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Hematologic Emergencies: Recognition and Initial Management

This article is a corrected version of the article that appeared in print.

Darrell Edward Jones, DO
Jennifer J. Walker, MD, MPH
Alain Michael P. Abellada, MD

American Family Physician. 2024;110(1):58-64.

Author disclosure: No relevant financial relationships.

This clinical content conforms to AAFP criteria for CME.

Hematologic emergencies are bleeding or clotting disorders that are hereditary or acquired and must be treated emergently to avoid significant morbidity or mortality. Patients experiencing a hematologic emergency may present with spontaneous bleeding, jaundice, petechiae, or purpura. Initial diagnostic testing should include a complete blood count. Patients who have bleeding associated with a hereditary disorder should receive clotting factor replacement before diagnostic testing. Acute chest syndrome is an uncommon but serious complication of sickle cell disease. Hemolysis caused by autoimmune disorders or iatrogenic errors from blood product transfusions has a distinct clinical presentation and requires immediate action. Severe thrombocytopenia presenting as immune or thrombotic thrombocytopenic purpura should be differentiated and treated appropriately. Disseminated intravascular coagulation and trauma coagulopathy are sometimes confused with each other, but both can cause serious injury and require unique treatments. Primary care physicians should promptly recognize patients who require emergent referral to a hematologic specialist.

DARRELL EDWARD JONES, DO, is a family medicine hospitalist and program director of the U.S. Army Family Medicine Hospitalist Fellowship at Womack Army Medical Center, Fort Liberty, N.C.

JENNIFER J. WALKER, MD, MPH, is a family physician and medical director of the East Hawaii acute and rural health clinic at Hilo (Hawaii) Medical Center.

ALAIN MICHAEL P. ABELLADA, MD, is a hospitalist and family medicine residency faculty at Womack Army Medical Center.

Address correspondence to Darrell Edward Jones, DO, at armydocjones@gmail.com.

Author disclosure: No relevant financial relationships.

  1. 1.Phillips J, Henderson AC. Hemolytic anemia: evaluation and differential diagnosis. Am Fam Physician. 2018;98(6):354-361.
  2. 2.Gauer RL, Whitaker DJ. Thrombocytopenia: evaluation and management. Am Fam Physician. 2022;106(3):288-298.
  3. 3.Spring J, Munshi L. Hematology emergencies in critically ill adults: benign hematology. Chest. 2022;161(5):1285-1296.
  4. 4.Neutze D, Roque J. Clinical evaluation of bleeding and bruising in primary care. Am Fam Physician. 2016;93(4):279-286.
  5. 5.Solves Alcaina P. Platelet transfusion: and update on challenges and outcomes. J Blood Med. 2020;11:19-26.
  6. 6.Marik PE. Transfusion of blood and blood products. Evidence-Based Critical Care. 2014:585-619.
  7. 7.Arya RC, Wander G, Gupta P. Blood component therapy: which, when and how much. J Anaesthesiol Clin Pharmacol. 2011;27(2):278-284.
  8. 8.Sysol JR, Machado R. Sickle cell disease and acute chest syndrome: epidemiology, diagnosis, management, outcomes. Hematologic Abnormalities and Acute Lung Syndromes. 2016:67-87.
  9. 9.National Bleeding Disorders Foundation. MASAC document 257 - guidelines for emergency department management of individuals with hemophilia and other bleeding disorders. December 5, 2019. Accessed February 25, 2023. https://www.hemophilia.org/healthcare-professionals/guidelines-on-care/masac-documents/masac-document-257-guidelines-for-emergency-department-management-of-individuals-with-hemophilia-and-other-bleeding-disorders
  10. 10.Doherty TM, Kelley A. Bleeding disorders. StatPearls [Internet]. Updated April 3, 2023. Accessed April 19, 2024. https://www.ncbi.nlm.nih.gov/books/NBK541050
  11. 11.James PD, Connell NT, Ameer B, et al. ASH ISTH NHF WFH 2021 guidelines on the diagnosis of von Willebrand disease. Blood Adv. 2021;5(1):280-300.
  12. 12.National Bleeding Disorders Foundation. MASAC document 266 - recommendations regarding the treatment of von Willebrand disease. March 4, 2021. Accessed February 27, 2023. https://www.hemophilia.org/healthcare-professionals/guidelines-on-care/masac-documents/masac-document-266-masac-recommendations-regarding-the-treatment-of-von-willebrand-disease
  13. 13.Pishko AM, Doshi BS. Acquired hemophilia A: current guidance and experience from clinical practice. J Blood Med. 2022;13:255-265.
  14. 14.Raval JS, Griggs JR, Fleg A. Blood product transfusion in adults: indications, adverse reactions, and modifications. Am Fam Physician. 2020;102(1):30-38.
  15. 15.Strobel E. Hemolytic transfusion reactions. Transfus Med Hemother. 2008;35(5):346-353.
  16. 16.Ackfeld T, Schmutz T, Guechi Y, et al. Blood transfusion reactions—a comprehensive review of the literature including a Swiss perspective. J Clin Med. 2022;11(10):2859.
  17. 17.Hill A, Hill QA. Autoimmune hemolytic anemia. Hematology Am Soc Hematol Educ Program. 2018(1):382-389.
  18. 18.Zanella A, Barcellini W. Treatment of autoimmune hemolytic anemias. Haematologica. 2014;99(10):1547-1554.
  19. 19.Brodsky RA. Warm autoimmune hemolytic anemia. N Engl J Med. 2019;381(7):647-654.
  20. 20.Michel M, Terriou L, Roudot-Thoraval F, et al. A randomized and double-blind controlled trial evaluating the safety and efficacy of rituximab for warm auto-immune hemolytic anemia in adults (the RAIHA study). Am J Hematol. 2017;92(1):23-27.
  21. 21.Salama A, Berghöfer H, Mueller-Eckhardt C. Red blood cell transfusion in warm-type autoimmune haemolytic anaemia. Lancet. 1992;340(8834–8835):1515-1517.
  22. 22.Röth A, Barcellini W, D’Sa S, et al. Sutimlimab in cold agglutinin disease. N Engl J Med. 2021;384(14):1323-1334.
  23. 23.Neunert C, Terrell DR, Arnold DM, et al. American Society of Hematology 2019 guidelines for immune thrombocytopenia [published correction appears in Blood Adv. 2020; 4(2): 252]. Blood Adv. 2019;3(23):3829-3866.
  24. 24.Peyvandi F, Scully M, Kremer Hovinga JA, et al.; TITAN Investigators. Caplacizumab for acquired thrombotic thrombocytopenic purpura. N Engl J Med. 2016;374(6):511-522.
  25. 25.Levi M, Scully M. How I treat disseminated intravascular coagulation. Blood. 2018;131(8):845-854.
  26. 26.Levi M. Diagnosis and treatment of disseminated intravascular coagulation. Int J Lab Hematol. 2014;36(3):228-236.
  27. 27.Szczepiorkowski ZM, Dunbar NM. Transfusion guidelines: when to transfuse. Hematology Am Soc Hematol Educ Program. 2013;2013:638-644.
  28. 28.Scully M, Hunt BJ, Benjamin S, et al.; British Committee for Standards in Haematology. Guidelines on the diagnosis and management of thrombotic thrombocytopenic purpura and other thrombotic microangiopathies. Br J Haematol. 2012;158(3):323-335.
  29. 29.Sukumar S, Lämmle B, Cataland SR. Thrombotic thrombocytopenic purpura: pathophysiology, diagnosis, and management. J Clin Med. 2021;10(3):536.
  30. 30.Bendapudi PK, Hurwitz S, Fry A, et al. Derivation and external validation of the PLASMIC score for rapid assessment of adults with thrombotic microangiopathies: a cohort study. Lancet Haematol. 2017(4):e157-e164.
  31. 31.Zheng XL, Vesely SK, Cataland SR, et al. ISTH guidelines for treatment of thrombotic thrombocytopenic purpura. J Thromb Haemost. 2020;18(10):2496-2502.
  32. 32.George JN. TTP: the evolution of clinical practice. Blood. 2021;137(6):719-720.
  33. 33.Taylor FB, Toh CH, Hoots WK, et al.; Scientific Subcommittee on Disseminated Intravascular Coagulation of the International Society on Thrombosis and Haemostasis. Towards definition, clinical and laboratory criteria, and a scoring system for disseminated intravascular coagulation. Thromb Haemost. 2001;86(5):1327-1330.
  34. 34.Wada H, Matsumoto T, Yamashita Y. Diagnosis and treatment of disseminated intravascular coagulation (DIC) according to four DIC guidelines. J Intensive Care. 2014;2(1):15.
  35. 35.Levi M, Toh CH, Thachil J, et al.; British Committee for Standards in Haematology. Guidelines for the diagnosis and management of disseminated intravascular coagulation. Br J Haematol. 2009;145(1):24-33.
  36. 36.Savioli G, Ceresa IF, Caneva L, et al. Trauma-induced coagulopathy: overview of an emerging medical problem from pathophysiology to outcomes. Medicines (Basel). 2021;8(4):16.
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