Aplastic Anemia


Article Author:
Christine Moore


Article Editor:
Koyamangalath Krishnan


Editors In Chief:
Chaddie Doerr


Managing Editors:
Avais Raja
Orawan Chaigasame
Carrie Smith
Abdul Waheed
Khalid Alsayouri
Trevor Nezwek
Radia Jamil
Patrick Le
Anoosh Zafar Gondal
Saad Nazir
William Gossman
Hassam Zulfiqar
Hussain Sajjad
Steve Bhimji
Muhammad Hashmi
John Shell
Matthew Varacallo
Heba Mahdy
Ahmad Malik
Sarosh Vaqar
Mark Pellegrini
James Hughes
Beata Beatty
Beenish Sohail
Nazia Sadiq
Hajira Basit
Phillip Hynes


Updated:
5/14/2019 3:28:48 PM

Introduction

Aplastic anemia refers to the syndrome of chronic primary hematopoietic failure from injury leading to diminished or absent hematopoietic precursors in the bone marrow and attendant pancytopenia.[1][2]

Etiology

Injury to bone marrow occurs in a multitude of settings (table 1). The most common etiology, idiopathic, accounts for 65%. Fanconi anemia is the most common hereditary cause. It presents in the late first decade with pancytopenia, organ hypoplasia, and bone defects including abnormal radii, absent thumbs, and short stature. Seronegative hepatitis is responsible for 5% to 10% of total cases. Telomerase defects are found in 5% to 10% of adult-onset aplastic anemia. Some of these associations are very rare for example, eosinophilic fasciitis.[3][4]

Epidemiology

Accurate information regarding the epidemiology regarding the incidence of aplastic anemia is generally not available. Studies suggest the incidence is 0.6 to 6.1 cases per million population; this rate is largely based on data from retrospective reviews of death registries.[5][6]

The male-to-female ratio is approximately 1:1. Although aplastic anemia occurs in all age groups, a small peak in the incidence is observed in childhood. A second peak is found in the 20 to 25-year-old age group.

Pathophysiology

Two interrelated explanations exist for aplastic anemia: extrinsic immune-mediated suppression of hematopoietic stem cells and intrinsic abnormality of marrow progenitors.[7][8]

Damaged hematopoietic stem cells mature into self-reactive T-helper cells (T1) that release cytokines interferon-? (IFN?) and tumor necrosis factor (TNF) to propagate a cytotoxic cascade to kill and suppress other hematopoietic stem cells. The exact antigens T1 cells target are unclear, but one appears to be the glucose phosphate inositol (GPI)-linked protein on cell membranes (the mechanism behind pancytopenia in PNH). Also, the genes for apoptosis and death pathways are upregulated. Moreover, immunosuppressive therapy targeting T-cells leads to a response in two-thirds of patients with idiopathic aplastic anemia, and patients with graft-versus-host disease develop aplasia in the setting of healthy bone marrow progenitors.

In the second theory, stem cells with inherent defects lose the capacity to differentiate and proliferative. Their inability to dedifferentiate can lead to clonal evolution into hematologic neoplasms, for example, myelodysplastic syndrome. This is common in patients with Fanconi anemia. Partial defects in telomeres, the component of DNA intertwined with cell division, lead to premature hematopoietic stem cell exhaustion and marrow aplasia as well. Shortened telomeres are present in cells of half the patients with aplastic anemia.

Histopathology

Bone marrow biopsy from patients with aplastic anemia will be markedly hypocellular. Fat cells and fibrotic stroma replace normal bone marrow tissue. Stray lymphocytes and plasma cells remain, the remainder is devoid of marrow progenitors.

History and Physical

Aplastic anemia presents at any age with equal distribution among gender and race. Symptoms related to the absent cell lineage (anemia, progressive weakness, pallor, and dyspnea; neutropenia, frequent and persistent minor infections, or sudden onset febrile illness; thrombocytopenia, ecchymoses, mucosal bleeding, and petechiae). Splenomegaly is not seen, and its presence suggests an alternative diagnosis. Labs will demonstrate macrocytic normochromic anemia with reticulopodia, neutropenia, and thrombocytopenia. There must be no cytologic abnormalities as this would suggest an underlying hematologic process.

Evaluation

The diagnostic criteria for aplastic anemia are the following: the presence of bone marrow hypocellularity and  2 or more cytopenias (reticulopodia less than 1% or less than 40,000/microliter, neutropenia less than 500/microliter, or thrombocytopenia less than 20,000/microliter). The moderate disease has less than 30% bone marrow cellularity; the severe disease has less than 25% cellularity or less than 50% cellularity containing fewer than 30% hematopoietic cells, and very severe meets severe criteria plus neutropenia less than 200/µL. Aspirate of bone marrow has little yield (“dry tap”). Bone marrow biopsy is essential: it will be markedly hypocellular and devoid of marrow progenitors. Genetic testing with flow cytometry and fluorescence in situ hybridization (FISH) is useful to exclude hematologic malignancies responsible for pancytopenia. Additional testing depends on the underlying condition responsible for bone marrow failure, for example, telomerase mutation testing for dyskeratosis congenital.[9][10]

Treatment / Management

Management of aplastic anemia is directed at the underlying cause. Remove the offending agent(s), if possible. Some drugs have been discontinued in the United States due to their associations with aplastic anemia (e.g., Ticlopidine, a platelet aggregation inhibitor used as primary/secondary stroke prevention or dual antiplatelet therapy following percutaneous coronary intervention; phenylbutazone, a NSAID used as an analgesic and antipyretic). Aplastic anemia associated with pregnancy is self-limited and ends with delivery. Patients with thymoma usually have full bone marrow recovery following thymectomy.[2][11][12]

For patients in whom no reversible cause is found, treatment depends on age, disease severity, donor availability, and performance status. Young patients (younger than 50 years) in good health with severe disease should undergo allogeneic hematopoietic cell transplant (HCT) before initial immunosuppressive therapy. Older patients (50 years or older) in good health and young patients without an HCT donor receive full-dose immunosuppressive therapy using eltrombopag, horse/rabbit anti-thymocyte globulin (ATG), cyclosporine A, and prednisone. This combination can be tailored to single-agent eltrombopag, ATG, or cyclosporine A for less healthy individuals. Eltrombopag is a thrombopoietin non-peptide agonist that increases platelet counts and activates intracellular signal transduction pathways to increase proliferation and differentiation of marrow progenitor cells. ATG eliminates antigen-reactive T-lymphocytes and induces hematologic responses in aplastic anemia. Cyclosporine A inhibits the production and release of interleukin-II (IL-2) and inhibits IL-2 induced activation of resting T-lymphocytes. Prednisone induces cell death of immature lymphocytes. Clinical studies are underway for alternative therapies used as second-line agents.

Supportive care includes infection prophylaxis/treatment and transfusions (leukoreduced red blood cells for Hb less than 7 mg/dL or platelets less than 10,000/microliters or less than 50,000/microliters for active blood loss). Monitor for secondary hemochromatosis and administer iron chelators as indicated. Use of growth factors such as erythropoietin or granulocyte colony-stimulating factors is not recommended because there are inadequate precursor cells to generate sufficient responses.

Survival in aplastic anemia depends largely on age, disease severity, and response to initial therapy. Those who recover following drug cessation or treatment of underlying condition have a stable clinical course, as well as those with self-limited processes. Five-year survival is greater than 75% for patients who undergo bone marrow transplant from a suitable donor. The majority of untreated patients die within one year from disease-related complications (e.g., bleeding, infections, or transformation to lymphoproliferative disorders).

Differential Diagnosis

Pancytopenia can occur due to myelophthistic syndrome, a pathologic process that replaces normal bone marrow. Etiologies are solid tumor metastases (ex. lung, breast, and prostate malignancies), lymphoid or myeloid neoplasms (ex. acute myelogenous leukemia), myelofibrosis, hemophagocytic lymphohistiocytosis, osteopetrosis, or Gaucher disease. The bone marrow biopsy will not be hypocellular and reflect the underlying disease.

Isolated failure of single hematopoietic lineage is common (ex. agranulocytosis, pure red cell aplasia). These share the same causes for aplastic anemia (ex. propylthiouracil and agranulocytosis, thymoma and pure red cell aplasia). Patients will have symptoms related to the cell line involved, not all three.

Prognosis

Survival in aplastic anemia depends largely on age, disease severity, and response to initial therapy. Those who recover following drug cessation or treatment of underlying condition have stable clinical courses, as well as those with self-limited processes. Five-year survival is >75% for patients who undergo bone marrow transplant from a suitable donor. The majority of untreated patients die within one year from disease-related complications (ex. bleeding, infections, or transformation to lymphoproliferative disorders).

Complications

The most common complications of aplastic anemia include bleeding, infections, or transformation to lymphoproliferative disorders. These are managed by surveillance and symptomatic treatment including antibiotics, chemotherapy, and/or transfusions.

Deterrence and Patient Education

Aplastic anemia is a condition in which the body is unable to make blood cells that perform vital functions including infection control, oxygen transport, and tissue repair following injury. While there are many causes for this disease, many patients never find the underlying issue. Recovery is excellent for patients with identifiable causes or disease that resolves spontaneously. Patients can opt for bone marrow transplant and additional medications to provide blood products to the body. Monitor for disease complications such as bleeding, cancers or infections, and notify physicians of any changes.

Pearls and Other Issues

Pancytopenia can occur due to the myelophthisic syndrome, a pathologic process that replaces normal bone marrow. Etiologies are solid tumor metastases (e.g., lung, breast, and prostate malignancies), lymphoid or myeloid neoplasms (e.g., acute myelogenous leukemia), myelofibrosis, hemophagocytic lymphohistiocytosis, osteopetrosis, or Gaucher disease. The bone marrow biopsy will not be hypocellular and reflect the underlying disease.

Isolated failure of a single hematopoietic lineage is common (agranulocytosis, pure red cell aplasia). These share the same causes for aplastic anemia (e.g., propylthiouracil and agranulocytosis, thymoma and pure red cell aplasia). Patients will have symptoms related to the cell line involved, not all three.

Enhancing Healthcare Team Outcomes

The management of patients with aplastic anemia is multidisciplinary. The disorder can affect many organ systems and besides the disease itself, many complications can result occur following immunosuppressive therapy and hematopoietic cell transplantation. These patients need close monitoring for infections and bleeding. Because of the neutropenia, the diet has to be tailored made and should exclude dairy products, raw meat and most vegetables and fruits because of colonization by a number of microorganisms. All patients should avoid intense physical activity because they are at a risk for bleeding. Premenopausal women can develop heavy periods and should be advised to be on hormonal therapy. Finally, all patients should be educated on the need to maintain good hand and personal hygiene because they are at a very high risk for infections. [13][14](Level V)

Outcomes

Over the past 3 decades, the prognosis for patients with aplastic anemia has markedly improved because of better treatment and supportive measures. Depending on the cause, with treatment 10-year survival of 65-75 have been reported with immunosuppressive and hematopoietic cell transplantation. In fact, for matched siblings, the outcomes of hematopoietic cell transplantations are excellent. With the use of immunosuppressive therapy, there is always a risk of relapse and late clonal disease. Some data show that in patients managed with immunosuppression, telomere length of leucocytes is associated with low survival, clonal evolution, and risk of relapse. The two major causes of death in aplastic anemia include bleeding and infection. Patients who undergo cell transplantation are at risk for graft versus host disease and graft failure. [15][16](Level V)


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    Contributed by Christine A Moore
Attributed To: Contributed by Christine A Moore

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Aplastic Anemia - Questions

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A 17-year-old female comes to the doctor's office complaining of weakness. She is 5 weeks postpartum from a pregnancy complicated by Rocky Mountain Spotted Fever for which she received chloramphenicol. On physical exam, the patient has pale conjunctivae and petechiae over her extremities. CBC reveals hemoglobin is 6 g/dl, platelets 50,000 and WBC 2000. What is the most common cause of death in a patient with these findings?



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A 36-year-old male is evaluated for the fatigue of 3 months duration. He admits to easy bruising, rash over his lower extremities, and frequent respiratory infections. He has rheumatoid arthritis, schizophrenia, and history of Rocky Mountain Spotted Fever when he was 5 years old requiring antibiotics. Medications are chlorpromazine and gold. He works at a paint manufacturing company. Temperature is 38°C (100.4°F), blood pressure is 100/58 mmHg, pulse rate is 94/min and regular, respiratory rate is 17/min, and oxygen saturation is 92% on ambient air. Physical examination reveals a gentleman in no acute distress. There are conjunctival pallor and mild oozing of blood at gingival mucosa. There are rales in the left lower lung field. The stool is guaiac negative. Petechiae is present over bilateral lower extremities. Chest x-ray demonstrates consolidation in the left lower lung. Results of laboratory studies are notable for hemoglobin 5.5 g/dL, platelets 50000, and white blood cell count 1000 with 30% neutrophils. The peripheral smear is negative for blasts or schistocytes. Bone marrow biopsy demonstrates paucity of cells with fat infiltration. What would be the most common cause of death in this patient?



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A 36-year-old male is evaluated for the fatigue of 3 months duration. He admits to easy bruising, rash over his lower extremities, and frequent respiratory infections. He had a prior history of rheumatoid arthritis, schizophrenia, and history of Rocky Mountain Spotted Fever. Medications are chlorpromazine and gold. His vital signs include temperature 38°C; blood pressure is 100/58 mmHg, pulse rate is 94/min and regular, respiratory rate 17/min and oxygen saturation 92% on ambient air. Physical examination reveals conjunctival pallor and mild oozing of blood at gingival mucosa along with rales in the left lower lung field. The stool is guaiac negative. Also, the petechiae are present over bilateral lower extremities. The chest x-ray demonstrates consolidation in the left lower lung. Further Laboratory results are notable for hemoglobin 5.5 g/dL, platelets 50000, and white blood cell count 1000 with 30% neutrophils. The peripheral smear is negative for blasts or schistocytes. Bone marrow biopsy demonstrates the paucity of cells with fat infiltration. Which of the following statements is true regarding this patient's most likely disease?



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A 36-year-old male is evaluated for the fatigue of 3 months duration. He admits to easy bruising, rash over his lower extremities, and frequent episodes of respiratory infections. He had a prior history of rheumatoid arthritis, schizophrenia, and history of Rocky Mountain Spotted Fever. His current medications include chlorpromazine and gold. His vital signs include temperature is 38°C (100.4°F), blood pressure is 100/58 mmHg, pulse rate is 94b/min, and regular, respiratory rate is 17/min, and oxygen saturation is 92% on ambient air. Physical examination reveals conjunctival pallor and mild oozing of blood at gingival mucosa and rales in the left lower lung field while the stool is guaiac negative. Also, petechiae are present over bilateral lower extremities. On further investigation, chest x-ray demonstrates consolidation in the left lower lung. Also, the results from laboratory studies are notable for hemoglobin 5.5 g/dL, platelets 50000, and white blood cell count 1000 with 30% neutrophils. The peripheral smear is negative for blasts or schistocytes. Bone marrow biopsy demonstrates the paucity of cells with fat infiltration. Which of the following statements are true regarding this patient's current condition?



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A 36-year-old male is evaluated for the fatigue of 3 months duration. He admits to easy bruising, rash over his lower extremities, and frequent respiratory infections. He has rheumatoid arthritis, schizophrenia, and history of rocky mountain spotted fever when he was 5 years old, and he was treated with antibiotics. His current medications include chlorpromazine and gold. His vitals include temperature=38°C, blood pressure is 100/58 mmHg, pulse rate is 94/min and regular, respiratory rate is 17/min, and oxygen saturation is 92% on ambient air. Physical examination reveals a gentleman in no acute distress. There is conjunctival pallor and mild oozing of blood at gingival mucosa. There are rales in the left lower lung field. The stool is guaiac negative. Petechiae is present over bilateral lower extremities. Chest x-ray demonstrates consolidation in the left lower lung. Results of laboratory studies are notable for hemoglobin 5.5 g/dL, platelets 50000, and white blood cell count 1000 with 30% neutrophils. The peripheral smear is negative for blasts or schistocytes. Bone marrow biopsy demonstrates the paucity of cells with fat infiltration. Which of the following characteristics are least likely in this disease?



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Which medications have not been associated with aplastic anemia?



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A 36-year-old male is evaluated for the fatigue of 3 months duration. He admits to easy bruising, rash over his lower extremities, and frequent respiratory infections. He had rheumatoid arthritis, schizophrenia, and history of Rocky Mountain Spotted Fever when he was 5 years old requiring antibiotics. Medications are chlorpromazine and gold. He works at a paint manufacturing company. Temperature is 38°C (100.4°F), blood pressure is 100/58 mmHg, pulse rate is 94/min and regular, respiratory rate is 17/min, and oxygen saturation is 92% on ambient air. Physical examination reveals a gentleman in no acute distress. There is conjunctival pallor and mild oozing of blood at gingival mucosa. There are rales in the left lower lung field. The stool is guaiac negative. Petechiae is present over bilateral lower extremities. Chest x-ray demonstrates consolidation in the left lower lung. Results of laboratory studies are notable for hemoglobin 5.5 g/dL, platelets 50000, and white blood cell count 1000 with 30% neutrophils. The peripheral smear is negative for blasts or schistocytes. Bone marrow biopsy demonstrates the paucity of cells with fat infiltration. Which of the following symptoms are not characteristic of this patient's most likely disease?



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A 27-year-old G1P0 female at 26 weeks gestational age is evaluated at the emergency department for dyspnea on exertion, easy bruising, frequent sinus infections, and rash. She was hospitalized twice in her first trimester for hyperemesis gravidarum. The patient has major depressive disorder and takes a multivitamin. She denies alcohol, illicit drugs, or tobacco use. Orthostatic vital signs reveal blood pressure 110/70 mm Hg and heart rate 105/min while sitting and blood pressure 102/58 mm Hg and heart rate 119/min after standing. Weight is 154 pounds (10-pound gain from preconception). Physical exam reveals a pregnant female with conjunctival pallor. Nasal mucosa is boggy with tenderness over left maxillary sinus. Cardiac auscultation reveals sinus tachycardia with a systolic flow murmur near left sternal border. The uterine fundus is palpated at the level of the umbilicus. Ecchymoses are present on her chest and legs, with scattered petechiae over bilateral anterior tibias. Labs are notable for hemoglobin 6.7 mg/dL, platelets 39,000/microL, and white blood cell count 1400/microL with 30% polymorphonuclear leukocytes. Peripheral blood smear reveals normocytic red blood cells without schistocytes. The remainder of labs including comprehensive metabolic panel and coagulation profile are unremarkable. What is the most appropriate next step in management?



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A 38-year-old G1P0 female 24 weeks gestational age is evaluated for easy bleeding and bruising, fatigue, recurrent respiratory infections, and skin rashes. Her pregnancy was complicated by hyperemesis gravidarum requiring hospitalization for fluid resuscitation and intravenous antiemetics. Medical history includes major depressive disorder in remission. She takes a prenatal multivitamin. Vital signs are stable; weight is 140 lb, an increase of 5 lb from pre-pregnancy weight. Physical exam reveals conjunctival pallor. Blood slowly oozes from gum and nasal mucosa. The oropharynx is hyperemic with tonsillar enlargement. Heart auscultation reveals tachycardia with systolic flow murmur lateral to the upper sternal border. There are ecchymoses scattered over her back, chest and upper thighs. Petechiae cover her anterior tibias bilaterally. Labs are notable for hemoglobin 7.6 mg/dL, platelets 20000/microL, and white blood cell count 2300/microL with 20% polymorphonuclear leukocytes (PMNs). A peripheral blood smear is negative for schistocytes, activated partial thromboplastin and prothrombin times are within normal limits. Bone marrow biopsy is shown below. What is the most likely diagnosis?

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A 38-year-old G1P0 female 24 weeks gestational age is evaluated for easy bleeding and bruising, fatigue, recurrent respiratory infections, and skin rashes. Her pregnancy was complicated by hyperemesis gravidarum requiring hospitalization for fluid resuscitation and intravenous antiemetics. Medical history includes major depressive disorder in remission. She takes a prenatal multivitamin. Vital signs are stable; weight is 140 lb, an increase of 5 lb from pre-pregnancy weight. Physical exam reveals conjunctival pallor. Blood slowly oozes from gum and nasal mucosa. The oropharynx is hyperemic with tonsillar enlargement. Heart auscultation reveals tachycardia with systolic flow murmur lateral to the upper sternal border. There are ecchymoses scattered over her back, chest and upper thighs. Petechiae cover her anterior tibias bilaterally. Labs are notable for hemoglobin 7.6 mg/dL, platelets 20000/microL, and white blood cell count 2300/microL with 20% polymorphonuclear leukocytes (PMNs). A peripheral blood smear is negative for schistocytes, activated partial thromboplastin and prothrombin times are within normal limits. Bone marrow biopsy is shown below. Which of the following is the most appropriate next step in management?

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A 27-year-old G1P0 female at 26 weeks gestational age is evaluated at the emergency department for dyspnea on exertion, easy bruising, frequent sinus infections, and rash. She was hospitalized twice in her first trimester for hyperemesis gravidarum. The patient has a major depressive disorder and takes a multivitamin. She denies alcohol, illicit drugs, or tobacco use. Orthostatic vital signs reveal blood pressure 110/70 mm Hg and heart rate 105/min while sitting and blood pressure 102/58 mm Hg and heart rate 119/min after standing. Weight is 154 pounds (10-pound gain from preconception). Physical exam reveals a pregnant female with conjunctival pallor. The nasal mucosa is boggy with tenderness over left maxillary sinus. Cardiac auscultation reveals sinus tachycardia with a systolic flow murmur near the left sternal border. The uterine fundus is palpated at the level of the umbilicus. Ecchymoses are present on her chest and legs, with scattered petechiae over bilateral anterior tibias. Labs are notable for hemoglobin 6.7 mg/dL, platelets 39,000/microL, and white blood cell count 1400/microL with 30% polymorphonuclear leukocytes. A peripheral blood smear is negative for schistocytes, activated partial thromboplastin and prothrombin times are within normal limits. Bone marrow biopsy is shown below. What is the most likely diagnosis?

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A 27-year-old G1P0 female at 26 weeks gestational age is evaluated at the emergency department for dyspnea on exertion, easy bruising, frequent sinus infections, and rash. She was hospitalized twice in her first trimester for hyperemesis gravidarum. The patient has a major depressive disorder and takes a multivitamin. She denies alcohol, illicit drugs, or tobacco use. Orthostatic vital signs reveal blood pressure 110/70 mm Hg and heart rate 105/min while sitting and blood pressure 102/58 mm Hg and heart rate 119/min after standing. Weight is 154 pounds (10-pound gain from preconception). Physical exam reveals a pregnant female with conjunctival pallor. The nasal mucosa is boggy with tenderness over left maxillary sinus. Cardiac auscultation reveals sinus tachycardia with a systolic flow murmur near the left sternal border. The uterine fundus is palpated at the level of the umbilicus. Ecchymoses are present on her chest and legs, with scattered petechiae over bilateral anterior tibias. Labs are notable for hemoglobin 6.7 mg/dL, platelets 39,000/microL, and white blood cell count 1400/microL with 30% polymorphonuclear leukocytes. A peripheral blood smear is negative for schistocytes, activated partial thromboplastin and prothrombin times are within normal limits. Bone marrow biopsy is shown below. Which of the following is the most appropriate next step in management?

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    Contributed by Ruozhi Xiao
Attributed To: Contributed by Ruozhi Xiao



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Aplastic Anemia - References

References

Ding SX,Chen T,Wang T,Liu CY,Lu WL,Fu R, The Risk of Clonal Evolution of Granulocyte Colony-Stimulating Factor for Acquired Aplastic Anemia: A Systematic Review and Meta-Analysis. Acta haematologica. 2018     [PubMed]
Georges GE,Doney K,Storb R, Severe aplastic anemia: allogeneic bone marrow transplantation as first-line treatment. Blood advances. 2018 Aug 14     [PubMed]
Shallis RM,Ahmad R,Zeidan AM, Aplastic anemia: Etiology, molecular pathogenesis, and emerging concepts. European journal of haematology. 2018 Dec     [PubMed]
Gadalla SM,Aubert G,Wang T,Haagenson M,Spellman SR,Wang L,Katki HA,Savage SA,Lee SJ, Donor telomere length and causes of death after unrelated hematopoietic cell transplantation in patients with marrow failure. Blood. 2018 May 24     [PubMed]
Li SS,Hsu YT,Chang C,Lee SC,Yen CC,Cheng CN,Chen JS,Lin SH,Chang KC,Chen TY, Incidence and treatment outcome of aplastic anemia in Taiwan-real-world data from single-institute experience and a nationwide population-based database. Annals of hematology. 2018 Sep 3     [PubMed]
Vaht K,Göransson M,Carlson K,Isaksson C,Lenhoff S,Sandstedt A,Uggla B,Winiarski J,Ljungman P,Brune M,Andersson PO, Incidence and outcome of acquired aplastic anemia: real-world data from patients diagnosed in Sweden from 2000-2011. Haematologica. 2017 Oct     [PubMed]
Schoettler ML,Nathan DG, The Pathophysiology of Acquired Aplastic Anemia: Current Concepts Revisited. Hematology/oncology clinics of North America. 2018 Aug     [PubMed]
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Gnanaraj J,Parnes A,Francis CW,Go RS,Takemoto CM,Hashmi SK, Approach to pancytopenia: Diagnostic algorithm for clinical hematologists. Blood reviews. 2018 Sep     [PubMed]
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