About harley syndrome

What is harley syndrome?

Paroxysmal cold hemoglobinuria (PCH) is a rare type of anemia characterized by the premature destruction of healthy red blood cells by autoantibodies. The disorder is classified as an autoimmune hemolytic anemia (AIHA), an uncommon group of disorders in which the immune system mistakenly attacks healthy red blood cells. Autoimmune diseases occur when the body's natural defenses against foreign organisms destroy healthy tissue for unknown reasons. Normally, red blood cells have a life span of approximately 120 days before they get removed by the spleen. In individuals with PCH, red blood cells are destroyed prematurely and sometimes suddenly (paroxysmally). Many reports emphasize that PCH is an unusual disease. However, in recent years, PCH has become recognized as one of the most common causes of acute AIHA in young children. The reason why acute transient PCH appears to be a more common type of childhood AIHA than it was thought to be several decades ago is uncertain, but probably relates to greater awareness of the disorder and more frequent use of the Donath-Landsteiner test (see Diagnosis section ), especially in children with acute AIHA with hemoglobinuria.

PCH was first described as a distinct disorder in the medical literature in 1872. The specific antibody associated with the disorder (Donath-Landsteiner autoantibody) was first described by Drs. Donath and Landsteiner in 1904.

A majority of cases of PCH recorded in the early medical literature were associated with late syphilis or congenital syphilis. In the early 1900s over 90 percent of patients with chronic PCH had a positive test for syphilis and approximately 30 percent showed clinical evidence of the disease. With the effective treatment of syphilis and the virtual elimination of the congenital form, "classical" syphilitic PCH is now an extremely rare disorder, as is chronic PCH. It was in patients with the chronic form of PCH that exposure to cold resulted in a paroxysm of hemoglobinuria.

In modern times, PCH is almost always encountered as an acute transient syndrome in young children with a recent history of a viral illness, so that paroxysms resulting from cold exposure are rarely encountered. Thus, although this type of AIHA is known as PCH, the words paroxysmal and cold are generally not relevant to the disorder as it is manifest in the modern era. As children with PCH do not usually have hemolysis directly related to exposure to the cold (e.g., they continue hemolysis when in a warm hospital environment), it has been suggested that a better term might be Donath-Landsteiner test positive hemolytic anemia.

What are the symptoms for harley syndrome?

The symptoms associated with Harlequin syndrome may be more likely to occur when a person has been exercising, is very warm, or is in an intense emotional situation.[13659] In these situations, one side of the body sweats and flushes appropriately as a response to the situation, whereas the other side of the body does not. The asymmetrical facial Sweating and flushing associated with this condition has been named the “Harlequin sign.” Harlequin syndrome is thought to be one of a spectrum of diseases that can cause Harlequin sign.

What are the causes for harley syndrome?

The exact cause of Harlequin syndrome is not completely understood. In some patients with this syndrome, the underlying cause seems to be a lesion or tumor that is affecting the ability of the cells of the autonomic nervous system to communicate with one side of the body. However, in many cases an exact cause of the symptoms is not found

What are the treatments for harley syndrome?

Treatment may consist of removing any lesion that may be causing the symptoms of the syndrome. If no lesion is present and the syndrome is not interfering with a person’s daily living, treatment may not be necessary.

What are the risk factors for harley syndrome?

Harley syndrome or Harlequin syndrome is a rare dysautonomic syndrome caused by the unilateral blockade of the T2-T3 fibers carrying sudomotor and vasomotor supply to the face.

  • The autonomic nervous system is responsible for regulating the natural processes of the body, including sweating, skin flushing, and the response of the pupils to any stimuli. The exact cause of the condition is not found yet.
  • The symptoms associated with the syndrome are more likely to occur when the affected individual has been exercising, has a warm body temperature, or is in an intensely emotional situation.
  • Diagnosis is based on observing such consistent symptoms, followed by a series of examinations, to detect the other diseases linked with Harlequin disorder.
  • There is no genetic connection to the disorder; however, it is mostly diagnosed in women rather than men.
  • Moreover, it is also a common representation among healthy newborns, which usually occurs from day 2 to day 5 of birth and rarely occurs after three weeks.
  • In the past years, the condition has been thought to be common among premature neonates, but researchers have found a similar incidence among healthy term neonates as well.


Symptoms
Sudden unilateral skin flushing and sweating,Breathing difficulties,Cluster headaches,Forehead sweating,Nasal discharge,Pupillary constriction,Drooping of the upper eyelid
Conditions
Hypothalamic functional immaturity,Vasomotor instability,Diabetic neuropathy,Hyperthyroidism,Multiple sclerosis,Motor deficit
Drugs
Botulinum toxin injection

Is there a cure/medications for harley syndrome?

Harley Syndrome is a rare syndrome that affects the autonomic nervous syndrome. In the condition, one side of the body sweats and flushes as a response to the effect. The asymmetrical facial sweating and flushing accompanied by the condition are the primary signs.

  • There is no cure for the syndrome, and despite its benign nature, it can cause significant anxiety among the patient, caretaker, and healthcare professional.
  • It is a self-limiting condition that will resolve automatically within minutes to hours once the trigger subsides.
  • Even though treatment is not necessary considering the auto-resolve nature of the condition.
  • Harley's face resulting from compressive or retraction injury at the time of neck surgeries will last for hours to days before complete resolution.
  • However, this might lead to permanent facial discoloration, resulting in social embarrassment.
  • Responsive patients might benefit from repetitive stellate ganglion blockage or botulinum toxin injection.
  • In refractory cases, the surgical sympathectomy on the contralateral side will be the optimal treatment option.
  • Familiarity with the syndrome will allow the healthcare experts to identify, diagnose and manage the syndrome, to ensure patient satisfaction and avoid potentially further complications.


Symptoms
Sudden unilateral skin flushing and sweating,Breathing difficulties,Cluster headaches,Forehead sweating,Nasal discharge,Pupillary constriction,Drooping of the upper eyelid
Conditions
Hypothalamic functional immaturity,Vasomotor instability,Diabetic neuropathy,Hyperthyroidism,Multiple sclerosis,Motor deficit
Drugs
Botulinum toxin injection

Video related to harley syndrome