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By: Chris Ebright

Blood-Related Disorders

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Anemia

Anemia is not a disease but rather a symptom of an underlying disease process. It is a condition in which a patient does not have enough red blood cells (low hematocrit) or when their red blood cells do not function properly. Signs and symptoms include weakness, shortness of breath, dizziness, fast or irregular heartbeat, headache, cold hands or feet, pale or yellow skin, restless leg syndrome, and/or chest pain.
Many people are at risk for anemia due to many reasons, including genetics, poor diet, intestinal disorders (Crohn disease or ulcerative colitis), chronic diseases (kidney failure, liver disease, thyroid disease), infections, cancer, and rheumatoid arthritis, or other autoimmune diseases (AIDS). Women who are menstruating or pregnant, the elderly, and patients on blood-thinning medications have a higher risk of developing anemia. Some of the more common types of anemia include: (8)

Iron-deficiency anemia

The most common type of anemia; occurs when there is a significant lack of iron within the body.
Iron is essential to produce hemoglobin in red blood cells. Any reduction in iron levels within the body results in the red cells’ inability to oxygenate tissues adequately.

  • Usually due to blood loss but may occasionally be due to poor absorption of iron.
  • Pregnancy, childbirth, and growth spurts consume a great deal of iron and may result in pregnancy-related anemia. 
  • People who have had gastric bypass surgery for weight loss or other reasons may also be iron deficient due to poor absorption.

Vitamin-deficiency anemia

May result from low vitamin B12 or folate (folic acid) levels, usually due to poor dietary intake. Both are necessary to process carbohydrates, proteins, and fats and help manufacture red blood cells. 

Pernicious anemia 

    • An inherited condition in which vitamin B12 cannot be absorbed in the gastrointestinal tract 
    • Other causes include stomach and minor intestine surgery, abnormal bacterial growth in the small intestine, Crohn’s disease, and celiac disease. 
    • It may be associated with Type I diabetes and thyroid disease.

Aplastic anemia

The bone marrow stops making sufficient levels of formed elements

    • Occurs because of autoimmune destruction or deficiency of blood-forming stem cells in the bone marrow
    • Viral infections, ionizing radiation, exposure to toxic chemicals, and medications such as antibiotics, anti-seizure, or cancer can also result in aplastic anemia.

Hemolytic anemia

Occurs when red blood cells are broken up in the bloodstream or the spleen

Mechanical causes

    • Leaky heart valves or aneurysms
    • Infections
    • Autoimmune disorders, or congenital abnormalities in the red blood cell

Inherited causes

Thalassemia
Usually affects people of Mediterranean, African, and Southeast Asian descent and is marked by abnormal and short-lived red blood cells. Also called Cooley’s Anemia, Thalassemia major is a severe form of anemia in which red blood cells are rapidly destroyed, and iron is deposited in the skin and vital organs. Regular transfusions are necessary.

Glucose-6 phosphate dehydrogenase deficiency

    • Causes red blood cells to break down in response to certain medications, infections, or other stressors.
    • When symptoms are triggered, they include fever, dark urine, abdominal and back pain, fatigue, and pale skin.

Sickle cell
Primarily affects African-Americans and those of Arabic, Greek, Italian, Latin-American, and Indian descent. Caucasians can also have sickle cell disease or sickle cell trait.

Mutations cause sickle cell disease in the gene that leads to a faulty hemoglobin protein, called hemoglobin S. Hemoglobin S changes red blood cells into rigid, sickle-shaped cells.

Sickle-shaped cells can stick to vessel walls, causing a blockage that slows or stops blood flow. When this happens, oxygen is unable to reach nearby tissues. The lack of oxygen in tissue can cause attacks of sudden severe pain, called sickle cell crisis. Sickle cell crisis may occur for no apparent reason or be triggered by conditions like dehydration, infection, stress, trauma, exposure to extreme temperatures, lack of oxygen, or strenuous physical activity. The frequency of pain varies from person to person, and recurrence of these crises can be disabling. Blindness, leg ulcers, and other health problems manifest, depending on where the blood flow blockage occurs in the body. (10)

Because sickle cells cannot change shape easily, they tend to burst. Normal red blood cells live about 90 to 120 days, but sickle cells last only 10 to 20 days. The body constantly makes new red blood cells to replace the old cells. However, the body may have trouble keeping up with how fast the cells are destroyed in sickle cell disease. Because of this, the number of red blood cells is usually lower than normal, leading to anemia. (9)

Signs and Symptoms

Common areas where pain develops are the extremities, back, chest, and abdomen.

Occlusion of blood flow results in:

    • shortness of breath
    • sudden severe abdominal and/or chest pain
    • icteric sclera, blindness
    • fever, weakness, and severe joint pain
    • TIA/CVA seizures and coma
    • priapism in males
    • gallstones
    • splenic damage – increases the risk of septicemia

Prehospital management of sickle cell crisis should decrease pain as required in any severe, acute pain-producing disease. Treatment of pain crisis includes administering analgesics, including narcotics and NSAIDs, rehydration, bed rest, and transportation to an ED for antibiotics to treat any underlying infection.(10,11) Oxygen should always be administered to sickle cell patients to fully oxygenate all normal red blood cells and decrease their sickling during a hypoxic state.

Polycythemia

Polycythemia is a bloodstream condition that results in an increased level of circulating red blood cells, hematocrit, or hemoglobin above normal limits. Polycythemia is divided into two categories:

    • Primary polycythemia: the increase in red blood cells is caused by inherent or genetic mutations.
    • Secondary polycythemia: occurs as a response to underlying conditions that promote red blood cell production. COPD, CHF, pulmonary hypertension, and sleep apnea are some examples.

With the increased levels of circulating red blood cells, the thickness of the blood is increased. This can be associated with a higher risk of thrombus formation leading to stroke, heart attack, pulmonary embolism, and possibly death. (19)

White Blood Cell Disorders

Leukopenia

A decrease in white blood cells, which can be caused by cells being destroyed or by not enough cells being made (12)
Causes include: aplastic anemia, autoimmune disorders, cancer treatments, antibiotics, vitamin deficiencies

Leukocytosis

An increase in white blood cells, which can be a normal response of the immune system but also caused by certain cancerous or non-cancerous diseases (13)
Causes include: smoking, allergies, certain types of leukemia, infections, removal of the spleen, and pregnancy

Signs and symptoms of white blood cell disorders can vary based on the underlying cause, and some patients may always be asymptomatic. If symptoms do develop, they are often non-specific. Fever, chills, fatigue, dizziness, sweating, dyspnea, tachycardia, body aches, cough, and loss of appetite are common.

The goal of prehospital management of patients with high or low white blood cell counts is to protect the patient from infection. It is imperative to use an aseptic technique when performing any invasive procedure. Many of these patients may be complaining about nausea, vomiting, and weakness that follow chemotherapy and/or radiation treatments. If the patient is dehydrated, treat with fluid therapy and transport to the hospital. If the patient is stable, transport in a position of comfort to the hospital for evaluation of their chief complaint. Consider analgesics for those patients in significant pain. (10)

Platelet Disorders

Thrombocytopenia

Lower than normal amount of circulating platelets.
Causes include: chemotherapy, hepatitis C, autoimmune diseases, pregnancy, medications such as warfarin and heparin, alcohol abuse, and severe sepsis

Thrombocytosis

Higher than normal amount of circulating platelets.
Causes include: Inflammatory conditions such as rheumatoid arthritis and inflammatory bowel disease, iron-deficiency anemia, removal of the spleen, cancer, infection, and trauma

Prehospital management is mainly supportive. Any interventions are usually on a case-by-case need and aimed at relieving any signs and symptoms that the patient is currently experiencing.

Hemophilia

Hemophilia is typically an inherited bleeding disorder in which the blood does not clot properly. This can lead to spontaneous bleeding as well as bleeding following injuries or surgery. The blood contains many proteins called clotting factors that can help to stop bleeding. There are several types of hemophilia; however, Hemophilia A and B are the most common. Hemophilia A, also called “classic hemophilia” is a lack or lower than normal level of clotting factor eight. Hemophilia B, also called “Christmas disease,” is a genetic disorder caused by missing or defective clotting factor nine.

People with hemophilia A or B bleed longer than other people. Bleeds can occur internally, into joints and muscles, the skin, the head, the urine or stool, or externally, from minor cuts, dental procedures, nosebleeds, circumcision, or injuries. (15) The severity of hemophilia that a person has is determined by the amount of clotting factor within the blood. The lower the amount of factor, the more likely it is that bleeding will occur. A patient’s plasma level of factor eight or nine must fall below 50 percent (half of what is needed to form a clot) to produce signs and symptoms. (14)

Von Willebrand disease

Von Willebrand disease (VWD) is the most common blood disorder, affecting 0.6 percent to 1.3 percent of the general population. (17) It is a bleeding disorder that affects both men and women, resulting from a protein deficiency in the blood called von Willebrand Factor (VWF).

When a person starts to bleed, the VWF in the blood attaches to platelets. This helps them stick together, like glue, to form a clot at the injury site and stop the bleeding. When a person has VWD, the clot might take longer to form or form incorrectly, and bleeding might take longer to stop. As a result, frequent and hard-to-stop nosebleeds, bruising, heavy menstrual bleeding, and longer than usual bleeding after injury, surgery, childbirth, or dental work may be apparent.

Most people who have VWD inherit it. While rare, a person can get VWD without a family history of the disease via a spontaneous mutation. It is also rare for a person to acquire VWD later in life because of an underlying medical condition. This can happen when a person’s immune system destroys their VWF, often because of another disease or medication use.

  • Type 1
    This is the most common and mildest form of VWD, in which a person has lower-than-normal levels of VWF. A person with Type 1 VWD also has low factor eight levels.
  • Type 2
    The body makes normal amounts of the VWF, but the factor does not work the way it should.
  • Type 3
    This is the most severe and rarest form of VWD. A person has very little or no VWF and low levels of factor eight.

EMS should treat bleeding in those with hemophilia or VWD with direct pressure over the injury location. The most important consideration for EMS providers is to transport these patients to the closest facility with a hemophilia treatment center. These centers will typically administer clotting factor cofactor and concentrate to the patient. While the patient should know where the closest center to them is, EMS providers can find the nearest one to the scene at: https://dbdgateway.cdc.gov/HTCDirSearch.aspx.. (16)

Conclusion

Hematological disorders are rare in the prehospital arena; however, it is important to understand the physiology behind these disease states. Increased knowledge will enable healthcare providers to conduct an improved assessment and better understand treatment for those patients.

Read Part One of Hematology Basics here!

References

  1. Mathew J, Sankar P, Varacallo M. Physiology, Blood Plasma. [Updated 2021 Apr 28]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2021 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK531504/.
  2. American Red Cross. (2021). What Does Hematocrit Mean? Hematocrit and Hemoglobin. Retrieved December 9, 2021, from https://www.redcrossblood.org/donate-blood/dlp/hematocrit.html.
  3. Cincinnati Center for Eosinophilic Disorders. (2021). Eosinophilic disorders: What is an eosinophil? Eosinophilic Disorders | What is an Eosinophil? Retrieved December 9, 2021, from https://www.cincinnatichildrens.org/service/c/eosinophilic-disorders/conditions/eosinophil#:~:text=Eosinophilic%20functions%20include%3A%20movement%20to,reactions%2C%20and%20modulating%20inflammatory%20responses.
  4. What are platelets, and why are they important? Johns Hopkins Medicine. (n.d.). Retrieved December 9, 2021, from https://www.hopkinsmedicine.org/health/conditions-and-diseases/what-are-platelets-and-why-are-they-important.
  5. Suzanne Dixon, M. P. H. (2021, September 2). What do your blood platelets do? Verywell Health. Retrieved December 10, 2021, from https://www.verywellhealth.com/thrombocyte-what-is-a-thrombocyte-797228#citation-1.
  6. American Red Cross. (2021). What Does Hematocrit Mean? Hematocrit and Hemoglobin. Retrieved December 9, 2021, from https://www.redcrossblood.org/donate-blood/dlp/hematocrit.html.
  7. Thompson, E. G., Husney, A., & Gabica, M. J. (2021, June 17). Blood type test. Retrieved December 10, 2021, from https://www.cham.org/HealthwiseArticle.aspx?id=hw3681.
  8. American Society of Hematology. (2021). Anemia. Hematology.org. Retrieved December 10, 2021, from https://www.hematology.org/education/patients/anemia.
  9. U.S. Department of Health and Human Services. (2020, September 1). Sickle cell disease. National Heart Lung and Blood Institute. Retrieved December 11, 2021, from https://www.nhlbi.nih.gov/health-topics/sickle-cell-disease.
  10. EMS World. (2005, August). Hematological Disorders. Hmpgloballearningnetwork.com. Retrieved December 11, 2021, from https://www.hmpgloballearningnetwork.com/site/emsworld/article/10323788/hematological-disorders.
  11. The Sickle Cell Information Center; The Georgia Comprehensive Sickle Cell Center at Grady Health System; The Sickle Cell Foundation of Georgia, Inc., Emory University School of Medicine, Department of Pediatrics, Atlanta Georgia; Morehouse School of Medicine. www.scinfo.org/prod05.htm.
  12. Christen D, Brummendorf TH, Panse J. Leukopenia – a diagnostic guideline for the clinical routine. Dtsch Med Wochenschr. 2017 Nov;142(23):1744-9. doi:10.1055/s-0043-113123
  13. Riley LK, Rupert J. Evaluation of patients with leukocytosis. Am Fam Physician. 2015 Dec 1;92(11):1004-11.
  14. Centers for Disease Control and Prevention. (2020, July 17). What is hemophilia? Centers for Disease Control and Prevention. Retrieved December 11, 2021, from https://www.cdc.gov/ncbddd/hemophilia/facts.html.
  15. Hemophilia A. National Hemophilia Foundation. (2021). Retrieved December 11, 2021, from https://www.hemophilia.org/bleeding-disorders-a-z/types/hemophilia-a.
  16. Greenhaus, D. (2020, June). Hemophilia: When the Bleeding Won’t Stop. Hmpgloballearningnetwork.com. Retrieved December 11, 2021, from https://www.hmpgloballearningnetwork.com/site/emsworld/article/1224422/hemophilia-when-bleeding-wont-stop.
  17. Blood disorders and blood safety. Blood Disorders and Blood Safety | Healthy People 2020. (2020). Retrieved December 11, 2021, from https://www.healthypeople.gov/2020/topics-objectives/topic/blood-disorders-and-blood-safety#three.
  18. James AH. Von Willebrand Disease. Obstet Gynecol Surv. 2006 Feb;61(2):136-5.
  19. Siamak N. Nabili, M. D. (2019, October 18). Polycythemia symptoms, causes, treatment & diagnosis. MedicineNet. Retrieved December 28, 2021, from https://www.medicinenet.com/polycythemia_high_red_blood_cell_count/article.htm

About the Author

Chris Ebright is the lead instructor for The University of Toledo EMS Program in Toledo, Ohio, where he provides all aspects of primary EMS education in-house and external continuing education for numerous EMS services in northwest Ohio. Chris has been a Nationally Registered paramedic for 27 years, providing primary EMS response and critical care transportation. He has educated hundreds of first responders, EMTs, paramedics, and nurses with his trademark whiteboard sessions, including students from the Cayman Islands, India, and Australia. Chris is also a recurring article contributor to the Limmer Education website and has been a featured presenter at numerous local, state, and national EMS conferences over the past 15 years. Chris holds a Bachelor of Education degree from the University of Toledo and can be contacted at c.ebrightnremtp@gmail.com or www.christopherebright.com.

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