Secondary erythrocytosis that is caused by oxygen deprivation may be treated with oxygen. Smokers are advised to quit and are offered treatments to assist quitting. Any underlying disorder that is causing the oxygen deprivation and secondary erythrocytosis is treated as effectively as possible.
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What are the treatment options for erythrocytosis?
You can also get tested for the gene mutations that cause erythrocytosis. Treatment aims to reduce your risk of blood clots and relieve symptoms. It often involves lowering your RBC count. Treatments for erythrocytosis include: Phlebotomy (also called venesection).
When is low-dose aspirin indicated in the treatment of erythrocytosis?
Some patients with erythrocytosis should be considered for judicious venesection to a designated target Hct depending on the clinical scenario and events. Low-dose aspirin can be considered in any patient who does not have a specific contra-indication.
What are the diagnostic criteria for erythrocytosis?
Erythrocytosis is suspected when haemoglobin is above 185 g/L or the packed cell volume is greater than 0.52 in a man or 165 g/L and 0.48, respectively, in a woman.
Is phlebotomy necessary for the treatment of secondary erythrocytosis?
The treatment of secondary erythrocytosis depends in part on its cause and in part on whether the erythrocytosis is causing symptoms due to increased blood viscosity. In this regard, the need for phlebotomy varies.
How do you fix erythrocytosis?
Treatments for erythrocytosis include:Phlebotomy (also called venesection). This procedure removes a small amount of blood from your body to lower the number of RBCs. ... Aspirin. Taking low doses of this everyday pain reliever may help prevent blood clots.Medications that lower RBC production.
What are the common symptoms of erythrocytosis?
Signs and symptoms of familial erythrocytosis can include headaches, dizziness, nosebleeds, and shortness of breath. The excess red blood cells also increase the risk of developing abnormal blood clots that can block the flow of blood through arteries and veins.
Can erythrocytosis go away?
There's no cure for polycythemia vera. Treatment focuses on reducing your risk of complications. These treatments may also ease your symptoms.
Is erythrocytosis reversible?
Spurious erythrocytosis may occur with hemoconcentration (eg, due to burns, diarrhea, or diuretic use). In patients who smoke, reversible erythrocytosis results mainly from tissue hypoxia due to elevation of blood carboxyhemoglobin concentration; levels will normalize with smoking cessation.
Is erythrocytosis serious?
Primary erythrocytosis can be a harmless disorder caused by a genetic trait that is passed down in families. If you only have too many red blood cells without having too many white blood cells or platelets, and you don't have secondary erythrocytosis, this may be why.
How do you treat erythrocytes?
If a health condition is causing a high red blood cell count, your provider may recommend a procedure or medication to lower it. If you have polycythemia vera (a rare type of blood cancer), your healthcare provider may prescribe a medicine called hydroxyurea.
What medicines cause erythrocytosis?
Drugs that can cause this type of hemolytic anemia include:Cephalosporins (a class of antibiotics), most common cause.Dapsone.Levodopa.Levofloxacin.Methyldopa.Nitrofurantoin.Nonsteroidal anti-inflammatory drugs (NSAIDs)Penicillin and its derivatives.More items...•
What level of hemoglobin is dangerously high?
The threshold for a high hemoglobin count differs slightly from one medical practice to another. It's generally defined as more than 16.6 grams (g) of hemoglobin per deciliter (dL) of blood for men and 15 g/dL for women.
What happens if you have too many red blood cells?
The increase in blood cells makes the blood thicker. Thick blood can lead to strokes or tissue and organ damage. Symptoms include lack of energy (fatigue) or weakness, headaches, dizziness, shortness of breath, visual disturbances, nose bleeds, bleeding gums, heavy menstrual periods, and bruising.
What is the difference between polycythemia and erythrocytosis?
Polycythemia is sometimes called erythrocytosis, but the terms are not synonymous, because polycythemia describes any increase in red blood mass (whether due to an erythrocytosis or not), whereas erythrocytosis is a documented increase of red cell count.
Can dehydration cause erythrocytosis?
Relative erythrocytosis occurs due to dehydration or endotoxemia (hemoconcentration) or excitement/exercise (splenic contraction). The vast majority of horses with erythrocytosis have the relative variety. Absolute (true) erythrocytosis can be a primary or secondary disorder.
How is secondary erythrocytosis treated?
They can be treated with oxygen therapy and phlebotomy. Tumor-associated erythrocytosis, a type of secondary polycythemia, occurs because of tumors or cysts in the kidneys, tumors in the liver, cerebellar hemangioblastoma (a type of brain tumor), or uterine leiomyoma (a tumor in the womb).
What are the nonspecific consequences of erythrocytosis?
Facial, conjunctival and mucous membrane plethora, and palmar erythema are other nonspecific consequences of erythrocytosis. Spider angiomata suggest that the erythrocytosis is due to the hepatopulmonary syndrome.
What is the diagnosis of relative erythrocytosis?
For relative erythrocytosis, the diagnosis relies primarily on a careful history with respect to diuretic, tobacco or androgen use, sleep apnea and the possibility of a pheochromocytoma, and for the latter, a 24 hour urine for fractionated catecholamines and metanephrines is the diagnostic test of choice.
How much does a phlebotomy reduce hematocrit?
Therefore, a single phlebotomy after equilibration should reduce the hematocrit by at least 10%. If the decrement in hematocrit is equal to or less than this, absolute erythrocytosis is present. If, however, the hematocrit falls below the patient’s baseline, absolute erythrocytosis is not present.
What is secondary erythrocytosis?
Erythrocytosis, also known as secondary polycythemia or secondary erythrocytosis to distinguish it from the chronic myeloproliferative disease, polycythemia vera, is an increase in the number of circulating red cells above the gender-specific normal level. Erythrocytosis can be either relative (pseudoerythrocytosis) or absolute.
Why is erythropoiesis tightly regulated?
Erythropoiesis is tightly regulated because red cell production is an exponential process, which left uncontrolled can produce a dangerous increase in blood viscosity. Erythropoiesis is regulated by the hormone erythropoietin, which acts as a mitogen to trigger dormant immature erythroid precursors into cell cycle and a viability factor to maintain these precursors as they develop into mature erythrocytes.
Does erythrocytosis cause blood viscosity?
The symptoms and signs associated with erythrocytosis in part reflect its cause and in part are a consequence of the increased blood viscosity associated with the increase in red cell mass or decrease in the plasma volume. If the onset of erythrocytosis is gradual or it is not severe, there may be no symptoms.
Does erythrocytosis require a high hematocrit?
Therefore, by definition, the presence of erythrocytosis requires a persist ent elevation of the red cell count, hematocrit or hemoglobin level. In this regard, prior measurements of red cell number, hematocrit or hemoglobin are extremely helpful in assessing not only the persistence but the duration of the erythrocytosis.
Why is it important to look for erythrocytosis?
However, it is important for doctors to look for it because both primary and secondary erythrocytosis result in too many red blood cells. Secondary erythrocytosis has many causes. It can be caused by oxygen deprivation, which can result, for example, from. Smoking.
When is congenital erythrocytosis diagnosed?
Congenital erythrocytosis is usually diagnosed when a person develops symptoms at an early age or has family members who also have erythrocytosis. In addition to blood tests, doctors may do genetic testing to determine the specific cause.
What causes erythrocytosis in the bone marrow?
Secondary: Caused by a disorder that triggers increased production by normal blood-forming cells. Primary erythrocytosis occurs as a result of polycythemia vera , a myeloproliferative neoplasm in which abnormal cells in the bone marrow produce too many red blood cells along with excessive numbers of white blood cells and platelets.
Why does oxygen deprivation cause erythrocytosis?
High altitude. Oxygen deprivation causes an increase in erythropoietin, which stimulates the bone marrow to produce more red blood cells so that the blood can carry more oxygen. People who spend long periods of time in environments or circumstances low in oxygen, such as people who live at high altitude, often develop erythrocytosis severe enough ...
Why is phlebotomy used in erythrocytosis?
In some people, phlebotomy (in which some of the person's blood is removed) is used to lower the number of red blood cells. It is rare that phlebotomy is needed in secondary erythrocytosis. In tumor-associated erythrocytosis, removal of the tumor can be curative.
What hormones are used to treat erythropoietin?
Treatment with male hormones such as testosterone. Kidney problems, including tumors, cysts, and narrowing of the arteries that go to the kidneys. Tumors of the liver, brain, or adrenal gland. Genetic disorders that affect erythropoietin production (congenital erythrocytosis) Male hormones such as testosterone stimulate production of erythropoietin.
Can erythropoietin be high?
A high level of erythropoietin is often enough for doctors to diagnose secondary erythrocytosis. If the level is low, other tests for the primary erythrocytosis ( polycythemia vera ) will be done. Sometimes other specialized tests are done, especially when doctors need to determine an uncommon cause of erythrocytosis.
What is the next step in erythrocytosis?
For patients without a JAK2 mutation and normal erythropoietin, the next step is usually measurement of red cell mass —the definitive way to determine whether a true erythrocytosis exists.
What are the causes of secondary erythrocytosis?
More common conditions that underlie secondary erythrocytosis include hypoxia from respiratory disease (the most common of these secondary causes) and cardiac disease, renal disorders , and exogenous administration of erythropoietin (box 1). When no cause can be identified, the condition is termed idiopathic erythrocytosis.
Does iron help with erythrocytosis?
As is the case in all instances of erythrocytosis, iron replacement therapy may be used judiciously, but the packed cell volume must be closely monitored. Adenosine facilitates the release of erythropoietin and is also thought to influence the bone marrow response to erythropoietin.
Can erythropoietin cause secondary erythrocytosis?
Rare congenital mutation s in the erythro poietin receptor gene can also cause primary erythrocytosis. A variety of rare genetic abnormalities, such as mutations that cause high oxygen affinity haemoglobin, can cause congenital secondary erythrocytosis.
Does erythrocytosis cause breathlessness?
For example, in cyanotic heart disease, the physiological response to hypoxia is an increase in red cell production, which aids oxygen delivery, so treatment of erythrocytosis in this situation could worsen oxygen delivery to the tissues and the patient’s symptoms, such as breathlessness.
What is the best treatment for erythrocytosis?
Low-dose aspirin and venesection to an achievable target are the main therapeutic options that can be considered in the management of erythrocytosis. Specific guidance on venesection options should be considered with certain causes such as high oxygen-affinity hemoglobins.
What is the cause of erythrocytosis?
An erythrocytosis occurs when there is an increased red-cell mass. The causes of erythrocytosis are divided into primary, when there is an intrinsic defect in the erythroid cell, and secondary, when the cause is extrinsic to the erythroid cell. An idiopathic erythrocytosis occurs when the increased red-cell mass has no identifiable cause.
What is the difference between erythrocytosis and secondary erythrocytosis?
The initial issue, once the presence of an erythrocytosis has been established, is to determine the cause. An erythrocytosis can be primary where there is an intrinsic defect in the bone marrow resulting in increased red-cell production. In contrast, a secondary erythrocytosis arises when something else drives the production of red cells.
What causes erythropoietin to be truncated?
Primary causes consist mainly of the classical acquired polycythemia vera, but there are also rare congenital primary causes where there is an intrinsic defect in the erythropoietin receptor on the red cell resulting in a truncated receptor that signals for red-cell production without the presence of EPO.
Is erythrocytosis a rare disease?
However, overall these are rare disorders in clinical practice.
Is erythrocytosis acquired or congenital?
The causes of erythrocytosis can also be classified into congenital and acquired depending on the age of onset and family history. Using these definitions an extensive list of the causes of erythrocytosis results (see Table 1). Table 1. Causes of erythrocytosis. Primary erythrocytosis. Congenital.
What is secondary erythrocytosis?
Secondary erythrocytosis is erythrocytosis that develops secondary to disorders that cause tissue hypoxia, inappropriately increased erythropoietin production, or increased sensitivity to erythropoietin. (See also Overview of Myeloproliferative Neoplasms .) In secondary erythrocytosis, only red blood cells (RBCs) are increased, ...
What imaging is done for elevated erythropoietin?
Patients with elevated erythropoietin levels (and no indication of hypoxia) or microscopic hematuria should undergo abdominal imaging, central nervous system imaging, or both to seek a renal lesion or other tumor sources of erythropoietin.
What is the difference between erythrocytosis and polycythemia?
In secondary erythrocytosis, only red blood cells (RBCs) are increased, whereas in polycythemia vera, RBCs, white blood cells (WBCs), and platelets will usually be increased. Any elevation of hemoglobin or hematocrit above normal values for age and sex is considered erythrocytosis.
Why is phlebotomy limited?
Because in some cases the elevated hematocrit is physiologic, phlebotomy should be limited to the extent necessary to relieve symptoms (in contrast to polycythemia vera, where the goal is to normalize the hematocrit).
What causes erythrocytosis?
Erythrocytosis results when there is an increased red cell mass and thus an increased hemoglobin. The causes can be divided into primary intrinsic defects of the erythroid progenitor cell and secondary defects, where factors external to the erythroid compartment are responsible. Both can then be further divided into congenital ...
What is the classical acquired primary erythrocytosis?
The classical acquired primary erythrocytosis is polycythemia vera. Patients with polycythemia vera have an acquired abnormal clone, an erythrocytosis and frequently an increased white cell and platelet count. There are now well-accepted diagnostic criteria 7. ,
How are erythrocytoses classified?
Classification of Erythrocytoses. An erythrocytosis can be classified depending on the identified cause. The main division is on the basis of primary causes, where an intrinsic defect in the erythroid progenitor cell is associated with an enhanced response to cytokines; or secondary, where the increased red cell production is driven by factors ...
What are the proteins involved in erythropoiesis?
This system involves a number of proteins: the prolyl hydroxylases (PHDs), which exist in the isoforms PHD1, PHD2 and PHD3; hypoxia-inducible factor (HIF), which is composed of an unstable alpha subunit and a stable beta subunit; and the von-Hippel-Lindau tumor suppressor protein (VHL). HIF-α has three isoforms: HIF-1α, HIF-2α and HIF-3α.
Is erythrocytosis clear?
Clinical consequences of the various erythrocytoses are not clear, but in some groups thrombo embolic events have been described in young patients. Evidence is lacking to define best management, but aspirin and venesection to a target hematocrit should be considered.
Is EPO elevated in a third of patients?
One third have EPO levels below the normal range. The other two thirds have normal or elevated EPO levels, and so have a secondary erythrocytosis of unknown cause. Defects have been identified in some patients in these groups; however, no mechanism for the erythrocytosis has been identified in the majority of patients.
What is primary erythrocytosis?
Primary erythrocytosis refers to autonomous production of erythrocytes, typically from a myeloproliferative neoplasm (polycythemia vera [PV]). In contrast, secondary erythrocytosis is caused by a physiologically appropriate response to elevated serum erythropoietin levels.
How to differentiate primary and secondary erythrocytosis?
Initial tests to differentiate primary from secondary erythrocytosis include a complete blood count, peripheral blood film, renal and liver function tests, and determination of the ferritin level. 14 Erythrocyte mass can be measured directly to confirm absolute erythrocytosis by nuclear isotope dilution, but this test is not widely available in Canada. 1 As PV is associated with panmyelosis (expansion of all myeloid elements of the bone marrow), patients often have mild to moderate leukocytosis and thrombocytosis rather than isolated erythrocytosis. 15 Many patients with PV are iron deficient at diagnosis owing to erythroid expansion and altered iron metabolism. 17 A low mean erythrocyte volume and low ferritin level (< 35–45 ng/mL) support a diagnosis of iron deficiency. 18 Baseline abdominal–pelvic imaging is indicated when the clinical examination for splenomegaly gives equivocal findings or when endogenous erythropoietin production is suspected. 19
What is the target hematocrit for PV?
To reduce the risk of thrombosis, most patients with PV are treated with low-dose acetylsalicylic acid and phlebotomy to achieve a target hematocrit value of less than 0.45, whereas patients at high risk for thrombosis may receive cytoreductive therapy.
What is PV treated with?
Patients with PV are treated with daily low-dose acetylsalicylic acid (ASA) and phlebotomy to achieve a target hematocrit value of less than 0.45 based on the results of 2 randomized trials. 14, 28 Patients are monitored regularly (every 3–6 mo) for symptoms, treatment complications, cardiovascular events and disease progression.
What is the goal of PV treatment?
The goals of treatment of PV are to reduce the risk of arterial and venous thromboembolism, and minimize symptoms. 14 Unfortunately, existing treatments do not successfully reduce the risk of transformation to myelofibrosis or acute leukemia.
Is hydroxyurea good for leukemia?
Hydroxyurea is well tolerated, but common adverse effects include complications from cytopenia, oral and leg ulcers, gastrointestinal upset, drug fever, and skin and nail changes. An international retrospective cohort study of 1545 patients with PV showed that hydroxyurea does not increase the incidence of leukemia. 15
Does hydroxyurea help with PV?
Observational studies suggest that patients with high-risk PV benefit from cytoreductive therapy in addition to low-dose ASA therapy and phlebotomy. 14, 28 In North America, hydroxyurea (hydroxycarbamide), an orally administered antimetabolite chemotherapy drug that causes myelosuppression, is used owing to its known efficacy, low cost, oral formulation and acceptable toxicity profile (discussed below). A study showed that 51 patients with PV treated with hydroxyurea had a lower incidence of thrombosis at 2 years than historical controls treated with phlebotomy alone (7% v. 14%). 31 Some clinicians titrate the dosage of hydroxyurea to achieve peripheral blood count remission (hematocrit < 45% without phlebotomy, platelet count ≤ 400 × 10 9 /L and leukocyte count < 10 × 10 9 /L). 32 Response definitions for PV have been created for use in clinical trials, but their routine application to clinical practice is not evidence-based. 32 Other clinicians titrate the dosage of hydroxyurea to minimize the need for phlebotomy. An observational study showed that patients receiving hydroxyurea who needed 3 or more phlebotomy procedures per year had a significantly higher rate of thrombosis than those who required fewer phlebotomy procedures (21% v. 5% at 3 yr, p < 0.001). 33
