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hemolytic disease of newborn


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hemolytic disease of newborn

  2. 2. Hemolytic Disease The term hemolytic disease is limited to conditions in which the rate of RBCs destruction is accelerated and the ability of bone marrow to respond is unimpaired.
  3. 3. Causes: Rh incompatibility Autoimmune Hemolytic Anemia Hereditary Spherocytosis Sickle Cell Disease G6PD Thalassemia
  4. 4. Rh incompatibility: Rh incompatibility is a condition which develops when an Rh negative mother conceives a fetus which is Rh positive.Isoimmunization: When the mother produces Abs directed against fetus RBC surface Ag.
  5. 5. THE MOST COMMON…. Cause of Maternal Isoimmunization  Feto- maternal BleedRisk Factors of Feto-maternal Bleed:  Amniocentesis Ectopic pregnancy
  6. 6. THE MOST COMMON…. RBC Rh Antigen :  Rh “ D ’’ Ag Mother produces:  Anti Rh (D) Abs
  7. 7. Is the baby at risk? Abs mustMother Coombs be Ab titer Dad mustmust be test must associated must be be Rh + Rh - be positive with above 1:8 Hemolysis Hemolytic • Anti KELL Abs • Anti Lewis Non-Hemolytic ABS • Anti RH(D) Abs Abs ABS
  8. 8. Presentation: Mild jaundice Erythroblastosis Fetalis  Generalized Edema  Hepatomegaly  Ascites
  9. 9. Management: Phototherapy for neonatewith mild jaundice Exchange transfusion inSevere cases
  10. 10. Prevention: To prevent Isoimmuization of yet unimmunized mother give Anti Rh D IgG (Rhogam) IntraMuscular at 28 weeks of gestation.
  11. 11. AutoImmune Hemolytic Anemia This Arises as an autoimmune phenomenon targeting the RBCs . It may arise as an isolated problem or as a complication of HBV, SLE .
  12. 12. Types:There are two types of AIHA: Warm AIHA: IgG is directed against RBCs Cold AIHA: IgM is directed against RBCs
  13. 13. Presentations: Acute Onset Weakness, Pallor, Fatigue Dark Urine Splenomegaly Underlying disease HIV/SLE
  14. 14. LABS: Normocytic Hemoglobinemia and Hemoglobinuria Coombs test is positive
  15. 15. Treatment: Warm AIHA:Prednisolone IV or IV ImmunoGlobulin Cold AIHA:Self-limited coursePlasma Exchange is effective
  16. 16. B-Thalassemia: It is an inherited disorders of hemoglobin synthesis that result from an alteration in the rate of Beta globin chain production. Pathology:Abnormality occurs when there is defective productionof beta chain and an excess of normally producedtype which accumulates in the cell as an unstable
  17. 17. Types: B-Thalassemia Minor: Reduced production of Beta chain B- Thalassemia Major: Complete absence of Beta chain
  18. 18. Presentation: Children present with severe Anemia, hepatosplenomegaly at the age of 3-6 months Jaundice Frontal Bossing, Maxillary prominence
  19. 19. Types of expression: Thalassemia trait: Patients have mild anemia Thalassemia intermedia: Patients have intermediate anemia Thalassemia Major: Severe symptoms
  20. 20. Labs:  Microcytic RBCs  Decreased MCH  Increased Nucleated RBCs  Increased Serum Ferritin & Transferrin levels.Diagnosis:  HPLC confirms diagnosis of Beta Thalassemia
  21. 21. Normal RBCs B- thalassemia Target cells
  22. 22. Management: Blood transfusions:  Keep Hb between 9-10mg/dl Chelation therapy and Iron Overload:  After multiple transfusions patient may develop Iron Overload  Leading to DIABETES, THYROID AND PARATHYROID dysfunction  To remove excess iron chelation therpay is very effective.  Deferoxamine IV subcutaneously or alternatively Deferiprone PO Cure:
  23. 23. Hereditary Spherocytosis:Defect in protein of the RBC membrane skeletonand plasma membrane such as Spectrin, ankyrin leading to rigid spherical shaped RBCs.The structural membrane defect predispose it todestruction when they pass through the splenic sinusoids.
  24. 24. Presentation: Newborn present with Anemia, jaundice Chronically splenomegaly and Gall stones are often present.
  25. 25. Labs: Increased MCHC Normal MCV Reticulocytosis Spherocytes on PBSDiagnosis: Family history (autosomal recessive) Osmotic Fragility test confirms the diagnosis.In this test, the spherocytes will rupture in mildly hypotonic solutions - this is due to increased permeability of the spherocyte membrane to salt and water.
  26. 26. SPHEROCYTOSISSpherocytes
  27. 27. Treatment: Folic Acid Supplementation 1-5mg/day Splenectomy for >6years , immunize against S.pneumonia priorly.Complications: Aplastic Crisis due to infection with Parvovirus B19 Cause transient arrest in RBC production for 4-6 weeks
  28. 28. Pyruvate Kinase deficiency : Deficieny of the PKenzyme in RBCs responsible for ATP production resulting in rigid RBCs predisposing them to splenic destruction.Presentation: Affected individuals present with Splenomegaly Pallor, jaundice and icterusDiagnosis: Pyruvate Kinase DeficiencyTreatment: Splenectomy Folic Acid supplementation
  29. 29. G6PD: Disease charaterized by hemolytic anemia following Oxidant stress such as :  Fava beans  Sulfa Drugs  Anti-Malarial drugs An X-linked disorder expressed in Males and carried in females Pathology: Decreased in IncreasedG6PD in RBCs Glutathione susceptibility to production Oxidant stress
  31. 31. Presentation: Following ingestion of such foods/drugs result in crisis such as:  Children present with Jaundice in neonatal period ,pallor and icterus  Dark Urine  Chronic patients may have splenomegaly. Labs:  Hemoglobinemia and hemoglobinuria  Heinz Bodies and Bite cells Diagnosis:  The nature of clinical Presentation  Family history (only present in males)  Quantitative G6PD enzyme assay (Confirmatory Diagnosis)
  32. 32. Heinz Bodies And Bite cells Bite cellHeinzbodies
  33. 33. Management: Supportive Care:  hydration  transfusion if needed and monitoring Folic Acid supplementation Counseling to avoid Similar Drugs in future
  34. 34. Sickle Cell Disease: It results from substitution of valine for glutamic acid at position 6 of Beta globin Chain. Sickle shaped RBCs are rapidly hemolyzed and have a life span of 10-20 days
  35. 35. Sickle shaped cells
  36. 36. Presentation:  Hemolytic anemia develop after 2-4 months of age  Pallor , jaundice develops  Asplenia due to auto-infarction of spleen , spleen not palpable, after 6 years Labs:  Anemia , thrombocytosis, reticulocytosis  Normal MCV  Bone Marrow hyperplasia On BMA  Sickle shaped Cells, Howel-Jolly bodies
  37. 37. Complications/ Acute painful Crisis When the microcirculation is obstructed by sickled RBCs it results in ischemic injury it may present as:  Dactylitis - Swollen hands and foot  Retinopathy- obstruction of ophthalmic artery  Acute Chest syndrome- involving legs causing pain, dyspnea, hypoxemia  Sequestration Crisis- SC block outflow to spleen  Aplastic Crisis- Bone marrow temporarily stops producing RBCs Diagnosis:  History of trigger preceding the crisis such as dehydration or fever  Hb electrophoresis confirms the diagnosis
  38. 38. Management: Hydration PO or IV, analgesics (narcotics) Specific therapy:  Aplastic crisis- Blood transfusion may be necessary  ACS or CVA – require Oxygen, mechanical ventilation and may require exchange transfusion Preventive Care:  After 2 y/o/a child is kept on penicillin and amoxicillin  Folate supplements  Immunization against S.pneumonia  Hydroxyurea – increase HbF
  39. 39. Hemolytic Weaknes Splenom Coomb Hemoglo PBS DiagnosiDiseases s egaly s -binuria s Pallor test fatigueHereditary Spherocytes OsmoticSpherocytosis + + - - fragility testPyruvate Normocytic PKKinase + + - - assaydeficiencyG6PD Bite cells G6PDdeficiency + + - + Heinz Bodies assayAutoimmune Normocytic IgG andHemolytic + + + + IgM Ab againstanemia RBCsB- Nucleated HbThalassemia ++ ++ - - RBCs, Target cell electrop horesisSickle Cell Sickle shaped Hbdisease + - - - RBCs Howel- electrop