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Related Concept Videos

Disorders of Erythrocytes01:27

Disorders of Erythrocytes

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Disorders of erythrocytes, or red blood cells (RBCs), include a range of conditions affecting their number, shape, or function.
Erythrocyte disorders can be broadly categorized into two main types: anemic and polycythemic conditions.
A low oxygen-carrying capacity of the blood due to the loss, lower production, or destruction of erythrocytes is termed anemia. Hemorrhagic anemia, for example, occurs when bleeding from an external wound or internal ulcer reduces erythrocyte counts.
On the other...
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Bone Marrow Sampling and Transplants01:22

Bone Marrow Sampling and Transplants

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Bone marrow transplant is a potential cure for several diseases, including cancer and specific genetic disorders. Notably, this procedure is applicable for patients suffering from aplastic anemia, certain types of leukemia, severe combined immunodeficiency disease (SCID), Hodgkin's disease, non-Hodgkin's lymphoma, multiple myeloma, thalassemia, sickle-cell disease, and certain cancers.
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Erythropoiesis01:14

Erythropoiesis

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Red blood cells  (RBCs) transport oxygen to all body tissues. These cells survive only for 120 days and then need to be replenished. Erythropoiesis is the process of RBC production. In healthy individuals, erythropoiesis ensures all tissues are amply supplied with oxygen. In addition, blood loss due to injury leads to a drop in the physiological oxygen level that will cause erythropoiesis. Any defect in erythropoiesis leads to several physiological disorders, including thalassemia, anemia,...
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Disorders of Hemostasis01:24

Disorders of Hemostasis

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Hemostasis, the process that stops bleeding after a blood vessel injury, is crucial for maintaining the integrity of the circulatory system. However, disorders of hemostasis can disrupt this delicate balance, leading to either excessive clotting or bleeding. These disorders can be broadly classified into thromboembolic disorders and bleeding disorders.
Thromboembolic Disorders
Two factors primarily cause thromboembolic conditions.
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Hematopoiesis01:21

Hematopoiesis

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The process of blood cell formation is called hematopoiesis. Hematopoiesis starts early during development, on the seventh day of embryogenesis. This phase of hematopoiesis is called the primitive wave, wherein the extraembryonic yolk sac allows the production of erythroid cells and endothelial cells from a common precursor called hemangioblast. The erythroid cells provide oxygen to support the growth of the rapidly dividing embryo. Hemangioblasts later develop into hematopoietic stem cells or...
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Factors Affecting Erythropoiesis01:24

Factors Affecting Erythropoiesis

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The cardiovascular system regulates the number of erythrocytes in the bloodstream to ensure optimal oxygen transport. It also prevents over-proliferation of these cells, which helps to maintain blood viscosity and flow rate.
Several factors influence the erythrocyte production rate, with tissue oxygen level being among the most critical. Intense exercise or high altitudes can cause tissue hypoxia, which triggers the kidneys to release more erythropoietin (EPO) into the bloodstream.
EPO then...
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Continuous Manual Exchange Transfusion for Patients with Sickle Cell Disease: An Efficient Method to Avoid Iron Overload
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[CME: Polycythemia vera].

Mirjam Wellauer Atencio1, Jeroen S Goede2

  • 1Akutgeriatrie, Kantonsspital Winterthur.

Praxis
|October 2, 2019
PubMed
Summary
This summary is machine-generated.

Polycythemia vera is a myeloproliferative disease involving three cell lines, often caused by a JAK2 mutation. Treatment focuses on phlebotomy and aspirin to prevent thrombotic events.

Keywords:
Chronische myeloproliferative NeoplasieErythrozytoseJAK2 mutationJAK2-MutationMyeloproliferative diseaseThromboembolien,increased red blood cellsthrombotic events

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Area of Science:

  • Hematology
  • Oncology

Background:

  • Polycythemia vera is a myeloproliferative neoplasm characterized by the overproduction of red blood cells, white blood cells, and platelets.
  • The disease is primarily associated with a mutation in the Janus kinase 2 (JAK2) gene.

Purpose of the Study:

  • To provide an overview of polycythemia vera, including its pathophysiology, clinical manifestations, risk factors, and current treatment strategies.
  • To emphasize the importance of preventing thrombotic events in patients with polycythemia vera.

Main Methods:

  • Review of current medical literature on polycythemia vera.
  • Synthesis of information regarding disease characteristics, diagnostic criteria, and therapeutic approaches.

Main Results:

  • Polycythemia vera involves the proliferation of erythroid, megakaryocytic, and granulocytic cell lines.
  • Common symptoms include pruritus and extremity pain due to increased red blood cell mass.
  • The most frequent complications are thrombotic events, with risk factors including age >60 and prior thrombosis.

Conclusions:

  • Phlebotomy and low-dose aspirin are foundational treatments for polycythemia vera, aimed at preventing thrombotic complications.
  • Cytoreductive therapy is often necessary for patients during the disease course to manage cell proliferation.