The complement system is a part of the immune system that is established in the blood and functions to keep the body healthy. The complement system consists of more than thirty proteins that flow throughout the blood and attack bacteria, viruses, and any foreign invader found throughout the body. More specifically, the complement system helps pathogen cells commit apoptosis, have inflammation, lyse, and opsonization. There are nine major complement proteins, which are C1-C9, that act a little different in each of the three pathways: the alternative, classical, and the lectin complement pathway. The difference between the lectin and the classical pathway is that in the lectin pathway, C1q is not involved but instead a mannan binding protein …show more content…
When the activity of the GPI-anchored proteins is reduced the mutation is then able to develop into the dominant gene in the creation of red blood cells. The complement system then recognizes the red blood cells as foreign cells causing the complement system to attack the mutation and when this occurs, red blood cells are destroyed faster than the normal 120 day life span, which leads to the deficiency of red blood cells and all the nutrients that they carry. In PNH, the red blood cells are missing two of the complement regulating proteins that are needed for GPI-anchored proteins to attach them to the cell membrane and since the two proteins are missing a lot of the nutrients and are in a deficient amount, it can lead to hemolytic anemia, iron deficiency anemia, aplastic anemia, blood clots, myelodysplasia, acute myelogenous leukemia, and even …show more content…
These include medications that help to slow the breakdown of the red blood cell which is the most effective treatment of PNH. Bone marrow transplantations are another method for treating PNH. The least effective treatment is the monitoring of the red blood cells without taking any medication or having transfusions. However, when Soliris, a complement inhibitor, and vitamin supplements are taken, the breakdown of red blood cells in PNH is greatly reduced. Side effects of using this drug include bright light sensitivity, a burning pain when you excrete urine, nausea, vomiting, a high fever with flu like symptoms, back or neck stiffness, headaches, purple spots on the skin, and a possibility of a seizure. Soliris falls in a group of medication called monoclonal antibodies which are antibodies that are identical clones of the parent cells made by immune cells. Soliris blocks the actions of the immune system that helps to destroy red blood cells causing PNH. Although medications may seem to be the best idea, a person may also try to undergo a blood transfusion as well as take natural supplements such as folic acid and iron in order to prevent anemia while also taking blood thinners to help prevent
The immune system is made up of several types of cells that work together to fight infections. Lymph cells (called lymphocytes) are the main type of cell in the adaptive immune system. There are 2 types of lymph cells: T cells and B cells. When B cells respond to an infection, they change into plasma cells. The plasma cells are found mainly in the bone marrow—the soft, inner part of some bones. The plasma cells
Protection- By white blood cells, antibodies and complement proteins that circulate in blood and defend again foreign microbes and toxins. Also white blood cells clot when body is injured.
Otherwise known as G6PD deficiency, people with an extreme case of this illness would experience hemolytic anemia, fever, and fatigue after eating fava beans or take drugs like primaquine. This is due to the fact that people with favism lack sufficient amount of the enzyme G6PD, which is important for protecting cells from chemical elements that would otherwise destroy the cells. The substances mentioned above act as the catalysts for the production of free radicals (un-paired electrons). As these un-paired electrons seek to pair with electrons in red blood cells with deficient amount of G6PD, it causes the cell membranes to burst. The loss of red blood cells when left untreated can cause the person to have kidney failure, heart failure, and death. As of now, there is no treatment for G6PG
Also known as normocytic anemia. This is the most frequent type of anemia most often happening to males over 85 years old. It is a common problem that occurs to men and women over 85 years old. Symptoms include and are caused by: a reduced production of normal-sized red blood cells even though presence of hemoglobin in the red blood cells is within the standard range; an increased production of HbS as is seen in sickle cell diseases; greater destruction and loss of red blood cells; an increase in plasma volume that is not compensated by anything else; a B2 (riboflavin) deficiency; and a B6 (pyridoxine) deficiency. (Brill & Baumgardner 2000).
Monitoring laboratories this quarter showed persistent eosinophilia and slightly low cell blood count for which client continues on daily oral Folic acid and Vitamin B12. Also elevated Ammonia levels that can be attributed to drug side effects (asymptomatic); he continued on daily Lactulose 60 ml total daily
As the body naturally makes new red blood cells, the anaemia will improve. If symptoms are more severe, a child may need to be hospitalized for supportive medical care.
Mutation of the CFH gene causes a disruption of the activation pathway in the body, therefore it begins to attack cells in the body specifically on the kidney or liver (Bu, Borsa, Gianluigi, & Smith, 2012). The attack on the kidney can lead to thrombotic microangiopathy (TMA), small vessel clots. Platelets will help minimize any leaking of vessels done by the attack of the disrupted activation pathway but after several clots form oxygen is cut off from certain parts of the kidney. When the kidney no longer receives oxygen, those parts begin to die off leading to thrombocytopenia, anemia and renal failure. When the kidney begins to fail, erythropoietin levels decrease. EPO binds to a receptor in the bone marrow to inhibit erythropoiesis. Therefore, RBC production in the bone marrow decreases when EPO levels decrease. (Bu, Borsa, Gianluigi, & Smith, 2012)
Ms. A is 26-year-old female with a history of menorrhagia and dysmenorrhea since she was 14-16 years old. In addition, she has taken aspirin during menstruation and for preventing and alleviating joint pain during golf season. Her signs and symptoms include shortness of breath, lethargy, light headache, tachycardia, tachypnea, and hypotension. Her laboratory values show low Hemoglobin (HB), Hematocrit (HCT) and Erythrocyte (RBC) but normal in Reticulocyte (REIC) count. According to Chen, Zieve, & Ogilvie (2014, p. 1), the normal value of HB is 12.1 to 15.1g/dl, HCT is 36.1% to 44.3%, RBC count is 4.2 to 5.2 x10/mm and RETIC count is 0.5% to 1.5% in female. The low Hemoglobin and Hematocrit can direct that Ms. A has anemia. The RBC smear can determine her classification of anemia. Her RBC smear showed microcytic and hypochromic cells. The evidence can indicate that Ms. A has Iron Deficiency Anemia (IDA).
Mutations lead to a genetic deficiency in clotting factor VIII, causing increased bleeding and affects males a majority of the time. HA can occur in homozygous females. 5-10% of patients with HA have dysfunctional proteins (factor VII) and 90% to 95% of HA cases are characterized by a quantitative deficiency pf factor VII. Patients usually experience bleeding episodes: the most serious bleeding sites include, joints, muscles, digestive tract, and brain. 30% of cases are caused by new mutations. Medications are prescribed to prevent bleeding episodes and for more severe cases blood coagulate and blood plasma treatments are
Pernicious anemia due to decreased B12 affects the hematopoietic,gastrointestinal and nervous systems of the body. The hematopoietic system is affected in that, B12 is essential for the formation of red blood cells, therefore without adequate B12, there is a reduction in the production of red blood cells causing abnormally large shaped red blood cells to be produced. Red blood cells carry oxygen , therefore the NP will anticipate patients with anemia to have symptoms like dizziness, fatigue, pallor, tinnitus, cardiomegaly with murmur, tachycardia, and chest pain. Additionally, symptoms of the affected gastrointestinal system include anorexia, weight loss, abdominal cramping, weight loss, and glossitis. Moreover, symptoms of the affected
Platelets disorders relate directly to how the platelets function where clotting factor deficiencies deal with the proteins in the blood that are used for controlling bleeding. Platelet disorders are identified based on the amount present and how they function. Idiopathic thrombocytopenic purpura is a platelet disorder where a person suffers from a low platelet count which causes them to easily bruise or bleed. This is due the immune system attacking platelets. Clotting factor deficiencies occur due to the body’s’ inability to make enough proteins to aid in clotting blood. These deficiencies are either due to hereditary or from some other medical disorder. Hemophilia is classified as a hereditary disorder because of a deficiency in one of the
Favism, a condition affecting around 400 million people worldwide is an enzyme deficiency. This enzyme is glucose-6-phosphate dehydrogenase or G6PD. G6PD is important because it prevents blood cells from getting destroyed by chemical elements. When a person has G6PD deficiency their blood cells break down causing a loss of red blood cells. People with G6PD deficiency is also likely to be diagnosed with hemolytic anemia which is the result of the early breakdown of red blood cells. Hemolytic anemia can cause weakness, fatigue and if it is left untreated kidney failure, heart failure, and possibly
In the mutated hybrid version of these two genes, they still act as gene repressors, and bind the sections of the DNA related to white blood cell proliferation. Instead of responding to signals that would normally cause RARa or PML to dissociate, the gene repressor remains on the DNA, effectively almost always blocking transcription of the gene. Since the proteins involved in assisting a white blood cell differentiate past the promyelocyte phase are rarely, if ever, transcribed, new white blood cells remain in this unmatured stage and cannot complete their differentiation into fully functioning leukocytes. In addition, the PML protein also loses other functions, such as inducing apoptosis and preventing uncontrolled cell proliferation. Because of this, cells that should begin apoptosis remain alive, and cell proliferation is not regulated, allowing uncontrolled growth. As a result of the cumulation of protein function abnormalities, PML-RARa blocks promyelocyte stage differentiation, and allows excess promyelocytes to proliferate. These immature cells then go on to accumulate in the bone marrow and joints, leading to pain and possible metastasis as they travel through the bloodstream.
Have you ever wondered how your body works? Did you ever think about how your body defends itself from outside invaders? Remember the last time you were sick. Did you know how your body fought and killed the sickness? All these questions can be answered by learning about one of the most important systems in your body, the immune system.
Hemophilia is a rare blood disease that interferes with the body’s ability to create protein-clotting factors, which is a special protein that is essential