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Elevated LDH (an enzyme found in cells)

      • High blood pressure

      • Enlarged spleen or liver (along with abdominal discomfort or the sensation of feeling full quickly)

      • General skin itchiness (pruritis), which may be worse when taking a shower

      • Redness of hands and feet (erythromelagia)

      • Blood clots

      • Hemorrhage

      • Facial redness

      • Gout or arthritis

      • Fatigue

      • Psychosocial symptoms (depression, anxiety, etc.)

      • Sleep disturbance

      • Dizziness

      • Headache

      How is PV diagnosed?

      PV can be diagnosed by:

      • A blood test which looks at blood “counts,” including the number of red blood cells, white blood cells and platelets.

      • Molecular tests (done on the blood or with a bone marrow biopsy to determine gene mutations)

      • Bone marrow biopsy to evaluate if there are changes in bone marrow that confirms the diagnosis or rules out other diseases, especially the possibility of fibrosis (scarring) in the bone marrow, which may change treatment decisions or prognosis.

      • A blood test to look at erythropoietin level, or “EPO” level. Erythropoietin is a protein that stimulates red blood cell production in the bone marrow. EPO levels should be normal or low in PV. If the level is high, then other causes, such as lung disease, smoking, sleep apnea or high altitude, should be considered.

      World Health Organization (WHO) Criteria for Polycythemia Vera:

      The diagnostic criteria for PV were updated in 2016, with two major changes: the hemoglobin (a part of the blood that transports oxygen) threshold was lowered to 16.5 g/dl, and a bone marrow biopsy is now considered a major component for diagnosis.

      A diagnosis of PV requires meeting all three major criteria or two major criteria and the minor criterion.

Major Criteria	Minor Criterion
Hemoglobin > 16.5 g/dl in women or hematocrit >49% in men; >48% in women or increased red cell mass (RCM)	Low level or normal blood erythropoietin (EPO) level
Bone marrow biopsy showing changes consistent with PV
Presence of JAK2 V617F or JAK2 exon 12 mutation

      How is PV treated?

      PV is a chronic and sometimes progressive disease, which means it may get worse over time. However, PV can be managed by keeping blood counts under control. Like ET, understanding your risk for events such as heart attack and stroke is important when determining treatment. High risk factors include being 60 years of age or older and having a history of blood clots, heart attack or stroke.

      For patients who are considered “low risk,” treatment often consists of removing blood (phlebotomy). Your healthcare team will monitor your blood counts, and if hematocrit levels (the volume of red blood cells) get too high (>45%), a phlebotomy is recommended. The frequency of this procedure will depend on your disease and your physician’s treatment plan. In addition, a daily aspirin is often recommended, depending on your medical history, and should be discussed with your doctor.

      Other therapies may be considered, such as hydroxyurea, interferon, ruxolitinib, piprobroman, and related drugs, if you are considered high risk for a thrombotic event (blood clot).

      What is the magic of the 45% or less

      hematocrit goal?

      A study was performed (Cyto PV study published by Marchioli et al) which revealed that patients who had phlebotomy (blood removal) with a hematocrit goal higher than 45% had a 4x increase in cardiovascular death or thrombosis (blood clot). Be sure you are hitting the proper goal, and discuss with your physician if your hematocrit level is higher than 45%.

      Making Your Phlebotomy Easier

      Removing blood can be stressful for some people. To make things easier, be sure to drink plenty of water the day before your phlebotomy. Staying hydrated is important because it helps decrease dizziness by keeping the blood volume as normal as possible before removing it. Hydration also helps the veins “plump,” which makes it easier to find a vein that can easily be punctured, Additionally, try to limit caffeine on the day prior to a blood draw, as it acts as a mild diuretic and increases the amount of urine you produce, which may lead to dehydration.

      It’s 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

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