also important to practice gentle breaths during the procedure. Some people hold their breath in anticipation of the needle poke, but this can make you feel faint. You’ll be far less likely to feel lightheaded if you continue to breath normally. If the potential for pain is making you nervous, or you are particularly sensitive, you can ask the phlebotomist who is performing the procedure to use a numbing medication.
If you have had problems in the past, such as fainting or difficulty finding a vein, be sure to tell your infusion nurse or doctor prior to the procedure.
During the procedure, be sure to sit still and don’t watch the blood drain if you know it makes you queasy or lightheaded. Try thinking of something pleasant or singing a song in your head, which takes the focus off the procedure.
Once the procedure is complete, take a moment before standing up. Rise slowly and make sure you are not dizzy or lightheaded before starting to walk. Usually, resting for a few minutes and drinking water prior to standing up helps to decrease dizziness after your phlebotomy procedure.
Myelofibrosis (MF)
What is MF?
Similar to ET and PV, myelofibrosis involves the abnormal production of blood cells. However, MF is a chronic blood cancer in which the bone marrow function is impacted by scarring, producing blood counts that are either too high or too low. In fact, myelo means “bone marrow” and fibrosis means “scarring.”
As we discussed earlier, although no one knows exactly what causes MF, we do know that JAKs (Janus-associated kinases) are involved. Remember, JAKs tell blood cells in the bone marrow to divide and grow. When JAKs are working properly, they help the body make the right number of blood cells. However, when these proteins are malfunctioning, they cause the body to produce the wrong number of cells, which can lead to bone marrow scarring and an enlarged spleen, among other symptoms.
In its very early stages, MF may produce no symptoms. However, as the disease progresses, the signs and symptoms typically begin to increase. Symptoms of MF can range from mild to severe and may include (but are not limited to):
• Low red blood cells
• High or low white blood cells or platelets
• Enlarged spleen or liver (and abdominal pain or feeling full quickly after
a meal)
• Fever
• Night sweats
• Bone pain
• Fatigue (which may be debilitating)
• Itchiness (pruritis)
• Blood clots (thrombotic events)
• Psychosocial symptoms (depression, anxiety, etc.)
• Sleep disturbance
Patients with MF often have too many cytokines, which are proteins that cause inflammation. An overproduction of cytokines may cause itching, night sweats, and bone and muscle pain.
How is MF diagnosed?
MF is most commonly seen in men and women over the age of 60. Approximately 85% or more of people with MF have an enlarged spleen at diagnosis. If your bone marrow is not making enough blood cells or making them too quickly so they don’t have time to fully form, the spleen takes over and begins to make and dispose of blood cells. As it works harder, it becomes larger, causing patients to feel abdominal discomfort, pain under the left ribs and/or early feelings of fullness.
The tests used to diagnose MF are similar to those used in ET and PV, including:
• A blood test to evaluate blood counts (red blood cells, white blood cells and platelets)
• Molecular tests (done on blood or with a bone marrow biopsy)
• Bone marrow biopsy, which is essential to diagnose MF. The bone marrow is viewed under a microscope to see cellular changes consistent with MF, as well as to determine how much scarring or “fibrosis” is present. This will be graded on a scale of 1 to 3, with 3 being the most fibrotic or scarred bone marrow.
Other factors can be considered when making a diagnosis of MF, such as an enlarged spleen, changes in appearance of blood cells under a microscope, or changes in routine blood tests, such as higher LDH levels.
World Health Organization (WHO) Criteria for Myelofibrosis:
A diagnosis of MF requires meeting all three major criteria, and at least one minor criterion.
| Major Criteria | Minor Criterion |
| Presence of megakaryocytic proliferation and atypia, accompanied by:• Fibrosis• Not meeting WHO criteria for other blood diseases• Presence of JAK2, CALR, or MPL mutation or other marker | Presence of at least one of the following, confirmed in two consecutive tests:• Low red blood cells not attributed to another condition• High white blood cell count• An enlarged spleen• Changes in appearance of cells under a microscope that are consistent with MF |
How is MF treated?
The only curative treatment for MF is an allogeneic stem cell transplant, which involves transferring the stem cells from a healthy person (the donor) to the patient’s body after high-intensity chemotherapy or radiation. This can be a high-risk procedure. The intense doses of chemotherapy or radiation are used to inactivate the immune system to reduce the chance of cell graft rejection and enable donor cells to travel to the bone marrow. However, because this treatment temporarily shuts down the immune system, there can be adverse reactions.
In the last decade, targeted therapy with Janus kinase (JAK) inhibitors has become available for treatment. This therapy can improve symptoms by inhibiting or slowing down the overproduction of blood cells, thereby decreasing the size of the spleen. This treatment may also prolong a patient’s life.
Many other medical therapies are often used to treat MF, depending on a patient’s symptoms and the treating physician. For example, agents such as erythropoietin can be used to stimulate red blood cell count. Patients may also receive transfusions, steroids, or immunomodulating agents.
In addition, many clinical trials are ongoing for patients with MF, and you are encouraged to talk to your healthcare team about which trials may be available to you.
The MPN Symptom Burden
As an MPN patient, you may experience a wide range of symptoms which can impact your quality of life (QOL). The symptoms associated with MPN, and the severity of those symptoms, can differ greatly from one person to another. Keeping track of how symptoms affect you day-to-day, ideally with a symptom diary (see Chapter 6), is the best way to monitor and treat the disease. That way, you can discuss any changes in symptoms with your healthcare team at each visit. Understanding your individual symptoms is very important to managing the disease and reducing its effects.
To help us better understand this burden of symptoms, as well as how physicians evaluate and manage the disease, the MPN QOL group was formed. The group is a collaborative effort between UT Health – San Antonio, Mayo Clinic Arizona, Arizona State University and the University of Arizona. They are dedicated to advancing our knowledge of MPN symptom burden, quality of life, and interventions to improve MPN patient wellness.
Obviously, having a better understanding of the variety of symptoms patients
