Janet Kwiatkowski, MD, MSCE

Papers

2020

Al-Samkari H, van Beers EJ, Morton DH, Barcellini W, Eber SW, Glader B, Yaish HM, Chonat S, Kuo KHM, Kollmar N, Despotovic JM, Pospíšilová D, Knoll CM, Kwiatkowski JL, Pastore YD, Thompson AA, Wlodarski MW, Ravindranath Y, Rothman JA, Wang H, Holzhauer S, Breakey VR, Verhovsek MM, Kunz J, Sheth S, Sharma M, Rose MJ, Bradeen HA, McNaull MN, Addonizio K, Al-Sayegh H, London WB, Grace RF. Characterization of the severe phenotype of pyruvate kinase deficiency. Am J Hematol. 2020 Jul 3. doi: 10.1002/ajh.25926. Epub ahead of print. PMID: 32619047.

Al-Samkari H, Addonizio K, Glader B, Morton DH, Chonat S, Thompson AA, Kuo KHM, Ravindranath Y, Wang H, Rothman JA, Kwiatkowski JL, Kung C, Kosinski PA, Al-Sayegh H, London WB, Grace RF. The pyruvate kinase (PK) to hexokinase enzyme activity ratio and erythrocyte PK protein level in the diagnosis and phenotype of PK deficiency. Br J Haematol. 2020 May 28. doi: 10.1111/bjh.16724. Epub ahead of print. PMID: 32463523.

Tisdale JF, Pierciey FJ Jr, Bonner M, Thompson AA, Krishnamurti L, Mapara MY, Kwiatkowski JL, Shestopalov I, Ribeil JA, Huang W, Asmal M, Kanter J, Walters MC. Safety and feasibility of hematopoietic progenitor stem cell collection by mobilization with plerixafor followed by apheresis vs bone marrow harvest in patients with sickle cell disease in the multi-center HGB-206 trial. Am J Hematol. 2020 Sep;95(9):E239-E242. doi: 10.1002/ajh.25867. Epub 2020 Jun 3. PMID: 32401372.

Bianchi P, Fermo E, Lezon-Geyda K, van Beers EJ, Morton HD, Barcellini W, Glader B, Chonat S, Ravindranath Y, Newburger PE, Kollmar N, Despotovic JM, Verhovsek M, Sharma M, Kwiatkowski JL, Kuo KHM, Wlodarski MW, Yaish HM, Holzhauer S, Wang H, Kunz J, Addonizio K, Al-Sayegh H, London WB, Andres O, van Wijk R, Gallagher PG, Grace RFF. Genotype-phenotype correlation and molecular heterogeneity in pyruvate kinase deficiency. Am J Hematol. 2020 May;95(5):472-482. doi: 10.1002/ajh.25753. Epub 2020 Mar 6. PMID: 32043619.

Chou ST, Alsawas M, Fasano RM, Field JJ, Hendrickson JE, Howard J, Kameka M, Kwiatkowski JL, Pirenne F, Shi PA, Stowell SR, Thein SL, Westhoff CM, Wong TE, Akl EA. American Society of Hematology 2020 guidelines for sickle cell disease: transfusion support. Blood Adv. 2020 Jan 28;4(2):327-355. doi: 10.1182/bloodadvances.2019001143. PMID: 31985807; PMCID: PMC6988392.

2019

Chonat S, Risinger M, Sakthivel H, Niss O, Rothman JA, Hsieh L, Chou ST, Kwiatkowski JL, Khandros E, Gorman MF, Wells DT, Maghathe T, Dagaonkar N, Seu KG, Zhang K, Zhang W, Kalfa TA. Corrigendum: The Spectrum of SPTA1-Associated Hereditary Spherocytosis. Front Physiol. 2019 Oct 18;10:1331. doi: 10.3389/fphys.2019.01331. Erratum for: Front Physiol. 2019 Jul 03;10:815. PMID: 31736770; PMCID: PMC6843059.

Kwiatkowski JL, Voeks JH, Kanter J, Fullerton HJ, Debenham E, Brown L, Adams RJ; Post-STOP Study Group. Ischemic stroke in children and young adults with sickle cell disease in the post-STOP era. Am J Hematol. 2019 Dec;94(12):1335-1343. doi: 10.1002/ajh.25635. Epub 2019 Nov 1. PMID: 31489983.

Grace RF, Rose C, Layton DM, Galactéros F, Barcellini W, Morton DH, van Beers EJ, Yaish H, Ravindranath Y, Kuo KHM, Sheth S, Kwiatkowski JL, Barbier AJ, Bodie S, Silver B, Hua L, Kung C, Hawkins P, Jouvin MH, Bowden C, Glader B. Safety and Efficacy of Mitapivat in Pyruvate Kinase Deficiency. N Engl J Med. 2019 Sep 5;381(10):933-944. doi: 10.1056/NEJMoa1902678. PMID: 31483964.

Chonat S, Risinger M, Sakthivel H, Niss O, Rothman JA, Hsieh L, Chou ST, Kwiatkowski JL, Khandros E, Gorman MF, Wells DT, Maghathe T, Dagaonkar N, Seu KG, Zhang K, Zhang W, Kalfa TA. The Spectrum of SPTA1-Associated Hereditary Spherocytosis. Front Physiol. 2019 Jul 3;10:815. doi: 10.3389/fphys.2019.00815. Erratum in: Front Physiol. 2019 Oct 18;10:1331. PMID: 31333484.

Khandros E, Kwiatkowski JL. Beta Thalassemia: Monitoring and New Treatment Approaches. Hematol Oncol Clin North Am. 2019 Jun;33(3):339-353. doi: 10.1016/j.hoc.2019.01.003. Epub 2019 Apr 2. PMID: 31030806.

Ikawa Y, Miccio A, Magrin E, Kwiatkowski JL, Rivella S, Cavazzana M. Gene therapy of hemoglobinopathies: progress and future challenges. Hum Mol Genet. 2019 Oct 1;28(R1):R24-R30. doi: 10.1093/hmg/ddz172. PMID: 31322165.

Kwiatkowski JL. Matched Sibling Donor Hematopoietic Stem Cell Transplantation to Prevent Stroke in Children With Sickle Cell Anemia. JAMA. 2019 Jan 22;321(3):251-252. doi: 10.1001/jama.2018.20390. PMID: 30667487.

2018

Van Beers EJ, Van Straaten S, Morton DH, Barcellini W, Eber SW, Glader B, Yaish HM, Chonat S, Kwiatkowski JL, Rothman JA, Sharma M, Neufeld EJ, Sheth S, Despotovic JM, Kollmar N, Pospíšilová D, Knoll CM, Kuo K, Pastore YD, Thompson AA, Newburger PE, Ravindranath Y, Wang WC, Wlodarski MW, Wang H, Holzhauer S, Breakey VR, Verhovsek M, Kunz J, McNaull MA, Rose MJ, Bradeen HA, Addonizio K, Li A, Al-Sayegh H, London WB, Grace RF. Prevalence and management of iron overload in pyruvate kinase deficiency: report from the Pyruvate Kinase Deficiency Natural History Study. Haematologica. 2019 Feb;104(2):e51-e53. doi: 10.3324/haematol.2018.196295. Epub 2018 Sep 13. PMID: 30213831.

Riley LG, Heeney MM, Rudinger-Thirion J, Frugier M, Campagna DR, Zhou R, Hale GA, Hilliard LM, Kaplan JA, Kwiatkowski JL, Sieff CA, Steensma DP, Rennings AJ, Simons A, Schaap N, Roodenburg RJ, Kleefstra T, Arenillas L, Fita-Torró J, Ahmed R, Abboud M, Bechara E, Farah R, Tamminga RYJ, Bottomley SS, Sanchez M, Huls G, Swinkels DW, Christodoulou J, Fleming MD. The phenotypic spectrum of germline YARS2 variants: from isolated sideroblastic anemia to mitochondrial myopathy, lactic acidosis and sideroblastic anemia 2. Haematologica. 2018 Dec;103(12):2008-2015. doi: 10.3324/haematol.2017.182659. Epub 2018 Jul 19. PMID: 30026338.

Thompson AA, Walters MC, Kwiatkowski J, Rasko JEJ, Ribeil JA, Hongeng S, Magrin E, Schiller GJ, Payen E, Semeraro M, Moshous D, Lefrere F, Puy H, Bourget P, Magnani A, Caccavelli L, Diana JS, Suarez F, Monpoux F, Brousse V, Poirot C, Brouzes C, Meritet JF, Pondarré C, Beuzard Y, Chrétien S, Lefebvre T, Teachey DT, Anurathapan U, Ho PJ, von Kalle C, Kletzel M, Vichinsky E, Soni S, Veres G, Negre O, Ross RW, Davidson D, Petrusich A, Sandler L, Asmal M, Hermine O, De Montalembert M, Hacein-Bey-Abina S, Blanche S, Leboulch P, Cavazzana M. Gene Therapy in Patients with Transfusion-Dependent β-Thalassemia. N Engl J Med. 2018 Apr 19. PMID: 29669226.

Vichinsky E, Cohen A, Thompson AA, Giardina PJ, Lal A, Paley C, Cheng WY, McCormick N, Sasane M, Qiu Y, Kwiatkowski JL. Epidemiologic and clinical characteristics of nontransfusion-dependent thalassemia in the United States. Pediatr Blood Cancer. 2018 Jul;65(7):e27067. Epub 2018 Apr 10. PMID: 29637688.

Shenoy S, Walters MC, Ngwube A, Soni S, Jacobsohn D, Chaudhury S, Grimley M, Chan K, Haight A, Kasow KA, Parikh S, Andreansky M, Connelly J, Delgado D, Godder K, Hale G, Nieder M, Pulsipher MA, Trachtenberg F, Neufeld E, Kwiatkowski JL, Thompson AA. Unrelated Donor Transplantation in Children with Thalassemia using Reduced-Intensity Conditioning: The URTH Trial. Biol Blood Marrow Transplant. 2018 Jun;24(6):1216-1222. Epub 2018 Jan 31. PMID: 29374585; PMCID: PMC5993578.

2016

Stanley HM, Friedman D, Webb J, Kwiatkowski, JL.  Transfusional Iron Overload in a Cohort of Children with Sickle Cell Disease: Impact of Magnetic Resonance Imaging, Transfusion Method, and Chelation. Pediatr Blood Cancer. 2016 Apr 21. doi: 10.1002/pbc.26017. [Epub ahead of print].

Nabavizadeh SA, Vossough A, Ichord RN, Kwiatkowski J, Pukenas BA, Smith MJ, Storm PB, Zager EL, Hurst RW. Intracranial Aneurysms in sickle cell anemia: clinical and imaging findings. J Neurointerv Surg. 2016 Apr;8(4):434-40. doi: 10.1136/neurintsurg-2014-011572. Epub 2015 Mar 19.

Luchtman-Jones L, Pressel S, Hilliard L, Brown RC, Kwiatkowski JL, Davis BR, Ware RE. Effects of hydroxyurea treatment for patients with hemoglobin SC disease. Am J Hematol. 2016 Feb;91(2):238-42. doi: 10.1002/ajh.24255.

Ware RE, Davis BR…Kwiatkowski JL, Jackson S…Pressel S, Adams RJ.  Hydroxycarbamide versus chronic transfusion for maintenance of transcranial Doppler flow velocities in children with sickle cell anaemia-TCD with Transfusions Changing to Hydroxyurea (TWiTCH): a multicenter, open-label, phase 3, non-inferiority trial. Lancet. 2016 Feb 13;387(10019):661-70. doi: 10.1016/S0140-6736(15)01041-7. Epub 2015 Dec 6.

2015

Wood JC, Pressel S, Rogers ZR, Odame I, Kwiatkowski JL, Lee MT, Owen WC, Cohen AR, St. Pierre T, Heeney MM, Schultz WH, Davis BR, Ware RE for the TWiTCH Investigators. Liver iron concentration measurements by MRI in chronically transfused children with sickle cell anemia: baseline results from the TWiTCH trial. Am J Hematol. 2015 Sep;90(9):806-10. doi: 10.1002/ajh.24089.

Morris CR, Kim HY, Klings ES, Wood J, Porter JB, Trachtenberg F, Sweeters N, Olivieri NF, Kwiatkowski JL, Virzi L, Hassell K, Taher A, Neufeld EJ, Thompson AA, Larkin S, Suh JH, Vichinsky EP, Kuypers FA for the Thalassemia Clinical Research Network. Dysregulated arginine metabolism and cardiopulmonary dysfunction in patients with thalassemia. Br J Haematol. 2015 Jun;169(6):887-98. doi: 10.1111/bjh.13452. Epub 2015 Apr 24.

Belmont AP, Nossair F, Brambilla D, Friedman M, Boswinkel J, Bradford AB, and Kwiatkowski JL. Safety of deep sedation in young children with sickle cell disease: a retrospective cohort study. J Pediatr. 2015 May;166(5):1226-32. doi: 10.1016/j.jpeds.2015.01.054.

Tubman VN, Fung EB, Vogiatzi M, Thompson AA, Rogers ZR, Neufeld EJ, Kwiatkowski JL, for the Thalassemia Clinical Research Network. Guidelines for the Standard Monitoring of Patients with Thalassemia: Report of the Thalassemia Longitudinal Cohort. J Pediatr Hematol Oncol. 2015 Apr;37(3):e162-9. doi: 10.1097/MPH.0000000000000307.

Aygun B, Mortier NA, Kesler K, Lockhart A, Schultz WH, Cohen Ar, Alvarez O, Rogers ZR, Kwiatkowski JL, Miller ST, Sylvestre P, Iyer R, Lane PA, Ware RE and the Stroke with Transfusions Changing to Hydroxyurea Trial Investigators. Therapeutic phlebotomy is safe in children with sickle cell anemia and can be effective treatment for transfusional iron overload. Br J Haematol. 2015 Apr;169(2):262-6. doi: 10.1111/bjh.13280. Epub 2015 Jan 22.

2014

King AA, Rodeghier MJ, Panepinto JA, Strouse JJ, Casella JF, Quinn C, Dowling M…Kwiatkowski JL, Howard TH… DeBaun MR.  Silent cerebral infarction, income and grade retention among students with sickle cell. Am J Hematol. 2014 Oct;89(10):E188-92. doi: 10.1002/ajh.23805. Epub 2014 Aug 4.

DeBaun MR, Gordon M, McKinstry RC, Noetzel MJ…Kwiatkowski JL, King AA, …Casella JF.  Controlled trial of transfusions for silent cerebral infarcts in sickle cell anemia. N Engl J Med. 2014 Aug 21;371(8):699-710. doi: 10.1056/NEJMoa1401731.

Green ST, Martin MB, Haines D, Carson S, Coates T, Oliveros O, Gerstenberger E, Trachtenberg F, Kwiatkowski JL for the Thalassemia Clinical Research Network. Variance of pain prevalence and associated severity during the transfusion cycle of adult thalassemia patients. Br J Haematol. 2014 Sep;166(5):797-800. doi: 10.1111/bjh.12903. Epub 2014 May 8.

Arkuszewski M, Krejza J, Chen R, Ichord R, Kwiatkowski JL, Bilello M, Zimmerman R, Ohene-Frempong K, Melhem ER.  Sickle cell anemia: intracranial stenosis and silent cerebral infarcts in children with low risk of stroke. Adv Med Sci. 2014 Mar;59(1):108-13. doi: 10.1016/j.advms.2013.09.001. Epub 2014 Mar 27.

Books

Chapters

2012

Kwiatkowski, JL. Thalassemia Minor”and “Beta Thalassemia Major. In: Gulati GL, Filicko-O’Hara J, Krause JR, editors. Case Studies in Hematology and Coagulation. Chicago (IL): American Society for Clinical Pathology Press; 2012.

2011

Kwiatkowski JL. Hemoglobinopathies. In: Lanzkowsky, et al., editors. Manual of Pediatric Hematology and Oncology. 5th ed. London (UK): Elsevier, London; 2011. pp. 200-246.

2009

Kwiatkowski JL, Porter J. Transfusion and Iron Chelation Therapy in Thalassemia and Sickle Cell Disease. In: Steinberg MH, Forget BG, Higgs DR, Weatherall DJ, editors. Disorders of Hemoglobin. 2nd ed. Cambridge University Press; 2009.

2008

Kwiatkowski JL. Iron Deficiency Anemia. In Schwartz, et al., editors. The Five-Minute Pediatric Consult. 5th ed. Philadelphia, PA: Lippincott Williams & Wilkins; 2008. pp. 466-7.

Kwiatkowski JL. Microcytic Anemia. In Schwartz, et al., editors. The Five-Minute Pediatric Consult. 5th ed. Philadelphia, PA: Lippincott Williams & Wilkins; 2008. pp. 536-7.

Kwiatkowski JL. Aplastic Anemia. In Schwartz, et al., editors. The Five-Minute Pediatric Consult. 5th ed. Philadelphia, PA: Lippincott Williams & Wilkins; 2008. pp. 54-5.

Kwiatkowski JL. Thalassemia. In Schwartz, et al., editors. The Five-Minute Pediatric Consult. 5th ed. Philadelphia, PA: Lippincott Williams & Wilkins; 2008. pp. 846-7.

2005

Kwiatkowski JL.  Section Editor. Clinical Management of Patients with Thalassemia. In: Perspectives on the Treatment of Transfusion-Dependent Hematologic Disorders. Health Science Center for Continuing Medical Education; 2005.

Kwiatkowski JL. Iron Deficiency Anemia. In: The Five-Minute Pediatric Consult. 4th Ed. Schwartz, et al., editors. Philadelphia (PA): Lippincott Williams & Wilkins; 2005. pp. 500-501.

Kwiatkowski JL, Shusterman S. Microcytic Anemia. In: The Five-Minute Pediatric Consult. 4th Ed. Schwartz, et al., editors. Philadelphia (PA): Lippincott Williams & Wilkins; 2005. pp. 62-63.

Kwiatkowski JL, Minturn J. Aplastic Anemia. In: The Five-Minute Pediatric Consult. 4th Ed. Schwartz, et al., editors. Philadelphia (PA): Lippincott Williams & Wilkins; 2005. pp. 134-35.

Bickert B, Kwiatkowski JL. Coagulation Disorders. In: Depiro, et al, editors Pharmacotherapy: A Pathophysiologic Approach. 6th ed. McGraw-Hill; 2005. pp. 1833-1854.

2004

Kwiatkowski JL, Ohene-Frempong K. Stroke in sickle cell disease. In: Okpala I, editor. Practical Management of Haemoglobinopathies. Malden (MA): Blackwell Publishing, Ltd.; 2004. pp. 134-144.

2002

Bickert B, Kwiatkowski JL. Coagulation Disorders. In: Depiro JT, et al, editors. Pharmacotherapy: A Pathophysiologic Approach. 5th ed., McGraw-Hill Companies, Inc.; 2002. pp. 1747-1768.

Posters and Presentations

2015

Kwiatkowski JL. Mentoring Lunch for Women, Underrepresented Minorities, and Early Career Investigators. New York Academy of Sciences, Tenth Cooley’s Anemia Symposium; 2015 Oct 20; Chicago, IL.  

Kwiatkowski JL. Current Recommendations for Chelation Therapy in Transfusion-Dependent Thalassemia. New York Academy of Sciences, Tenth Cooley’s Anemia Symposium; 2015 Oct 20; Chicago, IL.  

Kwiatkowski JL. The Role of Zinc in Thalassaemia. 8th Annual Advanced Sickle Cell And Thalassaemia Course. Guys and St. Thomas NHS Foundation Trust; 2015 Oct 2; London, UK.

Kwiatkowski JL. Optimizing TCD Screening and Treatment Protocols. 8th Annual Advanced Sickle Cell And Thalassaemia Course. Guys and St. Thomas NHS Foundation Trust; 2015 Oct 2; London, UK.

2014

Kwiatkowski JL. Iron Assessment and Chelation Options.  Cooley’s Anemia Foundation Patient-Family Conference; 2015 Jun 21; Chicago, IL.