Any parent of boys and girls will confirm that there are differences between the genders that are not based on social conditioning. Yet much of the research for decades had mostly male subjects; what was learned was applied to women. That’s why I was so excited to learn about the newer MPN research that teases out the particulars of female and male patients.
Many of the virtual conversations in the various MPN on-line support groups speak in generalities (e.g., “does anyone else have…?”). As more data becomes available, newly-diagnosed patients and care givers may more quickly understand that this is not a “one size fits all” disease process.
The “Women and MPN Conference” run by MPN Advocacy & Education International, was a day and a half in sunny San Diego, California in September, 2015. The event was recorded and is available through their website (www.mpnadvocacy.com).
Gender Difference in MPN
Dr. Holly Geyer is from Mayo Clinic Arizona and leads the International Working Group of MPN Quality of Life Working Group. Her report on Gender Differences in MPN was particularly interesting.
First, Dr. Geyer shared some MPN basics:
- Essential Thrombocythemia (ET): Diagnosis at age 60. 66% patients are female. More likely to be CAL-R positive or triple negative (no JAK2, CAL-R, or MPL). Life expectancy: normal
- Polycythemia Vera (PV): Diagnosis at age 60. 50% patients are female and 50% male. Higher JAK2 allele burden. More venous blood clots in liver; more likely to have splenomegaly; more likely to have occult (hidden) disease. Life expectancy: normal.
- Primary Myelofibrosis (MF): Diagnosis usually in late 60’s. Patient mix is 50/50 female/male. Can be primary or show up after ET or PV. Fatigue, anemia, cytothemias are common. Life expectancy: 6-10 years, but this may be changing with the new JAK2 inhibitors coming on the scene.
Hormones can play a role in MPN, but hasn’t been researched.
Among female patients, they tend to have higher estrogen levels and increased immune response/antibodies compared to healthy females. This is another opportunity for further research.
Among MPN men, their androgen levels tend to be lower than average and have inverse response to MPN women’s experience with estrogen.
MPN & Thrombosis
The rates of thrombosis (something I survived) were interesting:
- Venous thrombosis (vein clots) occurs in 9% of female patients and 5.4% of male patients.
- Arterial thrombosis (artery clots) occurs in 14% of female patients and 18% of male patients.
MPN & Pregnancy
Pregnancy is riskier for women with MPN than otherwise healthy women. Thrombosis is a significant threat. Take a multi-disciplinary approach when planning a pregnancy — include your hematologist in the planning. Make sure you deliver in a hospital equipped to manage high risk deliveries with a hematologist on call.
Quality of Life Symptoms
They are learning more about symptoms and quality of life with a revised and validated Patient Reported Symptom Tool (MPN SAFTSS or MPN 10) that is providing a lot of patient-reported data. Some findings to date:
- Fatigue is expressed in all MPN patients, strongest with Myelofibrosis.
- Fatigue, anemia, microvascular symptoms (Itching, mental acuity), and early satiety (feel full with little food) are most commonly reported.
- Polycythemia Vera patients report the most symptoms.
- Severity of symptoms do indicate likelihood of disease progression for myelofibrosis patients.
- Women have higher symptom scores – both in the number of symptoms and their severity – then men.
- Women have lower RBCs but are less transfusion dependent then men.
- Women report more abdominal symptoms and increased microvascular and microvascular symptoms.
- Women have a younger mean age at diagnosis compared to the men.
- Men report a higher mean age, higher transfusion needs, and lower symptom scores.
- Men and women report the same Quality of Life scores.
Because women report fatigue at all ages, assumptions that MPN fatigue is tied to patient age is discounted. Also, men require more transfusions even though their red counts tend to be higher than women.
This study raises further questions. Why do both genders report the same quality of life scores when women have higher symptom scores? How do women manage more symptoms and still report the same quality of life as men? Different compensating mechanisms? What role does attitude play in one’s quality of life? What about one’s personal community – immediate family, friends, co-workers, extended family? We had speculating throughout the conference.
While the study sets the stage for further inquiry, it is exciting to see that researchers are taking interest in the different ways MPN affects the lives of women and men. Perhaps this will lead to more specialized treatment. At the very least, such information may make some physicians less dismissive of the complaints brought forth by female patients. This information is also affirming for women and the families who depend on them. It’s not “all in your head.”