Hematology is the study of blood and how it relates to health and disease (“Blood Disorders”). Hematologists study blood disorders, what causes them, and how to prevent, treat, and cure them (“Blood Basics”). Advances in this field have helped increase the quality of life in people who suffer from blood disorders. For example, CML (Chronic Myelogenous Leukemia) used to be treated with chemotherapy, which kills rapidly dividing cells, both healthy and diseased. Because of studies in hematology, CML patients can be cured much more quickly and easily using imatinib, a “smart” drug that only targets cells with the mutation causing the disease. Additionally, hemophilia patients used to need a trip to the hospital for several days for large doses of plasma to stop bleeding episodes. Now, clotting factor concentrates are portable, easily stored, and do not require a hospital to use (“Advances in Hematology"). Blood is a bodily fluid that flows through the circulatory system. Its main function is to transport oxygen and nutrients to all body tissues, but it also transports waste and fights infections. The liquid part of blood is called “plasma” and is made mainly of water. Plasma transports blood cells, nutrients, wastes, antibodies, proteins, and hormones. Red blood cells, or erythrocytes, carry oxygen from the lungs to the rest of the body and carbon dioxide from the body to the lungs. The protein hemoglobin helps them do this, as well as gives them their red color. White blood cells, or leukocytes, are much less common than red blood cells, and protect the body from infected cells, tumors, bacteria, viruses, and other foreign materials. Platelets, or thrombocytes, are small pieces and fragments of cells that help with clotting when a vessel is damaged (“Blood Basics”). First, the vessel contracts to slow the bleeding. Then, platelets gather on the walls of the damaged vessel in a process called platelet adhesion. Platelets release substances that signal other platelets to join the clumping in the process of platelet aggregation. Lastly, clotting factors, proteins that help with clotting, that are flowing through the blood’s plasma activate on the surface of platelets to form a fibrin clot ("The Clotting Problem in Hemophilia”). Hemophilia was recognized by multiple different people at multiple different time periods and places.
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In the 12th century, Albucasis, an Arab physician, wrote about a family in which all of the males died of prolonged bleeding from minor injuries. In 1803, Dr. John Conrad Otto, a physician in Philadelphia wrote about how certain families had bleeding problems. Dr. Otto also acknowledged how the disease was hereditary and more common in males. “Hemophilia” was first coined in a description of the disease by Hopff at the University of Zurich in 1828 ("The History of Hemophilia”). Hemophilia causes bleeding to last much longer than normal, as well as bleeding in joints, muscles, and organs. …show more content…
This can cause major damage if untreated or if it happens in great frequency. Sometimes, bleeding can start for no clear reason; this is called a spontaneous bleed. People suffering from hemophilia call a bleeding episode a “bleed” and can feel when one happens ("How Blood Works in a Person With Hemophilia"). People with hemophilia need to infuse clotting factors very frequently; depending on which factor they need and which product they use, this ranges from weekly, daily, hourly, and within minutes. When new products enter the market, it can sometimes be difficult for patients of hemophilia to decide to switch products. Patients must weigh cost, the possible benefits of the new treatment, and the risk of switching compared to the current treatment (Clement, "New Factor Concentrates, The Future Is Now!"). In hemophilia, the genes that produce clotting factors VIII and IX are mutated and do not perform correctly. These genes are located on the X-chromosome, making hemophilia a sex-linked disorder that is mostly found in men. Hemophilia A is more common and causes a deficiency of clotting factor VIII, and hemophilia B is rarer and causes a deficiency of clotting factor IX ("Carriers of Hemophilia A and B"). As stated earlier, clotting factors are vital to producing an effective fibrin clot. Hemophilia only affects factors VIII and IX, but clotting factors work in a chain with a specific order. If one clotting factor is absent,
In the 12th century, Albucasis, an Arab physician, wrote about a family in which all of the males died of prolonged bleeding from minor injuries. In 1803, Dr. John Conrad Otto, a physician in Philadelphia wrote about how certain families had bleeding problems. Dr. Otto also acknowledged how the disease was hereditary and more common in males. “Hemophilia” was first coined in a description of the disease by Hopff at the University of Zurich in 1828 ("The History of Hemophilia”). Hemophilia causes bleeding to last much longer than normal, as well as bleeding in joints, muscles, and organs. …show more content…
This can cause major damage if untreated or if it happens in great frequency. Sometimes, bleeding can start for no clear reason; this is called a spontaneous bleed. People suffering from hemophilia call a bleeding episode a “bleed” and can feel when one happens ("How Blood Works in a Person With Hemophilia"). People with hemophilia need to infuse clotting factors very frequently; depending on which factor they need and which product they use, this ranges from weekly, daily, hourly, and within minutes. When new products enter the market, it can sometimes be difficult for patients of hemophilia to decide to switch products. Patients must weigh cost, the possible benefits of the new treatment, and the risk of switching compared to the current treatment (Clement, "New Factor Concentrates, The Future Is Now!"). In hemophilia, the genes that produce clotting factors VIII and IX are mutated and do not perform correctly. These genes are located on the X-chromosome, making hemophilia a sex-linked disorder that is mostly found in men. Hemophilia A is more common and causes a deficiency of clotting factor VIII, and hemophilia B is rarer and causes a deficiency of clotting factor IX ("Carriers of Hemophilia A and B"). As stated earlier, clotting factors are vital to producing an effective fibrin clot. Hemophilia only affects factors VIII and IX, but clotting factors work in a chain with a specific order. If one clotting factor is absent,