Pancytopenia

What is pancytopenia?

Pancytopenia is the medical term for reduced levels of all blood cells, which include erythrocytes or red blood cells (RBCs), leukocytes or white blood cells (WBCs), and thrombocytes or platelets.

These blood cell types have different functions in the body. Red blood cells are the ones that carry oxygen all throughout the body, white blood cells help protect the body against foreign invaders and infectious diseases, and platelets play a very important role in blood clotting.

A combination of different blood diseases develops in people with pancytopenia. These diseases include:

• Leukopenia (low WBC count)
• Anemia (low RBC count)
• Thrombocytopenia (low platelet count)

All of these blood cells are needed by the body, so pancytopenia can be quite serious or life-threatening if it is left untreated. There are also a number of conditions that can cause pancytopenia, and they include certain infections, some cancers, and bone marrow diseases. Another cause of pancytopenia is chemotherapy. 

Treatment for pancytopenia usually depends on its specific cause. Its treatment often focuses on increasing the levels of blood cells to help relieve the symptoms. 

Symptoms

Mild cases of pancytopenia often do not cause any symptoms. Some cases might only be discovered by doctors while ordering blood tests for other reasons.

Severe pancytopenia can cause symptoms that are related to a blood cell deficiency:

• Symptoms of anemia (shortness of breath, pale skin, increased heart rate, and fatigue)
• Symptoms of leukopenia (tiredness, weakness, fever, and signs of infection)
• Symptoms of thrombocytopenia (heavy bleeding and bruising)

Other symptoms may include:

• Dizziness
• Purpura (purple spots on the skin)
• Petechiae (tiny purple spots on the skin)
• Epistaxis (nosebleeds)
• Bleeding gums

Seek emergency medical help if you or someone else has any of the following signs and symptoms of severe pancytopenia:

• Loss of consciousness
• Confusion
• Severe shortness of breath
• Seizures
• Fever (more than 101 degrees Fahrenheit or 38.3 degrees Celsius)
• Heavy bleeding

Causes

Anything that interferes with the process of blood cell formation in the bone marrow and availability of these blood cells in the bloodstream may cause pancytopenia. Causes may include bone marrow suppression during chemotherapy, bone marrow damage due to toxins, or some cancers.

Bone marrow suppression or damage may occur due to infections, inflammation, or autoimmune diseases. Most of these conditions tend to develop later in life. However, a few can also be inherited and present from birth. Approximately half of these cases are idiopathic, which means they occur with unknown causes.

Other possible causes of pancytopenia are:

• Aplastic anemia
• Systemic lupus erythematosus (SLE) and other autoimmune disorders
• Myelodysplastic syndromes (MDS)
• Drug-induced (certain antibiotics, chemotherapy drugs, or medications for heart disease)
• Bone marrow suppression that is chemotherapy-induced
• Splenic sequestration
• Infections (HIV, infectious mononucleosis, malaria, hepatitis, including sepsis)
• Metastatic bone marrow tumors
• Leukemia
• Multiple myeloma
• Lymphoma (Hodgkin’s or non-Hodgkin’s lymphoma)
• Solid tumors to the bone marrow, particularly stomach or gastric cancer, colon cancer, prostate cancer, breast cancer, and melanoma. The most common symptom of bone marrow metastases is anemia, which is then followed by thrombocytopenia.
• Inherited syndromes (Diamond-Blackfan anemia and Fanconi anemia)
• Paroxysmal nocturnal hemoglobinuria
• Radiation sickness
• Exposure to benzene or arsenic
• Vitamin deficiencies (lack of folate or vitamin B12)
• Liver disease

Diagnosis

When doctors suspect that their patients have pancytopenia, they will likely refer them to a hematologist. A hematologist specializes in the diagnosis, treatment, and prevention of blood diseases.

During your doctor’s appointment, you will be asked about your symptoms, personal medical history and family history. You will also undergo a physical examination, which includes checking your skin, nose, mouth, throat, and ears.

Your doctor will also order a blood test called a complete blood count (CBC) to check the amount of RBCs, WBCs, and platelets in your blood. Another test called a peripheral blood smear may be ordered if your CBC has abnormal results. In this test, a drop of your blood is needed on a slide to check and look at the types of blood cells your blood contains.

If your doctor suspects a bone marrow problem, a bone marrow aspiration and biopsy may be performed. This test involves the use of a needle to extract a certain amount of tissue and liquid inside your bone. The collected sample will then be tested and examined in the laboratory.

Separate tests may be recommended by your doctor to look for other causes of pancytopenia. A CT scan or other imaging tests may also be ordered to look for organ problems, including cancer. 

Complications

Severe pancytopenia is a potentially life-threatening condition. Abnormally low levels of RBCs, WBCs, and platelets may lead to certain complications, which include:

• Increased risk of developing infections due to low WBC counts
• Excessive bleeding or blood clotting problems due to low platelet counts

Treatment

Pancytopenia treatment focuses on treating the underlying cause. If the main cause is unknown, treatment is often aimed at relieving the symptoms. This mode of treatment is also applicable to expected causes of pancytopenia, such as chemotherapy.

Treatments may include:

• Antibiotics for infections.
• Blood transfusions to replenish RBCs, WBCs, and platelets.
• Stem cell transplant or bone marrow transplant to replace the damaged bone marrow and produce healthy stem cells.
• Immunosuppressants for autoimmune disorders.
• Bone marrow stimulants to help improve the production of blood cells in the bone marrow.

Prognosis

The prognosis of pancytopenia largely depends on the type of disease that caused the condition, including the type of treatment given by doctors.

Most people with chemical- or drug-induced pancytopenia tend to get better after stopping the exposure. However, certain conditions, such as cancer may take longer to treat.

Prevention

Certain types of infection may be prevented by practicing good hygiene and by avoiding direct contact with people who are sick. Chemicals or drugs that can cause pancytopenia should also be avoided. However, inherited bone marrow diseases, cancer, and other serious causes of pancytopenia are not preventable. 

Conclusion

All types of blood cells are reduced in people with pancytopenia. This condition also has a number of potential causes, and all of these causes carry health risks. 

References

Gedik, H., & Yokus, O. (2016). Etiological causes of pancytopenia: A report of 137 cases. Avicenna Journal of Medicine, 6(4), 109. doi:10.4103/2231-0770.191447

Kirpal Das Makheja, V. (2013). The common causes leading to pancytopenia in patients presenting to tertiary care hospital. Pakistan Journal of Medical Sciences, 29(5), 1108.

Approach to the adult with unexplained pancytopenia.UpToDate. (2018). Uptodate.com. Retrieved 7 December 2018, from https://www.uptodate.com/contents/approach-to-the-adult-with-unexplained-pancytopenia?source=search_result&search=pancytopenia&selectedTitle=1~150