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Aa

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Aa

  1. 1. CASE MANAGEMENT Group 1
  2. 2. GENERAL DATA <ul><li>J.G., 34 y/o, male </li></ul><ul><li>Filipino, from Dagupan City </li></ul><ul><li>admitted for the first time at PMC on November 2, 2009. </li></ul>
  3. 3. <ul><li>INFORMANT: Patient </li></ul><ul><li>RELIABILITY: 80 % </li></ul><ul><li>CHIEF COMPLAINT: Dizziness </li></ul>
  4. 4. HISTORY OF PRESENT ILLNESS <ul><li>5 days prior to admission </li></ul><ul><li>dizziness associated with loss of appetite </li></ul><ul><li>Vomiting </li></ul><ul><ul><li>non-billous </li></ul></ul><ul><ul><li>non-projectile </li></ul></ul><ul><ul><li>amounting to 5-10 ml per bout. </li></ul></ul><ul><li>generalized body weakness </li></ul>
  5. 5. HPI..cont <ul><li>Few hours PTA </li></ul><ul><li>Dizziness---px can’t walk and stand alone </li></ul><ul><li>Consultation --admission </li></ul>
  6. 6. Past Medical History <ul><li>Year 2000 </li></ul><ul><li>Dizziness </li></ul><ul><li>Philippine General Hospital due ---- diagnosed to have “ Paroxysmal Nocturnal Hemoglubinuria” </li></ul><ul><li>Medication: </li></ul><ul><li>Folic acid OD and Vit B6 & B1 OD </li></ul><ul><li>September 2009 </li></ul><ul><li>Skin discoloration </li></ul><ul><li>At Philippine General Hospital </li></ul><ul><li>Px already had 14x blood transfusion(4-5bags/time) </li></ul>
  7. 7. Family History <ul><li>Mother has breast cancer </li></ul><ul><li>Father has hypertension. </li></ul><ul><li>3 siblings have anemia. </li></ul>
  8. 8. Social, Personal, and Environmental History <ul><li>P atient is a farmer </li></ul><ul><li>Lives with his wife and 3 children </li></ul><ul><li>Alcoholic drinker </li></ul><ul><li>Smoker </li></ul>
  9. 9. Review of system <ul><li>+Dizziness </li></ul><ul><li>+loss of appetite </li></ul><ul><li>+Vomiting </li></ul><ul><li>+generalized body weakness </li></ul>
  10. 10. PHYSICAL EXAMINATION <ul><li>SKIN: (+) jaundice </li></ul><ul><li>HEENT: Pale palpebral conjunctivae </li></ul><ul><li>ABDOMEN: Globular , liver span - 13cm </li></ul>
  11. 11. Vital signs <ul><li>BP - 110/80 mmHg </li></ul><ul><li>HR - 78 bpm </li></ul><ul><li>RR - 16 cpm </li></ul><ul><li>TEM - 36.8 C( upon admission 37.8) </li></ul>
  12. 12. Laboratory test
  13. 13. Hematology : Parameter Result Normal value <ul><li>Total WBC </li></ul>2.9 4-10 x 10 9 /L SEGMENTERS 0.39 0.40-0.80 LYMPHOCYTES 0.16 0.20-0.40 MONOCYTES 0.04 0.04-0.10 EOSINOPHILS 0.01 0.02-0.06 <ul><li>Total RBC </li></ul>0.51 4-6x10 12 /L HEMOGLOBIN 21 140-180g/L HEMATOCRIT 0.06 0.40-0.54 <ul><li>PLATELET </li></ul>78 150-450 x 10 9 /L
  14. 14. Blood chem Result Normal value Urea nitrogen 4.8 mmol/L 2.2-8.2mmol/L Creatinin 1.9 5-17 Phos Alk 32.8 10-35 SGPT 50.9 5-47
  15. 15. HBsAg screening <ul><li>Nonreaction </li></ul>
  16. 16. What are you impression?
  17. 17. APLASTIC ANEMIA
  18. 18. DIFFERENTIAL DIAGNOSIS <ul><li>Role out </li></ul><ul><li>Leukemia--- WBC increased in BM </li></ul><ul><li>Multiple Myeloma--- plasma cell increased in BM </li></ul><ul><li>Lymphoma(Hodgkins)--- Lymphocyte increased in BM </li></ul>
  19. 19. APLASTIC ANEMIA What happens when the bone marrow shuts down?
  20. 20. What is Aplastic Anemia ? - Aplastic Anemia is a bone marrow failure disease .
  21. 21. APLASTIC ANEMIA <ul><li>Aplastic anemia is a severe, life threatening syndrome in which production of erythrocytes, WBCs, and platlets has failed. </li></ul><ul><li>The disease is characterized by peripheral pancytopenia and accompanied by a hypocellular bone marrow. </li></ul>
  22. 22. Peripheral blood Smear Peripheral blood Smear
  23. 23. HYPOCELLULAR BONE MARROW IN APLASTIC ANEMIA
  24. 24. Aplastic Anemia patients <ul><li>Aplastic Anemia patients have decreased amounts of: - Red Blood Cells </li></ul><ul><li>- White Blood Cells </li></ul><ul><li>- Platelets </li></ul>
  25. 25. Functions of Blood Cells <ul><ul><li>Red Blood Cells </li></ul></ul><ul><ul><li>Carry oxygen to all body organs </li></ul></ul><ul><ul><li>White Blood Cells </li></ul></ul><ul><ul><li>Fight infection and keep you healthy </li></ul></ul><ul><ul><li>Platelets </li></ul></ul><ul><ul><li>Help control bleeding </li></ul></ul>
  26. 26. Clinical Manifestations Symptoms caused by suppression of any or all bone marrow elements <ul><li>Low Red Blood Cell </li></ul><ul><ul><ul><li>Fatigue, Headache, Inability to Concentrate, Dyspnea </li></ul></ul></ul><ul><ul><ul><li>,Pale skin </li></ul></ul></ul><ul><ul><ul><li>Low White Blood Cell </li></ul></ul></ul><ul><ul><ul><li>Frequent or prolonged infections </li></ul></ul></ul><ul><li>Viral Infections, Bacterial Infections </li></ul><ul><li>Low Platelets </li></ul><ul><ul><ul><li>Easy Bruising, Petichiae, Nosebleed and bleeding gums,Prolonged bleeding </li></ul></ul></ul>
  27. 27. PATHOGENESIS <ul><li>Stem cell failure resulting from: </li></ul><ul><li>1-An acquired intrinsic stem cell defect </li></ul><ul><li>2-An environmental cause </li></ul><ul><ul><li>Immune mechanisms </li></ul></ul><ul><ul><li>Growth factor deficiency </li></ul></ul><ul><ul><li>Defects in the microenvironment </li></ul></ul>
  28. 28. Pathophysiology of aplastic anemia
  29. 29. Epidemiology <ul><li>Incidence: </li></ul><ul><li>5-10 persons:10 6 per year </li></ul><ul><li>Aplastic anemia may occur in all age groups and both genders </li></ul>
  30. 30. Etiology : Hereditary <ul><li>1-Schwacman – Diamond Syndrome </li></ul><ul><li>2-Fanconi’s anemia syndrome </li></ul><ul><li>Autosomal recessive disorder </li></ul><ul><li>congenital developmental anomalies </li></ul><ul><li>progressive pancytopenia </li></ul><ul><li>increase risk of malignancy </li></ul><ul><li>3-Dyskeratosis congenita </li></ul><ul><li>characterized by mucous membrane leukoplasia </li></ul><ul><li>dystrophic nails, reticular hyperpigmentation </li></ul><ul><li>development of aplastic anemia during childhood </li></ul>
  31. 31. Etiology: Acquired <ul><li>1-Idiopathic </li></ul><ul><li>2- Drugs: dose related idiosyncratic </li></ul><ul><li>Nonsteroidal analgesics </li></ul><ul><li>Sulfonamides </li></ul><ul><li>Thyrostatic drugs </li></ul><ul><li>Some psychotropics </li></ul><ul><li>Penicillamine </li></ul><ul><li>Allopurinol </li></ul><ul><li>Chlorampenicol </li></ul>
  32. 32. Etiology: Acquired <ul><li>3-Radiation- Damages DNA </li></ul><ul><li>4-Chemicals- BENZENE </li></ul><ul><li>5-Viruses- Hepatitis – most common </li></ul><ul><li>6-Pregnancy </li></ul><ul><li>7-PNH ( Paroxysmal Nocturnal Hemoglobinuria ) </li></ul><ul><li>8-Disorders of immune system- SLE. </li></ul>
  33. 33. <ul><li>What causes </li></ul><ul><li>Paroxysmal Nocturnal Hemoglobinuria </li></ul><ul><ul><li>Defect in PIG-A gene which is required for the biosynthesis of cellular anchors, so partial or complete inability to construct Glycosylphosphatidylinositol(GPI) anchor for the attachment of CD55, CD59 </li></ul></ul><ul><ul><li>Diagnosis: Ham’s test , sucrose hemolytic test , Flow cytometry using antibodies against cell surface antigens CD55, CD59 which are lacking in disease </li></ul></ul>
  34. 34. Clinical manifestations of PNH <ul><li>Thrombosis </li></ul><ul><ul><li>Hepatic vein most common </li></ul></ul><ul><ul><ul><li>common cause of fatality </li></ul></ul></ul><ul><ul><li>Cerebral vein thrombosis </li></ul></ul><ul><ul><ul><li>sagittal sinus in particular </li></ul></ul></ul><ul><ul><li>Abdominal veins </li></ul></ul><ul><ul><li>Dermal veins </li></ul></ul><ul><ul><li>Pulmonary embolism unusual </li></ul></ul>
  35. 35. Clinical manifestations of PNH <ul><li>Relative/absolute bone marrow failure </li></ul><ul><ul><li>present to some degree in all patients </li></ul></ul><ul><ul><li>relative granulocytopenia/thrombocytopenia </li></ul></ul><ul><ul><li>decreased capacity to form myeloid colonies </li></ul></ul><ul><li>Two stage model </li></ul><ul><ul><li>somatic mutation in PIG-A gene </li></ul></ul><ul><ul><li>some cause for bone marrow failure </li></ul></ul>
  36. 36. Diagnosis of AA: Lab findings Peripheral blood Smear Bone marrow biopsy <ul><ul><li>Pancytopenia </li></ul></ul><ul><ul><li>Normocytic-normochromic anemia (may be slightly macrocytic) </li></ul></ul><ul><ul><li>Low reticulocyte index </li></ul></ul><ul><ul><li>Empty fatty spaces </li></ul></ul><ul><ul><li>Few hematopoietic cells </li></ul></ul><ul><ul><li>Lymphocytes and plasma cells </li></ul></ul><ul><ul><li>Hypocellular bone marrow </li></ul></ul>
  37. 37. CLASSIFICATION Designation Criteria PBS BM biopsy Severe aplastic anemia <ul><li>-2 / 3 values- </li></ul><ul><ul><li>Neutro < 500/  L </li></ul></ul><ul><ul><li>-Platelets < 20,000/ ul- </li></ul></ul><ul><ul><li>-Reticulocyte index < 1% </li></ul></ul>-Marked hypocellular < 25% cellularity -Moderate hypocellular <25-50% -normal cellularity with <30% of remaining cell hematopoietic Very severe aplastic anemia As above but neutrophils < 200/  L Infection present
  38. 38. Presentation of Anemia, Neutropenia and Thrombocytopenia Hemorrhagic lesion of the gums in a patient with aplastic anemia caused by infection with Capnocytophaga ochraceus; such lesions are easily confused with those of herpes simplex.
  39. 39. 26-year-old woman with acute aplastic anemia and 1 day of facial pain/swelling. Mouth open involuntarily due to perioral edema. Needle aspirate of small purplish area near right alar revealed P. aeruginosa.
  40. 40. Aplastic anemia. Oral leukoplakia in dyskeratosis congenita.
  41. 41. Treatment Options Bone Marrow Transplant Growth Hormones Immune Suppressive Therapy Supportive Care
  42. 42. TREATMENT <ul><li>1-Withdrawal of the etiologic agent </li></ul><ul><li>2-Supportive treatment </li></ul><ul><ul><li>transfusion of the blood products, CMV seronegative should be given transfusion from the family members should be avoided to prevent sensitization. </li></ul></ul><ul><ul><li>pooled donor platelets but leads to sensitization </li></ul></ul><ul><ul><li>in refractory cases need HLA matched transfusion </li></ul></ul><ul><ul><li>packed cells filtrated to remove leukocyte and platelets </li></ul></ul><ul><ul><li>iron overload : give chelating therapy deferoxamine </li></ul></ul><ul><ul><li>CMV prophylaxis </li></ul></ul><ul><ul><li>Staph. Aeureus * hospitalization * menses </li></ul></ul>
  43. 43. <ul><li>3.Allogeneic BMT </li></ul><ul><li>This is the best therapy for the young patient with a fully histocompatible sibling donor </li></ul><ul><li>usually indicated for most patients with severe disease. </li></ul><ul><ul><li>-Preferably from sibling </li></ul></ul><ul><ul><li>-Curative in 60-90% of patients </li></ul></ul><ul><ul><li>-Applicable only for a third of patients </li></ul></ul>
  44. 44. 4.SPLENECTOMY <ul><li>Removal of the spleen </li></ul><ul><li>does not increase hematopoiesis but may increase neutrophil </li></ul><ul><li>increase platelet counts two- to threefold </li></ul><ul><li>improve survival of transfused red cells </li></ul><ul><li>platelets in highly sensitized individuals </li></ul>
  45. 45. 5.Immunosuppression <ul><li>Antithymocyte globulin(ATG) induces hematologic recovery (independence from transfusion and a leukocyte count adequate to prevent infection. </li></ul><ul><ul><li>Cyclosporin + ATG </li></ul></ul><ul><ul><li>Corticosteroids </li></ul></ul><ul><ul><li>High dose cyclophosphamide </li></ul></ul><ul><li>6. G-CSF/ GM-CSF/ EPO </li></ul><ul><ul><li>**Response rate 50-70% Occurs 2-3 months post Rx. </li></ul></ul>
  46. 46. <ul><li>Newer </li></ul><ul><ul><li>*Mycophenolate mofetil (MMF) - cytotoxic to T cells </li></ul></ul><ul><ul><li>*Monoclonal Ab against IL-2 receptor which is present on activated lymphocytes </li></ul></ul>
  47. 47. Outcomes <ul><li>Age, Younger is better </li></ul><ul><li>BMT </li></ul><ul><ul><li>< 20 yr with a sib… 75% </li></ul></ul><ul><ul><li>20 - 40 yr with a sib…60% </li></ul></ul><ul><ul><li>< 20 yr unrelated BMT… 40% </li></ul></ul><ul><ul><li>20 - 40 yr unrelated BMT…35% </li></ul></ul><ul><li>Immunosuppression - 60 - 80% </li></ul>
  48. 49. Thank you ?

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