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What Does My Trephine Biopsy Report Indicate?

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Posted on Mon, 12 Jun 2017
Question: My husband is 42 years old. Has been diagnosed with PMF. Here is the report.
TREPINE BIOPSY Surgical pathology no. S/2893/17
Specimen : Bone marrow biopsy
Gross : single linear piece measuring 1.0 cm. All processed in one block.
Microscopic examination : Section shows fibroconnective tissue cortical,
cancellous bone with
intervening cellular marrow displaying megakaryocytic proliferation with
atypia and clustering
at places. Megakaryocytes show abnormal chromatin clumping with
hyerpchromatic condensed
nuclei. Erythroid, myeloid cells are adequate. There is no lymphoid
cluster or granulomatous
lesion. Bone trabeculae are normal.
Reticulin : MF 1-2 (WHO 2016).
Impression : Chronic myeloproliferative neoplasm s/o Primary
myelofibrosis (PMF).
Advise correlation with JAK2 mutation and CAL R mutation analysis.

Hydroxeaurea has been prescribed.

His PMF is without any symptom as of now. I am concerned as he is sooo young.

Does the disease progress fast? Does he have only 10-15 years with us?
doctor
Answered by Dr. Hardik Sanghvi (1 hour later)
Brief Answer:
it will progress and prognosis depends on many factors.

Detailed Answer:
Hi, dear
I have gone through your question.
I can understand your concern.

He has primary myelofibrosis. It is commonly seen in middle aged and elder patients. In initial phase he may not have any symptoms. But in later stage he will develop symptoms. Prognosis depends on many other factors like treatment, age, symptoms other blood reports and many more.

Approximately median survival is 69 months.

Hope I have answered your question. If you have any doubts then feel free to ask me. I will be happy to answer. Thanks for using health care magic. Wish you a very good health.
Above answer was peer-reviewed by : Dr. Prasad
doctor
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Follow up: Dr. Hardik Sanghvi (14 hours later)
Hello Dr. XXXXXXX

The analyzing doctor we met showed us the DIPSS and the expectancy he calculated is 180 months.

The articles I have read all says somewhere between 69 months to 120 months.

Of course, as you can understand, my efforts will be to go beyond 180 months.

What all can I expect from the progress of the ailment? Can you please classify it as phases?

I know how one progress from phase 1 to phase 2 will be very very individualistic and cannot be predicted. But still I want to know the general trends or statistical details.

Some thing like -

phase 0 - no symptoms
Phase 1 - anaemia related sysmtoms may start occuring
Phase 2 - gout may start developing
Phase 3 - aches and pain all over the body as the smeared platelets have reached the organs and lack of oxygen due to lowered harmoglobin
Phase 4 - ....
Phase 5 - ...

Something like this.

Also - doctor has indicated that as soon as the risk increases for my husband, they will start analyzing for bone marrow transplant.

I tried looking at the statistics available for the bone marrow transplant for PMF patients but could not understand more of the literature available. An easy study can help me. Can you point out one for me?

Also - does a second test for bone marrow biopsy and MPN RELFEX PANEL recommended to be sure that he really does have this problem? From another hospital maybe and not the same one? (as you can see we all are finding it difficult to deal with the situation.)

Also - what about counselling session fro me and my husband on dealing with this situation? When do we go for these sessions. I feel right now is too soon.

Sorry for so many questions and a lengthy email. I hope you can spend time on my question.

Thank you once again for your answer so far. Looking forward to your answers to these questions.

Regards,
Anjali


doctor
Answered by Dr. Hardik Sanghvi (25 hours later)
Brief Answer:
Ofcourse there are different stages.

Detailed Answer:
Hi, dear
I have gone through your question.
I can understand your concern.

All cases are different and react different but in general stages are there.

First is early prefibrotic stage. In this there is moderate elevation of wbc and mild to moderate anemia. Patient may don't have symptoms.

Then comes fibrotic stage in which there is leukoerythroblastic blood picture. Tear drop cells and other picture. Patient have liver and spleen enlargement, weight loss, night sweat, anorexia etc.

Then comes osteomyelosclerosis stage. All symptoms are aggravated in this stage.

Then it can turn in to accelerated phase where it convert in to leukemia.

Stem cell transplantation carries good prognosis in young patient but it depends on many other factors and chances of complications are there.

If you want to reconfirm your diagnosis then you can go for slide review. No need of repeat procedure. Just review your trephine biopsy slight at other good hospital.

Counselling is required and there is no harm in that. It prepare patients for future symptoms. So we can avoid or treat at early stage.

Hope I have answered your question. If you have any doubts then feel free to ask me. I will be happy to answer. Thanks for using health care magic. Wish you a very good health.
Above answer was peer-reviewed by : Dr. Chakravarthy Mazumdar
doctor
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Follow up: Dr. Hardik Sanghvi (17 minutes later)
Thank you Doctor XXXXXXX This answers lot of my questions.

Going forward as I understand there are going to be monthly blood test (CBC only) to keep a check on White blood cell count and iron level.

Which all tests can show early stages of anemia?

Is monthly check up good for stage 0? Will we be able to keep a close tab on progression from stage 0 to stage 1?

So the difference between osteomyelosclerosis and earlier phase is severity of the symptoms?

Also - we will most likely go for bone marrow transplant before we hit osteomyelosclerosis. Right? Because if the symptoms worsen then we may not have better recovery? (Assuming we get good donor and no significant infection risk)

You have been a good guide. Thank you so much for your help so far.

Regards.
doctor
Answered by Dr. Hardik Sanghvi (7 minutes later)
Brief Answer:
Monthly CBC is advisable.

Detailed Answer:
Hi, dear
I have gone through your question.
I can understand your concern.

Yes monthly CBC is advisable. There are other differences in fibrotic stage and osteomyelosclerosis stage. That are in bone marrow. There is an increased amount of bone formation and it may occupy more then50% of bone marrow space. This all changes make your symptoms worsen.

Wish you all the best for stem cell transplantation.

Hope I have answered your question. If you have any doubts then feel free to ask me. I will be happy to answer. Thanks for using health care magic. Wish you a very good health.
Note: For further queries related to kidney problems Click here.

Above answer was peer-reviewed by : Dr. Remy Koshy
doctor
Answered by
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Dr. Hardik Sanghvi

Hematologist

Practicing since :2008

Answered : 7043 Questions

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What Does My Trephine Biopsy Report Indicate?

Brief Answer: it will progress and prognosis depends on many factors. Detailed Answer: Hi, dear I have gone through your question. I can understand your concern. He has primary myelofibrosis. It is commonly seen in middle aged and elder patients. In initial phase he may not have any symptoms. But in later stage he will develop symptoms. Prognosis depends on many other factors like treatment, age, symptoms other blood reports and many more. Approximately median survival is 69 months. Hope I have answered your question. If you have any doubts then feel free to ask me. I will be happy to answer. Thanks for using health care magic. Wish you a very good health.