Megaloblastic anemia and pernicious anemia

Megaloblastic anemia and pernicious anemia are type of anemia which are rare but irreversible type of anemia that can be controlled.

1. Megaloblastic anemia & pernicious anemia Name : Sudha Sanjibanee Roll no:17 2 nd year Bsc.nursing Gopabandhu Institute of Medical Science And Research School and College of Nursing, Madhapur,Athagarh, Cuttack

2. MEGALOBLASTIC ANEMIA Megaloblastic anemias refer to anemia with characteristics morphological changes caused by defective DNA synthesis and abnormal RBC maturated. The RBCs are large (macrocyte) and abnormal and are referred to as megaloblasts. Macrocytic RBCs are early destroyed because of their fragile membranes.

3. DEFINITION Megaloblastic anemia is a subset of macrocytic anemias characterized by increased RBC size and an arrest in nuclear maturation arising from abnormal cell division in erythroid precursors. OR Megaloblastic anaemia is a red blood cell disorder due to the inhibition of DNA synthesis during erythropioesis. M E G A L O B L A S T I C A N E M I A

4. INCIDENCE The peak incidence of megaloblastic anaemia was in the age group of 10-30 years (48%), with female preponderance (71%). Over the last two to three decades, incidence of MA seems to be increasing(in developing countries due to deficiency of vitamin B(12) or folic acid) M
E
G
A
L
O
B
L
A
S
T
I
C
A
N
E
M
I
A

5. ETIOLOGY Vitamin B12 (cobalamin) deficiency: Intrinsic factor deficiency (pernicious anemia)
Malabsorption (Crohn disease, celiac disease, chronic pancreatitis, prior gastric or ileal surgery)
Vegan diet (vegan)
Nitrous oxide abuse/toxicity
Diphyllobothrium latum (fish tapeworm) infestation
Drugs (immunosuppressants, isoniazid, metformin, colchicine, H2 blockers, proton pump inhibitors)
HIV Genetic disorders M
E
G
A
L
O
B
L
A
S
T
I
C
A
N
E
M
I
A

6. Vitamin B9(Folic acid) deficiency: Increased demand: pregnancy, hemolytic anemia, chronic dermatitis, hemodialysis
Alcoholism
Dietary deficiency (restricted diets, countries without folate fortification of foods)
Drugs (antimetabolites such as methotrexate, tetracyclines, penicillins, nitrofurantoin, phenobarbital, phenytoin, trimethoprim)
Intestinal dysfunction: Malabsorption occurs in surgery (gastric bypass). M
E
G
A
L
O
B
L
A
S
T
I
C
A
N
E
M
I
A

7. Rare disorders: Orotic aciduria: Deficiency of uridine monophosphate synthase leads to ↓ de novo pyrimidine synthesis that is unresponsive to B12 and folate replacement.
Methylmalonic acidemia: inborn error of amino acid metabolism M
E
G
A
L
O
B
L
A
S
T
I
C
A
N
E
M
I
A Fig : megaloblastic blood cell compared to normal blood cell

8. RISK FACTORS Folate deficiency
Nutritional deficiency
Strict vegan diet:do not eat any meat Or dietary product
Malabsorptive disorders such as blind loops/bacterial overgrowth, sprue, Whipple’s and crohn’s Diphillobothrium latum infection (a competitor for B12 absorption)-very rare Elderly age :ageing decreases ability to absorb vitamin B12 Narcotic abuse and Alcohol use :increases folic acid requirements and have diet deficient in vitamin. M
E
G
A
L
O
B
L
A
S
T
I
C
A
N
E
M
I
A

9. Physiologic or pathologic states of increased metabolic demand Pregnancy
Infancy
Low grade hemolysis
Malignancy
Chronic hemodialysis Autoimmune disease: Hashimoto’s,Vitiligo,Diabetes,Adrenal insufficiency (Schmitt’s Syndrome) M
E
G
A
L
O
B
L
A
S
T
I
C
A
N
E
M
I
A

10. PATHOPHYSIOLOGY vitamin B12 or folate deficiency thymidine synthase function is impaired and DNA synthesis is interrupted but RNA and Protein synthesis remains unimpaired inability to synthesize DNA leads to ineffectual erythropoiesis resulting in excess hemoglobin and enlarged erythroid precursors being produced developing red cell has difficulty in undergoing cell division but RNA continues to be translated and transcribed into protein leading to growth of the cytoplasm while the nucleus lags behind. M
E
G
A
L
O
B
L
A
S
T
I
C
A
N
E
M
I
A

11. Often one or more cell division are skipped leading to a larger than normal cell. There is often erythroid hyperplasia in the marrow but most of these immature cells die before reaching maturity leading to – elevated Lactate Dehydrogenase (LDH) and hyperbilirunemia. M
E
G
A
L
O
B
L
A
S
T
I
C
A
N
E
M
I
A

12. CLINICAL MANIFESTATIONS General manifestations B12 deficiency develops over years, whereas folate deficiency develops in weeks to months.
Depending on the degree of deficiency and time of onset, patients can be asymptomatic. M
E
G
A
L
O
B
L
A
S
T
I
C
A
N
E
M
I
A

13. Signs and symptoms of anemia: Fatigue
Shortness of breath
Tachycardia
Palpitations
Pallor
Jaundice GI symptoms (related to underlying GI conditions such as inflammatory bowel disease): Diarrhea
Bloating
Epigastric/abdominal pain M
E
G
A
L
O
B
L
A
S
T
I
C
A
N
E
M
I
A

14. Specific manifestations Neurologic symptoms most commonly due to B12 deficiency:
Subacute combined degeneration (classic finding): Dorsal column: vibration, proprioception (wide-based gait)
Lateral corticospinal tracts: spasticity
Dorsal spinocerebellar: ataxia Neuropathy: tingling, numbness
Psychosis, depression, irritability
Cognitive impairment, forgetfulness
Dementia M
E
G
A
L
O
B
L
A
S
T
I
C
A
N
E
M
I
A

15. Additional findings in B12 deficiency: Oral mucosa pathology (present in 50%–60% of patients): Glossitis
Angular cheilitis
Recurrent oral ulcers
Diffuse erythematous mucositis/mucosal atrophy
Mouth soreness/burning sensation Cutaneous hyperpigmentation
↑ Risk of gastric cancer in pernicious anemia Folate deficiency: ↑ risk of neural tube defects (congenital anomaly) M
E
G
A
L
O
B
L
A
S
T
I
C
A
N
E
M
I
A

16. Fig : Peculiar cutaneous hyperpigmentation from cases with megaloblastic anemia M
E
G
A
L
O
B
L
A
S
T
I
C
A
N
E
M
I
A

17. DIAGNOSTIC EVALUATION History collection : Diet: vegan or vegetarian,Medical and social history: look for autoimmune disorders, alcoholism,Surgical history: gastric or ileal resection,GI symptoms,Neurologic symptoms,Medications Physical examination : evidence of anemia,lemon yellow pallor, pigmentation etc. M
E
G
A
L
O
B
L
A
S
T
I
C
A
N
E
M
I
A

18. Complete Blood Count (CBC):↓ Hb/Het, ↑ MCV. ↓ retics, WBC, Plts, macroovalocytosis, anisocytosis, poikilocytosis, hypersegmentation of granulocytes. Also there may be variable thrombocytopenia. Bone marrow smear: Bone marrow examination reveals myeloid cell changes (giant bands, metamyelocytes and hypertsegmentation) and megakariocytes are decreased and show abnormal morphology. Peripheral blood smear :sample of blood is viewed under a microscope to count different circulating blood cells (red blood cells, white blood cells, platelets, etc.) and see whether the cells look normal. M
E
G
A
L
O
B
L
A
S
T
I
C
A
N
E
M
I
A

19. Schilling test Anti-intrinsic factor antibodies Antiparietal cell antibodies Intrinsic Factor Blocking Antibody Testing
Orotic aciduria: normal urine ammonia with ↑ orotic acid levels For pernicious anemia M
E
G
A
L
O
B
L
A
S
T
I
C
A
N
E
M
I
A

20. Fig :Bone marrow aspirate in megaloblastic anemia with cutaneous hyperpigmentation M
E
G
A
L
O
B
L
A
S
T
I
C
A
N
E
M
I
A

21. MEDICAL MANAGEMENT Increasing amount of folic acid in diet and administering 1 mg of folic acid daily.Folic acid administered intramuscularly only for people with malabsorption problems.After hemoglobin level returns to normal, folic acid replacement can be stopped.
Vitamin B12 deficiency treated by vitamin B12 replacement.
Vegetarian supplements through vitamins or fortified soy milk.
If underlying disorders (e.g., Crohn’s disease, tropical sprue, celiac sprue, blind loop syndrome, inborn errors of metabolism) are the cause of these vitamin deficiencies, appropriate treatment for the specific disorder is required. M
E
G
A
L
O
B
L
A
S
T
I
C
A
N
E
M
I
A

22. For vit B12 deficiency:Hydroxocobalamin injection 1mg/1ml is the 1 st drug of choice. For folic acid:Folic acid 5mg tablets is prescribed. Preventive (prophylactic) folate supplementation may be recommended for individuals who have higher-than-normal demands for folate such as pregnant women. M
E
G
A
L
O
B
L
A
S
T
I
C
A
N
E
M
I
A

23. NURSING DIAGNOSIS Activity intolerance related to imbalance between oxygen supply and demand as evidenced by generalized weakness. Fatigue related to decreased hemoglobin and diminished oxygen-carrying capacity of the blood as evidenced by Inability to maintain usual level of physical activity. Knowledge deficient related to complexity of treatment as evidenced by unfamiliarity with the disease condition. M
E
G
A
L
O
B
L
A
S
T
I
C
A
N
E
M
I
A

24. NURSING MANAGEMENT Asses vitals,blood analysis. Provide Nutritious diet.
Administration of 1 mg folic acid a day (oral) as prescribed.
For patients with malabsorption, folic acid is administered intramuscularly (IM) as directed. Encourage self care. Educate patient and his/her family about the condition and treatment. Provide psychological support. M
E
G
A
L
O
B
L
A
S
T
I
C
A
N
E
M
I
A

25. Pernicious anemia Pernicious is a term that means destructive, injurious, deadly. Pernicious Anemia is due to an inability to absorb vitamin B-12 and due to lack of intrinsic factor (IF) in the gastric mucosa.In this type Anaemia RBC’s are larger than normal and Die Earlier than the 120 Days Life Expectancy.

26. DEFINITION Pernicious anemia is defined as a type of vitamin B12 deficiency that results from impaired uptake of vitamin B-12 due to the lack of a substance known as intrinsic factor (IF) produced by the stomach lining.
Pernicious anemia is a condition caused by too little vitamin B12 in the body. It is one form of vitamin B12 deficiency anemia. P E R N I C I O U S A N E M I A

27. INCIDENCE Its prevalence is 0.1% in the general population and 1.9% in subjects over the age of 60 years according to a 2012 studyTrusted Source in the Journal of Blood Medicine. In India, pernicious anaemia is uncommon & is more common in people of Northern European and African descent. P
E
R
N
I
C
I
O
U
S
A
N
E
M
I
A

28. ETIOLOGY Defective gastric secretion of Intrinsic factor (IF) from parietal cells of gastric mucosa Congenital
Following partial or complete gastrectomy
Autoimmune gastric atrophy Hypothyroidism(20times more frequent)
Chronic atrophic gastritis Heavy alcohol, hot tea, smoking More common in elderly P
E
R
N
I
C
I
O
U
S
A
N
E
M
I
A

29. RISK FACTORS Having a family history of the disease Race: Northern European or Scandinavian descent Underlying disease :type 1 diabetes mellitus, an autoimmune condition, or certain intestinal diseases such as Crohn’s disease
having had part of your stomach removed Age :60 years or older P
E
R
N
I
C
I
O
U
S
A
N
E
M
I
A

30. PATHOPHYSIOLOGY Due to etiological factors Parietal cells located within the gastric mucosa are destroyed
Reduced IF secretion
Failure of absorption of dietary Vit B12
Deficiency of Vit B12 affects growth & maturity of all body cells Megaloblastic Glossitis peripheral neuropathy Anemia P
E
R
N
I
C
I
O
U
S
A
N
E
M
I
A

31. CLINICAL MANIFESTATIONS Signs and symptoms may take a while to be noticed by the patient.(*=most common in pernicious anemia)
Remember the mnemonic: Pernicious P ale E nergy gone (very fatigued)* R ed, smooth tongue* N umbness or tingling in hands and feet* P
E
R
N
I
C
I
O
U
S
A
N
E
M
I
A

32. I ntestinal issues (abdominal bloating, diarrhea/constipation, indigestion) C onfusion I ncreased sadness (depression)
l O ss of appetite (taste changes and weight loss) U nsteady gait (clumsy) S hortness of breath with activity P
E
R
N
I
C
I
O
U
S
A
N
E
M
I
A

33. Fig :pale skin and red smooth tongue

34. DIAGNOSTIC EVALUATION History collection: lifestyle, underlying disease, History of fever, fatigue,malaise. Physical examination: For sign and Symptoms CBC: to measure RBCs, hgb, hct Schilling test: confirms poor absorption as the cause of cobalamin deficiency P
E
R
N
I
C
I
O
U
S
A
N
E
M
I
A

35. Blood smear to look at RBC (will appear large and oval)
Vitamin b12 level
Intrinsic factor antibody assay
Bone marrow aspiration and biopsy Anti-intrinsic factor antibodies: high specificity for pernicious anemia
Antiparietal cell antibodies: can be present in gastritis P
E
R
N
I
C
I
O
U
S
A
N
E
M
I
A

36. COMPLICATION Gastric cancer Peripheral nerve damage
digestive tract problems
memory problems, confusion, or other neurological symptoms
heart problems,heart failure P
E
R
N
I
C
I
O
U
S
A
N
E
M
I
A

37. MEDICAL MANAGEMENT The goal of the treatment is to increse your vitamin B12 level.
Eating foods high in vitamin B12 and folic acid can help to prevent vitamin B12 deficiency caused by a poor diet.
Some people may also need to take vitamin B12 supplement by mouth.
Certain type of vitB12 may be given through the nose. P
E
R
N
I
C
I
O
U
S
A
N
E
M
I
A

38. Vitamin B12 is available for therapeutic use parenterally as either cyanocobalamin or hydroxocobalamin Multivitamins are used as dietary supplements. Intrinsic factor, replacement is by intramuscular (IM) injection of Vitamin B12. P
E
R
N
I
C
I
O
U
S
A
N
E
M
I
A

39. NURSING DIAGNOSIS Fatigue related to Decreased hemoglobin and diminished oxygen-carrying capacity of the blood As evidenced by Report of fatigue and lack of energy.
Deficient knowledge related to disease condition as evidenced by frequently asked questions.
Risk for heart failure and neurological deficits secondary to disease condition.
Inadequate body metabolism and absorption related Malabsorption
Anxiety, confusion related to disease conditions P
E
R
N
I
C
I
O
U
S
A
N
E
M
I
A

40. NURSING MANAGEMENT Check vitals
Assess input & output chart.
Educate patient about the disorderand treatment.
Encourage a healthy diet.If the patient is vegetarian, encourage cobalamin supplements Administer vitamin b 12 injections (intramuscular….not orally because they aren’t absorbing it in the GI system) as ordered by doctor. P
E
R
N
I
C
I
O
U
S
A
N
E
M
I
A

41. Educated on safety: due to risk of injuries from unsteady gait…more clumsy from the paresthesia
Check blood work to ensure the patient is not anemic
Look at tongue (it is often beefy red- it resolves with treatment) Maintain good oral hygiene due to changes to tongue.
Encourage self-care
Check reticulocyte count to ensure that the treatment is working
Provide oxygen is patient is short of breath
Consult with a dietitian to educate the patient P
E
R
N
I
C
I
O
U
S
A
N
E
M
I
A

42. T H A N K y o u