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group_4_presentation_2_-aplastic_anemia [2017/10/31 01:14]
mirosha [Treatment & Pharmacology]
group_4_presentation_2_-aplastic_anemia [2018/01/25 15:18] (current)
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 == Discovery == == Discovery ==
 {{ :​ehrlich.jpg?​100|}} {{ :​ehrlich.jpg?​100|}}
-There is a bit of discrepancy between who actually discovered aplastic anemia. Dr. Paul Ehrlich first treated the disease in 1888. He was observing a young woman who died prematurely from an illness. Her symptoms were limited amounts of red blood cells, excessive bleeding and fevers, similar to what we would now expect as aplastic anemia. Dr. Anatole Chauffard officially introduced the name “Aplastic Anemia” ​over one hundred ​years later in 1904. Originally it was thought that bone marrow only produced red blood cells, but it wasn’t until later that it was discovered that white blood cells and platelets were also limited in the body (Cuglievan, DePombo, & De Angulo , 2017). ​+There is a bit of discrepancy between who actually discovered aplastic anemia. Dr. Paul Ehrlich first treated the disease in 1888. He was observing a young woman who died prematurely from an illness. Her symptoms were limited amounts of red blood cells, excessive bleeding and fevers, similar to what we would now expect as aplastic anemia. Dr. Anatole Chauffard officially introduced the name “Aplastic Anemia” years later in 1904. Originally it was thought that bone marrow only produced red blood cells, but it wasn’t until later that it was discovered that white blood cells and platelets were also limited in the body (Cuglievan, DePombo, & De Angulo , 2017). ​
 Due to the complexity of the disease and its involvement in not one, but three types of cells, its not surprising it has been hard to find a cure that covers the entirety of the disease. ​ Due to the complexity of the disease and its involvement in not one, but three types of cells, its not surprising it has been hard to find a cure that covers the entirety of the disease. ​
  
  
 ===== Etiology ===== ===== Etiology =====
-Aplastic Anemia is caused either as a result of the accidental impairment of stem cells by the immmune system or because of genetic-related factors. ​+Aplastic Anemia is caused either as a result of the accidental impairment of stem cells by the immmune system or because of genetic-related factors ​(AAMDS, 2016)
  
 **Acquired Aplastic Anemia** ​ **Acquired Aplastic Anemia** ​
  
-Acquired Aplastic anemia is characertized as an autoimmune disease and can develop at any point in one's life. It is linked to  exposure of toxins(such as pesticides, arsenic, or benzene), radiation/​chemotherapy used for treating cancer/​disorders,​ medications used for the treating autoimmune diseases like rheumatoid arthritis and infectious diseases such as Hepatitis and HIV. Acquired aplastic anemia can also develop through pregnancy but this can be temporary until delivery. ​ +Acquired Aplastic anemia is characertized as an autoimmune disease and can develop at any point in one's life (NIH, 2012). It is linked to exposure of toxins(such as pesticides, arsenic, or benzene), radiation/​chemotherapy used for treating cancer/​disorders,​ medications used for the treating autoimmune diseases like rheumatoid arthritis and infectious diseases such as Hepatitis and HIV (NIH, 2012). Acquired aplastic anemia can also develop through pregnancy but this can be temporary until delivery. ​
- +
-https://​www.nhlbi.nih.gov/​health/​health-topics/​topics/​aplastic/​causes +
-http://​www.aamds.org/​diseases/​aplastic-anemia/​causes+
    
 **Hereditary Aplastic Anemia** ​ **Hereditary Aplastic Anemia** ​
  
-Hereditary Aplastic Anemia is far less common compared to acquired aplastic anemia. It is passed down from parent to child and is diagnosed during childhood. Fanconi anemia, Shwachman-Diamond syndrome, Dyskeratosis and Diamond-Blackfan anemia are inherited conditions that can harm stem cells contributing to aplastic anemia. ​+Hereditary Aplastic Anemia is far less common compared to acquired aplastic anemia ​(AAMDS, 2016). It is passed down from parent to child and is diagnosed during childhood. Fanconi anemia, Shwachman-Diamond syndrome, Dyskeratosis and Diamond-Blackfan anemia are inherited conditions that can harm stem cells contributing to aplastic anemia ​(AAMDS, 2016)
  
-Recent findings have identified a new type of hereditary aplastic anemia diagnosed in adulthood. It is prevalent in individuals who have family members with a history of aplastic anemia or fibrosis of the lungs or liver. It is characterized by excessive shortening of the telomeres and is only diagnosed through special tests. ​+Recent findings have identified a new type of hereditary aplastic anemia diagnosed in adulthood. It is prevalent in individuals who have family members with a history of aplastic anemia or fibrosis of the lungs or liver (AAMDS, 2016). It is characterized by excessive shortening of the telomeres and is only diagnosed through special tests. ​
  
-In most cases, the cause of aplastic anemia is unknown and is referred to as idiopathic aplastic anemia.+In most cases, the cause of aplastic anemia is unknown and is referred to as idiopathic aplastic anemia ​(AAMDS, 2016).
  
-http://​www.aamds.org/​diseases/​aplastic-anemia/​causes 
  
 **Genetic Research ** **Genetic Research **
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 Research by Solomou et al., suggest that genetic alterations to the Perforin-1 (PRF1) gene contributes bone marrow failure. In the study, researchers obtained DNA from 75 unrelated patients and analysed the DNA sequence for PRF1 (Solomou et al., 2007) . 4/5 patients with the PRF1 mutation diagnosed with aplastic anemia had hemphapgocytosis in the bone marrow (Solomou et al., 2007). This suggests that PRF1 mutation is involved with impaired cytotoxic T-cell activity, low Perforin protein level and a risk factor for bone marrow failure (Solomou et al., 2007). Research by Solomou et al., suggest that genetic alterations to the Perforin-1 (PRF1) gene contributes bone marrow failure. In the study, researchers obtained DNA from 75 unrelated patients and analysed the DNA sequence for PRF1 (Solomou et al., 2007) . 4/5 patients with the PRF1 mutation diagnosed with aplastic anemia had hemphapgocytosis in the bone marrow (Solomou et al., 2007). This suggests that PRF1 mutation is involved with impaired cytotoxic T-cell activity, low Perforin protein level and a risk factor for bone marrow failure (Solomou et al., 2007).
  
-Solomou, E., Gibellini, F., Stewart, B., Malide, D., Berg, M., & Visconte, V. et al. (2007). Perforin gene mutations in patients with acquired aplastic anemia. Blood, 109(12), 5234-5237. http://​dx.doi.org/​10.1182/​blood-2006-12-063495 
  
 ACD Genetic Alterations ACD Genetic Alterations
 The ACD gene interacts and changes the telomere binding protein (TPP1). Once ACD interacts with telomere and telomerase on the end of the chromosomes,​ it disrupts the interaction and results in alteration to the telomere function (Guo et al., 2014). ACD gene prevents the telomerase maintaining the telomere and protecting the cells, the blood cell loses structural integrity (Guo et al., 2014). Eventually, the blood cells will die and resulting in bone marrow failure and aplastic anemia. The ACD gene interacts and changes the telomere binding protein (TPP1). Once ACD interacts with telomere and telomerase on the end of the chromosomes,​ it disrupts the interaction and results in alteration to the telomere function (Guo et al., 2014). ACD gene prevents the telomerase maintaining the telomere and protecting the cells, the blood cell loses structural integrity (Guo et al., 2014). Eventually, the blood cells will die and resulting in bone marrow failure and aplastic anemia.
  
- 
-Guo, Y., Kartawinata,​ M., Li, J., Pickett, H., Teo, J., & Kilo, T. et al. (2014). Inherited bone marrow failure associated with germline mutation of ACD, the gene encoding telomere protein TPP1. Blood, 124(18), 2767-2774. http://​dx.doi.org/​10.1182/​blood-2014-08-596445 
  
  
 ===== Epidemiology ===== ===== Epidemiology =====
  
-Aplastic anemia commonly affects older demographics,​ particular individuals over the age of 60 years. However, it can also affect young adults between the ages of 20 to 25 and  is equally present ​among males and females. There is considerable geographic variability associated with aplastic anemia since there is 2-3 fold increase of the disease in East and South-East Asia compared to Europe and North America. In East Asia working men are disproportionately diagnosed with the disease, likely a result of exposure to toxic substances in the workplace. ​+Aplastic anemia commonly affects older demographics,​ particular individuals over the age of 60 years (Young & Kaufman, 2008). However, it can also affect young adults between the ages of 20 to 25 and  is equally present ​in males and females. There is considerable geographic variability associated with aplastic anemia since there is 2-3 fold increase of the disease in East and South-East Asia compared to Europe and North America ​(Young & Kaufman, 2008). In East Asia working men are disproportionately diagnosed with the disease, likely a result of exposure to toxic substances in the workplace. ​
  
 A study by Montane and et al., conducted between 1983-2003 reported the incidence rates of aplastic anemia. A total of 235 cases were identified with 2.34 million people diagnosed ​ yearly with the disease. Most cases reported were severe or very severe aplastic anemia, with occurrences increasing with age.  A study by Montane and et al., conducted between 1983-2003 reported the incidence rates of aplastic anemia. A total of 235 cases were identified with 2.34 million people diagnosed ​ yearly with the disease. Most cases reported were severe or very severe aplastic anemia, with occurrences increasing with age. 
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   * After 15 years=51%  ​   * After 15 years=51%  ​
  
-Older people diagnosed with a more severe form of the disease had a lower survival rate. There was a two-year survival rate among patients who were treated with bone marrow transplantation. In 49 of the 235 cases identified, individuals were diagnosed because of exposure to drugs, while 21 cases were a result of exposure to toxic agents. ​+Older people diagnosed with a more severe form of the disease had a lower survival rate (Montane et al., 2008). There was a two-year survival rate among patients who were treated with bone marrow transplantation. In 49 of the 235 cases identified, individuals were diagnosed because of exposure to drugs, while 21 cases were a result of exposure to toxic agents ​(Montane et al., 2008)
  
  
-https://​www.ncbi.nlm.nih.gov/​pubmed/​18322256 
-http://​www.haematologica.org/​content/​93/​4/​489#​sec-1 
-http://​www.haematologica.org/​content/​93/​4/​489.short 
  
  
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 {{:​aplastic_symptom.png?​300|}} {{::​bruise_clinical.png?​300|}} {{:​aplastic_symptom.png?​300|}} {{::​bruise_clinical.png?​300|}}
  
-Hussain, S. K. (2017, April 03). Aplastic Anemia and MDS Awareness Week: Let's Fight Against Anemia. Retrieved October 19, 2017, from https://​www.consumerhealthdigest.com/​health-awareness/​aplastic-anemia-and-mds-awareness-week.html +**Figure 1:** Various symptoms that are observed with aplastic anemia and clinical presentation ​(Hussain, S. K, 2017).
- +
-**Figure 1:** Various symptoms that are observed with aplastic anemia and clinical presentation.+
  
 === Myelodysplastic Syndrome === === Myelodysplastic Syndrome ===
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 {{:​myelodysplastic.png|}} {{:​myelodysplastic.png|}}
  
-Myelodysplastic Syndrome - Types, Causes, Symptoms, Diagnosis, Treatment & Prognosis. (2017, May 31). Retrieved October 19, 2017, from http://​www.medindia.net/​patientinfo/​myelodysplastic-syndrome.htm 
  
-**Figure 2:** The image above depicts abnormally formed blood cells within the bone marrow of a patient with myelodysplastic syndrome. ​+**Figure 2:** The image above depicts abnormally formed blood cells within the bone marrow of a patient with myelodysplastic syndrome ​(Myelodysplastic Syndrome, 2017)
  
 ===== Pathophysiology ===== ===== Pathophysiology =====
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 {{::​bloodcells_aplastic.png?​300|}} {{::​bloodcells_aplastic.png?​300|}}
  
-Aplastic Anemia & Myelodysplastic Syndromes. (2014, May 01). Retrieved October 19, 2017, from https://​www.niddk.nih.gov/​health-information/​blood-diseases/​aplastic-anemia-myelodysplastic-syndromes +**Figure 3:** This image shows the decrease in available blood cells circulating in an individual with aplastic anemia. It is important to note that the blood cells released into the stream are physiologically normal. The decreased number of blood cells is what causes the symptoms of aplastic anemia ​(Aplastic Anemia & Myelodysplastic Syndromes, 2014)
- +
-**Figure 3:** This image shows the decrease in available blood cells circulating in an individual with aplastic anemia. It is important to note that the blood cells released into the stream are physiologically normal. The decreased number of blood cells is what causes the symptoms of aplastic anemia. ​+
  
 Bone marrow is red and spongy material in your bone that produces stem cells which give rise to a number of cells in the blood (N., 2016). Aplastic bone marrow means the marrow is empty, or contains very few blood cells (hypoplastic). Stem cells in bone marrow produce red blood cells (RBCs), white blood cells (WBCs), and platelets. There are a number of factors that can temporarily or permanently affect the ability of bone marrow to produce blood cells, including: radiation & chemotherapy,​ exposure to toxic chemicals, use of certain drugs, autoimmune disorders, viral infections, and even pregnancy (N., 2016). When stem cells are damaged from these factors, they are unable to produce blood cells, and further replicate themselves, therefore cell numbers will continue to decrease, limiting the number of blood cells that can be released into the bloodstream. Bone marrow is red and spongy material in your bone that produces stem cells which give rise to a number of cells in the blood (N., 2016). Aplastic bone marrow means the marrow is empty, or contains very few blood cells (hypoplastic). Stem cells in bone marrow produce red blood cells (RBCs), white blood cells (WBCs), and platelets. There are a number of factors that can temporarily or permanently affect the ability of bone marrow to produce blood cells, including: radiation & chemotherapy,​ exposure to toxic chemicals, use of certain drugs, autoimmune disorders, viral infections, and even pregnancy (N., 2016). When stem cells are damaged from these factors, they are unable to produce blood cells, and further replicate themselves, therefore cell numbers will continue to decrease, limiting the number of blood cells that can be released into the bloodstream.
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 {{::​pathway_aa1.png?​300|}} {{::​pathway_aa1.png?​300|}}
  
-N. (2016, February 15). Getting to know Aplastic Anemia. Retrieved October 19, 2017, from http://​nursingcrib.com/​nursing-notes-reviewer/​getting-to-know-aplastic-anemia/​ +**Figure 4:** This image shows the three damaged pathways of the bone marrow stem cells to produce RBCs, WBCs & platelets ​(Getting to know Aplastic Anemia, 2016).
- +
-**Figure 4:** This image shows the three damaged pathways of the bone marrow stem cells to produce RBCs, WBCs & platelets.+
  
 === Stem Cells === === Stem Cells ===
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 {{:​multipotent.png|}} {{:​multipotent.png|}}
  
-Stem Cells and Types of Stem Cells. (n.d.). Retrieved October 19, 2017, from https://​explorecuriocity.org/​Explore/​ArticleId/​2439/​stem-cells-and-types-of-stem-cells-2439.aspx +**Figure 5:** This image depicts the pathway of a multipotent stem cell differentiating into many different specialized blood cells (Stem Cells and Types of Stem Cells, 2017)
- +
-**Figure 5:** This image depicts the pathway of a multipotent stem cell differentiating into many different specialized blood cells. ​+
  
 === Red Blood Cells === === Red Blood Cells ===
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 {{:​ironn.png|}} {{:​ironn.png|}}
  
-Stem Cells and Types of Stem Cells. (n.d.). Retrieved October 19, 2017, from https://​explorecuriocity.org/​Explore/​ArticleId/​2439/​stem-cells-and-types-of-stem-cells-2439.aspx +**Figure 6:** This image shows the development of red blood cells originating as stem cells in the bone marrow to full development as mature red blood cells released into the bloodstream ​(Stem Cells and Types of Stem Cells, n.d.)
- +
-**Figure 6:** This image shows the development of red blood cells originating as stem cells in the bone marrow to full development as mature red blood cells released into the bloodstream. ​+
  
 === White Blood Cells === === White Blood Cells ===
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 {{:​1234.jpg?​300|}} {{:​1234.jpg?​300|}}
  
-What is a platelet? (n.d.). Retrieved October 19, 2017, from http://​plateletcharity.co.uk/​uncategorized/​what-is-a-platelet/​ +**Figure 7:** This image shows the role of platelets in the blood stream, working with coagulation factors to clot injured blood vessels ​(What is a platelet?, n.d.) 
- +
-**Figure 7:** This image shows the role of platelets in the blood stream, working with coagulation factors to clot injured blood vessels. ​+
  
 ===== Screening & Diagnosis ===== ===== Screening & Diagnosis =====
  
-Aplastic anemia is characterized by reduction in hematopoiesis due to bone marrow damage, therefore diagnostic tests that detect abnormal blood levels and bone marrow damage are administered. These tests include a complete blood count, leukocyte differential,​ reticulocyte count and bone marrow aspirate and biopsy (NIH, 2017). A complete blood count is administered to evaluate overall health and measure levels of red blood cells, white blood cells, hemoglobin, hematocrit and platelets (NIH, 2017). A leukocyte differential test is administered to measure proportion of white blood cells to each other and detect presence of immature white blood cells (NIH, 2017). A reticulocyte count is administered to measure the speed at which reticulocytes,​ immature red blood cells, are made by the bone marrow and released into the blood stream (NIH, 2017). In order to measure bone marrow damage by determining bone marrow cellularity,​ bone marrow aspiration and biopsy are performed (NIH, 2017). During a bone marrow aspiration procedure, a needle is inserted into the bone with a tube attached to it, which creates suction and allows a small sample of bone marrow fluid to be extracted through the tube (NIH, 2017). As well, bone marrow biopsy is performed through insertion of biopsy needle into the bone (NIH, 2017). In this process, the centre of the needle is removed and the hollowed needle is moved deeper into the bone capturing a core of bone marrow within the needle (NIH, 2017). The extracted bone marrow sample undergoes further testing. Diagnosis of aplastic anemia requires performance of bone marrow aspiration and biopsy (NIH, 2017). Severe aplastic anemia is defined by the presence of 2 or more of the following: bone marrow cellularity (hematopoietic stem cell to adipocyte ratio) of 30% or less, absolute neutrophil count of 500 microliters ​or less, absolute reticulocyte count of 20,​000 ​microliters ​or less and platelet count of 20,​000 ​microliters ​or less (Baunstein, 2017). {{ :​r7_bonemarrowaspiration.jpg |}} **Figure 8:** The image displays bone marrow aspiration procedure. ​+Aplastic anemia is characterized by reduction in hematopoiesis due to bone marrow damage, therefore diagnostic tests that detect abnormal blood levels and bone marrow damage are administered. These tests include a complete blood count, leukocyte differential,​ reticulocyte count and bone marrow aspirate and biopsy (NIH, 2017). A complete blood count is administered to evaluate overall health and measure levels of red blood cells, white blood cells, hemoglobin, hematocrit and platelets (NIH, 2017). A leukocyte differential test is administered to measure proportion of white blood cells to each other and detect presence of immature white blood cells (NIH, 2017). A reticulocyte count is administered to measure the speed at which reticulocytes,​ immature red blood cells, are made by the bone marrow and released into the blood stream (NIH, 2017). In order to measure bone marrow damage by determining bone marrow cellularity,​ bone marrow aspiration and biopsy are performed (NIH, 2017). During a bone marrow aspiration procedure, a needle is inserted into the bone with a tube attached to it, which creates suction and allows a small sample of bone marrow fluid to be extracted through the tube (NIH, 2017). As well, bone marrow biopsy is performed through insertion of biopsy needle into the bone (NIH, 2017). In this process, the centre of the needle is removed and the hollowed needle is moved deeper into the bone capturing a core of bone marrow within the needle (NIH, 2017). The extracted bone marrow sample undergoes further testing. Diagnosis of aplastic anemia requires performance of bone marrow aspiration and biopsy (NIH, 2017). Severe aplastic anemia is defined by the presence of 2 or more of the following: bone marrow cellularity (hematopoietic stem cell to adipocyte ratio) of 30% or less, absolute neutrophil count of 500 cells per microliter ​or less, absolute reticulocyte count of 20,​000 ​cells per microliter ​or less and platelet count of 20,​000 ​cells per microliter ​or less (Baunstein, 2017). {{ :​r7_bonemarrowaspiration.jpg |}} **Figure 8:** The image displays bone marrow aspiration procedure. ​
  
 **Types** **Types**
  
-There are three types of Acquired Aplastic Anemia.+There are three types of Acquired Aplastic Anemia ​(AAMDS, 2016).
  
 **Moderate Aplastic Anemia (MAA)** **Moderate Aplastic Anemia (MAA)**
-Is the lowest level of severity characterized by low blood cells counts, but not as low compared to severe aplastic anemia. There are few to no symptoms associated with it and the doctor may not recommend treatment for the patient. However, the doctor may keep an eye on the patient’s blood count. The condition for MAA may stay the same for many years.+Is the lowest level of severity characterized by low blood cells counts, but not as low compared to severe aplastic anemia ​(AAMDS, 2016). There are few to no symptoms associated with it and the doctor may not recommend treatment for the patient. However, the doctor may keep an eye on the patient’s blood count. The condition for MAA may stay the same for many years (AAMDS, 2016).
  
 **Severe Aplastic Anemia (SAA)**  ​ **Severe Aplastic Anemia (SAA)**  ​
-Is present when a person exhibits at least two of the following: 1) neutrophil count is less  500 cells per microliter, 2) reticulocyte (young red blood cell) count is less 20,000 per microliter or/and 3) platelet count is less than 20,000 per microliter. ​+Is present when a person exhibits at least two of the following: 1) neutrophil count is less  500 cells per microliter, 2) reticulocyte (young red blood cell) count is less 20,000 per microliter or/and 3) platelet count is less than 20,000 per microliter ​(AAMDS, 2016)
  
 **Very Severe Aplastic Anemia (VSAA)** **Very Severe Aplastic Anemia (VSAA)**
-It the most severe form of aplastic anemia. The neutrophil count is less than 200 per microliter and blood count levels are similar those of someone with severe aplastic anemia.+It the most severe form of aplastic anemia. The neutrophil count is less than 200 per microliter and blood count levels are similar those of someone with severe aplastic anemia ​(AAMDS, 2016).
  
-http://​www.aamds.org/​diseases/​aplastic-anemia/​types 
  
  
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 ===== Current Research ===== ===== Current Research =====
 === Telomerase Based Treatment === === Telomerase Based Treatment ===
-  
-Bar, C., Povedano, J., Serrano, R., Benitez-Buelga,​ C., Popkes, M., & Formentini, I. et al. (2016). Telomerase gene therapy rescues telomere length, bone marrow aplasia, and survival in mice with aplastic anemia. Blood, 127(14), 1770-1779. http://​dx.doi.org/​10.1182/​blood-2015-08-667485 
 One new type of research being implemented for Aplastic Anemia is called Telomerase based treatment. The purpose of the treatment is to introduce telomerase, through gene therapy, into the bone marrow, in hopes that the enzymes will repair telomere length (Bar et al., 2016). Once telomerase is repaired then it will continue to generate blood cells. In the study, there was two strains of mice stimulated acquired Aplastic Anemia while the second strain had a mutation that associated with hereditary aplastic anemia. The results revealed that when both condition was treated with the telomerase treatment, there was an increase in the number of blood cells (Bar et al., 2016). One new type of research being implemented for Aplastic Anemia is called Telomerase based treatment. The purpose of the treatment is to introduce telomerase, through gene therapy, into the bone marrow, in hopes that the enzymes will repair telomere length (Bar et al., 2016). Once telomerase is repaired then it will continue to generate blood cells. In the study, there was two strains of mice stimulated acquired Aplastic Anemia while the second strain had a mutation that associated with hereditary aplastic anemia. The results revealed that when both condition was treated with the telomerase treatment, there was an increase in the number of blood cells (Bar et al., 2016).
    
 === Transplantation and Cychophosphamide === === Transplantation and Cychophosphamide ===
-DeZern, A., Zahurak, M., Symons, H., Cooke, K., Jones, R., & Brodsky, R. (2017). Alternative Donor Transplantation with High-Dose Post-Transplantation Cyclophosphamide for Refractory Severe Aplastic Anemia. Biology Of Blood And Marrow Transplantation,​ 23(3), 498-504. http://​dx.doi.org/​10.1016/​j.bbmt.2016.12.628 
    
 Another type of treatment being researched is a combination treatment with cychophosphamide,​ chemotherapy drug and then donor transplantation (DeZern et al., 2017). Researchers recruited 16 patients, aged 11-69 years old with severe aplastic anemia and were matched to bone marrow transplant between July 2011 through August 2016. They administer a patient'​s’ drugs to suppress the immune system and to prevent the donor marrow rejection. Then the patient receives bone marrow transplant. Afterwards, a high dose of chemotherapy drug called cyclophosphadmide is given to the patient for about a year. Researchers monitored the red and white blood cell and platelet count. The results reveal that the count for each had returned to normal without the need for blood transfusion. Researcher hypothesize that the reason the treatment is successful is because the drug destroys the patient'​s diseased immune cells but does not harm the donor’s blood cells therefore allow for disease free blood cells to survive in the patient (DeZern et al., 2017). Another type of treatment being researched is a combination treatment with cychophosphamide,​ chemotherapy drug and then donor transplantation (DeZern et al., 2017). Researchers recruited 16 patients, aged 11-69 years old with severe aplastic anemia and were matched to bone marrow transplant between July 2011 through August 2016. They administer a patient'​s’ drugs to suppress the immune system and to prevent the donor marrow rejection. Then the patient receives bone marrow transplant. Afterwards, a high dose of chemotherapy drug called cyclophosphadmide is given to the patient for about a year. Researchers monitored the red and white blood cell and platelet count. The results reveal that the count for each had returned to normal without the need for blood transfusion. Researcher hypothesize that the reason the treatment is successful is because the drug destroys the patient'​s diseased immune cells but does not harm the donor’s blood cells therefore allow for disease free blood cells to survive in the patient (DeZern et al., 2017).
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 Fatigue is one of the common symptoms of myelodysplastic syndrome (MDS), aplastic anemia (AA) and paroxysmal nocturnal hemoglobinuria (PNH) along with pain, depression, anxiety and stress. This can have a large impact on the quality of life of an individual. A study done by Escalante et al. looked into quality of life of individuals with MDS, AA and PNH. The average fatigue score of the patients was 28 (range 1-52) and the quality of life score was 67 (range 10-104) specifically for those with AA. The higher values of the ranges were indicative of high fatigue or better quality of life. Popular management strategies people used were preserving energy, exercise, and naps; the most popular strategy was preserving energy. (Escalante et al., 2015). People that are diagnosed with AA may be more limited to certain activities due to their increased amount of fatigue compared to those without AA.  Fatigue is one of the common symptoms of myelodysplastic syndrome (MDS), aplastic anemia (AA) and paroxysmal nocturnal hemoglobinuria (PNH) along with pain, depression, anxiety and stress. This can have a large impact on the quality of life of an individual. A study done by Escalante et al. looked into quality of life of individuals with MDS, AA and PNH. The average fatigue score of the patients was 28 (range 1-52) and the quality of life score was 67 (range 10-104) specifically for those with AA. The higher values of the ranges were indicative of high fatigue or better quality of life. Popular management strategies people used were preserving energy, exercise, and naps; the most popular strategy was preserving energy. (Escalante et al., 2015). People that are diagnosed with AA may be more limited to certain activities due to their increased amount of fatigue compared to those without AA. 
 +
 +
 +**Powerpoint Presentation**
 +{{:​aplastic_anemia_ppt._.pptx|}}
 ===== References ===== ===== References =====
  
 +Aplastic Anemia and MDS International Foundation. Causes. (2016). Retrieved October 25, 2017, from http://​www.aamds.org/​diseases/​aplastic-anemia/​causes
 +Aplastic Anemia and MDS International Foundation. Types. (2016). Retrieved October 25, 2017, from http://​www.aamds.org/​diseases/​aplastic-anemia/​types
 + 
 Bacigalupo, A. (2008). Treatment strategies for patients with severe aplastic anemia. Bone Marrow Transplantation,​ 42. doi:​10.1038/​bmt.2008.113 Bacigalupo, A. (2008). Treatment strategies for patients with severe aplastic anemia. Bone Marrow Transplantation,​ 42. doi:​10.1038/​bmt.2008.113
 + 
 +Bar, C., Povedano, J., Serrano, R., Benitez-Buelga,​ C., Popkes, M., & Formentini, I. et al. (2016). Telomerase gene therapy rescues telomere length, bone marrow aplasia, and survival in mice with aplastic anemia. Blood, 127(14), 1770-1779. http://​dx.doi.org/​10.1182/​blood-2015-08-667485
 + 
 +Braunstein, M.E. (2016) Aplastic Anemia. Retrieved 28 October 2017, from https://​www.merckmanuals.com/​en-ca/​professional/​hematology-and-oncology/​anemias-caused-by-deficient-erythropoiesis/​aplastic-anemia
 + 
 +Cuglievan, B., DePombo, A., & De Angulo , G. (2017). Aplastic anemia: the correct nomenclature matters. The Hematology Journal , 101 (9).
 +
 +DeZern, A. E., & Brodsky, R. A. (2011). Clinical management of aplastic anemia. Expert Review of Hematology, 4(2), 221–230. http://​doi.org/​10.1586/​ehm.11.11 ​   ​
 +
 +DeZern, A., Zahurak, M., Symons, H., Cooke, K., Jones, R., & Brodsky, R. (2017). Alternative Donor Transplantation with High-Dose Post-Transplantation Cyclophosphamide for Refractory Severe Aplastic Anemia. Biology Of Blood And Marrow Transplantation,​ 23(3), 498-504. http://​dx.doi.org/​10.1016/​j.bbmt.2016.12.628
 +
 +Escalante, C. P., Chisolm, S., Song, J., Richardson, M., Ellen, S., Lam, T., et al. (2015). Fatigue, quality of life and related symptoms: patient reported outcomes in myeloplastic syndrome, aplastic anemia and paroxysmal nocturnal hemoglobinuria. Blood , 126 (23).
 +
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