Abstract

Immune thrombocytopenic purpura (ITP) is an immune-mediated acquired disease present in both adults and children. It is identified by transient or persistent decrease in platelet count and bruising of the skin.[1]The incidence of ITP was 1.80 per 100,000 person-years.[2]We report a 4 year old male paediatric patient with Immune thrombocytopenic purpura.

Keywords

Introduction

ITP, a rare condition with a slightly female predominance, is characterized by acute, persistent, or chronic durations, increasing with age. Immunothrombocytopenia (ITP) has a modest female predisposition and an incidence rate that varies with age, from 1.6 to 3.9 per 100,000 patient-years. ITP is classified as acute, persistent, or chronic depending on whether it lasts for less than three months, three to twelve months, or more than twelve months. Immune thrombocytopenic purpura (ITP) is also known as idiopathic thrombocytopenic purpura, which is characterized by a low platelet count with a normal peripheral smear study. The condition presents with easy bruising, bleeding into the skin, which looks like purple-colored spots mostly on the lower legs,bleeding from the nose or gums, blood in stool or urine, and heavy menstrual flow, but ay also be asymptomatic? ^[3] ITP involves IgG-type antibody-mediated destruction of circulating platelets, resulting in thrombocytopenia. This antibody is active against platelet membrane glycoproteins ?b and ?a, which inhibit platelet aggregation and damage megakaryocytes that are responsible for the production of platelets. ^[4] Diagnosis include physical examination for finding purpura, peripheral smear to identify thrombocytopenia. In most cases, kidswith ITP don’t need a bone marrow test. Other tests like liver and kidney function, viral infection screening, and autoimmune profile can be helpful. Expensive tests like immunoglobulin levels and platelet survival studies aren’t usually necessary for typical ITP cases. Common variable immune deficiency (CVID) is a differential diagnosis to consider, especially in India, where kids may have low platelet counts. Usually, a complete blood count and examination of a blood film by a hematopathologist are enough to support the suspicion of classical ITP. The blood film may show large platelets, and the bone marrow may have an adequate or increased number of megakaryocytes may not produce enough platelets. The main goal of these tests is to ensure that the red blood cells, white blood cells, and their precursors are normal; ruling out other causes.ITP can be managed by medical and surgical treatment. Corticosteroids such as Prednisolone, IV immunoglobulin G and IV anti-D are the first line agents. Secondly managed with immunosuppressant, rituximab, TPO-RA such as eltrombopag, avatrombopag, romiplostim, platelet transfusion usually done when platelets depleted below 10000 lakhs/cumm. When medical treatments for ITP don’t work, Surgical management such as splenectomy is considered the definitive treatment. ^[5]However, it is important to be aware of the risk of overwhelming post-splenectomy infection, especially in children under 5 years old, and particularly those under 2 years old. To minimize this risk, it is recommended to delay splenectomy until after 6 years of age, and only if the child has severe thrombocytopenia (platelet count less than 10000 lakha/cumm), persistent mucosal bleeds for at least 12 months, and failure of second-line medications. Splenectomy has a high success rate of 75% in achieving a complete response. Before the procedure, it is crucial for children to be vaccinated against pneumococcus, meningococcus, and H. influenza at least 2 weeks in advance. ^[7]

CASE REPORT

A 4-year-old male pediatric patient presented with complaints of blackish discoloration in the lower region of the leg. The patient had no past medical history. Laboratory investigation revealed platelet depletion (30000 lakhs/cumm) on the first day and showed an increase to 40000 lakhs/cumm, but again depleted to 35000 lakhs/cumm on the third day. Peripheral smear study on RBS shows normocytic and normochromic, WBC count is within normal limits, lymphocytes predominate, few reactive lymphocytes are seen, no immature cells or blasts are noted, platelet count is markedly reduced and is scattered singly, and few large forms are seen.The patient was treated with Syp Azithral (Azithromycin) 3 ml OD, Syp Levolin (Levosalbutamol) 2.5 ml TID, and Syp Zincovit (Multivitamins and Minerals) 5 ml OD. Steroids were started at the time of discharge, and discharge medicines included Syp Omnacortil (Prednisolone) (7 ml BD for 5 days), Syp Azithral (Azithromycin) (3 ml OD for 2 days), Syp Levolin (Levosalbutamol) 2.5 ml TID, and Syp Zincovit (Multivitamins and Minerals) (5 ml OD for 5 days).

DISCUSSION

Immune thrombocytopenic purpura is an autoimmune disorder marked by immune-mediated destruction of platelets, which shows depletion in platelet count without any other known etiology of thrombocytopenia. ^[6] ITP is predominantly affected by females than men. Childhood ITP of acute form is seen in the 2–10 year age group, with a peak incidence at 2–4 years of age. The disease is usually self-limiting, mostly occurring after a viral infection. Recent ASH guideline recommends IVIG, steroids and anti-D for children with RhD positive blood groups can quickly improve symptoms and thus represent appropriate first-line therapy. ^[1]If initial therapy does not work, immunomodulatory drugs such as azathioprine, cyclosporine A (CSA), mycophenolate mofetil (MMF), dapsone, danazol and cytotoxic agents can be used. Rituximab is showing promise as a latter line of treatment option for non-responders, with four weekly doses. If medical measures fail to treat ITP, splenectomy is considered the ultimate treatment option. In life-threatening situations, it is recommended to use a combination of intravenous prednisolone, IVIG/anti D, and a high dose of platelet transfusion (up to 30mg/dl). To control bleeding, antifibrinolytics like tranexamic acid can be administered intravenously every 6 hours at a dose of 10-15mg/dl. ^[7] In thiscase patient was managed with the first-line agent, corticosteroid and discharged.

CONCLUSION

Immune thrombocytopenic purpura (ITP), also known as idiopathic thrombocytopenic purpura, is an autoimmune disorder characterized by a low platelet count and a normal peripheral smear study. It presents with symptoms such as easy bruising, purple-colored skin bleeding, nose or gum bleeding, blood in stool or urine, and heavy menstrual flow. The condition is caused by IgG-type antibody-mediated destruction of circulating platelets, resulting in thrombocytopenia. Diagnosis includes physical examination and peripheral smear. Treatments include corticosteroids, immunosuppressants, TPO-RA, platelet transfusions, and surgical management like splenectomy. ITP is predominantly affected by females and is usually self-limiting, usually occurring after a viral infection. A high dose of IVIg and corticosteroid can quickly improve symptoms and represent appropriate first-line therapy.

REFERENCE

1. Sugiura T, Yamamoto K, Murakami K, Horita S, Matsusue Y, Nakashima C, Kirita T. Immune thrombocytopenic purpura detected with oral Hemorrhage: A case report. Journal of Dentistry. 2018 Jun;19(2):159.
2. Zahidin MA, Razak NI, Noor NH, Johan MF, Zulkafli Z, Abdullah AD, Edinur HA, Dzarr A. An Epidemiology Study of Adult Immune Thrombocytopenia Patients in a Teaching Hospital in Northeastern Malaysia. Cureus. 2023 Nov 8;15(11).
3. Immune thrombocytopenia (ITP), Mayo Clinic.
4. McLeish R, Boyd D. Immune thrombocytopenic purpura: A case report. Advances in Oral and Maxillofacial Surgery. 2022 Oct 1;8:100340.
5. Warrier R, Chauhan A. Management of immune thrombocytopenic purpura: an update. Ochsner Journal. 2012 Sep 21;12(3):221-7.
6. Chhetri ST, Kunwor B, Sharma B, Joshi P, Timilsina S, Acharya B. Immune Thrombocytopenic Purpura in an Adult Male: A Case Report. Cureus. 2023 Oct 8;15(10).
7. Anoop P. Immune thrombocytopenic purpura: historical perspective, current status, recent advances and future directions. Indian pediatrics. 2012 Oct;49:811-8.