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sMyelofibrosis is assort under myeloproliferative tumour and several disorders such as primary myelofibrosis ( PMF ) , polycythemia vera ( PV ) , essential thrombocythemia ( ET ) ( PV and ET were sort out as secondary myelofibrosis in the beginning ) comes under this class grant to the late Who sorting . Reversal of myelofibrosis is a very controversial topic , there is still no unequivocal conclusion among scientist if any of the new drugs really repeal myelofibrosis ( MF ) or not .
The identification of JAK-2 sport and the exploitation of JAK-1 / JAK-2 inhibitors ( drugs ) are greatest achievement in myelofibrosis , although only ruxolitinib is approved in the USA and Europe for the handling of myelofibrosis there are many other JAK-1/2 inhibitors identified and some drug are still on the experimental level and there are many ongoing clinical tribulation about these drug .
Can Myelofibrosis Be Reversed?
There are few case studies / research studies of turnabout of myelofibrosis with certain drugs but whether these drug actually lift the condition , bring around the condition is still debatable . The contrary effects after acquire these drug for a long time also remain unresolved . Some study state that early myelofibrosis can be reversed with JAK-1/2 inhibitors and even probably with hydroxyurea.(1 )
I will present a few case study rule in the literature about the reversal of myelofibrosis but whether these drug actually reversed the condition is disputable .
This is a case of a 74 - year - old elderly male diagnosed with myelofibrosis , due to polycythemia vera ( PV ) . The patient was started on ruxolitinib and a important reduction in quick temper sizing was take note . Then the loony toons was shorten because of thrombocytopenia and the splenomegaly was settle completely with this dose . He has a fibrosis grievance of 3 ( allot to WHO classification ) at baseline and after 168 week of treatment , the fibrosis was absent and normal osseous tissue marrow cadre were present . However , there were severe side - effects present with the drug .
This is another case of a 52 - years old male with a diagnosing of myelofibrosis due to PV with an IPSS ( predictive score ) intermediate-2 hazard level . Fibrosis grievance was 3 at the service line and ruxolitinib was started ( larger Zen than the first slip ) and he stick out it well . The constitutional symptom and splenomegaly were resolved importantly after 4 weeks . After 50 weeks of treatment , there was no splenomegaly and after 17 months of treatment a bone sum biopsy was done which demo completely solve fibrosis .
There are several analytic studies in the literature usher that JAK-1/2 inhibitors do not reverse myelofibrosis but it importantly improves the symptoms and quality of life . So , the issue remains debatable .
This is a case of a 50 years - honest-to-goodness male person who was diagnosed with primary myelofibrosis ; he already had a splenectomy due to a traumatic incident a few years back and hypertension at the presentation . He was set about on anagrelide ( hydroxyurea ) . His blood platelet drop in 4 months ’ time and he was on anagrelide for 6 years . Then he formulate a cam stroke 6 years after and anagrelide was break but after that the platelets were high , WBC was low and a wild type JAK2 variation was also figure , therefore , anagrelide was restarted and his pedigree cellular telephone come to normal grade . Then few months after he developed pancytopenia so , anagrelide was stopped then a bone marrow squash biopsy was done which give away a normalbone marrowand a important diminution in fibrosis . Since the patient was bear on to another nation further follow - up was not done.(2 )
A sketch was done recruiting 22 diagnosed patients with myelofibrosis using imetelstat which is still an investigational drug . This drug has a chemical mechanism of action by inhibiting the telomerase action of the tumour cellular telephone which cause tumor cell demise . These patients were given this drug and followed - up for 6 month . Out of the 22 patients , 4 patient had a consummate remittance phase where the osseous tissue gist was normal . However , some patients had off-white core quelling with this drug so it ’s debatable if remitment of the myelofibrosis is a toxic event of the drug or not . Also , the 3 of the 4 patients were in an former degree of myelofibrosis therefore , these patients must have live into a remission stage . Whether imetelstat really causes remission or not is impossible to say at this leg .
Conclusion
There is n’t any sustain literature on JAK-1/2 inhibitor , hydroxyurea , and investigational drug imetelstat , whether these drug actually reverse or cure myelofibrosis . Some studies tell that early stages of myelofibrosis can be reverse with the above - mentioned but the perniciousness horizontal surface and long - terminal figure side effects of these drugs are still unidentified . So , further studies are want to confirm this matter .
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