What Is Pancytopenia?

Overview

Pancytopenia is a relatively common and critical medical condition caused by a significant decrease in all three types of blood cells: red blood cells (RBCs), white blood cells (WBCs), and platelets. In this article, we will help you understand what pancytopenia is, how it affects the body, its causes, diagnosis, treatment, and more. By the end of this article, you'll have a solid grasp of this condition and its implications.

Blood cells’ role in the body

Blood is an essential, specialised body fluid that circulates throughout the body. The three main blood cells circulating in the blood are red blood cells, white blood cells and platelets. 

In adults, these cells are mainly produced in the bone marrow from specialised cells called Stem cells. Each of these cell types is important for our overall health as they perform specific functions.

Red blood cells also known as erythrocytes contain a special protein called haemoglobin. It helps transport oxygen and nutrients to all the cells in the body. Having low levels of red blood cells or haemoglobin is called anaemia.

White blood cells or leukocytes help fight infections as they form an important part of our body’s defence system. Two important types of white blood cells are neutrophils and lymphocytes. Having reduced levels of white blood cells is called leukopenia.

Platelets also called thrombocytes, help in preventing blood loss. Multiple platelets gather at the site of injury to form a clot and thereby help control bleeding. Having low levels of platelets is called thrombocytopenia.

How pancytopenia affects the blood cells?

Pancytopenia is a medical condition with lower-than-normal levels of all three blood cell types. Therefore it’s a combination of anaemia, leukopenia, and thrombocytopenia.¹

The blood reports of a patient with pancytopenia will show a haemoglobin value of less than 13 g/dL (grams per decilitre) in individuals assigned men at birth (AMAB) and 12 g/dL in individuals assigned female at birth (AFAB), a platelet count of less than 150,000 per mcL (microlitres), and a white blood cell count of less than 4000 per ml. One point to remember here is that these thresholds may differ depending on age, sex, and race.¹

Symptoms of Pancytopenia

Mild pancytopenia may not produce any noticeable symptoms. They are often diagnosed by your doctor incidentally when a blood test is done for some other condition. However severe pancytopenia can be life-threatening. Anaemia can cause symptoms like:

• Weakness or easy fatigability
• Shortness of breath
• Palpitations
• Chest pain
• Headache
• Dizziness
• Pale skin

Patients with low platelet count may experience the following symptoms:

• Prolonged bleeding from cuts
• Bruising easily
• Bleeding from nose and gums
• Blood in the urine or stools
• Heavy periods

Symptoms due to reduced white blood cells include:

• Repeated infections
• Fever
• Sepsis which can be life-threatening^1,2

Causes of Pancytopenia

Now, the big question: What causes pancytopenia? Well, it's often a bit like solving a mystery. There can be many culprits. Pancytopenia is not a disease by itself, but it could indicate an underlying serious and life-threatening disease process. 

Our bone marrow has an incredible capacity to produce blood cells depending on how much our body needs. This regulated process is called hematopoiesis. Sometimes pancytopenia develops when our bone marrow fails to produce enough cells and other times it happens when the cells produced by the bone marrow are destroyed.¹ We'll go through these causes one by one to shed light on why pancytopenia occurs.

Vitamin B12 and Folic acid deficiency

These nutrients are crucial for the synthesis of DNA in our cells. Therefore if our body does not get these nutrients our bone marrow fails to produce sufficient blood cells leading to pancytopenia.Pancytopenia due to nutritional deficiency is quite common in developing countries. Chronic alcoholism and liver diseases can also lead to the deficiency of these vitamins causing pancytopenia.^3,4

Enlarged spleen

The spleen can grow in size in infections like malaria, leishmaniasis and certain conditions like liver cirrhosis and blood disorders. When this happens our blood cells can get trapped in the spleen and get destroyed causing pancytopenia.³

Infections

Pancytopenia is also seen in infections like parvovirus B19, tuberculosis, hepatitis, HIV, cytomegalovirus, brucellosis and Epstein-Barr virus.

Pancytopenia has also been reported in a few patients after Coronavirus 2 (SARS-CoV-2/COVID-19) infection.¹

Drugs/ Medications

A list of medicines including anticancer drugs like methotrexate, dapsone, carbimazole, carbamazepine, and chloramphenicol can also suppress the bone marrow and reduce the production of blood cells.¹ Similarly, environmental toxins and radiation exposure can also cause pancytopenia.^1,4

Cancer

Pancytopenia can be seen in cancers of the blood namely lymphoma, leukaemia, multiple myeloma and also when cancer from other parts of the body spreads to the bone marrow.^3,4

Autoimmune conditions

These are conditions where our body's immune system attacks and destroys its own cells. Pancytopenia can be seen in autoimmune conditions like systemic lupus erythematosus, and rheumatoid arthritis.³

Congenital diseases

A few conditions that a person is born with can also cause pancytopenia. Examples of this include Fanconi’s anaemia, Shwachman-Diamond syndrome  and dyskeratosis congenita.⁴

Other conditions where bone marrow does not produce enough healthy blood cells include aplastic anaemia, myelofibrosis, and myelodysplastic syndrome. Some cases of pancytopenia may not have an identifiable cause. This is referred to as idiopathic pancytopenia.⁴

Diagnosis and workup

 Now, let's talk about how doctors figure out if you have pancytopenia. It's like being a detective in a medical mystery. Your doctor will ask for a detailed history including a family history of blood disorder.

They will also perform a thorough physical examination to look for any signs of infections, nutritional deficiency, an enlarged spleen or even signs of liver failure and cancer.¹ They will use a variety of tests and exams to piece the puzzle together.

Complete blood count

One of the most critical tests is called a "complete blood count" or CBC for short. It's like a snapshot of what's happening in your blood. This test will measure the red blood cells, white blood cells and platelets.¹

Peripheral blood smear examination

This is a test in which a drop of blood is spread on a glass slide and examined under the microscope. The presence of any unhealthy-looking blood cells can give important clues to diagnose the cause of pancytopenia.¹

Additional blood tests

The blood tests may also include vitamin B12 and folate levels, liver function tests and tests to detect autoimmune diseases if needed.¹

Bone marrow aspiration and biopsy 

The doctor will also explore bone marrow aspiration and biopsy, which is basically like taking samples from your blood cell factory to see what's going on inside. It is almost always done as a workup in pancytopenia unless the cause is very apparent.

An infection workup will also be done if there is a history pointing towards infections that can cause pancytopenia. In addition to these, doctors may look at other tests and evaluations, and with your medical history, solve the puzzle that can lead to the right diagnosis.¹

Treatment options

Once you have the diagnosis, what's next? The first step is to address the root cause of pancytopenia. Tackling that issue is the top priority. So if it's because of a medication then that has to be discontinued. If it is due to nutritional deficiency then that has to be corrected.

Any infection, autoimmune condition or cancer if diagnosed should be treated accordingly. Medicines can be given that can stimulate the bone marrow to produce new blood cells. But treatment isn't just about fixing the issue. It's also about making you feel better day to day, and that's where supportive care measures come in.

Therefore, in case you have anaemia, a red blood transfusion will have to be given. A platelet transfusion will be given to prevent spontaneous bleeding if the platelet count is very low. If the white blood count or neutrophil count is very low antibiotic treatment will be given to prevent infections. In more serious cases, a bone marrow transplant could be on the cards, which is like a factory reset for your blood cell production.¹

Complications

Pancytopenia can lead to severe anaemia, spontaneous bleeding including bleeding inside the brain and infections all of which can be life-threatening if not promptly treated.¹

Prognosis

Your prognosis depends on the underlying cause. If the blood counts were low due to infection, then it could be reversed with treatment of infection. Likewise, if medications have caused pancytopenia then switching to alternate drugs will bring the blood counts back to normal. Pancytopenia due to cancer conditions depends on the stage and treatment of that cancer. So your doctor will be able to assess the prognosis based on the cause and test results.¹ 

When should I go to the ER?

Severe pancytopenia can be life-threatening if not treated promptly. Therefore, visit the ER or urgent medical care if you encounter the following symptoms:

• Fever 
• Breathlessness
• Lightheadedness
• Chest pain
• Severe headache
• Bleeding from gum or nose
• Blood in urine or stools
• Seizure

Summary

In a nutshell, pancytopenia is like a complex puzzle involving blood and bone marrow. It's a condition where you're running low on red blood cells, white blood cells, and platelets, and it can make you feel tired and unwell. But with the right diagnosis, treatment, and support, you can piece that puzzle back together. Remember, it's all about early detection and seeking medical help.