An abnormal proliferation of autoreactive T cells leading to the breakdown of immune tolerance to platelet antigens is suggested to be responsible for the up-regulated proliferation of autoantibody producing B cells.

What causes the abnormal proliferation of autoreactive T cells is unknown.

The disease sometimes follows a viral infection, use of certain drugs, during pregnancy, or as part of an immune disorder.


The pathogenesis of ITP is incompletely understood.


An abnormal proliferation of autoreactive cytoxic T cells leading to the breakdown of immune tolerance to platelet antigens is suggested to be responsible for the up-regulated proliferation of autoantibody producing B cells.

The principal mechanism is thought to involve specific autoantibodies produced by the patient's B cells (typically, IgG), most often directed against platelet membrane glycoproteins such as GPIIb/IIIa.1


An additional contribution to the pathophysiology of ITP is given by alterations of thrombopoiesis caused by platelet-reactive autoantibodies or cytotoxic T cells leading to impaired megakaryocyte differentiation and platelet production.

Also humoral and cellular autoimmunity directed at megakaryocytes, causes impaired platelet production.

Consequently, the immune response induces enhanced T cell-mediated cytotoxicity and antibody-mediated platelet destruction through phagocytosis, complement activation and apoptosis.


All these processes involved in ITP pathophysiology account for the complexity and heterogeneity in the clinical manifestation and therapy responsiveness of this disorder.

Reduced platelet lifespan due to antibody-mediated destruction is the predominant hypothesis.

Low platelets leads to bleeding


ITP affects women more often than men.

It is more common in children than adults.

In children, the disease affects boys and girls equally.


The goal of immune thrombocytopenia (ITP) treatment is to provide a safe platelet count in order to prevent clinically important bleeding, rather than to normalize the platelet count.

Bleeding risk is greatest in individuals with platelet counts less than 10,000/microL.


1. Corticosteroids.


IV Immunoglobulin.

5. Anti-Rho(D)

Anti-Rho(D), or Anti-D (WinRho®) is an intravenous (IV) drug infusion used to elevate platelet counts temporarily, and can be repeated over a period of time for extended relief.

Often used as a “first line” therapy, the shorter infusion time and often lower cost of Anti-D can offer an advantage over intravenous immunoglobulin (IVIG) for some patients. 

Anti-D is a blood product consisting of antibodies to the RH factor on red blood cells. It has been shown to achieve a temporary rise in the platelet count in about 80 percent of people and occasionally has a longer-term effect.1 

Anti-D products were first licensed in 1995 for the treatment of ITP, and are used in both children and adults.

Anti-D is made from human plasma derived from a limited list of donors in a special program that stimulates the production of high levels of antibodies. The antibodies from the donors are combined in batches that undergo a viral inactivation and micro filtration process using solvent/detergent to remove or deactivate disease-causing agents that can be transmitted through blood infusions.2

Following a treatment with Anti-D, the patient's RH-positive red blood cells link to the anti-D antibodies; the anti-body coated red blood cells are then removed in the spleen. Since red blood cells are eliminated, the process often causes mild anemia. However, it is usually successful in keeping the antibody-coated platelets of the ITP patient in circulation.

For the product to be safe and effective, the patient must be RH positive, have a spleen and not be anemic or deficient in immunoglobulin antibody (IgA), an antibody that plays a crucial role in the immune function of mucous membranes. Before administering anti-D, the ITP Consensus Report recommends doctors determine the patient’s blood group, complete a direct antiglobulin test (DAT) to check for antibodies against red blood cells that may already be present and perform a reticulocyte count to test the rate of red cell production.6


According to the package insert, the suggested dosing is 50 mcg/kg of body weight although some clinical studies report greater success at a 75 mcg/kg dose.3 Because the antibodies are concentrated, a treatment with anti-D requires less product and a shorter infusion time than IVIg. Premedication with paracetamol/acetaminophen, or corticosteroids is advised to reduce the risk of fever and chills.6

Side Effects

Side effects developed following seven percent of infusions and included headaches, chills, fever and body aches. A remote risk of anaphylaxis (shock response) exists for patients with hypersensitivity to blood products.4,5 

A very small number of people receiving anti-D experience intravascular hemolysis, the destruction of red blood cells in circulation. This type of red cell destruction can cause anemia, multi-system organ failure, difficulty breathing, and even death. In December 2009, the FDA revised the WinRho SDF package insert to highlight these warnings and suggest additional tests and patient monitoring to better able to identify and treat those patients at risk.4

Patients should immediately report symptoms of back pain, shaking chills, fever, discolored urine, decreased urine output, sudden weight gain, fluid retention/edema and/or shortness of breath to their physicians.4,5

Anti-D can interfere with the efficacy of live virus vaccines, therefore the manufacturers do not recommend live virus immunizations within three months after an anti-D treatment.4,5 

Maltose (a sugar produced by the breakdown of starch) in IVIG products, such as the liquid formulation of WinRho SDF, has been shown to give false high blood glucose levels in certain types of blood glucose testing systems. Only systems that are glucose-specific should be used to test or monitor blood glucose levels in patients receiving this product.4,5 

WinRho SDF was voluntarily withdrawn from the European market in August 2009 due to safety concerns.7

Managing Side Effects

As with any treatment, Anti-Rho (D) or Anti-D intravenous (IV) infusion has side effects. The key to getting the best results while minimizing adverse effects is working closely with your healthcare team. Here you will find a downloadable, printable document of helpful tips in decreasing the serious side effects of Anti-Rho (D) or Anti-D IV infusion. Discuss this information with your healthcare team prior to treatment.



  1. Cooper N et al. “Does treatment with intermittent infusions of intravenous anti-D allow a proportion of adults with recently diagnosed immune thrombocytopenic purpura to avoid splenectomy?” Blood. 2002 Mar 15;99(6):1922-7.
  2. Gaines AR. “Acute onset hemoglobinemia and/or hemoglobinuria and sequelae following Rho(D) immune globulin intravenous administration in immune thrombocytopenic purpura patients.” Blood. 2005 Sep 1;106(5):1532-7.
  3. Newman GC et al. “A dose of 75 microg/kg/d of i.v. anti-D increases the platelet count more rapidly and for a longer period of time than 50 microg/kg/d in adults with immune thrombocytopenic purpura.” Br J Haematol. 2001 Mar;112(4):1076-8.
  4. WinRho SDF Package Insert
  5. Rhophylac Package Insert
  6. Provan D et al. “International consensus report on the investigation and management of primary immune thrombocytopenia.” Blood January 14, 2010 vol. 115 no. 2 168-186.
  7. Federal Agency for Medicines and Health Products, "WinRho SDF 1500 IU / 5000 IU: withdrawal from the market"



You have diagnosed a 10-year-old child with immune thromobocytopenic purpura (ITP), and his platelet count is currently 10,000/μL. You are considering therapy to improve this patient's platelet count. All of the following are treatments for ITP EXCEPT:

The correct answer is A.

Since ITP is an immune-mediated process in which platelets are destroyed, transfused platelets are simply destroyed as well. However, steroids, immunoglobulin therapy, and splenectomy, and Rho(D) immunoglobulin (if he is Rh positive) all have a role in treating ITP.