Decreased Platelet Production

Bone Marrow Disorders:

• Aplastic Anemia: Bone marrow fails to produce sufficient blood cells.
• Leukemia: A type of cancer affecting blood and bone marrow.
• Myelodysplastic Syndromes: A group of disorders caused by poorly formed or dysfunctional blood cells.
• Bone Marrow Infiltration: Cancer or infections can infiltrate the bone marrow, reducing its ability to produce platelets.

Nutritional Deficiencies:

• Vitamin B12 Deficiency: Can lead to a reduction in platelet production.
• Folate Deficiency: Another vital nutrient whose deficiency can cause low platelet count.

Congenital Disorders:

• Fanconi Anemia: A rare genetic disorder affecting bone marrow.
• Congenital Amegakaryocytic Thrombocytopenia: A genetic condition leading to low platelet production.
• Thrombocytopenia with Absent Radius (TAR) Syndrome: A rare genetic disorder with low platelet count and absent radius bone in the forearm.

Infections:

• Viral Infections: Such as Epstein-Barr virus, cytomegalovirus, and HIV can decrease platelet production.

Increased Platelet Destruction

Immune-Mediated:

• Immune Thrombocytopenic Purpura (ITP): An autoimmune disorder where the body attacks its platelets.
• Drug-Induced Thrombocytopenia: Medications like heparin and quinine can cause this condition.
• Post-Transfusion Purpura: A rare complication following blood transfusion.

Non-Immune-Mediated:

• Disseminated Intravascular Coagulation (DIC): A serious disorder causing widespread clotting and bleeding.
• Thrombotic Thrombocytopenic Purpura (TTP): A condition causing small blood clots throughout the body.
• Hemolytic Uremic Syndrome (HUS): Often triggered by infection, leading to blood clot formation.

Infections:

• Bacterial Sepsis: Severe infection spreading through the blood.
• Viral Infections: Such as dengue fever, can also lead to increased platelet destruction.

Sequestration

Hypersplenism:

• Splenomegaly: Enlarged spleen due to liver disease or hematologic disorders can trap and reduce the number of platelets in circulation.

Hereditary Thrombocytosis:

• Genetic mutations (e.g., in the THPO or MPL genes) lead to increased platelet production.

Secondary (Reactive) Thrombocytosis

Infections:

• Bacterial, viral, or fungal infections can trigger elevated platelet counts as part of an acute phase response.

Inflammatory Conditions:

• Chronic diseases like juvenile rheumatoid arthritis, inflammatory bowel disease, and Kawasaki disease.

Tissue Damage:

• Trauma, surgery, or burns can increase platelet counts as part of the healing process.

Iron Deficiency:

• A common cause of reactive thrombocytosis in children.

Post-Splenectomy:

• Removal of the spleen can lead to persistently elevated platelet counts.

Hemolytic Anemia:

• Conditions causing hemolysis, such as sickle cell disease, can increase platelet production.

Medications:

• Drugs like corticosteroids can elevate platelet counts.

Malignancies:

• Some cancers, especially those affecting bone marrow, can cause elevated platelet production.

Chronic Blood Loss:

• Chronic conditions causing blood loss can stimulate platelet production as a compensatory mechanism.

Overview

• Definition: ITP involves the immune system producing antibodies against platelets.
• Prevalence: It is relatively common in children, with about 1 in 10,000 affected each year.

Causes

• Autoimmune Reaction: The exact cause is often unknown, but it involves antibody production against platelets.
• Triggers: Infections (e.g., viral illnesses like measles, mumps, flu), vaccinations, or other immune challenges.

Symptoms

• Bruising: Easy or excessive bruising without a known cause.
• Petechiae: Small red or purple spots on the skin.
• Nosebleeds and Bleeding Gums: Particularly after brushing teeth.
• Heavy Menstrual Bleeding: In adolescent girls.
• Severe Bleeding: Rarely, severe internal bleeding can occur.

Diagnosis

• History and Physical Examination: Reviewing recent illnesses or vaccinations and examining signs of bleeding.
• Blood Tests: Complete blood count showing low platelet count and peripheral blood smear.
• Bone Marrow Exam: Rarely needed but can exclude other bone marrow disorders.

Treatment

• Observation: Many mild cases resolve on their own.
• Medications: Corticosteroids, intravenous immunoglobulin (IVIG), and anti-D immunoglobulin for Rh-positive children.
• Chronic ITP: Additional treatments include Rituximab, thrombopoietin receptor agonists, and possibly splenectomy.

Prognosis and Management

• Acute ITP: Most children recover spontaneously within 6 months.
• Chronic ITP: Requires ongoing management, with regular follow-ups to monitor platelet counts and adjust treatment.

Congenital Disorders

1. Glanzmann Thrombasthenia: Deficiency of the platelet glycoprotein IIb/IIIa complex.
2. Bernard-Soulier Syndrome: Deficiency of the glycoprotein Ib-IX-V complex on the platelet surface.
3. Storage Pool Diseases: Deficiencies in alpha or delta granules.
4. Other Rare Disorders: Like May-Hegglin Anomaly and Wiskott-Aldrich Syndrome.

Acquired Disorders

1. Drug-Induced Platelet Dysfunction: Caused by NSAIDs, aspirin, and other medications.
2. Uremia: Chronic kidney disease leading to platelet function impairment.
3. Liver Disease: Reduced production of clotting factors and altered platelet function.
4. Cardiopulmonary Bypass Surgery: Platelet dysfunction due to mechanical forces during surgery.

Diagnosis and Treatment

• History and Physical Examination: Detailed bleeding history and family history.
• Laboratory Tests: Platelet function tests, blood smear, and electron microscopy.
• Treatment: Avoidance of antiplatelet drugs, desmopressin, platelet transfusions, antifibrinolytic agents, and specific therapies for underlying disorders.

Management and Support

• Regular Monitoring: For bleeding symptoms and platelet function.
• Patient Education: On recognizing symptoms and avoiding situations that may exacerbate bleeding.
• Genetic Counseling: For understanding the risk of transmission to offspring in inherited disorders.

For more detailed information and personalized advice, please consult a healthcare provider specializing in pediatric hematology and immunology.

• Corticosteroids to suppress the immune system

• Intravenous immunoglobulin (IVIG) therapy

• Platelet transfusions

• Medications that stimulate platelet production