Anemia

BLOOD DISORDERS

1. anemia RATHEESH R.L

2. definition Deficiency in the number of erythrocytes(RBC), the quantity of hemoglobin and/or the volume of packed RBCs

3. classification Etiologic (underlying cause) Morphologic (cellular characteristic ) Classification based on signs and symptoms

4. Etiologic classification Decreased erythrocyte production Blood loss Increased erythrocyte destruction

5. Decreased erythrocyte production Decreased hemoglobin synthesis * Iron deficiency * Thalassemia (decreased globin synthesis) Defective DNA synthesis * Cobalamin deficiency * Folic acid deficiency Decreased number of erythrocyte precursors * Aplastic anemia * Anemia of myeloproliferative diseases(leukemia)

6. Blood loss Acute * Trauma * Blood vessel rupture Chronic * Gastritis * Hemorrhoids * Menstrual flow

7. Increased erythrocyte destruction Abnormal hemoglobin( Sickle cell anemia) Infectious agents Physical trauma

8. Morphologic classification Normocytic normochromic Macrocytic normochromic Microcytic hypochromic

9. Classification based on signs and symptoms Mild Moderate severe

10. Normocytic normochromic Normal size and colour Causes include acute blood loss, chronic kidney disease, aplastic anemia, sickle cell anemia

11. Macrocytic normochromic Large size normal colour Causes include cobalamin deficiency, folic acid deficiency, liver disease, post splenectomy

12. Microcytic hypochromic Small size pale colour Causes include iron deficiency anemia, Thalassemia

13. Based on the clinical manifestations Mild( Hb - 10-14gm/dl) * cardiovascular: palpitations * Pulmonary : Exertional dyspnea Moderate ( Hb - 6- 10 gm/dl) * Cardiovascular: Increased palpitations * Pulmonary: Dyspnea * General: Fatigue

14. Based on the clinical manifestations…. Severe( Hb - less than 6gm/dl) * Integumentary : Pallor, pruitus * Eyes: Icteric conjuctiva and sclera, blurred vision * Mouth: Glossitis , Smooth tongue * Cardiovascular: Tachycardia, increased pulse pressure, murmurs * Pulmonary: tachypnea , orthopnea , dyspnea at rest

15. Severe…. * Neurologic: Headache, Vertigo, irritability, depression, impaired thought process * Gastro intestinal: Anorexia, hepatomegaly , splenomegaly , difficulty in swallowing, sore mouth * Musculo skeletal: Bone pain * General : sensitivity to cold, weight loss and lethargy

16. anemia caused by decreased erythrocyte production Decreased Hb synthesis Iron deficiency anemia, Thalassemia Defective DNA synthesis in RBC megaloblastic anemia Diminished availability of erythrocyte precursors Aplastic anemia, Anemia of myeloproliferative diseases(leukemia)

17. Decreased Hb synthesis

18. Iron deficiency anemia Normal iron metabolism Obtained from food and dietary supplements Ingested iron absorbed in the duodenum and upper jejunum Iron present in all RBC as heme in hemoglobin and in a stored form( ferritin and hemosiderin ) in the bone marrow, spleen, liver and macrophages

19. Daily Iron Demands Male 1 mg Adolesc. 2-3 mg Women in repr.age 2-3 mg Pregnant 3-4 mg

20. S mall loss of iron each day in urine, faeces , skin and in menstrua ting females as blood (1-2 mg daily) Normal diet contains about 15 mg of iron/day 6mg elemental iron/1000 cal 1/10 of ingested iron is absorbed Gastric acid releases iron from food

21. definition Iron deficiency anemia is defined as anemia associated with either inadequate absorption or excessive loss of i ron from the body.

22. etiology Inadequate dietary intake Malabsorption * After certain types of gastro intestinal surgery(removal of bypass of duodenum) * Malabsorption syndromes( diesases of the duodenum)

23. Etiology… Blood loss * sources of chronic blood loss are from GI and GU systems * Causes of GI blood loss – peptic ulcer, gastritis, esophagitis , hemorrhoids and neoplasms * causes of GU blood loss – menstrual bleeding * At the time of delivery and lactation

24. Etiology… Pregnancy - Diversion of iron to the fetus for erythropoiesis Dialysis treatment - blood lost in the dialysis equipment and frequent blood sampling

25. Clinical manifestations Fatigability Dizziness Headache Irritability palpitation Glossitis Stomatitis Dry pale skin Spoon shaped nails, koilonychia Hair loss Splenomegaly

26. Diagnostic measures Hb,H ct ,RBC: Low Plt: Normal/Low/High WBC: Normal/Low S iron: Decreased

27. Diagnostic measures…. History collection and physical examination Stool routine: to identify the presence of blood Endoscopy or colonoscopy: used to detect GI bleeding

28. treatment Replace iron and folic acid and treat underlying disease. Oral route is preferred for replacement. Intake of liver and muscle meats, eggs, dried fruits, legumes, dark green leafy vegetables, bread and cereals and potatoes If iron deficiency from acute blood loss require transfusion of packed RBC

29. Treatment…. Response can be followed by retic. increase in 1-2 weeks ( 5-7 days ) Hb response to treatment half normal by a month returns to normal by 2-4 months Replacement therapy is prolonged by 6-12 months to replenish stores of iron. Ongoing bleeding may cause indefinite therapy.

30. Treatment…. DRUG THERAPY Oral iron is usually prescribed Total daily dose:150-200 mg elemental iron Give in 3-4 divided doses (each tablet contains 50 to100mg of iron) Each one hour before meals. Taking iron with Vit C enhances iron absorbtion

31. Treatment… Undiluted liquid iron may stain patients teeth, therefore diluted and ingested through a straw Iron preparations cause the stools to become black(GI tract excretes excess iron) Constipation is common, therefore started on stool softeners

32. Treatment… Parenteral iron therapy: Indications Malabsorbtion Intolerance to oral replacement Colitis/enteritis Needs in excess of amount that can be given orally Patient uncooperative/poor compliance Hemodialysis

33. Treatment… Parenteral iron therapy: Given intramuscularly or intravenously Iron dextran complex contains 50mg/ml of elemental iron in 2ml Test dose of parenteral iron is often done to assess for allergic reaction Given deep IM in the outer quadrant of the buttocks with a 18 to 20 gauze needle

34. thalassemia Group of diseases that have an autosomal recessive genetic basis involving inadequate production of normal hemoglobin Normal hemoglobin is composed of 2 alpha and 2 beta globins Mutations in a given globin gene can cause a decrease in production of that globin, resulting in deficiency Absence of alpha globin chain alpha thalassemia Absence of beta globin chain beta thalassemia

35. Clinical manifestations Growth both physical and mental is retarded Person with thalassemia major is pale and displays other symptoms of anemia( Inadequate production + ineffective erythropoiesis + haemolysis  Anaemia ) Symptoms develop in childhood by 2 years of age Pronounced hepatosplenomegaly ( ↑ Haemolysis  ↑demands of phagocytic function hyperplasia of phagocytes  Hepatosplenomegaly )

36. Clinical manifestations…. To compensate anaemia extramedullary haemopoiesis in liver, spleen & brain  Organomegaly ↑ Erythropoiesis  marrow expansion & thinning of cortex of skull bone  Thalassaemia facies Jaundice from RBC hemolysis

37. Diagnostic measures Hb concentration – Decreased ESR – Mild increase RBC count – Markedly decreased Reticulocyte count – Increased

38. treatment Thalassemia minor requires no treatment Treatment of thassemia major includes Chronic Transfusion Therapy Maximizes growth and development Suppresses the patient’s own ineffective erythropoiesis and excessive dietary iron absorption RBC transfusions often monthly to maintain Hgb 10-12

39. Chelation Therapy Binds free iron and reduces hemosiderin deposits 8-hour subcutaneous infusion of deferoxamine , 5 nights/week Start after 1year of chronic transfusions Splenectomy --indications Trasfusion requirements increase 50% in 6months Severe leukopenia or thrombocytopenia

40. Defective DNA synthesis in RBC

41. Megaloblastic anemia/ cobalamine deficiancy anemia Group of disorders caused by impaired DNA synthesis and characterized by the presence of large RBC Impaired DNA synthesis defective RBC maturation Large ( macrocytic ) and abnormal RBC megaloblasts

42. Cobalamin ( Vit b12 ) deficiency Vitamin B12 Sources : Meat, fish Daily requirement : 2-5 micro gram Body stores : 3-5 mg( liver) Places of absorption: distal ileum

43. etiology Result from cobalamin ( Vit B12) and folic acid deficiencies Supression of DNA synthesis by * drugs * inborn errors of cobalamin and folic acid metabolism * erythroleukemia ( malignanat blood disorder characterized by proliferation of erythropoietic cells in bone marrow)

44. etiology 1. Malabsorption a) Inadequate production of intrinsic factor - pernicious anemia - gastrectomy, partial or total b) Inadequate releasing vit. B 12 from food (partial gastrectomy, abnormality of stomach function, chronic pancreatic insufficiency)

45. Malabsorption … c) Terminal ileum disease (celiac disease, ilea l resection, Crohn disease) d) Competition for intestinal B 12 : - bacterial overgrowth: jejunal diverticula, intestinal stasis an d obstruction due to strictures - Fish tapeworm

46. Etiology…. 2. Inadequate intake - vegetarians 3. Inadequate ut i lisation Drugs: Neomycin, Colchicin e , Nitrous oxide ,long term use of H2 receptor blockers

47. Clinical manifestations Atrophic glossitis (shiny tongue) Abnormal gait Anemia related manifestations Personality changes Anorexia Nausea, vomiting Abdominal pain

48. Clinical manifestations… Neurologic manifestations Paresthesias Memory loss Numbness Weakness Symmetric neuropathy legs>arms Severe weakness, spasticity, paraplegia and incontinence Subacute combined degeneration of the dorsal (posterior) and lateral spinal columns

49. Diagnostic measures 1. Blood cell count: macrocytic anemia Thrombocytopenia leucopenia (granulocytopenia) low reticulocyte count 2. Blood smear: H ypersegmentation of granulocytes

50. Diagnostic measures…. 3. Bone marrow smear Hypercellular E rythroid cell changes (megaloblasts, RBC precursor a abnormally large with nuclear- cytoplasmic asynchrony) M egakar y ocytes are decreased and show abnormal morphology

51. treatment If the patient has dietary deficiency of cobalamin dietary sources rich in cobalamin should be provided

52. Treatment…. 1. Vitamin B 12 administration intramuscular in dose 1000 μg per day for a week , then 100 μg per day for 2 weeks, per week 100μg for month 2. Reticulocytosis begins 2 or 3 days after therapy started and maximal number reached on day 5 to 8. * Serum iron monitoring, after 7-10 days of vit.B12 treatment, * if Fe deficiency is diagnosed , start iron substitution 3. 100 ug vit.B 12 i.m. every month, regimen that must be mainted for the rest on the patients life.

53. Folic acid deficiency Folic acid deficiency Megaloblastic anemia Folic acid is required for DNA synthesis, leading to RBC formation and maturation

54. Folic acid Sources : G reen vegetables, yeast Daily requirement : 50-100 ug Body stores : 10-12mg (liver) Places of absorptio n : duodenum and proxymal segment of small intestine

55. etiology 1. Inadequate intake - diet lacking leafy green vegetables, liver, citrus fruits, dried beans, nuts and grains ; chronic alcoholism, total parenteral nutrition 2. Malabsorption - small bowel disease ( celiac disease) - alcoholism

56. Etiology…. 3. Increased requirements: - pregnancy and lactation - infancy - chronic hemolysis - malignancy - hemodialysis 4. Defective utilisation Drugs:folate antagonists(methotrexate, trimethoprim), purine analogs (azathioprine), primidine analogs (zidovudine), RNA reductase inhibitor (hydroxyurea), miscellaneous (phenytoin)

57. Clinical manifestations Clinical features similar to those of cobalamin deficiency GI disturbances include dyspepsia and a smooth red tongue Absence of neurologic problem

58. Diagnostic measures Hemoglobin – Decreased S. Folate level - Low

59. treatment Treated by replacement therapy 1. Oral administration of folate 1 (5) mg per day, for 3 months, and maintance therapy if it’s necessary. 2. Reticulocytosis after 5-7 days 3. Correction of anemia is over after 1-2 months of therapy

60. Treatment… Eat foods containing large amounts of folic acid

61. Diminished availability of erythrocyte precursors

62. ANEMIA OF CHRONIC DISEASE Chronic inflammatory, autoimmune, infectious, or malignant diseases can lead to anemia and it is known as anemia of chronic disease Associated with under production of RBC and shortening of RBC survival

63. ETIOLOGY Chronic conditions leading to anemia Renal diseases Autoimmune hemolysis Chemotherapy and radiation therapy Infections Hypopitutirism & Hypothyroidism

64. Diagnostic measures Findings of elevated serum ferritin and increased iron stores distinguish it from iron deficiency anemia. Normal folate and cobalamin blood levels distinguish it from those types of anemias .

65. management Correction of the underlying disorder Blood transfusions Erythropoetin therapy( Epogen , Procit )

66. Aplastic anemia Rare disease caused by a decrease in or damage to marrow stem cells, damage to the microenvironment within the marrow, and replacement of the marrow with fat. It results in bone marrow aplasia (markedly reduced hematopoiesis ).

67. Aplastic anemia Characterized by peripheral blood pancytopenia ( decrease of all blood types- RBCs, white blood cells and platelets)

68. etiology Congenital causes Fanconi syndrome( disease of the proximal renal tubules in which glucose, uric acid and amino acids are not absorbed properly) Congenital dyskeratosis ( abnormal pigmentation of the skin will occur) Amegakaryocytic thrombocytopenia( it’s a hematological disease characterized by severe thrombocytopenia due to altered immunological status)

69. Etiology…. Acquired causes Exposure to ionizing radiation Chemotherapy Chemical agents( benzene, arsenic, alcohol) Viral and bacterial infections Medications( Anti seizure agents, anti microbials )

70. Clinical manifestations General manifestations of anemia( fatigue, dyspnea along with cardiovascular and neurologic responses) Patient with neutropenia susceptible to infection and may be febrile Thrombocytopenia is manifested by a predisposition to bleeding( petechiae , epistaxis )

71. Diagnostic measures Decreased Hb , WBC, and platelet values Normocytic , normochromic anemia. Low reticulocyte count. Prolonged Bleeding time . Elevated serum iron and total iron-binding capacity (TIBC) Bone marrow biopsy - hypocellular with increased yellow marrow (fat content).

72. management Identify and remove the causative agent Hematopoietic stem cell transplant and immuno suppressive therapy with anti thymocyte globulin(ATG) and cyclosporine or high dose cyclo phosphamide

73. ANEMIA CAUSED BY BLOOD LOSS

74. Acute blood loss Chronic blood loss

75. etiology Trauma Complications of surgery Conditions or diseases that disrupt vascular integrity.

76. Clinical manifestations Volume lost 10 % None Volume lost20% No detectable signs or symptoms at rest, tachycardia with exercise and slight postural hypotension Volume lost30% Normal supine blood pressure and pulse rate at rest ,postural hypotension and tachycardia with exercise.

77. Clinical manifestations Volume lost 40% Blood pressure,central venous pressure,and cardiac output below normal at rest, rapid,thread pulse and cold clamy skin. Volume lost 50% Shock and potential death

78. Clinical manifestations Alert to patients expression of pain Internal hemorrhage cause pain Retro peritoneal bleed- may not experience abdominal pain

79. management IV fluids - dextran , hetastarch , albumin, and/or crystalloid electrolyte solutions such as lactated Ringer's. Blood transfusions (packed RBCs) may be needed if the blood loss is significant. If the bleeding is related to a platelet or clotting disorder, replacement of that deficiency is addressed. supplemental iron  

80. CHRONIC BLOOD LOSS Bleeding ulcer Hemorrhoids menstrual and postmenopausal blood loss etc.

81. INCREASED ERYTHROCYTE DESTRUCTION

82. Hemolytic anemia Condition caused by destruction or hemolyis of RBCs at a rate that exceeds production. Occur because of problems intrinsic or extrinsic to the RBC

83. etiology Intrinsic hemolytic anemia( heriditary ) *Abnormal hemoglobin Sickle cell anemia Thalassemia *Red blood cell membrane abnormality Heriditory spherocytosis *Enzyme deficiencies G6 PD deficiency ( glucose-6-phosphate dehydrogenase)

84. Etiology… Extrinsic hemolytic anemia( Acquired) * damage is caused by external factors such as trapping of cells within the sinuses of the liver or spleen * Antibody mediated destruction(Auto immune hemolytic anemia)

85. Clinical manifestations General symptoms of anemia Jaundice Enlarged liver and spleen

86. management Focus of treatment is to maintain renal function

87. Sickle cell disease Group of inherited, autosomal recessive disorders characterized by the presence of an abnormal form of Hb in the erythrocyte. This abnormal Hb , hemoglobin S ( Hb S), causes the erythrocyte to stiffen and elongate taking on a sickle shape in response to low oxygen levels .

88. etiology Sickle cell anemia Sickle cell thalassemia Sickle cell Hb C disease Sickle cell trait

89. Sickle cell anemia Occurs when is homozygous for hemoglobin S; the person has inherited Hb S from both parents.

90. Sickle cell thalassemia and sickle cell Hb C Occurs when a person inherits Hb S from one parent and another type of abnormal hemoglobin( thalassemia or Hb C) from other parent

91. Sickle cell trait Occurs when a person is heterozygous for Hb S; the person inherits hemoglobin S from one parent and normal hemoglobin( Hb A) from another parent

92. Sickling episodes Triggered by low oxygen tension in the blood Hypoxia can be caused by * viral or bacterial infection * high altitude * emotional or physical stress * blood loss

93. Sickling episodes… Sickled RBC become rigid and take an elongated cresent shape Cannot easily pass through capillaries Cause vascular occlusion Acute or chronic tissue injury

94. sIckle cell crisis Blood flow is impaired by sickled cells Vasospasm occurs Severe capillary hypoxia Changes in membrane permeability Plasma loss Hemo -concentration and development of thrombi Further circulatory stagnation

95. Sickle cell crisis… Tissue ischemia, infarction and necrosis occurs from lack of oxygen Shock can occur (life threatening consequence)

96. Clinical manifestations Features of anemia( pallor of mucous membranes, decreased exercise tolerance) Jaundice Primary symptom associated with sickling is pain - pain is severe - affect an area of the body with the back, chest, extremities and abdomen being mostly affected

97. Clinical manifestations…. Damaged vision: The sickled blood cells, often clog the blood vessels that connect to the retina, causing optical damage. Limited Growth: The scarcity of oxygen caused by sickle-cell anemia is detrimental to healthy human growth.

98. Diagnostic measures Peripheral blood smear Sickling test Electrophoresis of hemoglobin Skeletal X- rays MRI scan Doppler studies

99. complications Infections Pulmonary complications Pneumonia Acute chest syndrome Pulmonary hypertension Cardiac complications MI HF corpulmonale .

100. Complications….. Ophthalmic complications Retinal vessel obstruction may result in Hemorrhage Scarring retinal detachment blindness. Renal complications Renal failure. Neurologic complications Stroke

101. Complications….. Musculoskeletal complications Osteoporosis Osteosclerosis Integumentory system complications Chronic leg ulcers Genitourinary system complications Priapism

102. management No specific treatment for the disease Focus on alleviating the symptoms and minimizing end organ damage Instruct to avoid high altitude, maintain adequate fluid intake and treat infections promptly Chronic leg ulcers treated with bed rest, antibiotics, warm saline soak, mechanical debridement and grafting

103. sickle cell crisis management Oxygen administration Fluid administration Transfusion therapy Pain management Acute chest syndrome treated with broad spectrum antibiotics, oxygen therapy and fluid therapy Antisickling agents( Hydroxy urea)

104. Management…. Hematopoietic stem cell transplantation * Allogenic * Syngenic * Autologous

105. NURSING DIAGNOSIS High risk of infection related to an inadequate secondary defenses (decreased hemoglobin leucopenia, or a decrease in granulocytes (inflammatory response depressed ). Imbalanced nutrition less than body requirement related to failure to digest or inability to digest the food / nutrient absorption necessary for the formation of red blood cells

106. Activity intolerance  related to imbalance between oxygen supply (delivery) and demand. Altered tissue perfusion related to decreased cellular components required for the delivery of oxygen / nutrients to the cells High risk of  damage  to skin integrity related to circulatory and neurological changes

107. Constipation  or diarrhea related to decreased  dietary  inputs; changes in the digestive process; the side effects of  drug therapy . knowledge in relation to the lack of exposure / recall; incorrect interpretation of information; do not know the source of information