Capillary leak syndrome

Overview

Capillary leak syndrome occurs when plasma, the liquid component of blood, escapes from minuscule blood vessels called capillaries, seeping into the body’s cavities, muscles, and tissues. These episodes of leakage may occur sporadically, ranging from once a year to multiple times annually.

The two primary types of capillary leak syndrome are as follows:

• Systemic capillary leak syndrome: Recurring episodes are a feature of systemic capillary leak syndrome, also known as primary capillary leak syndrome or Clarkson’s illness, and they typically affect otherwise healthy individuals.
• Secondary capillary leak syndrome: Single episodes of secondary capillary leak syndrome are brought on by other illnesses, medications, or conditions.

A capillary leak syndrome incident occurs in three stages:

• Prodromal phase: This stage takes place a day or two before to the attack. Symptoms including thirst, fatigue, and sudden weight gain might begin to appear.
• Leak or resuscitation phase: This stage takes place throughout the attack. Your capillaries leak fluids and albumin, a protein present in blood plasma, into the gaps between your tissues. The blood flow slows down, preventing oxygen from reaching your tissues. Your red blood cell count increases and your blood pressure decreases. Hypovolemia is the outcome of the leakage, which causes your body’s blood volume to drop
• Post-leak phase or recruitment phase: This stage comes after the attack. Fluid overload is brought on by your capillaries’ reabsorption of bodily fluids and albumin. Your body may therefore develop pulmonary edema (fluid in the lungs) and polyuria (excessive urine production).

Without prompt treatment for capillary leak syndrome, there’s a risk of rapid decline in blood pressure, potentially leading to organ failure or fatality. While there’s no cure for the syndrome, seeking immediate medical care is crucial for symptom management. Consistent use of medication or infusions could help prevent subsequent episodes of systemic capillary leak syndrome.

Symptoms

 The signs and symptoms of prodromal phase may include the following:

• Abdominal pain
• Fatigue or weakness.
• Headache.
• Irritability.
• Muscle pain (myalgia).
• Nausea.
• Sudden body weight increase.
• Thirst.
• Viral or upper respiratory tract infection.

The signs and symptoms for leak or resuscitation phase may vary from person to person. The common indicators include the following:

• Diarrhea.
• Hypercoagulability, or thickened blood, brought on by an increased count of red blood cells (hematocrit) or white blood cells (leukocytosis).
• Low blood pressure or hypotension.
• Swelling of the body, legs, and arms (peripheral edema).

Causes

The following may cause capillary leak syndrome:

• Systemic capillary leak syndrome: It is unclear what causes systemic capillary leak syndrome. Researchers believe that the disorder may be brought on by the immune system’s reaction to an infection or sickness.
• Secondary capillary leak syndrome: Secondary capillary leak syndrome is most frequently caused by sepsis, a potentially fatal infection-related consequence. The following diseases can also result in capillary leak syndrome:
  • Autoimmune diseases: An immune system attack on your body’s healthy cells.
  • Differentiation syndrome: A side effect of medication used to treat acute myeloid leukemia or acute promyelocytic leukemia.
  • Engraftment syndrome: A potential side effect following a bone marrow transplant.
  • Hemophagocytic lymphohistiocytosis: A potentially fatal condition brought on by an excessive immunological reaction.
  • Ovarian hyperstimulation syndrome: A side effect of fertility medicine.
  • Ricin poisoning.
  • Snakebites.
  • Viral hemorrhagic fevers: A collection of virally-induced infectious illnesses.

Secondary capillary leak syndrome can also be brought on by some pharmaceuticals, such as the chemotherapy agents tagraxofusp (Elzonris®) and gemcitabine (Gemzar®).

Risk factors

The following may increase the risk for capillary leak syndrome:

• Systemic capillary leak syndrome: There are less than 500 cases of systemic capillary leak syndrome worldwide, making it an uncommon condition. Rarely occurring in children, systemic capillary leak syndrome primarily affects those in their middle years. The incidence may be greater since systemic capillary leak syndrome patients frequently receive the incorrect diagnosis.
• Secondary capillary leak syndrome: People of any age can develop secondary capillary leak syndrome. Capillary leak syndrome may result from specific illnesses, infections, or drugs.