Sep 2, 2025
Hemoglobin Chain Composition Primary Site Gower-1 χ2 χ2 Yolk sac Gower-2, α2 χ2 Yolk sac Portland χ2 ψ2 Yolk sac Fetal (F) α2 ψ2 Liver Adult (A) α2 π2 Marrow
Haemoglobin concentration (g/L) Population No anaemia Mild anaemia Moderate anaemia Severe anaemia Children, 6-23 months ≥ 105 95-104 70-94 <70 Children, 24-59 months ≥ 110 100-109 70-99 <70 Children, 5-11 years ≥ 115 110-114 80-109 <80 Children, 12-14 years, non-pregnant girls ≥ 120 110-119 80-109 <80 Children, 12-14 years boys ≥ 120 110-119 80-109 <80 Adult, 15-65 year, non-pregnant women ≥ 120 110-119 80-109 <80 Adults, 15-65 years men ≥ 130 110-129 80-109 <80 Pregnancy First trimester ≥ 110 100-109 70-99 <70 Second trimester ≥ 105 95-104 70-94 <70 Third trimester ≥ 110 100-109 70-99 <70
Markers of extravascular hemolysis |
Markers of intravascular hemolysis |
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Mild-HS Moderate Moderately Severe Severe Frequency It is seen in 20-30% of the cases. It is seen in 60-70% of the cases. It is seen in 10% of the cases. It is seen in less than 5% of the cases. Inheritance It has an autosomal dominant inheritance. It has autosomal dominant inheritance and can also be caused due to de Novo mutations. It has autosomal dominant inheritance and can also be caused due to de Novo mutations. It has an autosomal recessive inheritance. HB(g/dL) It is more than 10.5 g/dL. Hb levels are between 8-12 g/dL. Hb levels are between 6-8 g/dL. It is less than 6 g/dL. Rectic Count It is 1.5-6%. It is equal to or more than 6%. It is equal to or more than 10%. It is equal to or more than 10%. TSB (mg/dl) It is less than 2mg/dl. It is equal to or more than 2 mg/dL. It is equal to or more than 2 mg/dL. It is equal to or more than 3 mg/dL. Spherocytosis There will be occasional spherocytosis. There will be spherocytosis. There will be spherocytosis. There will be significant spherocytosis and poikilocytosis. Osmotic Fragility It is normal or mildly elevated. It is elevated. It is elevated. It is severely elevated. Need for BT It is usually not required. It is occasionally needed. It is occasionally needed. It is regularly needed.
It is also called transfusion-dependent HS.Splenectomy It is usually not needed. It is sometimes required, and partial splenectomy is done. It is usually needed between 6-9 years. It is often needed in children more than 3 years old.
Pancytopenia with Hypocellular or Acellular Bone Marrow |
Pancytopenia with Cellular BM |
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Platelet Factor Bleeding Clotting Factor Bleeding Superficial mucocutaneous bleeds, e.g., Petechiae and epistaxis. Deeper internal organ-based bleeds, e.g., hemarthrosis. The most common sites are skin, ENT mucosa, and GIT mucosa. The most common sites are soft tissues, muscles, and joints. Petechiae are usually present. Petechiae are absent. Ecchymosis is superficial. Ecchymosis is deep. Common in trivial trauma. Rare in Trivial trauma. Postoperatively they show immediate bleeding. Post-operative, they show delayed bleeding on the 1st to 3rd day.
Category |
Symptoms / Treatment Options |
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Newly diagnosed ITP or Persistent ITP |
Severe bleeding: Short course Steroids / IVIG anti Dig (along with platelet transfusion in life threatening bleeds) |
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Chronic ITP |
No mild bleeding |
Observation |
Moderate to severe bleeding |
Available therapies include:
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Type Hemophilia A-Factor VIII Conc Hemophilia B-Factor IX Conc Hemarthrosis 50-60 IU/kg on D1, then 20 IU/kg on D2. Alternate day therapy till joint normalizes. Consider prophylaxis 80-100 IU/kg on D1, then 40 IU/kg on D2. Alternate day therapy till joint normalizes. Consider prophylaxis Muscle/Large SC Hematoma 50 IU/kg on D1, then 20 IU/kg alternate day till resolves 80 IU/kg OR 40 IU/kg for 2-3 days 40 IU/kg + Antifibrinolytic Rx Epistaxis Apply pressure for 15-20 min; pack with petrolatum gauze; give antifibrinolytic therapy If fails- 20 IU/kg Concentrates Apply pressure for 15-20 min; pack with petrolatum gauze; give antifibrinolytic therapy If fails- 30 IU/kg Concentrates Major Surgery CNS/Life-Threatening Hemorrhage 50-75 IU/kg on D1, then 25 IU/kg every 8-12 hrly to maintain trough level >50 IU/dL for 5-7 d Then 50 IU/kg OD to maintain trough >25 IU/dL for 7 days 80-120 IU/kg on D1, then 50-60 IU/kg every 8-12 hrly to maintain trough level >40 IU/dL for 5-7 d Then 50 IU/kg OD to maintain trough >30 IU/dL for 7 days Iliopsoas hemorrhage 50 IU/kg on D1, then 25 IU/kg BD until asymptomatic; then 20 IU/kg alternate day, for a total of 10-14 days 100 IU/kg on D1, then 25 IU/kg BD until asymptomatic; then 50-60 IU/kg alternate day, for a total of 10-14 days Hematuria Bed Rest, Maint fluids × 1.5 times 20 IU/kg single dose Bed Rest, Maint fluids × 1.5 times 40 IU/kg single dose Prophylaxis 20-40 IU/kg every alternate day to achieve trough level ≥1% 30-50 IU/kg every alternate day to achieve trough level ≥1%
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