Jakafi (ruxolitinib) was approved by FDA in 2022 as a second line therapy for PV when patients did not respond adequately or resistant or intolerant to hydroxyurea.
Among patients with phlebotomy-dependent polycythemia vera (PV), treatment with rusfertide, a hepcidin mimetic administered weekly as a subcutaneous injection, continues to be effective, results in sustained hematocrit control, and eliminates the need for repeated phlebotomies. These results from the ongoing phase II REVIVE trial were presented at the 2022 American Society of Clinical Oncology (ASCO) Annual Meeting. Patients received rusfertide for 28 weeks, after which they could continue therapy in the open-label extension phase for up to 36 months after a 12-week withdrawal phase. Of 70 patients, 84% did not require a phlebotomy, 14% required one, and 2% required two during the initial 28 weeks of rusfertide treatment. The therapy was equally effective in controlling hematocrit levels in patients who were treated previously with phlebotomy alone or those who required myelosuppressive agents. Patients had been treated for up to 1.5 years and essentially did not need phlebotomies. Patients had normalized iron stores, as measured by ferritin levels, and reduced red blood cell counts, yet therapy did not affect white blood cell or platelet counts. Most adverse events (AEs) were grade 1 or 2. Injection site reactions were the most common, occurring with 33% of injections. There were no grade 3 AEs related to rusfertide. One grade 4 event occurred in a patient with asymptomatic thrombocytosis possibly related to rusfertide. Two patients withdrew from the trial as a result of AEs possibly related to the therapy. “Rusfertide can potentially be used to treat virtually all patients with PV,” Dr. Hoffman said. “This drug will likely allow one to reduce the doses of myelosuppressive agents and eliminate the need for supplemental phlebotomy in order to maintain [normal] hematocrit levels associated with a reduced risk of thrombotic events.” A phase III clinical trial in patients with PV (VERIFY) has been developed to validate these phase II results.
One spoken error: At 30 sec, I said "a tight bonding between oxygen (O2) and hemoglobin". It should be "a tight bonding between carbon monoxide (CO) and hemoglobin". Because CO molecule binds to hemoglobin very tightly, O2 molecules gradually lose their place in RBCs. Its like when a naughty girl (CO) steals a man's heart and attaches to him (Hemoglobin) too tightly, a good girl (O2) has no chance to associate with the man (hemoglobin) and the good girl (O2) loses her chance to let him know her. So, the carboxyhemoglobin (COHb) occupying the RBC has no O2 to give to the tissue of the organ resulting in low tissue O2 concentration. Patients with secondary polycythemia from chronic carbon monoxide poisoning have normal O2 saturation when measured with a pulse oxymetry device because it falsely overestimates the SPO2 in the presence of high COHb. Therefore, when you suspect carbon monoxide poisoning, you need to get the arterial blood gas (ABG) analysis in order to obtain the accurate SPO2.
Could you please answer, my cbc fluctuates 4 months ago my hb was 11.8 and rbc was 4.6 now my hb is 12.8 and rbc are 5.7 my mch is always low. I’m a female
I have high platelets with JAK2 V617F. Platelets average around 450,000. RBCs run on average about 5.1, Hemoglobin averages around 15.6 to 16.0 and Hematocrit around 46 to 49. I have been diagnosed with ET via BMB but wonder if my condition is not PV instead? My CBCs have been pretty stable for decades.
I was diagnosed with hyperthyroidism with secondary polycythemia. I already had 2 therapeutic phlebotgomies. I’m currently taking methimazole for my hypertyhroidism. Will my hgb/hct go back to normal once my thyroid hormones are corrected? Thank you very much.
I am not sure if hydroxyurea is a very toxic drug. I have old patients whose age older than 80 have been taking more than 20 years without problem and children with sickle cell anemia takes it daily for many years.
You probably have thalassemia trait, either alpha or beta. Then you do not have polycythemia. Patients with thalassemia trait have a small size RBC shown as a low MCV. Please watch my UA-cam lecture about thalassemia.
@@chetanrs in my report normal range is from 14-16 . Initially it was 16.6 after 9 days it was 16.5. and after 1 week during donating blood drop to 16. I hve no PVC and Rbc count in the report. WBC count=6700
Jakafi (ruxolitinib) was approved by FDA in 2022 as a second line therapy for PV when patients did not respond adequately or resistant or intolerant to hydroxyurea.
Among patients with phlebotomy-dependent polycythemia vera (PV), treatment with rusfertide, a hepcidin mimetic administered weekly as a subcutaneous injection, continues to be effective, results in sustained hematocrit control, and eliminates the need for repeated phlebotomies. These results from the ongoing phase II REVIVE trial were presented at the 2022 American Society of Clinical Oncology (ASCO) Annual Meeting.
Patients received rusfertide for 28 weeks, after which they could continue therapy in the open-label extension phase for up to 36 months after a 12-week withdrawal phase.
Of 70 patients, 84% did not require a phlebotomy, 14% required one, and 2% required two during the initial 28 weeks of rusfertide treatment. The therapy was equally effective in controlling hematocrit levels in patients who were treated previously with phlebotomy alone or those who required myelosuppressive agents. Patients had been treated for up to 1.5 years and essentially did not need phlebotomies.
Patients had normalized iron stores, as measured by ferritin levels, and reduced red blood cell counts, yet therapy did not affect white blood cell or platelet counts.
Most adverse events (AEs) were grade 1 or 2. Injection site reactions were the most common, occurring with 33% of injections. There were no grade 3 AEs related to rusfertide. One grade 4 event occurred in a patient with asymptomatic thrombocytosis possibly related to rusfertide. Two patients withdrew from the trial as a result of AEs possibly related to the therapy.
“Rusfertide can potentially be used to treat virtually all patients with PV,” Dr. Hoffman said. “This drug will likely allow one to reduce the doses of myelosuppressive agents and eliminate the need for supplemental phlebotomy in order to maintain [normal] hematocrit levels associated with a reduced risk of thrombotic events.”
A phase III clinical trial in patients with PV (VERIFY) has been developed to validate these phase II results.
Very informative video! Thank you.
You’re welcome
One spoken error:
At 30 sec, I said "a tight bonding between oxygen (O2) and hemoglobin". It should be "a tight bonding between carbon monoxide (CO) and hemoglobin".
Because CO molecule binds to hemoglobin very tightly, O2 molecules gradually lose their place in RBCs. Its like when a naughty girl (CO) steals a man's heart and attaches to him (Hemoglobin) too tightly, a good girl (O2) has no chance to associate with the man (hemoglobin) and the good girl (O2) loses her chance to let him know her.
So, the carboxyhemoglobin (COHb) occupying the RBC has no O2 to give to the tissue of the organ resulting in low tissue O2 concentration.
Patients with secondary polycythemia from chronic carbon monoxide poisoning have normal O2 saturation when measured with a pulse oxymetry device because it falsely overestimates the SPO2 in the presence of high COHb. Therefore, when you suspect carbon monoxide poisoning, you need to get the arterial blood gas (ABG) analysis in order to obtain the accurate SPO2.
Will Rusfertide help patients with polycythemia caused from sleep apnea. I have to have a phlebotomy about every 6 weeks.
Thank you Dr. Very detailed❤
My pleasure
Hello I’m a 44 yr old female. My hemoglobin is 16.3, Hct 50% and total rbc 5.43. Is that normal?
Thanks alot Sir ..Your videos are amazing
You’re welcome
Could you please answer, my cbc fluctuates 4 months ago my hb was 11.8 and rbc was 4.6 now my hb is 12.8 and rbc are 5.7 my mch is always low. I’m a female
Correction* my hb now is 12.8 and rbc is 5.20
Thank you so much for this such a great lecture. 🙏
You’re welcome
I have high platelets with JAK2 V617F. Platelets average around 450,000. RBCs run on average about 5.1, Hemoglobin averages around 15.6 to 16.0 and Hematocrit around 46 to 49. I have been diagnosed with ET via BMB but wonder if my condition is not PV instead? My CBCs have been pretty stable for decades.
Yes, good question. You may have PV instead of ET.
So, please test for EPO. If it’s low, you likely have PV.
@@stanleykim1924 THank you
❤😊 Thank you so much 🙏
I was diagnosed with hyperthyroidism with secondary polycythemia. I already had 2 therapeutic phlebotgomies. I’m currently taking methimazole for my hypertyhroidism. Will my hgb/hct go back to normal once my thyroid hormones are corrected? Thank you very much.
If it is truly associated with hyperthyroidism, it should improve.
Hello doc I have polycythemia it's now 3 years how can I contact you,thank you in advance.
Find a hematologist nearby or ask PCP for referral
I wonder how long you can be on Hydroxyurea since it’s a toxic drug. I’ve been taking it for 12-13 years. Bad reaction to Anagrelide (chest pain).
I am not sure if hydroxyurea is a very toxic drug.
I have old patients whose age older than 80 have been taking more than 20 years without problem and children with sickle cell anemia takes it daily for many years.
Dr. is it okay for a PV patient to have the covid 19 vaccine? Thank you.
If appropriately treated, there is no reason why you can’t have C19 vaccination.
My red blood cells count is 5.13 is that normal?
Check hemoglobin and hematocrit but check with your doctor please.
@@stanleykim1924 my hemoglobin was in the normal range and hematocrit, I did find out I had iron deficiency without anemia thank you 🙂🙏🏼
I have 6.7 RBC but hemoglobin 12.3 ...mcv , mchc , mch low . Hct normal . Is it polychythemia ?
You probably have thalassemia trait, either alpha or beta. Then you do not have polycythemia. Patients with thalassemia trait have a small size RBC shown as a low MCV.
Please watch my UA-cam lecture about thalassemia.
doc i have 5.96 rbc and my hemoglobin is 15.2 and my mcv and mchc are low and my htc is 46.6 do u think i have polychythemia plss answer my question
My hemoglobin 16.2. Hct 48% Total rbc 5.1. is it normal?
It looks like normal if you are man.
My hb level 17.1, rbc 5.59, hematocrit 50.5, epo 7.23 and abg normal, jak2 negative and normal bone marrow result actually what condition i have
It looks like secondary polycythemia.
@@stanleykim1924is it curable? and reason is for secondary polycythemia
If you correct the cause of the secondary polycythemia, it will be cured.
@@stanleykim1924 how can i find the reason. All other test are normal.
Does addressing OSA reverse or cure secondary polycythemia ?
우리말이면 좋겠어요
I am 30 yrs old my hb count is 16.5
Am i a PV patient?
I have recently donated my blood also.
What is the cut off value of Hemoglobin in your report ? What is your -- PCV and RBC count ?
@@chetanrs in my report normal range is from 14-16 .
Initially it was 16.6 after 9 days it was 16.5. and after 1 week during donating blood drop to 16.
I hve no PVC and Rbc count in the report.
WBC count=6700
@@nishanttamang9198 why are you donating blood then ?
@@chetanrs sir because i thought my hb count is going high slightly which may cause complications in my health.