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Idiopathic Thrombocytopenic Purpura


OVERVIEW OF Idiopathic Thrombocytopenic Purpura :

Idiopathic thrombocytopenic purpura is an immune disorder in which the blood doesn’t clot normally. This condition is now more commonly referred to as immune thrombocytopenia ( ITP) .

It can cause excessive bruising and bleeding. An usually low level of platelets or thrombocytes in the blood results in ITP. Platelets are produced in the bone marrow, they have stop bleeding by clumping together to form a clot that seals cuts or small tears in blood vessel walls and tissues. If the blood doesn’t have enough platelets, it’s slow to clot.

Internal bleeding or bleeding on or under the skin can result. People with Idiopathic thrombocytopenic purpura often have many purple bruises called purpura on the skin or mucous membrane inside the mouth. These bruises may also appear as pinpoint sized red or purple dots on the skin called as petechiae. It often look loke a rash.

Idiopathic thrombocytopenic purpura can occur in both children and adults. There appears to be differences at certain ages between women and men and the development of ITP. At younger ages, ITP may be more common in women. At older ages, it may be more common in men. Children are most likely to develop this condition after a general viral illness. Some specific viruses, such as chickenpox, mumps, and measles, have been linked to ITP as well.


CAUSES :

There are two types of Idiopathic Thrombocytopenic Purpura. They includes acute which is of shorter duration and the chronic which includes longer duration.

Acute Idiopathic thrombocytopenic purpura is common form of disorder in children. It usually occurs less than six months and Chronic Idiopathic thrombocytopenic purpura lasts more .

Chronic idiopathic lasts six months or longer. It is most commonly seen in adults, although teenagers and younger children can also be affected.

Platelet production defects are an uncommon cause of thrombocytopenia in the dog and when present are typically present in association with other cytopenias. Potential causes of marrow production defects in the dog include infectious agents (canine parvovirus, distemper virus, ehrlichia), vaccination (typically a mild transient response arising 3-5 days after vaccination and not associated with any bleeding tendencies), drug therapy (chemotherapeutics, immunosuppressants,estrogens, sulfa antibiotics), irradiation, toxins, dysthrombopoiesis associated with myelodysplasia, and immune-mediated destruction of megakaryocytes.


PATHOPHYSIOLOGY :

Chronic immune thrombocytopenic purpura (ITP) is an autoimmune disorder in which the patient’s immune system reacts with a platelet autoantigen(s) resulting in thrombocytopenia due to immune-mediated platelet destruction and/or suppression of platelet production. Platelet membrane proteins, for reasons that are unclear, become antigenic and stimulate the immune system to produce autoantibodies and cytotoxic T cells. The initial antigenic response probably occurs in the spleen followed by stimulation of other antibody-producing tissues, particularly the bone marrow. Autoantibodies against platelet glycoprotein(GP) IIb-IIIa and/or GPIb-IX are produced by the majority of ITP patients and can be detected using antigen specific assays. Many patients produce multiple antibodies; this has been attributed to the phenomenon of epitope spreading. Once produced, autoantibody may either bind to platelets, causing their destruction by either phagocytosis or possibly complement activation and lysis, or bind to megakaryocytes, resulting in decreased thrombopoiesis. Evidence for platelet destruction in ITP includes the following: (1) infusion of ITP blood or plasma into normal recipients may result in thrombocytopenia; (2) there is decreased intravascular survival of radiolabeled platelets in most ITP patients; (3) morphologic and in vitro evidence of platelet phagocytosis can be demonstrated; and (4) cytotoxic T cells can induce lysis of autologous platelets. Evidence for suppressed platelet production in ITP includes the following: (1) morphologic studies show megakaryocyte damage in most ITP patients; (2) there is normal or decreased platelet turnover in the majority of patients; (3) in vitro studies show antibody-induced inhibition of megakaryocyte production and maturation; and (4) an increase in the platelet count occurs in many ITP patients receiving treatment with thrombopeitin mimetics. In summary, activation of the immune system by platelet autoantigens in ITP may result in platelet destruction and/or inhibition of platelet production. The importance of each mechanism in the individual patient probably varies.


COMMON CLINICAL SIGNS AND SYMPTOMS :

The most common symptoms of ITP are:

  • bruising easily
  • pinpoint-sized petechiae, often on the lower legs
  • spontaneous nosebleeds
  • bleeding from the gums (for example, during dental work)
  • blood in the urine
  • blood in the stool
  • abnormally heavy menstruation
  • prolonged bleeding from cuts

profuse bleeding during surgery


DIAGNOSTIC :

  • A complete blood count (CBC):

In which they measures the size, number, and maturity of different blood cells in a specific volume of blood (to measure platelets).

  • Additional blood and urine tests:

The  measurement of  bleeding time and detect possible infections

  • Careful review of the child's medications:

A bone marrow aspiration may be performed to look at your child's production of platelets and to rule out any abnormal cells your child's bone marrow could be producing that could lower platelet counts.


TREATMENT AND PROGNOSIS :

 Treatment:

When the treatment is necessary , the two most common forms of treatment are:

Steroids:

It helps to prevent bleeding by decreasing the rate of platelet destruction. Steroids if  present it is effective it might result in the increase in the platelet counts which is seen within two to three weeks. Side effects may include irritability, stomach irritation , weight gain and hypertension and Acne.

Intravenous gamma globulin (IVGG):

 A protein that contains many antibodies and also shows the destruction of platelets . OVGG might work more quickly than steroids. It works within 24-48 hours.

Other treatments include:

Rh immune globulin:

This medication temporarily stops the spleen from destroying the platelets.

Medication changes:

If any medications tends to cause any alteration in the thrombocytopenic purpur then discontinuation or changing the medication may be necessary.

Infection treatment:

If infection is the cause of this, then the treatment to this infection might result in the higher platelet counts.

Splenectomy:

The child’s spleen may need to be removed since this is the site of the platelet’s destruction. This is considered more often in children with chronic idiopathic thrombocytopenic purpura to decrease the rate of platelet destruction.

Hormone therapy:

The girls might take hormones to stop their menstrual cycle when their platelets are low.

 


PREVENTION :

Avoid heavy drinking. Alcohol slows the production of the platelets.

Try to avoid contact with the toxic chemicals. Chemicals such as pesticides, arsenic, benzene slow the production of the platelets.

Maintain the healthy lifestyle and seek the medical help any complications arises.


REFERENCE :

https://www.healthline.com/health/idiopathic-thrombocytopenic-purpura-itp#:~:text=Idiopathic%20thrombocytopenic%20purpura%20is%20an,the%20blood%20results%20in%20ITP.

https://www.hopkinsmedicine.org/health/conditions-and-diseases/idiopathic-thrombocytopenic-purpura

https://www.mayoclinic.org/diseases-conditions/idiopathic-thrombocytopenic-purpura/symptoms-causes/syc-20352325

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4147825/

https://www.webmd.com/a-to-z-guides/thrombocytopenia-symptoms-causes-treatments#1