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===== 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 (National Institues of Health, 2012). 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. |
**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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===== 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 ===== | ||
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**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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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). | ||
+ | |||
+ | 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 | ||
+ | Health 24 . (n.d.). The seven types of anemia. Retrieved October 25, 2017, from Health 24: http://www.health24.com/Lifestyle/Your-Blood/Anaemia-20130216-2 | ||
+ | |||
+ | How Is Aplastic Anemia Diagnosed? - NHLBI, NIH. (2017). Nhlbi.nih.gov. Retrieved 28 October 2017, from https://www.nhlbi.nih.gov/health/health-topics/topics/aplastic/diagnosis | ||
+ | |||
+ | How Is Aplastic Anemia Treated? - NHLBI, NIH. (2017). Nhlbi.nih.gov. Retrieved 28 October 2017, from https://www.nhlbi.nih.gov/health/health-topics/topics/aplastic/treatment | ||
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 | 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 | ||
+ | |||
+ | Killick SB, Win N, Marsh JC, et al. (1997) Pilot study of HLA alloimmunization after transfusion with pre-storage leucodepleted blood products in aplastic anaemia. Br. J. Haematol. 97(3), 677–684. | ||
+ | |||
+ | Kojima, S. (2016). Why is the incidence of aplastic anemia higher in Asia? Retrieved October 24, 2017, from http://www.tandfonline.com/doi/full/10.1080/17474086.2017.1302797 | ||
+ | |||
+ | Laundy GJ, Bradley BA, Rees BM, Younie M, Hows JM. (2004) Incidence and specificity of HLA antibodies in multitransfused patients with acquired aplastic anemia. Transfusion. 44(6), 814–825. | ||
+ | |||
+ | Mandal, A. (2013). What is hematology? Retrieved October 25, 2017, from News Medical Life Sciences : https://www.news-medical.net/health/What-is-Hematology.aspx | ||
+ | |||
+ | Marsh J, Socie G, Tichelli A, et al. (2010) Should irradiated blood products be given routinely to all patients with aplastic anaemia undergoing immunosuppressive therapy with anti-thymocyte globulin (ATG)? A survey from the European Group for Blood and Marrow Transplantation Severe Aplastic Anaemia Working Party. Br. J. Haematol. 150(3), 377–379. | ||
+ | |||
+ | Montané, E., Ibáñez, L., Vidal, X., Ballarín, E., Puig, R., García, N., . . . Catalan, A. N. (2008). Epidemiology of aplastic anemia: a prospective multicenter study. Retrieved October 24, 2017, from https://www.ncbi.nlm.nih.gov/pubmed/18322256 | ||
Myelodysplastic Syndrome - Types, Causes, Symptoms, Diagnosis, Treatment & Prognosis. (2017, May 31). Retrieved October 19, 2017, from http://www.medindia.net/patientinfo/myelodysplastic-syndrome.htm | Myelodysplastic Syndrome - Types, Causes, Symptoms, Diagnosis, Treatment & Prognosis. (2017, May 31). Retrieved October 19, 2017, from http://www.medindia.net/patientinfo/myelodysplastic-syndrome.htm | ||
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/ | 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/ | ||
+ | |||
+ | National Heart, Lung and Blood Institute. (2012). What is aplastic anemia? Retrieved October 24, 2017, from National Heart, Lung and Blood Institute: https://www.nhlbi.nih.gov/health/health-topics/topics/aplastic | ||
+ | |||
+ | National Heart, Lung, and Blood Institute. (2012). What causes aplastic anemia? Retrieved October 18, 2017, from https://www.nhlbi.nih.gov/health/health-topics/topics/aplastic/causes | ||
Skalak, R., Tozeren, A., Zarda, R., & Chien, S. (1973). Strain Energy Function of Red Blood Cell Membranes. Biophysical Journal, 13(3), 245-264. doi:10.1016/s0006-3495(73)85983-1 | Skalak, R., Tozeren, A., Zarda, R., & Chien, S. (1973). Strain Energy Function of Red Blood Cell Membranes. Biophysical Journal, 13(3), 245-264. doi:10.1016/s0006-3495(73)85983-1 | ||
+ | Socie G, Henry-Amar M, Bacigalupo A, et al. (1993) Malignant tumors occuring after treatment of aplastic anemia. N. Engl. J. Med. 329, 1152–1157. | ||
+ | |||
+ | 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 | ||
Stemple, D. (2009). Faculty of 1000 evaluation for Hematopoietic stem cell development is dependent on blood flow. F1000 - Post-publication peer review of the biomedical literature. doi:10.3410/f.1161543.623346 | Stemple, D. (2009). Faculty of 1000 evaluation for Hematopoietic stem cell development is dependent on blood flow. F1000 - Post-publication peer review of the biomedical literature. doi:10.3410/f.1161543.623346 | ||
+ | |||
+ | Types. (2017). Aplastic Anemia and MDS International Foundation. Retrieved 28 October 2017, from http://www.aamds.org/diseases/aplastic-anemia/types | ||
+ | |||
+ | Young, N. and Kaufman, D. (2008). The epidemiology of acquired aplastic anemia. Retrieved October 26, 2017, from http://www.haematologica.org/content/93/4/489#sec-1 |