Both groups were followed for a median of 27 months (range in the hydroxyurea group, 2.5 to 42 months; range in the control group, 3.8 to 40 months). Eight percent of the patients (two women and seven men; median age, 78 years; range, 66 to 85) died of cardiac failure.
Full Answer
Is hydroxyurea safe for patients with essential thrombocythemia?
This drug has a broad dose–response range, mild side effects, and theoretically little mutagenic risk. Discontinuation of the drug quickly reverses any unwanted myelosuppression. 2 Although hydroxyurea reduces the platelet count, there is no convincing evidence that it also decreases thrombotic episodes in patients with essential thrombocythemia.
How long do you follow a patient with hydroxyurea?
Both groups were followed for a median of 27 months (range in the hydroxyurea group, 2.5 to 42 months; range in the control group, 3.8 to 40 months). Eight percent of the patients (two women and seven men; median age, 78 years; range, 66 to 85) died of cardiac failure.
When should hydroxyurea be used as a first-line cytoreductive therapy?
The ELN experts recommended hydroxyurea as first-line cytoreductive therapy at any age, although they also underlined that its use should be carefully considered in patients younger than 40 years of age. 38 In our current practice, we start hydroxyurea at a dose of 1 g per day, and then modify the dose according to the hematologic response.
How much hydroxyurea should I take for high platelets?
The starting dose of hydroxyurea (Oncocarbide; Simes, Vicenza, Italy) was 15 mg per kilogram of body weight per day. Thereafter, a maintenance dose of the drug was administered to maintain the platelet count below 600,000 per cubic millimeter without lowering the white-cell count below 4000 per cubic millimeter.
Can essential thrombocytosis go into remission?
Essential thrombocythemia is a chronic disease with no cure. If you have a mild form of the disease, you may not need treatment. If you have severe symptoms, you may need medicine that lowers your platelet count, blood thinners or both.
What is the best treatment for essential thrombocythemia?
Medication. Your doctor may suggest one of the following prescription drugs, perhaps along with low-dose aspirin, to reduce your platelet count: Hydroxyurea (Droxia, Hydrea.) This drug is the most common prescription used for essential thrombocythemia.
Does essential thrombocythemia always progress?
About 10%-20% of patients progress, the majority of people do not. Many can live close to their normal life expectancy, or live their normal life expectancy."
Does essential thrombocytosis shorten life expectancy?
ET does not generally shorten life expectancy. However, medical supervision is important to prevent or treat thrombosis, a serious complication that can affect vital organs such as the brain or the heart. Also, for untreated pregnant patients with ET, there is a risk to the survival of the fetus.
How long do you take hydroxyurea?
There are a few people who won't respond, so the medication doesn't help. But, you should try the hydroxyurea for at least six months before stopping It.
What is the most common side effect of hydroxyurea?
The more common side effects of hydroxyurea can include: stomach pain. constipation. diarrhea.
Can you live a normal life with essential thrombocythemia?
Essential thrombocythemia, also known as ET, is a rare disease. The most important first fact about ET: on average, people with ET have a normal life expectancy. Patients with ET have increased numbers of platelets.
Does essential thrombocythemia get worse over time?
ET results from an overproduction of platelets. As these platelets do not work normally, blood clots and bleeding are common problems. Untreated, ET worsens over time.
How long can you live with ET?
In general, ET is a chronic disease that does not shorten life expectancy in the first decade following diagnosis; however, over longer periods of time, survival may be shortened. Median survival of patients with ET is about 20 years.
What is the difference between essential thrombocytosis and thrombocythemia?
Thrombocythemia refers to a high platelet count that is not caused by another health condition. This condition is sometimes called primary or essential thrombocythemia. Thrombocytosis refers to a high platelet count caused by another disease or condition.
What happens if you don't treat essential thrombocythemia?
Essential thrombocytosis usually develops around 60 years of age. It can also develop in younger people, especially women of child-bearing age. Pregnant women with untreated essential thrombocytosis have a risk for complications, including miscarriage, the fetus developing slower than normal and premature birth.
Is thrombocytosis considered a disability?
In order for chronic thrombocytopenia to be considered disabling for Social Security Disability purposes, it must be severe.
How long did the hydroxyurea group follow?
Both groups were followed for a median of 27 months (range in the hydroxyurea group, 2.5 to 42 months ; range in the control group, 3.8 to 40 months). Eight percent of the patients (two women and seven men; median age, 78 years; range, 66 to 85) died of cardiac failure. Five were in the hydroxyurea group, and four were in the control group. No patient was lost to follow-up.
How many patients with thrombosis were taking aspirin?
Ten of 16 patients with thrombosis (62.5 percent) — 2 in the hydroxyurea group and 8 in the control group — had been taking aspirin (n = 6) or ticlopidine (n = 4) before thrombosis occurred, as compared with 69 of 98 asymptomatic patients (70 percent). Multivariate analysis did not reveal a significant effect of antiplatelet drugs on the outcome. Smoking status was the only variable other than hydroxyurea treatment associated with thrombosis ( Table 3 ).
What is the most frequent thrombotic complications in high-risk patients?
As in previous studies of essential thrombocythemia 3,4 the most frequent thrombotic complications in high-risk patients involved the central nervous system and peripheral and coronary arteries. Hemorrhagic complications occurred in only five patients (4.4 percent), and these patients were all taking aspirin or ticlopidine prophylactically, indicating once again that the main hemostatic problem in essential thrombocythemia is thrombosis, at least in patients with platelet counts below 1,500,000 per cubic millimeter.
Does hydroxyurea reduce platelet count?
The ability of hydroxyurea to reduce the platelet count in patients with essential thrombocythemia is well established, but its efficacy in reducing the rate of thrombotic complications is not. Recently, Turlure et al. 15 reported their experience with hydroxyurea in 79 patients with essential thrombocythemia who had a high risk of thrombotic or hemorrhagic complications because they were older than 65 years, had platelet counts above 1,500,000 per cubic millimeter, or had had previous thrombotic, ischemic, or hemorrhagic episodes. Despite treatment with hydroxyurea, 16 percent of the patients had major ischemic episodes. However, the lack of an untreated control group hinders interpretation of this study.
Is essential thrombocythemia asymptomatic?
Moreover, up to two thirds of patients with essential thrombocythemia are asymptomatic. 2 Thus, essential thrombocythemia is a clinically heterogeneous disorder in which the use of drugs to lower the platelet count or to inhibit platelet function is often problematic.
Is hydroxyurea an alkylating agent?
This myelosuppressive drug, which is not an alkylating agent, is in wide use for the treatment of essential thrombocythemia and other myeloproliferative disorders. 5 Its advantages include convenience, efficacy in reducing the platelet count, and low level of toxicity. 2 However, hydroxyurea must be taken continuously, and if it is inadvertently stopped an excessive rebound increase in the platelet count may occur. 14
Is hydroxurea good for thrombocythemia?
Hydroxyurea is effective in preventing thrombosis in high-risk patients with essential thrombocythemia.
What is hydrourea used for?
Treatment/exposure: Hydroxyurea is a drug used to treat certain blood disorders, some cancers, and HIV disease. If you not on this medicine you may be exposed to it in other ways such as handling it for a loved one. If neither is the case, please consult a healthcare provider asap.
Does hydroxyurea cause high pulse rate?
Possibly: High pulse rate (tachycardia) is not reported as a common side effect of hydroxyurea, but if your red blood cell count drops as a side effect, your heart rate may increase in order to increase the oxygen delivery to your tissues.
Does hydroxyurea cause leukemia?
Side Effects: There are some serious, but rare, side effects with hydroxyurea, including secondary leukemia, pulmonary fibrosis, pancreatitis, and peripheral neuropathy, or damage to the nerves. Discuss the benefits of decreasing the severity and length of the sickle cell attacks, and the risk of rare side effects as above.
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What is the effect of hydroxyurea on thrombosis?
Hydroxyurea: The oral chemotherapy agent hydroxyurea reduces the risk of thrombosis in essential thromobcytosis by reducing the platelet count. Hydroxyurea is started at a low dose and escalated until the platelet count is near normal (100,000 - 400,000 cells per microliter).
What is essential thrombocythemia?
Essential Thrombocythemia (ET) is one of a group of blood disorders called myeloproliferative neoplasms. Other types of myeloproliferative neoplasms include primary myelofibrosis and polycythemia vera . Genetic mutations in essential thrombocythemia lead to extremely high platelet counts (thrombocytosis). Many people are without symptoms ...
How is plateletpheresis done?
Plateletpheresis: Plateletpheresis is a procedure that removes the platelets from the circulation. Blood is removed via an intravenous (IV) catheter and collected in a machine where the platelets are separated from the other parts of the blood (white blood cells, red blood cells, and plasma). The blood, minus the platelets, is returned to the body via IV either with saline solution or plasma. Plateletpheresis is reserved for people with extremely high platelet counts, serious clot formation (in lungs, brain), or bleeding. The effect of plateletpheresis is temporary so another treatment will need to be started for long-term control of the platelet count.
What are the complications of thrombocytosis?
Complications include clot formation (leading to stroke, heart attack, a clot in lungs, or deep vein thrombosis), development of myelofibrosis, ...
What is the blood minus platelets returned to the body?
The blood, minus the platelets, is returned to the body via IV either with saline solution or plasma . Plateletpheresis is reserved for people with extremely high platelet counts, serious clot formation (in lungs, brain), or bleeding.
How often can you take Alpha Interferon?
Alpha interferon is also available in a long-acting form called pegylated interferon that can be given once per week. Interferon therapy is typically reserved for pregnant women or people who do not respond to hydroxyurea therapy.
How long can you go without a clot?
Most people will go several years without requiring treatment, which is typically started if you develop a clot. Aspirin: Daily low-dose aspirin is typically used to treat people with vasomotor symptoms. Vasomotor symptoms include headaches, dizziness, chest pain, burning of the hands and feet, and visual changes.
How many thrombotic events occurred in the first year of HU?
Six thrombotic episodes occurred within the first year of treatment and 27 events (70% arterial) were registered during follow-up ( Table 3 ). Overall, the thrombotic rate was 1.66% patients/year, whereas the rate of hematologic transformations was 0.68% patients/year. Deaths were registered in 23 patients (5.5%) and occurred after a median of 5.7 years from HU start (rate, 0.9% patients/year).
Does leukocyte count increase over time?
Increase in leukocyte count over time predicts thrombosis in patients with low-risk essential thrombocythemia.
Is leukocytosis a risk factor for thrombocythemia?
Leukocytosis is a risk factor for thrombosis in essential thrombocythemia: interaction with treatment, standard risk factors, and Jak2 mutation status.
Is leukocytosis a thrombotic risk factor?
Leukocytosis as a major thrombotic risk factor in patients with polycythemia vera.
What is essential thrombocythemia?
Essential thrombocythemia (ET) is an indolent myeloproliferative neoplasm that may be complicated by vascular events, including both thrombosis and bleeding. This disorder may also transform into more aggressive myeloid neoplasms, in particular into myelofibrosis. The identification of somatic mutations of JAK2, CALR, or MPL, found in about 90% of patients, has considerably improved the diagnostic approach to this disorder. Genomic profiling also holds the potential to improve prognostication and, more generally, clinical decision-making because the different driver mutations are associated with distinct clinical features. Prevention of vascular events has been so far the main objective of therapy, and continues to be extremely important in the management of patients with ET. Low-dose aspirin and cytoreductive drugs can be administered to this purpose, with cytoreductive treatment being primarily given to patients at high risk of vascular complications. Currently used cytoreductive drugs include hydroxyurea, mainly used in older patients, and interferon α, primarily given to younger patients. There is a need for disease-modifying drugs that can eradicate clonal hematopoiesis and/or prevent progression to more aggressive myeloid neoplasms, especially in younger patients. In this article, we use a case-based discussion format to illustrate our approach to diagnosis and treatment of ET.
What is the PLT count for thrombocytosis?
Thrombocytosis is defined as a platelet (PLT) count ≥450 × 10 9 /L. The major types of thrombocytosis include reactive (or secondary) thrombocytosis, clonal myeloid neoplasms, and familial or hereditary thrombocytosis. 21 As the most common cause is a reactive process, at the time of the first visit we always consider secondary thrombocytosis through a complete interview, physical examination, and first-level tests as reported in Table 2 and Figure 1.
When to discontinue antiplatelet treatment?
We discuss with obstetricians and anesthetists the optimal time to discontinue antiplatelet treatment (generally about 2 weeks before delivery) in order to account for any possible event, including instrumental delivery and epidural or spinal anesthesia. After delivery, we recommend treating all women with ET with LMWH for 6 weeks to prevent deep vein thrombosis. 79
Usual Adult Dose for Chronic Myelogenous Leukemia
Oral Capsules (i.e., Hydrea): Individualize treatment based on tumor type, disease state, response to treatment, patient risk factors, and current clinical practice standards Comments: -As monotherapy or in conjunction with other antitumor agents or radiation therapy to treat neoplastic diseases. -Prophylactic administration of folic acid is recommended. -Blood counts should be monitored at least once a week during therapy. -Severe anemia should be corrected before initiating therapy. Uses: -For the treatment of resistant chronic myeloid leukemia (CML). -For the treatment of locally advanced squamous cell carcinomas of the head and neck (excluding lip), in combination with concurrent chemoradiation..
Usual Adult Dose for Head and Neck Cancer
Oral Capsules (i.e., Hydrea): Individualize treatment based on tumor type, disease state, response to treatment, patient risk factors, and current clinical practice standards Comments: -As monotherapy or in conjunction with other antitumor agents or radiation therapy to treat neoplastic diseases. -Prophylactic administration of folic acid is recommended. -Blood counts should be monitored at least once a week during therapy. -Severe anemia should be corrected before initiating therapy. Uses: -For the treatment of resistant chronic myeloid leukemia (CML). -For the treatment of locally advanced squamous cell carcinomas of the head and neck (excluding lip), in combination with concurrent chemoradiation..
Usual Adult Dose for Sickle Cell Anemia
Oral Tablets (i.e., Siklos): -Initial dose: 15 mg/kg orally once a day -Increase 5 mg/kg/day every 8 weeks or if a painful crisis occurs; increase only if blood counts are in an acceptable range -Maximum dose: 35 mg/kg/day BLOOD COUNTS IN THE ACCEPTABLE RANGE: -Neutrophils greater than or equal to 2000 cells/mm3 -Platelets greater than or equal to 80,000/mm3 -Hemoglobin greater than 5.3 g/dL -Reticulocytes greater than or equal to 80,000/mm3 if the hemoglobin concentration is less than 9 g/dL Oral Capsules (i.e., Droxia): -Initial dose: 15 mg/kg orally once a day -Increase 5 mg/kg/day every 12 weeks; increase only if blood counts are in an acceptable range -Maximum dose: 35 mg/kg/day BLOOD COUNTS IN THE ACCEPTABLE RANGE: -Neutrophils greater than or equal to 2500 cells/mm3 -Platelets greater than or equal to 95,000/mm3 -Hemoglobin greater than 5.3 g/dL -Reticulocytes greater than or equal to 95,000/mm3 if the hemoglobin concentration is less than 9 g/dL Comments: -Dosage is based on the actual or ideal patient weight, whichever is less. -Blood counts should be monitored every 2 weeks. -Tablets may be spilt or quartered along score lines, tablet doses can be rounded to the nearest 50 mg or 100 mg strength based on clinical judgement; capsules should be swallowed whole and should not be opened or chewed. -Prophylactic administration of folic acid is recommended. Use: To reduce the frequency of painful crises and to reduce the need for blood transfusions in patients with sickle cell anemia with recurrent moderate to severe painful crises..
Usual Pediatric Dose for Sickle Cell Anemia
Oral Tablets (i.e., Siklos): -Initial dose: 20 mg/kg orally once a day -Increase 5 mg/kg/day every 8 weeks or if a painful crisis occurs; increase only if blood counts are in an acceptable range -Maximum dose: 35 mg/kg/day BLOOD COUNTS IN THE ACCEPTABLE RANGE: -Neutrophils greater than or equal to 2000 cells/mm3 -Platelets greater than or equal to 80,000/mm3 -Hemoglobin greater than 5.3 g/dL -Reticulocytes greater than or equal to 80,000/mm3 if the hemoglobin concentration is less than 9 g/dL Comments: -Dosage is based on the actual or ideal patient weight, whichever is less. -Blood counts should be monitored every 2 weeks. -Tablets may be spilt or quartered along score lines, tablet doses can be rounded to the nearest 50 mg or 100 mg strength based on clinical judgement. -Prophylactic administration of folic acid is recommended. Use: To reduce the frequency of painful crises and to reduce the need for blood transfusions in pediatric patients 2 years or older with sickle cell anemia with recurrent moderate to severe painful crises..
Dose Adjustments
SICKLE CELL ANEMIA: -Treatment for Sickle Cell Anemia should be interrupted if blood counts are in the toxic range: TOXIC RANGE --Neutrophils less than 2000 cells/mm3 (younger patients with lower baseline counts may safely tolerate absolute neutrophil counts down to 1250/mm3) --Platelets less than 80,000/mm3 --Hemoglobin less than 4.5 g/dL --Reticulocytes less than 80,000/mm3 if the hemoglobin concentration is less than 9 g/dL Dosing After Hematologic Recovery: -Upon recovery, reduce from dose associated with hematologic toxicity: --Tablets: Reduce by 5 mg/kg/day; may titrate up or down every 8 weeks in 5 mg/kg/day increments --Capsules: Reduce by 2.5 mg/kg/day; may titrate up or down every 12 weeks in 2.5 mg/kg/day increments -Patients should be at a stable dose with no hematologic toxicity for 24 weeks. -Permanently discontinue if the patient develops hematologic toxicity twice. NEOPLASTIC DISEASES: -Myelosuppression: Provide supportive care and modify dose or discontinue therapy as needed. -If cutaneous vasculitic ulcers occur, institute treatment and discontinue this drug..
Precautions
US BOXED WARNINGS: MYELOSUPPRESSION and MALIGNANCIES -Myelosuppression: May cause severe myelosuppression, monitor blood counts at baseline and throughout treatment; interrupt treatment and reduce dose as necessary -Malignancies: This drug is carcinogenic; advise sun protection and monitor patients for malignancies CONTRAINDICATIONS: -Hypersensitivity to the active substance or any product ingredients Safety and efficacy have not been established for use of the capsule formulation in patients younger than 18 years. Safety and efficacy for use of the tablet formulation for treating sickle cell anemia have not been established in pediatric patients younger than 2 years. Consult WARNINGS section for additional precautions..
Other Comments
Administration advice: Take orally once a day. Oral Capsules: -Swallow whole; do not open, break, or chew because this drug is cytotoxic Oral Tablets: -The 100 mg tablets have 1 score line and can be split into 2 parts (each 50 mg); the 1000 mg tablets have 3 score lines and can be split into 4 parts (each 250 mg); calculate the rounded doses to the nearest 50 mg or 100 mg strength based on clinical judgment -Tablet breaking instructions are provided in the instructions for use handout and should be reviewed with each prescription. General: -This is a cytotoxic drug; special handling and disposal procedures should be followed. -Different products are labeled for specific indications; the manufacturer product information should be consulted prior to using a product. Monitoring: -Monitor blood counts weekly (neoplastic diseases) or every 2 weeks (sickle cell anemia) -Monitor efficacy of sickle cell anemia by obtaining hemoglobin (HbF) levels every 3 to 4 months; an increase in HbF of at least 2-fold over baseline is desired -Monitor for malignancies, pulmonary toxicities Patient advice: -Patients should be instructed to read the US FDA-approved patient labeling (Medication Guide and Instructions for Use). -Patients should be advised to promptly report signs and symptoms of infection or bleeding. -Patients should be advised to use sun protection. -Patients should discuss vaccinations with their healthcare provider. -Both male and females of reproductive potential should be advised to use contraception during and after treatment..
Treatment
- Although there's no cure for essential thrombocythemia, treatments can control symptoms and reduce the risk of complications. Life span is expected to be normal despite the disease. Treatment of essential thrombocythemia depends on your risk of blood clots or bleeding episod…
Clinical Trials
- Explore Mayo Clinic studiestesting new treatments, interventions and tests as a means to prevent, detect, treat or manage this condition.
Lifestyle and Home Remedies
- If you have essential thrombocythemia, your doctor may recommend that you regularly take low-dose aspirin. Aspirin makes platelets less sticky and your blood less likely to form clots. If you're also pregnant, aspirin has a low risk of causing side effects to the fetus. Also try to choose healthy lifestyle habits to lower your risk of developing conditions that may contribute to blood c…
Preparing For Your Appointment
- Here's some information to help you get ready for your appointment and to know what to expect from your doctor.
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- In all patients in the hydroxyurea group, treatment with the drug resulted in a decrease in the platelet count to below 600,000 per cubic millimeter (median count, 459,000 per cubic millimeter; range, 285,000 to 628,000 per cubic millimeter) after a median of 30 days (range, 16 to 60). The response continued with long-term therapy, and there was no...
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