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What is the treatment for Idiopathic Thrombocytopaenic Purpura (ITP)?

Q: One of my friend's mother has been diagnosed with a condition called ITP (Idiopathic Thrombocytopaenic Purpura). To start with, she developed purple patches in the hand (Day 1), leg & neck area, which was followed by fever, and then red spots started appearing all over the body. This was followed by numbness (off and on) in the hand and stuttering (off & on). At the same time, she had severe headache all along. When the numbness surfaced (Day 6), she was taken to a neuro-physician who ordered a MRI, which indicated bleeding in the brain. When the blood tests were done, the platelet count was 1000 (just one thousand)! The platelet count taken when the purple patches started appearing was perfectly normal (125,000). She was admitted to hospital and was administered platelets, but that did not help much. Then the doctors concluded (Day 8) suspected that it is a case of ITP and the bone-marrow was done. This revealed that there is no problem there. At this point (Day 9), the haematologist concluded that it is a case of ITP and put her on Dexamethasone & Methylprednisolone for three days. This also did not improve the count much. Then Immunoglobin was administered for 2 days (Day 11 & 12). This is when she started responding well. The platelet counts on Day 11, 12 & 13 were in the range of 150,000. They stopped the Immunoglobin but continued the steroids. On Day 16 she was discharged but the steroids were continued (Wysolone 50 mg) for another 5 days (Days 17-21). When the blood tests were conducted on Day 22, the platelet count had dropped to 10,000 again. The haematologist who is advising her asked her to get admitted again and now she is on Immunoglobin again. He says that she may have to undergo splenectomy (removal of spleen). I would like to know, if the diagnosis has been correct so far? Are the lines of treatments that are being tried out correct under the circumstances? Can anybody give suggestions as to what are the best means of treatment in such a case?

A:Platelets (thrombocytes) are a type of blood cells, which are formed in the bone marrow from large cells called megakaryocytes. They are critical for the clotting of blood and have lifespan of about 10 days. The normal platelet count ranges from 150,000 - 400,000/ml and a reduction in platelets is referred to as thrombocytopaenia. This may be mild (100,000 - 150,000/ml), moderate (50,000 - 100,000/ml) or severe (less than 50,000/ml). Platelets may be low due to: a)Increased destruction (immunologic infections, drugs; gestational thrombocytopaenia) or utilisation (disseminated intra-vascular coagulation; abnormal vasculature as seen in haemolytic uraemic syndrome & thrombotic thrombocytopaenic purpura), b)decreased production (leukaemia, aplastic anaemia, megaloblastic anaemia, drugs, infections or Sequestration in the spleen (cirrhosis of liver). Even though there is a diverse list of potential underlying causes of thrombocytopaenia, most cases fall within two major categories: impaired platelet production or accelerated platelet destruction. The usual cause is an immune-mediated destruction secondary to an infection or drug or some primary disease called idiopathic thrombocytopaenic purpura (ITP). Distinguishing ITP from other conditions is usually done clinically, paying careful attention to the onset of symptoms, family history, medication use, and recent illnesses. Idiopathic thrombocytopaenic purpura (ITP) is the presence of isolated thrombocytopaenia with a normal bone marrow and the absence of other causes to account for it. ITP is caused by increased peripheral platelet destruction, with most patients having antibodies to specific platelet membrane glycoproteins while relative marrow failure may contribute to this. As the bone marrow is normal, there is no primary haematologic cause for thrombocytopaenia and her doctor will have to exclude infection and review her medications to find the cause. There are two distinct types - acute ITP in children, which has an abrupt onset and chronic ITP in adults, which is gradual in its appearance. No treatment is required when the platelet counts are more than 50,000/ml. Treatment is indicated a)when platelets are less than this and there is significant mucous membrane bleeding; b)in patients with hypertension, peptic ulcer disease or vigorous lifestyle who have a risk factor for bleeding and c)when platelet count <20,000-30,000/ml. Glucocorticoids and intra-venous gammaglobulins (I.V. Ig) are the mainstays of medical therapy. Their use and dose is dictated by the symptoms, patient's clinical condition and the platelet count. Platelet transfusion is indicated for controlling severe haemorrhage. Platelet survival is increased if the platelets are transfused immediately after I.V. Ig infusion. Splenectomy is reserved for patients in whom medical therapy fails. Long-term steroid use and splenectomy are associated with certain complications. Steroids may lead to osteoporosis, glaucoma, cataracts, loss of muscle mass, and an increased risk of infection while splenectomy can cause infection or sepsis. Laparoscopic splenectomy is readily available in most major hospitals in India and you can easily have this surgery done here.

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