Poikilocytosis: Symptoms, Causes, Types, Diagnosis and Treatment

Poikilocytosis is the term used for unusual shaped red blood cells (RBCs) in the blood. Ordinary RBCs (also called erythrocytes) are typically disk-shaped, which are thinner in the Center than in the edges, with a diameter of 6.2 to 8.2 Micrometers, a thickness at the thickest point of 2 2.5 Micrometers, and a thickness in the Center of 0.8 to 1 Micrometers.

Poikilocytosis generally refers to an expansion in abnormal-shaped red blood cells that make up to 10% or more of the total red blood cells. Poikilocytes may be flat, elongated, teardrop, crescent-shaped, or they may have point-like or thorn-like projections, or may have any other unusual feature.

Usually, a person’s RBCs (also called erythrocytes) are disk-shaped with a flattened Center on the two sides. Poikilocytes may:

• Be flatter than normal.
• Be prolonged, crescent-shaped, or teardrop-shaped
• Have sharp projections
• Have other abnormal features

1. Poikilocytosis Symptoms

One of the primary poikilocytosis symptoms is having a significant amount (more than 10%) of abnormal-shaped red blood cells. In general, the indications depend on the fundamental condition. Poikilocytosis can likewise be considered a symptom of numerous different disorders.

Common symptoms of other blood-related issues, such as anemia, include:

• Fatigue
• Pale skin
• Weakness
• Short of breath

2. What Causes Poikilocytosis?

Poikilocytosis usually is the result of another condition. The conditions of Poikilocytosis can be inherited or acquired. A genetic mutation causes hereditary diseases. The acquired conditions grow later in life.

Hereditary Reasons for Poikilocytosis Include:

• Sickle-cell Anemia, a genetic infection characterized by red blood cells with an abnormal crescent shape.
• Thalassemia, a genetic disorder of the blood where the body produces irregular hemoglobin.
  Pyruvate kinase deficiency
• McLeod syndrome, an uncommon genetic issue that affects the nerves, the heart, the blood, and the brain.
• Symptoms usually appear slowly and begin in mid-adulthood.
• Hereditary elliptocytosis
• Hereditary spherocytosis

Acquired Causes of Poikilocytosis Include:

• Iron-deficiency Anemia, the complete form of Anemia that happens when the body needs more iron.
• Megaloblastic Anemia, caused by a deficiency of folate or vitamin B-12
• Autoimmune hemolytic Anemias, a group of problems that happen when the immune system erroneously destroys red blood cells
• Liver and kidney disease
• Alcoholism or liver infection related to alcohol
• Lead poisoning
• Chemotherapy treatment and Cancer
• Serious infections
• Myelofibrosis

3. Types of Poikilocytosis

Poikilocytosis can be classified into various types depending on the shape that the cells assume. There is ultimately a lot of diversity in the cell shapes, and as such, Poikilocytosis can be grade into the following types:

a. Stomatocytes

They are oval-shaped and generally resemble wrinkled lips because of their three-dimensional structure covered with an end.

Reason for the condition: Hereditary stomatocytosis, alcoholism, Rh null phenotype, and hepatic problems as well as liver disease.

They usually happen in marine creatures and woodchucks. But, in the case of dogs, they lead to dwarfism, particularly in the Alaskan Malamutes species.

b. Drepanocytes

They are too known as sickle cells as they are looking like a crescent with spikes.

Reason for the condition: Sickle-cell Anemia

Mostly found in sheep and deer, yet could be present in humans because of hereditary Hemoglobin defect. The malformed ‘S’ shaped Hemoglobin could be because of the combination of hydrophobic valine and hydrophilic glutamic acid in the blood.

c. Ovalocytes

These RBCs are somewhat oval. There are three kinds of Elliptocytes, and all three hang on the length of the unusual cell.

Reason for Condition: Anemia, hereditary elliptocytosis, and thalassemia Generally found in dogs and cats.

d. Spherocytes

These are solid, rounded little cells.

Reason for Condition: Severe burns, transfused cells, genetic spherocytosis, newborn jaundice, immune-mediated hemolytic Anemia, and hereditary spherocytosis.

Generally found in horses, cats, and cattle. However, in the uncommon case of dogs, the middle portion is mostly pale.

e. Dacryocytes

These cells have a swell head with a sharp end, practically like a teardrop.

Reason for the condition: Beta thalassemia major, myelophthisic Anemia, myelofibrosis with myeloid metaplasia, extramedullary hematopoiesis, and Leukemia.

f. Leptocytes

These are flat, narrow cells with Hemoglobin present at the edge of the cell. Reason for the condition: Most commonly occur in patients with thalassemia.

Source: Wikipedia

g. Codocytes

They resemble a bull’s eye and hence, mention as target cells.

Reason for the condition: Hemoglobin C disorder, Cholestatic liver illness, and thalassemia are the primary reasons for this type of Poikilocytosis.

h. Echinocytes

They are also referred to as Burr cells. Their external casing has blunt, evenly spaced protrusions on the edges.

Reason for the condition: Microangiopathic hemolytic Anemia, severe stomach infections like Cancer or ulceration, uremia, neonates, pyruvate kinase deficiency, and kidney diseases.

Generally happens in horses and kidney of dogs. Coral snake, rattlesnake, and viper venom consist of Echinocytes. Subsequently, snake poison can be a primary cause for the growing of burr cells. Echinocytes can form and duplicate in the plasma instantly within 24 hours of a snake bite.

i. Schizocytes

They are disconnected or split RBCs having no specific shape.

Reason for the condition: Disorders in platelets such as coagulation problem, purpura, microangiopathic hemolytic thrombocytosis, severe burns, hemolytic uremic syndrome, and Anemia. In this case, the smooth endothelial edge becomes rough, causing turbulence in the blood flow. They are additionally responsible for the fragmentation of red blood cells.

Usually, it happens in cats and dogs though relatively rare in cats.

j. Acanthocyte

Also known as spur cells for their thorny projections and an unpredictable arrangement. They generally have a short lifespan because of their stiff

Reason for the condition: Anemia, extreme liver disorder, microangiopathic hemolytic, vitamin E deficiency and abetalipoproteinemia, neuroacanthocytosis.

They are generally observed in horses, cats, cattle, and dogs.

k. Keratocytes

These cells are shaped like a helmet.

Reason for the condition: Could occur due to blisters. The presence of keratocytes can indicate oxidant injury, fragmentation injury, or liver disease.

Generally found in dogs and cats, sometimes even in healthy cats.

l. Degmacytes

They have a nibbled appearance and hence, the name Bite Cells.

Reason for the condition: Liver cirrhosis G6PD, hemolytic Anemia, and poisoning.

m. Phantom cells/Semilunar bodies/Ghost cells

The cells are in the shape of a crescent and discolored due to a lack of Hemoglobin.

4. Diagnosis of Poikilocytosis

The following tests are undertaken to analyze Poikilocytosis –

• The primary test is a blood film test to distinguish abnormal RBCs and also help identify the class of Poikilocytosis. Blood from the individual is separated and examined under a high-resolution microscope.
• A blood count test is led to determine the degree of difference in the number of abnormal RBCs in your blood.
• A Hemoglobin test and blood composition test are additionally done to confirm the Hemoglobin count and amount of enzymes in the bloodstream, respectively.
• Other tests, including bone marrow testing, are also carried out.

Poikilocytosis vs. Anisocytosis

The significant difference between Poikilocytosis and Anisocytosis is that RBCs in the former are abnormal in shape while the latter are of varying size.

5. How is Poikilocytosis Treated?

The treatment for Poikilocytosis depends on what’s causing the condition. For example, Poikilocytosis brought by low levels of vitamin B-12, folate, or iron will likely be treated by taking supplements and increasing the amount of these vitamins in your diet. Or, doctors may treat the primary disease (like celiac disease) that may have caused the deficiency in the first place.

People with inherited forms of Anemia, similar to sickle cell Anemia or thalassemia, may require blood transfusions or a bone marrow transplant to treat their condition. People with liver illness may require a transplant, while those with severe infections may require antibiotics.