Hello dear all, today I am sharing a video on idiopathic thrombocytopenic purpura. We call it idiopathic thrombocytopenic purpura but actually it is not idiopathic, it is an immune phenomenon, an autoimmune disorder which leads to the reduction in the number of the platelet counts. Now this is the commonest bleeding disorder which affects mainly the age group between 1 to 7 years of age. If it is lasting for less than 6 months, we call it acute. If it is lasting for more than 6 months, we call it chronic.
Maximum cases of ITPK usually resolve within 2-4 months. So, the maximum presentation is in acute form. Then what actually happens is, what is the pathophysiology is, there will be history of some febrile illness like viral infection or else.
That viral antigen enters into the body and leads to the formation of the antibody. What happens, that antibody which are formed against the viral antigen will cross react with the antigen of the platelet. The glycoprotein 2b3a complex will be attached to the antibody.
Once an antibody attaches to the platelet antigen, that will lead to the trapping of the platelet into the spleen. And ultimately, it will remove the platelet macrophage and it will destroy the platelet. And ultimately, what we will get is thrombocytopenia. So, when a healthy child comes to us who has an antecedent history of viral illness or else, and suddenly he comes with a patek or bruises. The first diagnosis we will think is of idiopathic thrombocytopenic purpura.
This is very common. And again, the main role we have got in this is idiopathic thrombocytopenia that is played by the T helper cells, T helper type 1 cells which produce cytokines and antibodies formation and ultimately for removal. Now if we talk of normal platelet count, then normal platelet count is 1.5 to 4 lakhs. Then what happens is that usually the presentation of ITP will be given. So, we will have a child who will present with bruises, patecki or mucosal bleeding in the form of epistaxis.
Or after trivial trauma, with prolonged bleeding. Usually, the bleeding manifest is less than 20,000 and in 20,000 to 50,000 you may get patecki and bruises. Now, we get seasonal clustering in ITP.
number of cases of ITP increases with the change in season. ITP is idiopathic thrombocytopenic purpura which is an immune disorder. But we have to differentiate it from other disorders. So mainly we have to differentiate it from Marrow failure.
Then we have to differentiate it from malignancies. Then we have to differentiate it from hypersplenism. When we will do lab investigation on CBC, then all other hematological parameters will be normal. Only thing you will get is reduced platelet count. Then peripheral smear will also be normal.
There will be no malignant cells or something like that. Then if we look at bone marrow, we will get increased megakaryocytes. Because production is not hampered, which is against marrow failure.
In marrow failure, you will get reduced megakaryocytes. But in this, because macrophage is getting destroyed in the spleen, usually what you will get is increased production in the marrow. Then if we have a sick child, usually children of ITP also don't get...
a toxic look or sick look usually they are healthy child just with the patic and bruises but if a sick child is coming then we need to think of malignancy we need to think of sepsis we need to think of malaria dengue dic and other disorders which can lead to thrombocytopenia now how to treat if we are getting an igp child what we will do is if he has some patic or bruises usually If the platelet count is more than 20000 and there is no bleeding per se, then we will just observe the child. Now suppose if the child has bleeding, then we will go for IVIG i.e. intravenous immunoglobulin which we give 1g per kg per day for 1 or 2 days. Then if the child is Rh positive, we can also go for anti-D immunoglobulin which we give from 50 to 75 mg per kg. Then we go for steroids. In steroids, initially we give dexa and followed by prednisolone.
And if we have a child who is suffering from serious hemorrhage, then we have to replenish the platelet immediately. So, we will give platelet transfusion and also give steroids. Then if someone is suffering from chronic ITP, that is more than 6 months have passed and ITP is persisting, then the therapies we have is Lodoss, steroids, we can go for spleenectomy because spleen is the place where it is being destroyed.
Then others are, we can use Denazol, Winchristine, Cyclosporine, Azathioprine and Rituximab.