Hemoglobinopathies: Hemoglobin S Disorders

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Author: Lillian Mundt, EdD, MLS(ASCP)SH
Reviewers: David J. Moffa, PhD, BCLD; Pinal Patel, MT (AMT); Margaret Reinhart, MS, MT(ASCP)

Sickle cells are some of the most readily identifiable abnormal red blood cells that may be encountered on a manual differential and observation of them will assist in the positive identification of a hemoglobin S disorder. However, they are not present in all hemoglobin S disorders, or may not be present in all stages of a disorder.

This course will update you on the laboratory testing that is currently being used to diagnose and differentiate between the various forms of hemoglobin S disorders. You will also learn about the clinical symptoms that are associated with each one.

The course concludes with several case studies that will provide you with the opportunity to apply what you learned in the course.

Continuing Education Credits


  • Differentiate among structural abnormalities comprising the hemoglobinopathies.
  • Describe sickle cell disorders.
  • Explain the molecular basis for sickle cell hemoglobins.
  • Describe the process of sickle cell formation.
  • Identify contributors to the sickling process.
  • List the clinical manifestations of the various forms of sickle cell disorders.
  • Compare and contrast laboratory findings for sickle cell disorders.
  • Differentiate among the various forms of sickle cell disorders.
  • Describe therapies used in the treatment of sickle cell disorders.
  • Interpret laboratory results for sickle cell hemoglobin case studies.

Course Outline

  • Hemoglobinopathies
      • Hemoglobinopathies
      • Examples of Structural Abnormalities
      • Hemoglobinopathies can be caused by all of the following structural defects EXCEPT:
  • Sickle Cell Disorders
      • Sickle Cell Disorders
      • To produce hemoglobin S, glutamic acid that is normally present in the sixth position on the beta globin chain is substituted with which of the follow...
      • Sickle Cell Disorder Frequencies: Hb SS and Hb SA
      • Sickle Cell Disorder Frequency: Hb S with Other Hemoglobins
      • Sickle Cell Disorder Frequency: Hb S / Thalassemia
      • Sickle Cell Disorder Frequency: Hb S with Hereditary Persistence of Fetal Hemoglobin
  • The Sickling Process
      • Sickling Process Overview
      • The Sickling Process
      • The Sickling Process, continued
      • Normal Blood Flow
      • Blood Flow During Sickling
      • Vascular Occlusion
      • Cellular Dehydration
      • The first time a cell containing Hb SS is deoxygenated, it forms an irreversibly sickled cell.
  • Clinical Manifestations of Sickle Cell Disease
      • Clinical Manifestations of Sickle Cell Disease (SCD)
      • Vaso-occlusive Crisis
      • Vaso-occlusive Crisis, continued
      • Sequestration Crisis
      • Hemolytic Crisis
      • Aplastic Crisis
      • In sickle cell anemia, an increased amount of which of the following surface antigens on reticulocytes may allow platelets to form a bridge between th...
      • Which of the following will increase during hemolysis?
      • Complications
      • Risk Factors
      • Which of these could cause a sickling event?
  • Treatment of Sickle Cell Disease
      • Treatment for Sickle Cell Disease
      • Supportive Care
      • Pain Management
      • Pharmacotherapy
      • Non-Pharmacotherapy
      • Blood Tranfusions
      • Emerging New Treatments
      • Preventive Treatment
  • Laboratory Findings in Sickle Cell Disorders
      • Laboratory Diagnosis
      • Comparison of Laboratory Results Among Hemoglobin S Disorders
    • Solubility Test
      • Solubility Test
      • Solubility Test Errors
    • Hemoglobin Electrophoresis
      • Hemoglobin Electrophoresis
      • Migration of Hemoglobin in Alkaline Electrophoresis
      • Migration of Hemoglobin in Acid Electrophoresis
      • Normal Hemoglobin Electrophoresis (alkaline)
      • Normal Hemoglobin Electrophoresis Densitometer Tracing
      • Densitometer Tracing of Various Hemoglobins
      • Hemoglobin Electrophoresis Patterns in Sickle Cell Disorders (Alkaline)
      • Hemoglobin Electrophoresis Patterns in Sickle Cell Disorders (Acid)
      • The gel electrophoresis pattern for hemoglobin S shows which of the following migration patterns?
    • HPLC and other methods
      • High Pressure Liquid Chromatography, Capillary Electrophoresis, and Isoelectric Focusing
      • Genetic Testing
      • Other Methods of Detecting Hb S
      • A technique which utilizes high voltage and separates out the hemoglobin proteins on a polyacrylamide gel is
    • Red Blood Cell Morphologies in Sickle Cell Anemias
      • RBC Morphology in Sickle Cell Trait (Hb SA)
      • RBC Morphology in Sickle Cell Disease (Hb SS)
      • RBC Morphology in Sickle Cell Anemia variant (Hb SC)
      • What is the characteristic RBC that is uniquely associated with Hb SS?
  • Case Studies
    • Sickle Cell Trait
      • CBC Results and RBC Morphology
      • Hemoglobin Electrophoresis
    • Sickle Cell Disease
      • CBC and RBC Morphology
      • Hemoglobin Electrophoresis
    • Hemoglobin SC Disease
      • CBC and RBC Morphology
      • Hemoglobin Electrophoresis
    • HbS / Beta thalassemia
      • CBC and RBC Morphology
      • Hemoglobin Electrophoresis
    • HbS with Hereditary Persistance of Fetal Hemoglobin (HPFH)
      • CBC and RBC Morphology
      • Hemoglobin Electrophoresis
  • References
      • References

Additional Information

Level of instruction: Intermediate

Intended Audience: Medical laboratory professionals and other healthcare personnel who have an interest in this subject matter. This course is also appropriate for medical laboratory science and medical laboratory technician students and pathology residents.
Author Information: Lillian Mundt, EdD, MLS(ASCP)SH is currently employed by AMITA Adventist Hinsdale Hospital in the Molecular Pathology Department. She received her BA degree in Medical Technology from Aurora College, Aurora, Illinois. Her graduate work was completed at Governor's State University, University Park, Illinois, from which she received her Master of Health Science in Allied Health Professions Education; her doctorate in Educational Leadership was earned from the University of Phoenix. Her work experience includes a position as a medical technologist in hematology for 30 plus years at Hinsdale Hospital, Department of Pathology. Lillian also taught at Hinsdale Hospital School of Medical Technology and Rosalind Franklin University of Medicine and Science Department of Clinical Laboratory Sciences.
Reviewer Information: David J. Moffa, PhD, BCLD, has over 30 years of experience in the healthcare industry as an executive manager, clinical laboratory director, and medical laboratory scientist. He is currently a technical consultant for Kentmere Healthcare, Wilmington, DE, and until his retirement, was the Regional Director for LabCorp, Inc. He holds a PhD in medical biochemistry from the School of Medicine, West Virginia University.
Reviewer Information: Pinal Patel, MT (AMT), is a Laboratory Education Specialist at Cleveland Clinic Foundation in Cleveland, Ohio. She has been with Cleveland Clinic since 2005 and worked as Medical Technologist in Pathology and Laboratory Medicine and transitioned to Educational Specialist role in 2016. She received Bachelor degree in Science major in Microbiology from Gujarat State University, India. She is a member of ASCLS. 
Reviewer Information:  Margaret Reinhart, MS, MT(ASCP) is the MLS Program Director and Senior lecturer in Biological Sciences at the University of the Sciences in Philadelphia PA where she teaches hematology, clinical immuno

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