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What Causes Myeloproliferative Neoplasm?

Posted on Sat, 28 Nov 2015

Question: Dear Doctor, If a person has an acute transformation of overlap myeloproliferative/myelodysplasticity disorder then what sort of pathological evidence would one expect to find, for example, a high WCC? a low PLT? a high ALP? a high or low level of neutrophils, lymphocytes, metamyelocytes, MCV, MCH, RCC, RDW, and so on??

Answered by Dr. Shailja Puri (33 minutes later)

Brief Answer:
Acute transformation means increase in white blood cell count and blasts.

Detailed Answer:
Hello,
Thanks for posting your query on HealthcareMagic.

Acute transformation of myeloproliferative / myelodysplastic disorder is typically characterized by increase in white blood cell count and more than 20% blasts.
There can be fall in hemoglobin and fall in platelets.
There can be an increase in serum LDH but it largely depends on the clinical condition of the patient.
Level of neutrophils, lymphocytes, eosinophils and basophils, MCV, MCH, red cell count and RDW cannot be predicted or generalized.
You can post your report for more information.

Thanks
Dr Shailja Puri

Above answer was peer-reviewed by : Dr. Prasad

Follow up: Dr. Shailja Puri (24 hours later)

Thank you Dr Puri. I am very appreciative. There is no need for me to send my report to you since your answer is very helpfully detailed. But may I ask one more question, although I do not know whether it can be easily answered? If someone has overlap myeoloproliferative/myelodysplasticity disorder for a couple of years then how quickly could the condition turn into an acute transformation? For example, could it happen over a period of 10 days, a month or two months?

Answered by Dr. Shailja Puri (19 minutes later)

Brief Answer:
Prognosis depends on type of MDS/MPN disorder,genetics & clinical features.

Detailed Answer:
Hello and welcome again,
The prognosis, transformation to acute leukemia and survival depends on the type of myelodysplastic / myeloproliferative disorder (MDS/MPN disorder).
There are various types of myelodysplastic / myeloproliferative disorders.
In addition, the prognosis also depends on the type of chromosomal anomaly.
But generally acute transformation occurs in 15-40% patients of myelodysplastic / myeloproliferative disorder.
The acute transformation occurs within 10-25 months of diagnosis.
However, these figures are generalized for the whole group and it may from type of disorder and clinical condition of the disorder.
A regular follow up with your hematologist is required to note any progression towards acute transformation.
Once the disease transforms to acute disease, the prognosis is poor with patient succumbing to disease within few days or months.

If you have more queries, I will be glad to answer.

Thanks and take care
Dr Shailja Puri

Above answer was peer-reviewed by : Dr. Prasad

Follow up: Dr. Shailja Puri (11 hours later)

Thank you again Dr. Puti for your very detailed and helpful response. All I know about the chromosomal anomaly is that it involves a JAK-2 mutation. What I am especially interested in is whether there could be a situation in which someone does NOT have an acute transformation on the basis of the pathology results and then have an acute transformation, for instance, ten days later or two weeks later?
Can it happen that quickly? However, I appreciate that this is a complex matter and may not be easy to definitively answer. Thank you very much for all your help so far.

Answered by Dr. Shailja Puri (10 hours later)

Brief Answer:
Acute transformation can occur within days.

Detailed Answer:
Hello and welcome again,
A myelodysplastic / myeloproliferative disorder characterized by JAK-2 mutation is associated with poor prognosis.
It is possible to have an acute transformation within days.
However, acute transformation is heralded with worsening of clinical features.
These features may be increased weakness due to fall in hemoglobin, bleeding from various sites due to low platelets, decrease in appetite etc.
Acute transformations are not usually asymptomatic.

In case you have more concerns, I will be glad to help.

Thanks and take care
Dr Shailja Puri

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Above answer was peer-reviewed by : Dr. Prasad