eMedicine Specialties > Hematology > Red Blood Cells and Disorders

Paroxysmal Nocturnal Hemoglobinuria: Multimedia

Author: Emmanuel C Besa, MD, Professor, Department of Medicine, Division of Hematologic Malignancies, Kimmel Cancer Center, Thomas Jefferson University
Coauthor(s): Ulrich Woermann, MD, Consulting Staff, Division of Instructional Media, Institute for Medical Education, University of Bern, Switzerland
Contributor Information and Disclosures

Updated: Mar 27, 2009

Multimedia

This series of containers holds urine of a patien...
(Enlarge Image)
Media file 1: This series of containers holds urine of a patient with paroxysmal nocturnal hemoglobinuria, showing the episodic nature of the dark urine (hemoglobinuria) during intravascular hemolysis, usually occurring at night. Early morning urine is cola-colored. This may occur at different times of the day and vary from patient to patient. Permission to use this image has been granted by the American Society of Hematology Slide Bank, 3rd edition.
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This series of containers holds urine of a patien...

This series of containers holds urine of a patient with paroxysmal nocturnal hemoglobinuria, showing the episodic nature of the dark urine (hemoglobinuria) during intravascular hemolysis, usually occurring at night. Early morning urine is cola-colored. This may occur at different times of the day and vary from patient to patient. Permission to use this image has been granted by the American Society of Hematology Slide Bank, 3rd edition.

The Ham test (acidified serum lysis) establishes ...
(Enlarge Image)
Media file 2: The Ham test (acidified serum lysis) establishes the diagnosis of paroxysmal nocturnal hemoglobinuria (PNH), demonstrating a characteristic abnormality of PNH red blood cells by acidified fresh normal serum. Here is a PNH patient's (Pt) red blood cells lysed by normal serum at room temperature (RT) and at 37°C compared with normal red cells (no hemolysis) (control [C]). Heated serum at 56°C inactivates complement and prevents hemolysis in PNH cells. Permission to use this image has been granted by the American Society of Hematology Slide Bank, 3rd edition.
[ CLOSE WINDOW ]
The Ham test (acidified serum lysis) establishes ...

The Ham test (acidified serum lysis) establishes the diagnosis of paroxysmal nocturnal hemoglobinuria (PNH), demonstrating a characteristic abnormality of PNH red blood cells by acidified fresh normal serum. Here is a PNH patient's (Pt) red blood cells lysed by normal serum at room temperature (RT) and at 37°C compared with normal red cells (no hemolysis) (control [C]). Heated serum at 56°C inactivates complement and prevents hemolysis in PNH cells. Permission to use this image has been granted by the American Society of Hematology Slide Bank, 3rd edition.

More on Paroxysmal Nocturnal Hemoglobinuria

Overview: Paroxysmal Nocturnal Hemoglobinuria
Differential Diagnoses & Workup: Paroxysmal Nocturnal Hemoglobinuria
Treatment & Medication: Paroxysmal Nocturnal Hemoglobinuria
Follow-up: Paroxysmal Nocturnal Hemoglobinuria
Multimedia: Paroxysmal Nocturnal Hemoglobinuria
References
Further Reading
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References

  1. Rosse WF. Paroxysmal nocturnal hemoglobinuria. In: Handin RI, Lux SE, Stossel TP, eds. Blood: Principles and Practice of Hematology. Baltimore, Md: Lippincott Williams & Wilkins; 1995:367-76.

  2. Luzzatto L. Paroxysmal nocturnal hemoglobinuria. Hematology 2000. American Society of Hematology Education Program. 2000;28-38.

  3. Parker C, Omine M, Richards S, et al, for the International PNH Interest Group. Diagnosis and management of paroxysmal nocturnal hemoglobinuria. Blood. Dec 1 2005;106(12):3699-709. [Medline]. [Full Text].

  4. Roth A, Duhrsen U, Schrezenmeier H, Schubert J. [Paroxysmal nocturnal hemoglobinuria (PNH). Pathogenesis, diagnosis and treatment] [German]. Dtsch Med Wochenschr. Feb 2009;134(9):404-9. [Medline].

  5. Bessler M, Hillmen P. Somatic mutation and clonal selection in the pathogenesis and in the control of paroxysmal nocturnal hemoglobinuria. Semin Hematol. Apr 1998;35(2):149-67. [Medline].

  6. Rosse WF, Ware RE. The molecular basis of paroxysmal nocturnal hemoglobinuria. Blood. Nov 1 1995;86(9):3277-86. [Medline]. [Full Text].

  7. Nagarajan S, Brodsky RA, Young NS, Medof ME. Genetic defects underlying paroxysmal nocturnal hemoglobinuria that arises out of aplastic anemia. Blood. Dec 15 1995;86(12):4656-61. [Medline]. [Full Text].

  8. Parker C. Eculizumab for paroxysmal nocturnal haemoglobinuria. Lancet. Feb 28 2009;373(9665):759-67. [Medline].

  9. Ruiz-Delgado GJ, Vazquez-Garza E, Mendez-Ramirez N, Gomez-Almaguer D. Abnormalities in the expression of CD55 and CD59 surface molecules on peripheral blood cells are not specific to paroxysmal nocturnal hemoglobinuria. Hematology. Feb 2009;14(1):33-7. [Medline].

  10. Young NS. Hematopoietic cell destruction by immune mechanisms in acquired aplastic anemia. Semin Hematol. Jan 2000;37(1):3-14. [Medline].

  11. Hillmen P, Lewis SM, Bessler M, Luzzatto L, Dacie JV. Natural history of paroxysmal nocturnal hemoglobinuria. N Engl J Med. Nov 9 1995;333(19):1253-8. [Medline]. [Full Text].

  12. Nishimura J, Kanakura Y, Ware RE, et al. Clinical course and flow cytometric analysis of paroxysmal nocturnal hemoglobinuria in the United States and Japan. Medicine (Baltimore). May 2004;83(3):193-207. [Medline].

  13. Araten DJ, Thaler HT, Luzzatto L. High incidence of thrombosis in African-American and Latin-American patients with paroxysmal nocturnal haemoglobinuria. Thromb Haemost. Jan 2005;93(1):88-91. [Medline].

  14. Brodsky RA, Mukhina GL, Li S, et al. Improved detection and characterization of paroxysmal nocturnal hemoglobinuria using fluorescent aerolysin. Am J Clin Pathol. Sep 2000;114(3):459-66. [Medline].

  15. Rosse WF, Dacie JV. The role of complement in the sensitivity of the paroxysmal nocturnal haemoglobinuria red cell to immune lysis. Bibl Haematol. 1965;23:11-8. [Medline].

  16. Rosse WF, Dacie JV. Immune lysis of normal human and paroxysmal nocturnal hemoglobinuria (PNH) red blood cells. I. The sensitivity of PNH red cells to lysis by complement and specific antibody. J Clin Invest. May 1966;45(5):736-48. [Medline]. [Full Text].

  17. Rosse WF, Dacie JV. Immune lysis of normal human and paroxysmal nocturnal hemoglobinuria (PNH) red blood cells. II. The role of complement components in the increased sensitivity of PNH red cells to immune lysis. J Clin Invest. May 1966;45(5):749-57. [Medline]. [Full Text].

  18. Hillmen P, Hall C, Marsh JC, et al. Effect of eculizumab on hemolysis and transfusion requirements in patients with paroxysmal nocturnal hemoglobinuria. N Engl J Med. Feb 5 2004;350(6):552-9. [Medline]. [Full Text].

  19. Hill A, Hillmen P, Richards SJ, et al. Sustained response and long-term safety of eculizumab in paroxysmal nocturnal hemoglobinuria. Blood. Oct 1 2005;106(7):2559-65. [Medline]. [Full Text].

  20. Ebenbichler CF, Wurzner R, Sandhofer AD, et al. Anti-thymocyte globulin treatment of a patient for paroxysmal nocturnal haemoglobinuria-aplastic anaemia syndrome: complement activation and transient decrease of the PNH clone. Immunobiology. 1996-1997;196(5):513-21. [Medline].

  21. Graham ML, Rosse WF, Halperin EC, Miller CR, Ware RE. Resolution of Budd-Chiari syndrome following bone marrow transplantation for paroxysmal nocturnal haemoglobinuria. Br J Haematol. Mar 1996;92(3):707-10. [Medline].

  22. Hall C, Richards S, Hillmen P. Primary prophylaxis with warfarin prevents thrombosis in paroxysmal nocturnal hemoglobinuria (PNH). Blood. Nov 15 2003;102(10):3587-91. [Medline]. [Full Text].

  23. Socie G, Mary JY, de Gramont A, et al, for the French Society of Haematology. Paroxysmal nocturnal haemoglobinuria: long-term follow-up and prognostic factors. Lancet. Aug 31 1996;348(9027):573-7. [Medline].

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Keywords

paroxysmal nocturnal hemoglobinuria, PNH, myelodysplastic syndromes, hemoglobinuria, hemolytic anemia, paroxysmal cold hemoglobinuria, paroxysmal hemoglobinuria, hemosiderinuria, Marchiafava-Micheli syndrome, dark-colored urine, hemolysis,

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Contributor Information and Disclosures

Author

Emmanuel C Besa, MD, Professor, Department of Medicine, Division of Hematologic Malignancies, Kimmel Cancer Center, Thomas Jefferson University
Emmanuel C Besa, MD is a member of the following medical societies: American Association for Cancer Education, American College of Clinical Pharmacology, American Federation for Medical Research, American Society of Hematology, and New York Academy of Sciences
Disclosure: Nothing to disclose.

Coauthor(s)

Ulrich Woermann, MD, Consulting Staff, Division of Instructional Media, Institute for Medical Education, University of Bern, Switzerland
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

Marcel E Conrad, MD, (Retired) Distinguished Professor of Medicine, University of South Alabama
Marcel E Conrad, MD is a member of the following medical societies: Alpha Omega Alpha, American Association for the Advancement of Science, American Association of Blood Banks, American Chemical Society, American College of Physicians, American Physiological Society, American Society for Clinical Investigation, American Society of Hematology, Association of American Physicians, Association of Military Surgeons of the US, International Society of Hematology, Society for Experimental Biology and Medicine, and Southwest Oncology Group
Disclosure: No financial interests None None

CME Editor

Rajalaxmi McKenna, MD, FACP, Consulting Staff, Department of Medicine, Southwest Medical Consultants, SC, Good Samaritan Hospital, Advocate Health Systems
Rajalaxmi McKenna, MD, FACP is a member of the following medical societies: American Society of Clinical Oncology, American Society of Hematology, and International Society on Thrombosis and Haemostasis
Disclosure: Nothing to disclose.

Chief Editor

Koyamangalath Krishnan, MD, FRCP, FACP, Dishner Endowed Chair of Excellence in Medicine, Professor of Medicine and Chief of Hematology-Oncology, Program Director, Hematology-Oncology Fellowship, James H Quillen College of Medicine at East Tennessee State University
Koyamangalath Krishnan, MD, FRCP, FACP is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians-American Society of Internal Medicine, American Society of Hematology, and Royal College of Physicians
Disclosure: Nothing to disclose.

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