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What Are The Chances For Recurrence Of Pure Red Cell Aplasia?

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Posted on Mon, 20 Oct 2014
Question: Sir/madam:
I am writing this to inquire about a situation where a girl has been treated for pure red cell aplasia for 15 years as she represented progressive pallor and was off for treatment since 2007 and the doctor certified that she is normal. Now, is the girl fit for marriage and what is the chance that the problem can show up again and what is the chance that the child too may get affected by the same problem?
doctor
Answered by Dr. Rahul Tawde (2 hours later)
Brief Answer:
Benign course with excellent prognosis

Detailed Answer:
Thanks for posting the concern in the HCM.
Primary PRCA (pure red cell aplasia) that appears in childhood is usually benign has excellent prognosis. They are often reversible with treatment; and that has occurred in the case of this girl too.
However, there are a few important prognostic factors. PRCA is notorious for relapses. Relapse rates and also prognosis vary in accordance with the type and treatment.
Secondly, steroid is the mainstay of treatment. So, one has to bear with the long term and short term adverse effects of steroid therapy.
Thirdly, chance of infection is more among these patients.
Fourthly, PRCA can evolve into aplastic anemia and acute myelogenous leukemia, which have high morbidity and mortality rates.
and lastly another important treatment related issue is that the patients can develop hemosiderosis due to repeated transfusion.
Now, regarding your questions I would say,
1. No human being is unfit for marriage if he/ she is capable of having intercourse. If that is so, this girl has no issues with marriage.
2. But, regarding inheritance I would say that the child may get affected, but the chance is small. The entire genetic factor responsible for the disease is not clearly known. But, deletion of ribosomal gene is considered responsible. The genetic transmission is therefore not well known. But, a small proportion of the disease which appears in childhood runs in the family.
3. I have already told about the chance of relapse and important prognostic issues related to it.
Finally, I would say the girl should be counseled for HIV testing prior to marriage and karyotyping of both the partners are necessary as well.
Hope, I have successfully answered your question.
If you are satisfied, please close the thread and rate my answer.
Regards.
Above answer was peer-reviewed by : Dr. Shanthi.E
doctor
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Follow up: Dr. Rahul Tawde (4 hours later)
Sir:
Thanks for the information and your description is very informative.
What is the chance that the problem can repeat again and what has to be done if at all the problem arises again?
The relatives of the girl say that it was a problem once upon a time and is no longer a problem now an there is no chance that the problem can show up again is what the doctor told it seems.
My mother says that a doctor said that the girl is not fit for marriage an the children if any will also be affected because of this problem and may affect me too. Is there any chance that I maybe affected by the same problem?
I would have taken proper care if I had got the report before marriage and we have been married a year back and got that report 6 months after marriage.
Is there any connection with the treatment and mental bahviour of the girl as she usually is very strong with very little love & affection. My mother says that it must have been because if the treatment undergone as the per the source if another doctor.
I am not at all in a situation to decide something on this. My mother is of the opinion that their family just cheated us as the girl is behaving something different from the usual. She is just leaving my house whenever she likes after creating some problem here.
Please kindly help me and I beg you with folded hands to suggest me like a frien whether I can lead a happy life with her as I do not much on this subject and have to be based completely on what doctors like you say.
My mother says another doctor told the way I mentioned above that the girl is unfit for marriage and shouldn't get married an children will also he affected.
It has become a very sensitive issue for me sir as the marriage is over.
Hope to hear from you soon sir.
Thank you.

doctor
Answered by Dr. Rahul Tawde (3 hours later)
Brief Answer:
Fight the stigma, give social support

Detailed Answer:
Hi, thanks for your reply.

As the condition is very rare and has several subtypes and diverse etiologies, particular relapse rates are not available in the literature. However, researchers and specialists have found that the relapse rate of the disease is high.

Immunosuppressive therapy and corticosteroids are particularly helpful for relapses. And, as I have mentioned earlier, stem cell therapy has been showing some ray of hope for refractory cases.
Regarding your mother's expression, I feel she has been over-apprehensive thinking your future. Please make it sure that PRCA can occur via Parvovirus B19 infection, which transmits from acutely ill patients droplets and blood transfusion. Your wife has got a chronic PRCA. You cannot be infected from that. Secondly, marriage in XXXXXXX is not consummated by childbirth and women are not machines for bearing child. So, a PRCA patient has every right to get married. And, the patient's family actually hid the issue because of this social stigma. As the husband of this patient, it is your responsibility to fight this stigma. And believe me this is one of the most noble jobs in a country like XXXXXXX where women empowerment is still a remote practice.

I think the disease has got some effect on the mental status of the patient. I have already explained that in the previous thread. This actually arises from learned helplessness. A good social support is the key. I have also mentioned the remedy for this problem.

Now, a few key advice for a better life-
1. Both of you should consult a clinical psychologist or a person trained in CBT.
2. Please isolate the patient from unsupportive social environment, either by staying with your wife only or having a clear talk with everyone in the household regarding the support that she needs.
3. There is no issue like cheating here. The girl was legally capable of marrying and you married her. It is completely unethical and inhuman to think that one cannot marry a patient of debilitating illness.
4. Consult a good hematologist for regular follow up of your wife and continue with his/her advice.

For any further direct help regarding behavior change communication and cognitive behavior therapy you may also take my appointment.
For any further information, write back to me directly.
If you find the ans helpful, please close the thread and rate my answer.
Regards.
Above answer was peer-reviewed by : Dr. Vaishalee Punj
doctor
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Follow up: Dr. Rahul Tawde (1 hour later)
Sir:
Thank you very much for your response. It helps me a lot and is informative, supportive and of inspiration.
Would like to have your contact details so that I can contact you if at all I need any help in this regard.
Thanks.
doctor
Answered by Dr. Rahul Tawde (11 minutes later)
Brief Answer:
Detailed answer below

Detailed Answer:
You can post your query directly to me by clicking the below mentioned link-

https://www.healthcaremagic.com/Funnel?page=askDoctorDirectly&docId=68460

If the moderator allows to post the email, you can e-mail me in the id-
YYYY@YYYY
You can also take my appointment by customer care of HCM.

Finally, I would personally thank you for marrying a woman with rare disease, providing her immense support and hope you two would find a very happy life together.
Also, to make your effort more effective and help others around the world, you yourself can start a blog regarding the stigma, treatment and prognosis of this disease. You can also get relevant authentic information regarding the disease from Medscape and scholarly articles posted in google.
Please educate yourself and support your wife and spread your knowledge. Believe me, at the end of the days you will find a satisfaction that you cannot buy with money.
If you have found my answers helpful, please close the thread and rate my answer. Please write back to me directly whenever you have any query in future.
Regards.
Note: Do you have more questions on diagnosis or treatment of blood disorders? Ask An Expert/ Specialist Now

Above answer was peer-reviewed by : Dr. Vinay Bhardwaj
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Answered by
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Dr. Rahul Tawde

General & Family Physician

Practicing since :1980

Answered : 1 Question

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What Are The Chances For Recurrence Of Pure Red Cell Aplasia?

Brief Answer: Benign course with excellent prognosis Detailed Answer: Thanks for posting the concern in the HCM. Primary PRCA (pure red cell aplasia) that appears in childhood is usually benign has excellent prognosis. They are often reversible with treatment; and that has occurred in the case of this girl too. However, there are a few important prognostic factors. PRCA is notorious for relapses. Relapse rates and also prognosis vary in accordance with the type and treatment. Secondly, steroid is the mainstay of treatment. So, one has to bear with the long term and short term adverse effects of steroid therapy. Thirdly, chance of infection is more among these patients. Fourthly, PRCA can evolve into aplastic anemia and acute myelogenous leukemia, which have high morbidity and mortality rates. and lastly another important treatment related issue is that the patients can develop hemosiderosis due to repeated transfusion. Now, regarding your questions I would say, 1. No human being is unfit for marriage if he/ she is capable of having intercourse. If that is so, this girl has no issues with marriage. 2. But, regarding inheritance I would say that the child may get affected, but the chance is small. The entire genetic factor responsible for the disease is not clearly known. But, deletion of ribosomal gene is considered responsible. The genetic transmission is therefore not well known. But, a small proportion of the disease which appears in childhood runs in the family. 3. I have already told about the chance of relapse and important prognostic issues related to it. Finally, I would say the girl should be counseled for HIV testing prior to marriage and karyotyping of both the partners are necessary as well. Hope, I have successfully answered your question. If you are satisfied, please close the thread and rate my answer. Regards.