Definitions & Key takeaways

Aplastic anemia is a rare blood disorder caused by bone marrow failure to produce new blood cells. Even though it's referred to as aplastic anemia, it typically presents with pancytopenia, meaning that all three major blood cell lines - the red blood cells, white blood cells, and platelets are affected.

People with aplastic anemia may experience fatigue, shortness of breath, ecchymoses and mucosal bleeding, and frequent infections pallor Aplastic anemia can be caused by a variety of factors, including exposure to radiation or chemotherapy, viral infections, and autoimmune disorders.

Chapters:

Introduction0:00–0:22

Aplastic anemia is a condition characterized by pancytopenia, meaning decreased production of all blood cell lines, so red and white blood cells, as well as platelets.
This condition can be inherited, when it occurs because of genetic mutations, as well as acquired, when it occurs because of external factors.Let’s start by looking at the physiology of blood cell production, or hematopoiesis.

Physiology0:22–1:38

This process starts in the bone marrow, which is the innermost portion of bone, where hematopoietic stem cells reside. These serve as progenitor cells for all the different cell types found in the blood, including white blood cells, which primarily function as the defense mechanism against infections; as well as red blood cells, which are essential to carry oxygen to the tissues; and platelets, which play a key role in blood clotting.
Now, hematopoietic stem cells reach their final, mature form, when they receive the appropriate signals in the form of specific growth factors or stimulating factors.
One of these growth factors is called erythropoietin, or EPO. This is a hormone released by the kidneys, that tells hematopoietic stem cells to differentiate into red blood cells.
Another important factor is the granulocyte colony-stimulating factor, or G-CSF, which is produced by various cells and tissues, including the bone marrow itself, and it stimulates the production of a specific group of white blood cells called neutrophils.
Finally, there’s thrombopoietin, a hormone produced by the liver and kidneys, which stimulates platelet production.Alright, now the causes of aplastic anemia can be inherited or acquired.

Causes & risk factors1:38–2:50

The most common cause of inherited aplastic anemia is Fanconi’s anemia, a rare genetic disorder characterized by progressive bone marrow failure.
On the other hand, acquired causes of aplastic anemia include any other factor that leads to suppression or destruction of the bone marrow, including other conditions like an autoimmune disease, or undergoing treatments like chemotherapy or radiation therapy.
Additionally, many cases are idiopathic, meaning the exact cause is unknown.Some medications can also cause bone marrow suppression, like antiepileptics such as anticonvulsants hydantoins or carbamazepine; antithyroid medications like propylthiouracil and methimazole; and certain antibiotics like chloramphenicol and sulfonamides.
There are also toxic chemicals associated with bone marrow destruction, like benzene and certain solvents. And last but not least, some viruses like EBV, HIV or hepatitis viruses can also be a cause.

Pathology2:50–3:21

Okay, so the pathology of aplastic anemia starts with an inherited defect or acquired damage to the bone marrow. In most cases, this triggers an autoimmune reaction that destroys hematopoietic stem cells, followed by subsequent replacement of the bone marrow by non-functional fatty tissue.
Eventually, clients with aplastic anemia do not have enough hematopoietic stem cells to generate appropriate amounts of mature blood cells, and the end result is pancytopenia.Now, clinical manifestations of aplastic anemia vary depending on the disease severity.

Clinical manifestations3:21–4:09

A low red blood cell count can lead to fatigue, dizziness, and pallor. In severe cases, tissues cannot properly oxygenate, so the heart pumps harder to circulate the blood, which can lead to tachycardia, chest pain, and dyspnea.
Next, a low platelet count can lead to increased risk of bleeding due to minor injuries; as well as spontaneous mucosal bleeding, like epistaxis and bleeding gums; and petechiae, which are tiny red spots that appear on the skin because of broken capillaries.
Finally, low white blood cell count predisposes clients to develop recurrent or severe infections.The diagnosis of aplastic anemia starts with the client’s history and physical assessment; followed by laboratory tests, including complete blood cell count that shows pancytopenia.

Diagnosis4:09–5:10

Usually, the absolute neutrophil count, or ANC is less than 500/mm3, while platelet number is lower than 20,000/mm3. Other common findings include a hemoglobin level below 10g/dL and a decreased number of immature red blood cells, or reticulocytes, which demonstrates decreased red blood cell production; as well as increased levels of EPO, in an attempt to compensate and ramp up the production of red blood cells.
In addition, bleeding time may also be increased. Definitive diagnosis of aplastic anemia requires a bone marrow biopsy, which demonstrates profoundly low counts of hematopoietic stem cells with increased fat content, while at the same time ruling out other causes of bone marrow suppression, like malignancy or fibrosis.Treatment of aplastic anemia depends on the cause, and can vary depending on the client's age and clinical history.

Treatment5:10–6:01

If the condition is thought to be due to a medication or toxic agent, they should be removed immediately. Supportive treatment includes blood transfusions to correct the anemia and antibiotics, as well as antifungal and antiviral medications to prevent or treat infections.
For clients under the age of 50, first line therapy is a bone marrow transplant. For those over the age of 50, first line therapy includes immunosuppressive medications, like cyclosporine or glucocorticoids, in order to stop the autoimmune reaction.
Finally, a thrombopoietin receptor agonist, like eltrombopag, can sometimes also be used, irrespective of client’s age. Alright, let’s look at the nursing care you’ll be providing for a client with aplastic anemia.

Management and care6:01–8:00

The priority goals of care are to prevent complications from neutropenia, impaired coagulation, and decreased oxygenation-carrying capacity; and to provide supportive care until the underlying cause of the condition is treated.Begin by placing your client on neutropenic precautions.
Assess your client’s vital signs, and review their laboratory test results, including WBC count and inflammatory markers like ESR, CRP, and procalcitonin.
Immediately report to the healthcare provider if you note signs of infection, including fever, cough, or shortness of breath, or mental confusion.
Be prepared to obtain cultures and administer antibiotics as ordered. While caring for your client, be mindful of their impaired coagulation.
Use swabs to gently provide oral care, and avoid rectal temperatures until the client’s platelet count returns to normal.
Assess your client’s heart rate and blood pressure, and examine their skin and mucous membranes for signs of bleeding. Report to the healthcare provider if you note any new bruising, petechiae, epistaxis, gingival oozing, oozing from their IV insertion site, or frank bleeding.
Be prepared to hold pressure to any active site of bleeding, and administer fluids, platelets, or eltrombopag as ordered.
Next, review your client’s RBC count, hemoglobin, and hematocrit. Institute fall precautions, and assist them with ambulation.
Report to the healthcare provider if you note signs of decreasing oxygen carrying capacity, including fatigue, shortness of breath, and palpitations.
Administer supplemental oxygen per nasal cannula, and prepare to administer leukoreduced packed red blood cells, as ordered.
Alright, moving on to client and family teaching. Begin by explaining the function of the cells made by the bone marrow.

General client and family teaching8:00–9:11

Remind them that because they have a low white blood cell count, they are at risk for infection. Encourage them to practice good hand hygiene, avoid crowded areas and people with respiratory symptoms, and avoid eating unpasteurized dairy products, raw or uncooked meat and shellfish, raw sprouts, and unwashed fruits and vegetables.
To prevent bleeding, encourage them to take measures to avoid falls, and to include bleeding precautions in their daily activities, such as using a soft bristled toothbrush, electric razor for shaving, to avoid blowing their nose hard or scratching inside their nose.
Remind them to contact their healthcare provider immediately if they fall or injure themselves or if they experience symptoms such as prolonged bleeding; chest pain or palpitations, or lightheadedness; or if they have symptoms of infection, including fever, chills, cough, sore throat, or pain with urination.Alright, as a quick recap….

Review9:11–10:05

Aplastic anemia is a deficiency of all blood cell lines. This type of anemia can be caused by exposure to a toxin, virus, radiation, or medication.
It can also be caused by a congenital disorder, like Fanconi anemia, which affects bone marrow function and therefore blood cell production.
Diagnosis is often confirmed with a bone marrow biopsy. Treatment may include blood transfusions, antibiotics, antifungal and antiviral medications, as well as immunosuppressive therapy, thrombopoietin receptor agonists, or a bone marrow transplant.
Nursing care is aimed at preventing complications from neutropenia, impaired coagulation, and decreased oxygenation-carrying capacity.
Client teaching is centered around preventative care and when to