Kasabach-Merritt Syndrome | Symptoms and Treatments
Kasabach-Merritt Syndrome is a rare condition affecting the blood vessels in children, and if it goes untreated, it can be fatal. In this article, we will go over some of the symptoms and treatment options.
What is Kasabach-Merritt syndrome?
Kasabach-Merritt syndrome is a rare blood disorder in which excessive bleeding can occur in the blood vessels due to low levels of platelets. This bleeding occurs in the large blood vessels and often results in the formation of a clot. The clots intercept the outpour of blood to vital body areas, most of the lungs and the brain. Bleeding into the skin may also cause petechiae or red pinprick spots.
The platelet count is usually average; hence, the syndrome’s name means “without platelets.” People with Bernard-Soulier syndrome have moderate numbers of all blood cells except platelets. Their bleeding time is much longer than average, and they bruise. They have a higher risk of bleeding in various situations, such as surgery, trauma, tooth extraction. Or even during routine dental work such as having their teeth cleaned by a dentist.
Causes of Kasabach-Merritt syndrome
Kasabach-Merritt syndrome is a form of vasculitis that can inflammation the blood vessels and skin. Symptoms include erythema (redness), bruising, necrosis of the skin, and weight loss. The causes of Kasabach-Merritt syndrome are not currently well understood. It is known that a significant component of the syndrome involves genetic makeup.
Most people with Kasabach-Merritt syndrome have a familial tendency toward thrombocytopenia. Patients with the syndrome may have either a mild or severe form. A mild form has unexplained aches and pains, recurrent infections, fatigue, and sleep problems. An extreme condition is more powerful. It causes personality, behavior, movement, cognition, and sensation changes.
The syndrome may run in families and usually affects both men and women, although the inheritance pattern is unclear. In the past, the most common causes of this syndrome were endocrine disorders (such as Cushing’s disease or pituitary tumors), trauma, or psychological factors. More than half of all patients with Conn’s syndrome have no underlying cause identified at all in recent years.
What are the symptoms of Kasabach-Merritt syndrome?
Kasabach-Merritt syndrome is a rare disorder of blood vessels in the arms and legs that causes blood to collect and form clots. The symptoms of Kasabach-Merritt syndrome include large, painful, and swollen arms and legs, pain in the arms and legs, fever, chills, and chest pain. These symptoms begin, with the swelling and pain being more prominent in the extremities. The cause of Kasabach-Merritt syndrome is unknown.
There are a few hypotheses on how it develops. It may result from blood clots that block blood flow in small vessels. It could also be caused by changes in the lining of blood vessels, or it could be an immune response to abnormal deposits in small blood vessels. “It’s an understood condition, so there’s no test to diagnose it,” Dr. Coronado said.
Symptoms contain tingling in the hands and feet that can worsen at night— pain in the back, weakness, cramping in the legs, fatigue, headaches, and joint pain. The first signs may occur while sleeping and worsen as morning comes.
Kasabach-Merritt syndrome is a rare form of small vessel vasculitis in which blood leaks from the capillaries. This leakage can lead to hypovolemia and anemia. Symptoms include fever, fatigue, weight loss, erythema, and possible changes in hair color. Transient erythema may be the only symptom. A drop in hemoglobin levels may cause pallor, fatigue, palpitations, dizziness, or syncope.
Diagnosis of Kasabach-Merritt syndrome
Kasabach-Merritt syndrome is a rare cause of lower extremity edema. Characteristics include severe pain, erythema, tenderness, and severe localized swelling in the distal leg. Though the syndrome usually resolves within weeks. In some cases, treatment with steroids or an anticoagulant may be necessary. Kasabach-Merritt syndrome is a rare cause of lower extremity edema.
Kasabach-Merritt syndrome is named after U.S. hematologists Leon E. Kasabach and Ralph R. Merritt, who described it in 1965. They reported the case of a 23-year-old man who was thought to have an upper respiratory infection but had more than 40 blood clots in various organs.
When this disorder is suspected, anticoagulants (blood thinners) are started immediately, often before other tests are performed. The patient is observed in a hospital for at least four weeks after the initial episode.
Treatment of Kasabach-Merritt syndrome
Kasabach-Merritt syndrome is a low condition that requires treatment in a hospital. It is believed to be compelled by an allergic reaction.
If the affected blood vessels are not treated, they can narrow further and become blocked. This drives the red blood cells to stick together, clogging the small blood vessels and starving tissues of oxygen. As it can take a year for the disease to cause symptoms, most people already have some degree of damage when they are diagnosed.
And because it can take a year for the disease to cause symptoms, patients will already have some degree of damage before they are diagnosed.
Kasabach-Merritt syndrome is a rare type of thrombocytopenia that causes red blood cells to leak from the blood vessels.
Risk factors of Kasabach-Merritt syndrome
Kasabach-Merritt syndrome is characterized by bilateral lower extremity venous thrombosis with venous obstruction, diffuse cutaneous hemorrhages (purpura), and upper extremity edema. The diagnosis of thrombotic thrombocytopenic purpura is based on the history, physical examination, and laboratory findings. Complete blood count (CBC) with peripheral smear is done to determine the platelet count and red blood cell (RBC) count, hemoglobin level, and white blood cell (WBC) count.
Because platelets are essential for blood clotting, a low platelet count implies very few platelets in the blood. The RBC count will help assess for anemia. WBC count is elevated in disseminated intravascular coagulation. Prolonged PT and PTT are seen in liver disease. Fibrinogen levels may be reduced in liver disease. Reticulocyte count is elevated in hemolytic anemia. Serum bilirubin may be elevated or normal in liver disease, hemolytic anemia, and renal failure.