Polycythemia is a clinical disorder characterized by a relative increase in the number of red blood cells in the peripheral blood. In this condition, there is an increase in the hemoglobin content, red blood cell count and hematocrit.
Polycythemia can present with the following signs and symptoms:
Entire Body System
Clinical features include facial flushing, pruritus, erythromelalgia, headaches, and dizziness. Severe complications such as thrombotic events (e.g., stroke, Budd-Chiari syndrome) may occur. [amboss.com]
As the disease progresses, symptoms include: Shortness of breath Dizziness Itchy or flushed skin Fatigue Headaches A tingling or burning sensation in the hands and feet What causes polycythemia vera? PV begins in the bone marrow. [familydoctor.org]
If the skin is flushed or itchy, particularly after a hot bath, it may indicate a diagnosis of polycythemia. The doctor may also check for a history of smoking or alcohol abuse or a family history of kidney disease. [chealth.canoe.com]
Symptoms Symptoms of PV may include: Fatigue and/or weakness Headache Dizziness Shortness of breath and difficulty breathing while lying down Visual disturbance, such as double vision, blurred vision, and blind spots Inability to concentrate Night sweats Flushed [hopkinsmedicine.org]
One TAPS donor and one TAPS recipient had neurodevelopmental impairment; bilateral deafness at 9.5 years old and spastic paralysis at 2 years old, respectively. [ncbi.nlm.nih.gov]
This is a case of a 55-year-old Ghanaian who presented to our tertiary facility after a 2-month delay with a history of sudden onset of right-sided hemiparesis and expressive aphasia. [ncbi.nlm.nih.gov]
Polycythemia is diagnosed on the basis of clinical features and laboratory findings. The following tests are used in the work up of patients with polycythemia:
- Complete blood count (CBC): This determines the red cell mass, red cell count and hematocrit among suspected patients. Thrombocyte count and leukocyte count can also be derived from this laboratory test.
- Leukocyte alkaline phosphatase level: The leukocyte alkaline phosphatase level is generally elevated up to 100U/L in almost 70% of patients of polycythemia.
- Routine coagulation tests: Prothrombin time (PT) and activated partial thromboplastin time (aPTT) are temporarily lengthened in polycythemic patients.
- Imaging studies: The use of ultrasonography and computed tomography (CT scan) reveals the presence of an splenomegaly in the patients of polycythemia.
For example, isolated thrombocytosis, associated with a JAK2 mutation, can be the presenting manifestation of essential thrombocytosis, primary myelofibrosis, or polycythemia vera, while both essential thrombocytosis and primary myelofibrosis can evolve [cancertherapyadvisor.com]
Polycythemia is frequently used synonymously with erythrocytosis; however, polycythemia may imply leukocytosis and thrombocytosis, as well as erythrocytosis. Erythrocytosis and Polycythemia Overview of Erythrocytosis and Polycythemia [msdvetmanual.com]
Alternative form: polycythaemia Synonyms: erythrocytosis erythrocythemia hypercythemia hypererythrocythemia Compare: anemia, leukocytosis, thrombocytosis. [biology-online.org]
Differential diagnosis Differential diagnosis includes elimination of Ph1 negative myeloproliferative disease (MPD) including essential thrombocytosis and agnogenic myeloid metaplasia (see these terms). [orpha.net]
For patients with marked thrombocytosis the administration of anagrelide may be indicated. Although it is not a curable disease, it is, because of its chronic nature, usually effectively managed over long periods of time. [fcarreras.org]
The elevated blood cell mass results in hyperviscosity, which is associated with slow blood flow and an increased risk of thrombosis. The disorder typically initially has a long asymptomatic period, then assumes a chronic, insidious course. [amboss.com]
In the neonatal period, polycythemia-induced hyperviscosity can lead to altered blood flow and can subsequently affect organ function. [emedicine.medscape.com]
Symptoms of PV are often insidious in onset, and they are often related to blood hyperviscosity. Symptoms are related to 1. hyperviscosity 2. sludging of blood flow 3. thromboses which lead to poor oxygen delivery and symptoms that include headache [slideshare.net]
Decreased Oxygen Saturation
The diagnosis of polycythemia as a result of smoking was based on an elevated total red blood cell volume, decreased oxygen saturation ( 92%) on arterial blood gas measurement, evidence of COPD, and elevated carboxyhemoglobin levels ( 2.5%).11x11Smith [jvascsurg.org]
Polycythemia can be treated symptomatically by reducing the red blood cell mass by phlebotomy or the physical drainage of blood from a given vein. Patients who have undergone phlebotomy have a decreased chance of developing a thrombosis in the near future . In patients with polycythemia vera, regular phlebotomy may be indicated because of the chronic nature of the disease.
Patients with thicker blood viscosity benefit from the regular intake of low dose aspirin to prevent blood clots. The use of aspirin also addresses the burning sensation of the hands and feet felt by the patients suffering from polycythemia.
Anti-itch medications like oral anti-histamines may be given to control the itching felt in the palms and soles of patients. Ultraviolet light (UV) has been arbitrarily used to relieve the itch symptoms in the skin.
Polycythemia is a type of chronic disease requiring long term treatment regimen. Individuals who strictly adhere to the treatment can live a healthy life without any complications. Patients with secondary polycythemia (like smokers and asthmatics) have a greatly reduced the risk for cardiovascular events when regular phlebotomy is done.
Polycythemia can either be congenital in nature or can be acquired. The primary type of polycythemia occurs when there is a overproduction of red blood cells by the bone marrow. The most common type of primary polycythemia is known as polycythemia vera. Secondary polycythemia occurs due to certain underlying diseases.
Primary polycythemia is a rare occurrence. It has been estimated that in the United States, there are just about 45 - 57 cases per 100,000 population . Individuals above 60 years of age are at an increased risk of contracting this blood condition. The incidence of primary polycythemia, in individuals younger than 20 years is estimated to be about 0.01%.
Studies have shown that males are more prone to contract this condition than females, with the male to female ratio being 1.2:2.2 .
Mutations that are either acquired or inherited are the primary factors that favor the development of primary polycythemia. Polycythemia vera, which is the most common form of primary polycythemia, occurs as a result of excessive production of red blood cells (RBC's) due to certain abnormalities in the bone marrow . It is a type of myoproliferative disease which is characterized by increased production of myeloid cells . In such cases, excess production of RBC's is also accompanied by excessive production of white blood cells (WBC's) and platelets (thrombocytes).
Secondary form of polycythemia usually occurs due to an underlying disease condition and is characterized by 6 - 8 million erythrocytes per cubic millimeter of blood. Another factor which greatly triggers the development of polycythemia is the state of reduced oxygen supply, typically seen among chronic smokers and asthmatics. Such conditions cause an increased production of RBC's causing the development of polycythemia.
Patients diagnosed with polycythemia should have regular exercises to prevent blood clot formation and improve blood vessel circulation in the body. Tobacco should be strictly prohibited to prevent polycythemia from recurring. Polycythemic patients are prone to the development of sores in hands and feet. A good wound care is imperative to prevent complications especially in tissues with poor blood circulation.
The condition develops either due to increase in red blood cell count or decrease in plasma volume. In the former case, the condition is referred to as absolute polycythemia; whereas in the latter, it is referred to as relative polycythemia.
Polycythemia is a disorder characterized by an abnormally increased number of red blood cells in the blood. Polycythemia may either occur at birth or later in life secondary to conditions such as smoking, dehydration and obesity. Patients of polycythemia perennially complain of fatigue and disturbed balance. Itching is also a common feature. Headaches and dizziness due to frequent bouts of high blood pressure may also be observed. Blood is reguarly removed from the patients of polycthemia to keep the red cell counts at an acceptable level. Medicines are given to prevent the formation of blood clots and to relieve the symptoms of itching.
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