University of Wisconsin Hematology

Hemoglobinopathies and thalassemias

General, pathophysiology

  1. Thein SL. Genetic association studies in β-hemoglobinopathies. Hematology 2013:354
  2. Orkin SH. The Fetal-to-Adult Hemoglobin Switch — Mechanism and Therapy. NEJM 2025;392:2135
  3. Zhang et al. Neutrophils, platelets, and inflammatory pathways at the nexus of sickle cell disease pathophysiology. Blood 2016;127:801
  4. Manwani and Frenette. Vaso-occlusion in sickle cell disease: pathophysiology and novel targeted therapies. Blood 2013;122:3892
  5. Telen M. Role of adhesion molecules and vascular endothelium in the pathogenesis of sickle cell disease. Hematology 2007:84
  6. Kuypers F. Membrane lipid alterations in hemoglobinopathies. Hematology 2007:68
  7. May et al. Hemoglobin Variants and Disease Manifestations in Severe Falciparum Malaria. JAMA 2007;297:2220
  8. Sankaran et al. A functional element necessary for hemoglobin swtiching. NEJM 2011;365:807(with editorial)
  9. Steinberg MH. Fetal hemoglobin in sickle cell anemia. Blood 2021;136:2392
  10. Bunn et al. Pulmonary hypertension and nitric oxide depletion in sickle cell disease. Blood 2010;116:687
  11. Ranque et al. Arterial Stiffness Impairment in Sickle Cell Disease Associated With Chronic Vascular Complications. The Multinational African CADRE Study. Circulation 2016;134:923
  12. Vincent et al. Mast cell activation contributes to sickle cell pathobiology and pain in mice. Blood 2013;122:1853
  13. Allen et al. Methemoglobinemia and ascorbate deficiency in hemoglobin E β thalassemia: metabolic and clinical implications. Blood 2012;120:2939(Elevated methemoglobin levels in Hb E are common, may improve with ascorbate treatment)
  14. Bauer et al. An Erythroid Enhancer of BCL11A Subject to Genetic Variation Determines Fetal Hemoglobin Level. Science 2013;342:253(Potential target for treatment of thalassemias and hemoglobinopathies; with editorial)
  15. Dussiot et al. An activin receptor IIA ligand trap corrects ineffective erythropoiesis in β-thalassemia. Nat Med 2014;20:398(with editorial)
  16. Suragani et al. Transforming growth factor-β superfamily ligand trap ACE-536 corrects anemia by promoting late-stage erythropoiesis. Nat Med 2014;20:408(with editorial)
  17. Grevet et al. Domain-focused CRISPR screen identifies HRI as a fetal hemoglobin regulator in human erythroid cells. Science 2018;361:285(Potential therapeutic target for hemoglobinopathies; also see NEJM commentary)
  18. Whelihan et al. Thrombin generation and cell-dependent hypercoagulability in sickle cell disease. J Thromb Haemost 2016;14:1941
  19. Liu et al. Patrolling monocytes scavenge endothelial-adherent sickle RBCs: a novel mechanism of inhibition of vaso-occlusion in SCD. Blood 2019;134:579

Complications of sickle cell disease

  1. Miller et al. Prediction of adverse outcomes in children with sickle cell disease. NEJM 2000;342:83
  2. Pittman et al. Evaluation of Longitudinal Pain Study in Sickle Cell Disease (ELIPSIS) by patient-reported outcomes, actigraphy, and biomarkers. Blood 2021;137:2010(CRP a useful biomarker for vaso-occlusive crisis)
  3. Kato et al. Lactate dehydrogenase as a biomarker of hemolysis-associated nitric oxide resistance, priapism, leg ulceration, pulmonary hypertension, and death in patients with sickle cell disease. Blood 2006;107:2279
  4. Ataga and Key. Hypercoagulability in sickle cell disease: new approaches to an old problem. Hematology 2007;91
  5. Platt et al. Influence of sickle hemoglobinopathies on growth and development. NEJM 1984; 311:7
  6. Platt et al. Mortality in sickle cell disease. Life expectancy and risk factors for early death. NEJM 1994;330:1639
  7. Quinn et al. Improved survival of children and adolescents with sickle cell disease. Blood 2010; 115:3447(Increased mortality rate associated with transition from pediatric to adult care)
  8. Gladwin and Vichinsky. Pulmonary complications of sickle cell disease. NEJM 2008;359:2254
  9. Machogu and Machado. How I treat hypoxia in adults with hemoglobinopathies and hemolytic disorders. Blood 2018;132:1770
  10. Castro et al. The acute chest syndrome in sickle cell disease: incidence and risk factors. Blood 1994;84:643
  11. Vichinsky et al. Acute chest syndrome in sickle cell disease: clinical presentation and course. Blood 1997;89:1787
  12. Vichinsky et al. Causes and outcomes of the acute chest syndrome in sickle cell disease. NEJM 2000;342:1855 (Fat embolism relatively common in patients > 20 yrs)
  13. Gordeuk et al. Pathophysiology and treatment of pulmonary hypertension in sickle cell disease. Blood 2016;127:820
  14. Dessap et al. Pulmonary Hypertension and Cor Pulmonale during Severe Acute Chest Syndrome in Sickle Cell Disease. Am J Respir Crit Care Med 2008; 177:646(Markers of pulmonary hypertension and right heart failure correlate with outcome in chest crisis)
  15. Gladwin et al.  Pulmonary hypertension as a risk factor for death in patients with sickle cell disease.  NEJM 2004;350:886
  16. Klings et al. Abnormal Pulmonary Function in Adults with Sickle Cell Anemia. Am J Resp Crit Care Med 2006;173:1264(90% of patients had abnormal PFTs)
  17. Derchi et al. Prevalence and Risk Factors for Pulmonary Arterial Hypertension in a Large Group of β-Thalassemia Patients Using Right Heart Catheterization. A Webthal Study. Circulation 2013;129:338
  18. Pinto et al. Mortality in β-thalassemia patients with confirmed pulmonary arterial hypertension on right heart catheterization. Blood 2022;139:2080 (With editorial)
  19. Field and DeBaun. Asthma and sickle cell disease: two distinct diseases or part of the same process? Hematology 2009;45
  20. Strouse et al. Severe pandemic H1N1 and seasonal influenza in children and young adults with sickle cell disease. Blood 2010;116:3431
  21. DeBaun and Kirkham. Central nervous system complications and management in sickle cell disease. Blood 2016;127:829
  22. Kassim et al. How I treat and manage strokes in sickle cell disease. Blood 2015;125:3401
  23. Adams et al.  Prevention of a first stroke by transfusions in children with sickle cell anemia and abnormal results on transcranial doppler ultrasonography. NEJM 1998;339:5
  24. Verduzco and Nathan. Sickle cell disease and stroke. Blood 2009;114:5117
  25. Ford et al. Silent infarcts in sickle cell disease occur in the border zone region and are associated with low cerebral blood flow. Blood 2018;132:1714
  26. Vichinsky et al. Neuropsychological Dysfunction and Neuroimaging Abnormalities in Neurologically Intact Adults With Sickle Cell Anemia. JAMA 2010;303:1823(Adults with sickle disease had poorer cognitive performance than controls; worse scores associated with more severe anemia and older age)
  27. Naik et al. Venous thromboembolism incidence in the Cooperative Study of Sickle Cell Disease. J Thromb Haemost 2014;12:2010(High incidence of VTE in SS disease, higher mortality rates in patients with VTE)
  28. Smith-Whitley et al.  Epidemiology of human parvovirus B19 in children with sickle cell disease.  Blood 2004;103:422
  29. Falk and Hood. The heart in sickle cell anemia. Arch Intern Med 1982; 142:1680
  30. Niss et al. Association between diffuse myocardial fibrosis and diastolic dysfunction in sickle cell anemia. Blood 2017;130:205
  31. d’Humières et al. Determinants of ventricular arrhythmias in sickle cell anemia: toward better prevention of sudden cardiac death. Blood 2023;142:409
  32. Sharpe and Thein. How I treat renal complications in sickle cell disease. Blood 2014;123: 3720
  33. Xu and Frenette. Seasonal manifestations of sickle cell disease activity. Nat Med 2019;25:536(More disease-related complications in winter months)
  34. Smith-Whitley K. Reproductive issues in sickle cell disease. Blood 2014;124:3538
  35. Oteng-Ntim et al. Adverse maternal and perinatal outcomes in pregnant women with sickle cell disease: systematic review and meta-analysis. Blood 2015;125:3316
  36. Brunson et al. Increased risk of leukemia among sickle cell disease patients in California. Blood 2017;130:1597
  37. Pirenne and Pondarré. Alloimmunization and hyperhemolysis in sickle cell disease. Hematology Am Soc Hematol Educ Program (2023): 653

Treatment of sickle cell disease

  1. Chou et al. American Society of Hematology 2020 guidelines for sickle cell disease: transfusion support. Blood Adv 2020;4:327
  2. Brandow et al. American Society of Hematology 2020 guidelines for sickle cell disease: management of acute and chronic pain. Blood Adv 2020;4:2656
  3. Rai and Ataga. Using disease-modifying therapies in sickle cell disease. Hematology Am Soc Hematol Educ Program (2023): 519
  4. Piel et al. Sickle cell disease. NEJM 2017;376:1561
  5. Tisdale et al. Treating sickle cell anemia. Science 2020;367:1198
  6. Thein and Howard. How I treat the older adult with sickle cell disease. Blood 2018;132:1750
  7. James and Strouse. How I treat sickle cell disease in pregnancy. Blood 2024;143:769
  8. Kelly S. Logistics, risks, and benefits of automated red blood cell exchange for patients with sickle cell disease. Hematology Am Soc Hematol Educ Program (2023): 646
  9. Eaton and Bunn. Treating sickle cell disease by targeting HbS polymerization. Blood 2017;129:2719
  10. Manwani and Frenette. Vaso-occlusion in sickle cell disease: pathophysiology and novel targeted therapies. Hematology 2013:362
  11. Morris et al. Impact of arginine therapy on mitochondrial function in children with sickle cell disease during vaso-occlusive pain. Blood 2020;136:1402
  12. Machogu and Machado. How I treat hypoxia in adults with hemoglobinopathies and hemolytic disorders. Blood 2018;132:1770
  13. Ataga et al. Efficacy and safety of the Gardos channel blocker, senicapoc (ICA-17043), in patients with sickle cell anemia. Blood 2008;111:3991 (Drug appeared to decrease rate of hemolysis)
  14. Telen et al. Randomized phase 2 study of GMI-1070 in SCD: reduction in time to resolution of vaso-occlusive events and decreased opioid use. Blood 2015;125:2656 (Drug blocks selectin-mediated cell adhesion; 83% reduction in IV opioid use)
  15. Heeney et al. A Multinational Trial of Prasugrel for Sickle Cell Vaso-Occlusive Events. NEJM 2016;374:625(No apparent benefit)
  16. Koshy et al. Surgery and anesthesia in sickle cell disease. Blood 1995;86:3676
  17. Griffin et al. High-dose intravenous methylprednisolone therapy for pain in children and adolescents with sickle cell disease. NEJM 1994;330:733
  18. Saunthararajah et al. Effects of 5-aza-2′-deoxycytidine on fetal hemoglobin levels, red cell adhesion, and hematopoietic differentiation in patients with sickle cell disease. Blood 2003;102:3871
  19. Morris et al. Arginine Therapy. A New Treatment for Pulmonary Hypertension in Sickle Cell Disease? Am J Resp Crit Care Med 2003; 168:63
  20. Gladwin et al. Nitric Oxide for Inhalation in the Acute Treatment of Sickle Cell Pain Crisis. JAMA 2011;305:893(No apparent benefit)
  21. Niihara et al. A Phase 3 Trial of l-Glutamine in Sickle Cell Disease. NEJM 2018;379:226(Twice daily L-glutamine reduced rates of pain crises and hospitalization; with editorial)
  22. Quinn CT. l-Glutamine for sickle cell anemia: more questions than answers. Blood 2018;132:689
  23. Rees et al. A randomized, placebo-controlled, double-blind trial of canakinumab in children and young adults with sickle cell anemia. Blood 2022; 139:2642 (Moderate benefit from selective IL-1β blockade)
  24. Parekh et al. Recent developments in the use of pyruvate kinase activators as a new approach for treating sickle cell disease. Blood 2024;143:866
  25. Bernaudin et al. Long-term results of related myeloablative stem-cell transplantation to cure sickle cell disease. Blood 2007;110:2749
  26. Gardner et al. How we treat sickle hepatopathy and liver transplantation in adults. Blood 2014;123:2302
  27. Anele et al. How I treat priapism. Blood 2015;125:3551
  28. Shet and Wun. How I diagnose and treat venous thromboembolism in sickle cell disease. Blood 2018;132:1761
  29. Walter et al. Risk of vaso-occlusive episode after exposure to corticosteroids in patients with sickle cell disease. Blood 2022;139:3771

Hydroxyurea

  1. Wong et al. Update on the use of hydroxyurea therapy in sickle cell disease. Blood 2014;124:3850
  2. Ware R. How I use hydroxyurea to treat young patients with sickle cell anemia. Blood 2010;115:5300
  3. Brawley et al. National Institutes of Health Consensus Development Conference Statement: Hydroxyurea Treatment for Sickle Cell Disease. Ann Intern Med 2008;148:932
  4. Bakanay et al. Mortality in sickle cell patients on hydroxyurea therapy.  Blood 2005;105:545
  5. Voskaridou et al. The effect of prolonged administration of hydroxyurea on morbidity and mortality in adult patients with sickle cell syndromes: results of a 17-year, single-center trial (LaSHS). Blood 2010;115:2354(HU dramatically reduced complications and increased 10 year survival by 20%)
  6. Castro et al. Hydroxycarbamide treatment in sickle cell disease: estimates of possible leukaemia risk and of hospitalization survival benefit. Br J Haematol 2014;167:687(No increased risk of leukemia, survival benefit with huse of HU)
  7. Almeida et al. Acute hemolytic vascular inflammatory processes are prevented by nitric oxide replacement or a single dose of hydroxyurea. Blood 2015;126:711(Mouse study suggesting HU can reduce inflammatory response to heomolysis by acting as nitric oxide donor)

Pain control

  1. Tran et al. Targeting novel mechanisms of pain in sickle cell disease. Blood 2017;130:2377
  2. Smith et al. Daily Assessment of Pain in Adults with Sickle Cell Disease. Ann Intern Med 2008;148:94(Chronic pain is very common in sickle disease and is often undertreated)
  3. Solomon L. Treatment and prevention of pain due to vaso-occlusive crises in adults with sickle cell disease: an educational void. Blood 2008;111:997
  4. Ballas et al. Sickle cell pain: a critical reappraisal. Blood 2012;120:3647
  5. Lanzkron et al. Treatment of Acute Pain in Adults With Sickle Cell Disease in an Infusion Center Versus the Emergency Department. A Multicenter Prospective Cohort Study. Ann Intern Med 2021;174:1207(4-fold reduction in likelihood of admission with infusion center treatment)

Transfusion

  1. Pirenne and Yazdanbakhsh. How I safely transfuse patients with sickle-cell disease and manage delayed hemolytic transfusion reactions. Blood 2018;131:2773
  2. Yazdanbakhsh et al. Red blood cell alloimmunization in sickle cell disease: pathophysiology, risk factors, and transfusion management. Blood 2012;120:528
  3. Vichinsky et al. A comparison of conservative and aggressive transfusion regimens in the perioperative management of sickle cell disease. NEJM 1995;333:206
  4. DeBaun et al. Controlled Trial of Transfusions for Silent Cerebral Infarcts in Sickle Cell Anemia. NEJM 2014;371:699(Keeping Hb S < 30% reduces stroke risn in high risk children)
  5. Detterich et al. Chronic transfusion therapy improves but does not normalize systemic and pulmonary vasculopathy in sickle cell disease. Blood 2014;126:703
  6. Malinowski et al. Prophylactic transfusion for pregnant women with sickle cell disease: a systematic review and meta-analysis. Blood 2015;126:2424(Prophylactic transfusion beneficial in pregnancy)
  7. Swerdlow PS. Red Cell Exchange in Sickle Cell Disease. Hematology 2006;48-53

Crizanlizumab and related drugs

  1. Ataga et al. Crizanlizumab for the Prevention of Pain Crises in Sickle Cell Disease. NEJM 2017;376:429(More than 60% reduction in pain crises with administration of this antibody to P-selectin; with editorial)
  2. Dampier et al. A randomized clinical trial of the efficacy and safety of rivipansel for sickle cell vaso-occlusive crisis. Blood 2023;141:168 (E-selectin antagonist gave modest benefit)

Gene therapy

  1. Sharma A. How I treat sickle cell disease with gene therapy. Blood 2024;124:2693
  2. Benz et al. “Treatment with curative intent”: the emergence of genetic therapies for sickle cell anemia. Blood 2024;143:987
  3. Abraham and Tisdale. Gene therapy for sickle cell disease: moving from the bench to the bedside. Blood 2021;138:932
  4. Hoban et al. Genetic treatment of a molecular disorder: gene therapy approaches to sickle cell disease. Blood 2016;127:839
  5. Xu et al. Correction of Sickle Cell Disease in Adult Mice by Interference with Fetal Hemoglobin Silencing. Science 2011;334:993
  6. Ribeil et al. Gene therapy in a patient with sickle cell disease. NEJM 2017;376:848
  7. Esrick et al. Post-Transcriptional Genetic Silencing of BCL11A to Treat Sickle Cell Disease. NEJM 2021;384:205(With editorial)
  8. Frangoul et al. CRISPR-Cas9 Gene Editing for Sickle Cell Disease and β-Thalassemia. NEJM 2021;384:252 (With editorial)
  9. Sharma et al. CRISPR-Cas9 Editing of the HBG1 and HBG2 Promoters to Treat Sickle Cell Disease. NEJM 2023;389:820
  10. Frangoul et al. Exagamglogene Autotemcel for Severe Sickle Cell Disease. NEJM 2024;390:1649

Sickle cell trait

  1. Naik et al. Clinical Outcomes Associated With Sickle Cell Trait: A Systematic Review. Ann Intern Med 2018; 169:619(Risk factor for PE, kidney disease, rhabdomyolysis, not stroke or MI)
  2. Weeks et al. Sickle cell trait does not cause “sickle cell crisis” leading to exertion-related death: a systematic review. Blood 2025;145:1345
  3. Pecker and Naik. The current state of sickle cell trait: implications for reproductive and genetic counseling. Blood 2018;132:2331
  4. Austin et al. Sickle cell trait and the risk of venous thromboembolism among blacks. Blood 2007; 110:908(2-fold increased risk of VTE in sickle trait)
  5. Folsom et al. Prospective study of sickle cell trait and venous thromboembolism incidence. J Thromb Haemost 2014;13:2(2-fold increase in PE risk, no increase in DVT risk)
  6. Naik et al. Association of Sickle Cell Trait With Chronic Kidney Disease and Albuminuria in African Americans. JAMA 2014;312:2115(OR for CKD 1.57 in individuals with sickle trait)
  7. Nelson et al. Sickle Cell Trait, Rhabdomyolysis, and Mortality among U.S. Army Soldiers. NEJM 2016;375:435(SS trait associated with 1.54 x higher risk of rhabdomyolysis but not death)
  8. Liem et al. Association among sickle cell trait, fitness, and cardiovascular risk factors in CARDIA. Blood 2017;129:723(Sickle trait not associated with decreased fitness, hypertension, diabetes or metabolic syndrome)

Thalassemia and related hemoglobinopathies

  1. Kattamis et al. Thalassemia. Lancet 2022;399:2310
  2. Taher et al. β-Thalassemias. NEJM 2021;384:727
  3. Piel and Weatherall. The α-Thalassemias. NEJM 2014;371:1908
  4. Amid et al. Hemoglobin Bart’s hydrops fetalis: charting the past and envisioning the future. Blood 2024;144:822
  5. Taher and Cappellini. How I manage medical complications of β-thalassemia in adults. Blood 2018;132:1781
  6. Saliba et al. How I treat non-transfusion-dependent β-thalassemia. Blood 2023;142:949
  7. Piga et al. Luspatercept improves hemoglobin levels and blood transfusion requirements in a study of patients with β-thalassemia. Blood 2019;133:1279(81% of transfusion-dependent patients had reduced transfusion requirement)
  8. Cappellini et al. A Phase 3 Trial of Luspatercept in Patients with Transfusion-Dependent β-Thalassemia. NEJM 2020;382:1219(Significant reduction in transfusion requirements and ferritin levels vs placebo)
  9. Taher et al. Luspatercept for the treatment of anaemia in non-transfusion-dependent β-thalassaemia (BEYOND): a phase 2, randomised, double-blind, multicentre, placebo-controlled trial. Lancet Haematol 2022;9:e733 (77% of treated patients had at least a 1 g improvement in hemoglobin)
  10. Kuo et al. Safety and efficacy of mitapivat, an oral pyruvate kinase activator, in adults with non-transfusion dependent α-thalassaemia or β-thalassaemia: an open-label, multicentre, phase 2 study. Lancet 2022;400:493 (A majority of patients had improvement in hemoglobin)
  11. Songdej et al. An international registry of survivors with Hb Bart’s hydrops fetalis syndrome. Blood 2017;129:1251(A few patients survive to adulthood)
  12. Musallam et al. Fetal hemoglobin levels and morbidity in untransfused patients with β-thalassemia intermedia. Blood 2012;119:364 (Higher HbF associated with less morbidity)
  13. Thompson et al. Gene Therapy in Patients with Transfusion-Dependent β-Thalassemia. NEJM 2018;131:1479(12/13 patients with non–β0/β0 genotype became transfusion-independent; 73% drop in transfusion volume in patients with β0/β0 genotype; with editorial)
  14. Fu et al. CRISPR–Cas9-mediated gene editing of the BCL11A enhancer for pediatric β0/β0 transfusion-dependent β-thalassemia. Nat Med 2022;28:1573
  15. Locatelli et al. Exagamglogene Autotemcel for Transfusion-Dependent β-Thalassemia. NEJM 2024:390:1663
  16. Marktel et al. Intrabone hematopoietic stem cell gene therapy for adult and pediatric patients affected by transfusion-dependent ß-thalassemia. Nat Med 2019;25:234
  17. Mettananda et al. α-Globin as a molecular target in the treatment of β-thalassemia. Blood 2015;125:3694
  18. Cunningham et al.  Complications of beta-thalassemia major in North America. Blood 2004;104:39
  19. Taher et al. Overview on practices in thalassemia intermedia management aiming for lowering complication rates across a region of endemicity: the OPTIMAL CARE study. Blood 2010;115:1886(Evaluates the roles of transfusion, chlelation and hydroxyurea therapy)
  20. Rivella S. Iron metabolism under conditions of ineffective erythropoiesis in β-thalassemia. Blood 2019;133:51
  21. Oliveri and Brittenham. Iron-chelating therapy and the treatment of thalassemias. Blood 1997;89:739
  22. Pennell et al. Efficacy of deferasirox in reducing and preventing cardiac iron overload in β-thalassemia. Blood 2010;115:2364
  23. Kwiatkowski et al. Chelation use and iron burden in North American and British thalassemia patients: a report from the Thalassemia Longitudinal Cohort. Blood 2012;119:2746(Increasing use of oral chelators likely contributes to lower iron burden)
  24. Taher et al. Deferasirox reduces iron overload significantly in nontransfusion-dependent thalassemia: 1-year results from a prospective, randomized, double-blind, placebo-controlled study. Blood 2012;120:970
  25. Kwiatkowski et al. Deferiprone vs deferoxamine for transfusional iron overload in SCD and other anemias: a randomized, open-label noninferiority study. Blood Adv 2022;6:1243 (Deferiprone non-inferior to deferoxamine)
  26. Pasricha et al. Transfusion suppresses erythropoiesis and increases hepcidin in adult patients with β-thalassemia major: a longitudinal study. Blood 2013;122:124
  27. Fucharoen and Viprakasit. Hb H disease: clinical course and disease modifiers. Hematology 2009: 26
  28. Vichinsky E. Alpha thalassemia major—new mutations, intrauterine management, and outcomes. Hematology 2009;35
  29. Chen et al. Genetic and clinical features of hemoglobin H disease in Chinese patients. NEJM 2000;343:544
  30. Chui et al.  Hemoglobin H disease: not necessarily a benign disorder.  Blood 2003;101:791
  31. Lal et al. Heterogeneity of hemoglobin H disease in childhood. NEJM 2011;364:710
  32. Vichinsky E. Hemoglobin E syndromes. Hematology 2007;74
  33. Allen et al. Adaptation to anemia in hemoglobin E-β thalassemia. Blood 2010;116:5368(Right-shifted oxygen dissociation curve)

Other

  1. Kane et al. Benzocaine-Induced Methemoglobinemia Based on the Mayo Clinic Experience From 28 478 Transesophageal Echocardiograms. Arch Intern Med 2007;167:1977
  2. Steensma et al.  Acquired a-thalassemia in association with myelodysplastic syndrome and other hematologic malignancies.  Blood 2005;105:443
  3. Jauréguiberry et al. Postartesunate delayed hemolysis is a predictable event related to the lifesaving effect of artemisinins. Blood 2014;124: 167