Haematological disorders Topic name.pptx

Blood

1. Title:- Hematological Disorders & Their Surgical Management Subtitle: 1. Haemophilia, 2. Hemolytic Anemia , 3. Thrombocytopenia, 4.Sickle Cell Disease, 5. Polycythemia & 6.Thrombosis.

2. 1. Haemophilia Hemophilia is a rare genetic bleeding disorder. Caused by deficiency of clotting factors (Factor VIII or IX). Results in difficulty in blood clotting. Mostly affects males, but females can be carriers.

3. Types of Hemophilia Definition: X-linked recessive bleeding disorder due to deficiency of clotting factors. Types: 1. Hemophilia A – caused by deficiency of clotting factor VIII. 2. Hemophilia B – caused by deficiency of clotting factor IX. 3. Hemophilia C – caused by deficiency of clotting factor XI (rare).

4. Haemophilia (Symptoms & Evaluation) Symptoms: Symptoms: • Recurrent joint bleeds ( hemarthrosis ). Muscle hematomas. Prolonged bleeding after trauma/surgery. Evaluation: Prolonged APTT, normal PT/bleeding time. Factor assays.Family history.

5. Haemophilia (Management) Treatment: Factor replacement (VIII/IX concentrates). Desmopressin (for mild A). Antifibrinolytics ( tranexamic acid). Surgical considerations: Pre-op factor correction. Avoid IM injections. Meticulous hemostasis intra-op.

6. 2. Hemolytic Anemia (Definition & Types) Definition: Premature destruction of RBCs → anemia , jaundice, splenomegaly. *Types: 1. Intrinsic (hereditary): Sickle cell, thalassemia, spherocytosis. 2. Extrinsic (acquired): Autoimmune, infections, drugs.

7. Hemolytic Anemia (Symptoms & Evaluation) Symptoms: Fatigue, pallor, jaundice, dark urine, splenomegaly. Evaluation: Peripheral smear, reticulocytosis .↑ LDH, ↑ indirect bilirubin.Coombs test (autoimmune).

8. Hemolytic Anemia (Management) Medical: Treat underlying cause. Blood transfusion (cautious). Steroids (AIHA). Splenectomy in hereditary spherocytosis. Surgical: Perioperative transfusion planning. Monitor hemolysis risk during surgery.

9. 3. Thrombocytopenia (Definition & Types) Definition: Platelet count <150,000/µL. Types: 1. Decreased production (aplastic anemia, marrow failure). 2. Increased destruction (ITP, DIC). 3. Sequestration (splenomegaly).

10. Thrombocytopenia (Symptoms & Evaluation) Symptoms: Petechiae , purpura , mucosal bleeding, menorrhagia. Evaluation: CBC with platelets. Bone marrow biopsy (if needed). Rule out DIC, TTP.

11. Thrombocytopenia (Management) Treatment: Platelet transfusion (if <20,000/µL or active bleed). Steroids/ IVIg (ITP). Treat underlying cause. Surgical care: Platelet count >50,000/µL required for major surgery. Avoid drugs worsening bleeding (NSAIDs, heparin if HIT).

12. 4. Sickle Cell Disease (Definition & Pathogenesis) Definition: Hereditary hemoglobinopathy ( HbS mutation). Pathogenesis: HbS → sickling → vaso -occlusion & hemolysis .

13. Sickle Cell Disease (Symptoms & Evaluation) Symptoms: Anemia , jaundice, painful vaso -occlusive crisis, dactylitis , splenomegaly. Evaluation: Peripheral smear (sickled RBCs). Hb electrophoresis ( HbS ).

14. Sickle Cell Disease (Management) Medical: Hydroxyurea (↑ HbF ). Blood transfusion. Analgesics, Dehydration. Surgical considerations: Avoid hypoxia, dehydration, acidosis. Pre-op transfusion to reduce HbS .

15. 5. Polycythemia (Definition & Types) Definition: Abnormally increased RBC mass. Types: Primary: Polycythemia vera ( myeloproliferative ). Secondary: Hypoxia, high altitude, smoking, tumors .

16. Polycythemia (Symptoms & Evaluation) Symptoms: Headache, dizziness, pruritus (after hot bath), thrombosis. Evaluation: ↑ Hb , ↑ Hct.JAK2 mutation (PV). Erythropoietin level.

17. Polycythemia (Management & Surgical Care) Medical: Phlebotomy. Hydroxyurea , aspirin. Surgical: Optimize Hct (<45%). Maintain hydration. Prophylaxis against thrombosis.

18. 6.Thrombosis in Surgical Patients Causes: Hypercoagulable state (cancer, polycythemia ). Prolonged immobilization. Trauma/surgery itself. Risks: DVT, pulmonary embolism, arterial thrombosis. Prevention: Early mobilization. Compression stockings, pneumatic devices. Anticoagulant prophylaxis (LMWH, heparin).

19. Summary Haemophilia: Factor deficiency → bleeding. Hemolytic anemia : Premature RBC destruction. Thrombocytopenia: Low platelets → mucosal bleeding. Sickle cell: HbS mutation → vaso -occlusion. Polycythemia : High RBC mass → hyperviscosity , thrombosis. Surgical patients: Careful pre-op optimization & thrombosis prophylaxis needed.