition that causes hypochromic microcytic and hemolytic anémia, mild jaundice, and mild splenomegaly....Our case seems to indicate that the patient's pregnancy suddenly worsened preexisting hypochromic microcytic anemia and triggered red blood cell hemolysis. On the basis of the autopsy, laboratory, and anamnestic data, we offer the following tentative explanation of her clinical history.

The β-thalassemic trait and unstable HbC were responsible for her well-compensated anemic status, but the hemolytic anemia was suddenly worsened by pregnancy and triggered massive extramedullary hematopoiesis, giving rise to splenomegaly and finally, splenic rupture with massive intraperitoneal hemorrhage and death.

The explanation here may be the first to hypothesize what happens in these cases. This is a very critical time and they must make a decision soon, as you may have realized from the other cases, that your life may be in danger if you give birth. I don't want to say any of this to alarm you, but the doctors need to know exactly what is happening with your blood and spleen as soon as possible. Please print out the whole article and show it to the hematologist.

Although most doctors will tell you that thal minor has no symptoms, we have been hearing quite a few reports of problems as minors age, and especially problems during pregnancy, sometimes even requiring transfusions. It may well be up to the patients to educate the doctors about thal minor because there has been very little effort by researchers concerning thal minor.

Hi Courtenay,

I forgot to ask if the doctor had said whether or not splenectomy is an option at this stage of the pregnancy. The main danger here seems to be from rupture.

I would like to add my own guess as to what is happening in these cases. The pregnant woman's body has a higher blood demand while pregnant and the quality of red blood cells (RBCs) being produced is nowhere near optimum causing increased activity of RBC production in the bone marrow and possibly elsewhere (extramedullary hematopoiesis). This is a mass that develops and creates RBCs. It is often found in thal intermedias who don't transfuse and even in majors, as our member baal has experienced, but because it is still that person's blood, it is the same quality that the body is working to replace, and so the blood is of little value. This excess blood production is then being destroyed by the spleen, resulting in the rapid enlargement. This all happens because the hemoglobin level of the mother suddenly drops during pregnancy. Because of this, the ultimate treatment may be to identify carriers of both the beta thal trait and the HbC trait, and then closely watch their Hb levels during pregnancy and immediately begin a transfusion program for the length of the pregnancy if needed. Hypertransfusion may be necessary in extreme cases. The better quality transfused blood will prevent the excess RBC production, which in turn will prevent the excess destruction of RBCs in the spleen and the resulting enlarged spleen.

Hi Andy! Its great to know that you're down the road.

My dad is supposedly negative for b-thal trait, but i guess in the early 80's when i was born they either didn't know about or just didn't test for HbC.  They're running the test tommorow on both him and my mom, who has b-thal minor. they're also testing her for HbC tommorow b/c she had some problems last year for a couple months due to a swollen abdomen with no results from tests...naturally, they didn't check her spleen...

I think you are right about thal-minors really needing to educate docs that some of us have symptoms like mild thal-intermedias.  I don't know why they seem so insistent that because its rare, it doesn't ever happen.

 I have that particular autopsy bookmarked, and from what i can surmise, i agree with your deduction of what may be happening.  I had the shoulder pain before the transfusion...so i've tried to explain to them i doubt it was the transfusions themselves that made my spleen freak and turn on the rbc's.

Thank you so much for your quick reply. i'll keep you updated when i go to the hemo.

I'm amazed that the spleen wasn't checked with you or your mother, especially since a physical exam alone can detect enlargement.

There are so many different variations of the hemoglobin genes being discovered all the time, and these combinations with beta thal are creating havoc in people's lives but for the most part, are not yet being detected until a serious problem occurs, if at all. In most cases, people are told they are beta minors and don't worry. Even beta minor by itself varies from no significant problems to transfusion dependent intermedia. When combined with genes such as HbE, beta can become as severe as major for some patients, requiring transfusion from a very early age. There are so many variations that are not tested for that can add to the severity of the thal conditions and researchers are just touching the surface of understanding what different mutations and combinations of mutations mean to a patient's health.

Hi Courtenay,

The doctors are on top of this situation and they are doing exactly the right things. The doctors from Strong are a good group and will be a definite asset to the team. The founder of our group, Lisa, took her treatment at Strong, and their long history working with thal is a major plus.

If there is any danger of rupture, the spleen will be removed. 19 cm is actually quite large and usually indicate removal is necessary. However, in your case, this is a temporary condition, and if it isn't necessary, it won't be removed, as an added surgery at this stage will be avoided if possible.

This case is a lesson for all of us. Fortunately, you are under the care of doctors who understand what it going on and who are also quite willing to bring in outside help for advise on the case. In many situations this would not have happened and there would be a great danger to both mother and child and that just is not acceptable. I didn't know a thing about HbC trait until you posted but I found the relevant information on the internet. The internet is an incredible resource and we have to train our doctors to use it. The information I posted earlier in this thread is very new and most doctors would have no way of being aware of it unless they have searched very recent medical journals which are also mostly available online. Having this current information in this case can mean the difference between life and death for both mother and baby and it is absolutely necessary that all doctors everywhere are making this information available to themselves. I often hear excuses about doctors in some areas not having adequate resources, but the only resource necessary to understand what is going on here is internet access, which doctors everywhere have access to. We have to constantly encourage the medical professional to take advantage of all resources available. Regardless of a lack of any other resources, the doctors must at the least avail themselves of all readily available information and use it the best they can when treating patients. I have been very dismayed in the past to talk to doctors from around the world who are not familiar with information about thal treatment that is readily available if they only will look. I'm not a doctor or even a health care worker but I take advantage of all the information I can find to increase my knowledge of thal. Should you expect any less from the trained professionals treating you or your family and friends?

Courtenay, our prayers will be with you throughout. I have confidence you are in good hands and that all the right decisions will be made. Please get word to us a soon as you can after the C-section. You're one of our family now, and we'll be anxiously awaiting word.

God be with you.

Dear Courtenay

I am sure you will pass all this and you will enjoy your coming baby. I will pray for you and for the baby.
The important thing is to try to relax and know that you are in good hands.  Keep us updated as soon as you can .
Wish you all the best.

Take care

Manal

Hello everyone!

Thank you so much for all your thoughts and prayers.  We welcomed Mychael Christopher into the world at 11:53 am on Monday Nov. 20, 2006.  The hemotology team decided not to remove the spleen, and monitor it with ultrasounds.  The u/s done the day after delivery was still 18-19 cm. so they weren't too happy.  I'm still having shoulder pains, but hopefully the spleen will reduce in size as the weeks progress.  They had to give me nitrous (goody!) during surgery, so there's no good pics of Mychael and I (I look like a dope addict! LOL) But here's a cute one of my daughter Karol meeting him, and a good face shot.

I'll be in touch after my follow up appt on Tuesday morning, and we'll see how much the spleen's gone down, if any.  My CBC's didn't warrant another transfusion..."yet," they say. Ugh!

Courtenay,

Congratulations to you and you family!      

I am so happy that all went well. I've been thinking about you all week and almost jumped out of my chair when I saw there was a new post from you. 

This is the most wonderful news!!! 

Congratulations Courtenay :hug


I am soooooooooooooooo happy for you and for your family. You finally did . Thank God you and the baby are well. Your kids are very cute

Take care                        :five

Manal

Congratulations, Courtney! You did it! I'm so glad things went ok for you. What beautiful children you have.      Jean

We often talk about doctors who seem too busy or who don't seem to care, so it's only fair that we give a huge thanks to all the doctors and medical staff that were involved in Courtenay's care and delivery. This was an extremely dangerous situation that can threaten the lives of both mother and child, and it was handled with the utmost seriousness and professionalism.

Thank you all! 

:five

Yes, definitely! Dr. Roaman (Strong Hematology) and Dr. Cummings (OB) and all the great staff at St. Josephs Hospital in Syracuse did wonderfully. 

He is sooooo cute!!

:five


Take care, Peace!