![INTRODUCTION
DEFINITION
• Autoimmune hemolytic anemia[AIHA] is characterized by an
abnormal production of auto antibodies[Ig G,M.E,A] that bind
to Antigens on the erythrocyte surface. These antibodies then
lead to destruction of RBC’s thus shortening their life span.
• Red cell lifespan may decrease to 30days.
CLASSIFICATION
• Warm AIHA
• Cold AIHA – Cold Agglutinin Syndrome
Paroxysmal Cold Hemoglobinuria
• Mixed type AIHA
• Drug Induce Immune hemolytic anemia](data:image/gif;base64,R0lGODlhAQABAIAAAAAAAP///yH5BAEAAAAALAAAAAABAAEAAAIBRAA7)
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autoimmune hemolytic anemia
- 1. CASE PRESENTATION ON AUTO-IMMUNE HEMOLYTIC ANEMIA BY M.HEMA SREE PHARM D 4TH YEAR
- 2. INTRODUCTION DEFINITION • Autoimmune hemolytic anemia[AIHA] is characterized by an abnormal production of auto antibodies[Ig G,M.E,A] that bind to Antigens on the erythrocyte surface. These antibodies then lead to destruction of RBC’s thus shortening their life span. • Red cell lifespan may decrease to 30days. CLASSIFICATION • Warm AIHA • Cold AIHA – Cold Agglutinin Syndrome Paroxysmal Cold Hemoglobinuria • Mixed type AIHA • Drug Induce Immune hemolytic anemia
- 3. WARM AIHA • In this warm AIHA, the autoantibodies attach to & destroy the RBC at temperature ≥ to 37 degrees celcius. • More common among women. • MECHANISM OF DESTRUCTION: Due to Extravascular hemolysis • Associated with development of Ig G • May be due to primary or secondary etiology Primary – idiopathic Secondary –due to underlying disease[eg: lymphoproliferative or autoimmune disorders]
- 4. COLD AIHA • The autoantibodies become most active and attack RBC below normal body temperature. • Mainly effects in middle aged or elderly. • Due to development of IgM & G antibody. • Either primary or secondary in etiology • MECHANSIM OF DESTRUCTION: Extravascular and Intravascular hemolysis COLD AGGLUTINATION SYNDROME – IgM antibodies • Hemolysis – extravascular • Causes – mononucleosis infections, lymphoproliferative disorders) PAROXYSMAL COLD HEMOGLOBINURIA : Ig G antiodies • Intravascular hemolysis • Occurs due to infections( sypihils, pneumoniae, measles, infulenza) also due to vaccines
- 6. MIXED TYPE AIHA • Features similar to WAIHA & CAS • Both IgG & C3d[activated by IgM cold Ab] are detected DRUG INDUCED AIHA • Antibodies directed against or one of its metabolites • All may involve IgG &C3 • Eg: Pencillin , Cephalosporins , Quinidine , Methyldopa
- 7. PATHO or IMMUNOLOGIACL VIEW • WAIA IgG binds to RBC surface antigens This drives monocytes and macrophages to grab & pick off portions of RBC membrane RBC becomes spherocytes Destructed in spleen Extravascular hemolysis
- 8. COLD AGGLUTINATION DISEASE In cold temperature, IgM binds to polysaccharide region of glycoprotein on RBC surface This triggers complement system to lyse RBC If complement system fails to form membrane attack complex, complement proteins deposit on RBC surface This opsonisation enhances RBC phagocytosis in liver, spleen & lungs Extravascular hemolysis
- 9. PAROXYSMALCOLD HEMOGLOBINURIA During infections , microbes trigger formation of Abs which react with p antigen of RBC surface These polyclonol anti-P autoAb binds to P-Ag of RBC in cold temp Activation of complement system, Lysis of RBCs Intravascular hemolysis
- 10. CLINICAL MANIFESTATIONS • Paleness of skin • Fatigue • Fever and chills • Confusion • Lightheadedness • Weakness • Body ache COMMON SYPTOMS LESS COMMON SYMPTOMS • Dark urine • Yellowing of skin and eyes • Increased HR • Enlarged spleen • Enlarged liver
- 11. DIAGNOSIS • CBC : Hb - decreased Hematocrit – decreased Reticulocytes- increased • peripheral smear – microcytic, anisocytosis , spherocytes , hypochromic , poikilocytosis • Other lab data : elevated LDH, indirect bilirubin and decreased haptoglobin • Direct coombs test
- 12. TREATMENT WARM AHIA • Folic acid supplements • Corticosteriods like prednisolone[1- 1.5mg/kg/day] • Immunoglobulins like rituximab, azathioprine etc.. • If ineffective splenectomy considered • Blood transfusion • Splenectomy COLD AIHA • Removal of underlying disease. • Use antibiotics like pencillin in case of infections • Rituximab is given majorly • Avoid cold weather an cold drinks • Blood transfusion • Splenectomy
- 15. DEMOGRAPHIC DETAILS • Patient name: XYZ • Age: 21 Years • Gender: Female • Height:165cm • Weight: 85kg • BMI: 31.2Kg/sqmm[over weight] • IP No:472890 • Dept: General medicine-554 • DOA:11/NOV/19 • DOD:14/NOV/19
- 16. COMPLAINTS • B/L knee pain • Mild swelling at knees • Chills • Headache • Weakness and pale skin • Fatigue HISTORY • Past medical history: Hypothyroid • Past medication history: Tab Thyronorm 100mcg
- 17. PROVISIONAL DIAGNOSIS AUTO-IMMUNE HEMOLYTIC ANEMIA..? Osteoarthritis..? Osteonecrosis..? Jaundice..?
- 18. DAY TO DAY ASSESMENT DAY-1 • O/E patient conscious , coherent and afebrile • c/o bilateral knee pain, Headache(+), Generalised weakness • Hypothyroid (℞ on 1oomcg thyronorm) • No organomegaly and lymphadenopathy • Patient was evaluated outside and given Tab HCQS -400mg od 3 days • Beta thalassemia minor-negative
- 19. Day 2 • B/L Knee pain • Weakness reduced • No other issues • T.GEMFOS[Risedronate]-35mg/week on empty stomach with 1full glass of water, Don’t EAT, LIE DOWN, BEND for 2hrs, can walk and sit Day -3 • B/L Knee pain reduced No weakness No headache • Can be discharged tomorrow
- 20. Day -4 • DISCHARGE RECOMMENDATIONS 1. Consider treatment with biphosphonates and calcium and vitamin D - Tab SHELCAL 5OOmg OD 2. Tab GEMFOS 35mg/Week 3. Tab WYSOLONE 1.5mg/alternate day 4. No squatting, strain, inclines, walking on uneven surface 5. Reduce and maintain weight 6. Physiotherapy 7. Continue Tab THYRONORM 100mcg OD 8. Tab. FOLACIN 800mcg OD
- 21. PHYSICAL EXAMINATION VITALS • RR -19/min • BP -110/7OmmHg • SPO2 – 98% • Respiratory system – BAE (+) No added sounds • CNS – no focal deficits • CVS –S1S2 Heard • LIMBS – distal pulses (+) • ABDOMEN – non-tender, soft
- 22. LABORATORY VALUES PARAMETER RESULT NORMAL RANGE Haemoglobin 10.10gms% 12-16gms% RBC 3.59million/cumm 4.5-5.5 million/cumm PCV 37% 35-48% Platelets 3.63lakhs/cumm 1.5-4.5lakhs/cumm WBC 6,720cells/cumm 4k-10kcells/cumm Hematocrit 12% 37-48% Reticulocytes 3.76% 0.5-2.5% ESR 25mm 0-20mm LDH Total 197 IU/L 120-246 1U/L
- 23. PARAMETER RESULT NORMAL RANGE T.Bilirubin 0.50mg/dl 0.1-1.2 mg/dl BC.Conjugated 0.00mg/dl 0-0.35mg/dl BC.unconjugated 0.99mg/dl 0-0.65mg/dl T.Protein 7.20g/dl 6.3-7.9g/dl Albumin 4.10g/dl 3.5-5.0g/dl Gobulin 3.10g/dl 2-3.5g/dl A/G Ratio 1.30 Approx 1 GGT 25.00 IU/L 8-62 IU/L ALP 64.OO IU/L 40-129 IU/L SGOT 26 IU/L 5-40 IU/L SGPT 23 IU/L 5-40IU/L
- 24. IMMUNOSEROLOGY • Rheumatoid factor assay : <8.6 IU/L [ normal : <121 IU/L] PERIPHERAL SMEAR • RBC Morphology : Microcytic Hypochromic with Anisocytosis, spherocytes • WBC : normal in count & disturbances • PLATELET : Adequate BIOCHEMISTRY LUPUS ANTICOAGULANT • DIL RUSSEL VIPER VEN TIME : 58.00 sec • DRVVT CONTROL : 49.00 sec • DRVVT:DRVVTC Ratio – 1.18 sec • REFERENCE VALUE [ <1.30sec]
- 25. URINE • Total Input- 1300ml • Total Output – 1200ml MRI OF KNEE JOINTS • Findings : few irregular areas of altered signal intensity with T1 isointense &peripheral hyperintense rim, hyperintense on T2 weighed fat • Marrow edema noted in femoral condyle with ill defined margins of sub-chondral altered signal area • Findings suggestive of medullary infracts in distal femur with sub-chondral osteonecrosis in medial femoral condyle • Mildly reduced fibro femoral joint space with reduced articular cartilage thickness[grade-2]
- 26. MEDICATION CHART S. no BRAND NAME GENERIC NAME DOSE FREQ ROA D 1 D 2 D 3 1 T.SHELCAL Ca++ with vit D3 500mg OD PO + + + 2 T.GEMFOS Risedronate 35mg OD PO - + + 3 T. WYSOLONE Prednisolone 1.5 mg Alterna te day PO + - + 4 T.FOLACIN 800 Folic acid 800mc g OD PO + + + 5 T.THYRONOR M levothyroxine 100mc g OD PO + + +
- 27. SOAP ANALYSIS
- 28. SUBJECTIVE EVIDENCE • Fatigue • Chills • Headache • Weakness • Pale skin
- 29. OBJECTIVE EVIDENCE • Haemoglobin- 10.10gms% [low] • Hematocrit -12%[low] • Reticulocytes - 3.76%[ high] • Bc Unconjugated -1.67mg/dl [high] • RBC Morphology – microcytic hypochromic with anisocytosis, spherocytes • Direct Coombs test - positive
- 30. ASSESSMENT • Based on above subjective and objective evidence data patient was diagnosed with or found to be suffering from AUTO- IMMUNE HEAMOLYTIC ANEMIA
- 31. PLANNING GOALS OF TREATMENT • To reduce signs and symptoms • To correct the underlying disease • Reduce or stop the destruction of RBCs • To prevent relapse • Prevent further complications like jaundice, hepatosplenomgaly, hyperbilirubinemia
- 32. TREATMENT OPTIONS Tab. SHELCAL • MOA-calcium improves functioning of muscles, bones and vit D3 increases absorption of calcium and phosphorus . • USES – weak bone, osteonecrosis, hypothyroid, to maintain healthy immune system • SE –constipation, nausea, vomiting, rashes • MP- Ca++ levels, vit D3, bone density Tab. GEMFOS • MOA- it is a biphosphonate binds to hydroxyapatite crystals in bone & inhibits osteoclast - mediated bone resorption • Uses- bone or joint pain • SE – headache, arthralgia constipation • MP – Ca++ levels ,vit d & phosphorus levela
- 33. Tab.WYSOLONE • MOA- Prednisolone is a steriod , modify immune responses of body and decreases hemolysis. • USES- 1st line therapy for AIHA • SE- acne, dry skin, bloating, eye problems • MP- BP, opthalmic exam, blood glucose Tab.FOLACIN • MOA- It helps / stimulates body to produce and maintain blood cells • SE- allergic rxn, fever, bronchospasm • USES- AIHA • MP- Vit B12 and B9 levels
- 34. Tab.THYRONORM • MOA-levothyroxine is synthetic form of T4, binds to thyroid receptors in cell nucleus and causes metabolic effects • USES-Hypothyroid • SE-increased appetite, weight loss , headache, excessive sweating • MP- TSH, T4, and T3 levels
- 35. PROBLEMS IDENTIFIED DRUG INTERACTIONS Risedronate < > calcium ( Moderate ) • Calcium decreases effectiveness and absorption of risedronate into systemic circulation . • MANAGEMENT - 30 mins time gap should be there between the administration of drugs • Levothyroxine < > Calcium ( Moderate ) • Using calcium together with levothyroxine may decrease the effects of levothyroxine. • MANGEMENT : you should separate the administration of L- thyroxine and calcium by at least 4hrs
- 37. GOALS ACHIEVED •B/L knee pain reduced •No headache by the 3rd day •No more weakness
- 38. MONITORING PARAMETERS • Hemogoblin levels • Hematocrit levels • Reticulocytes levels • Indirect bilirubin levels • LDH levels • ESR • Peripheral smear • Haptoglobin
- 39. PATIENT COUNSELLING ABOUT THE DISEASE • The patient must be counselled about the disease i.e., AUTOIMMUNE HEMOLYTIC ANEMIA which is a condition that causes your RBCs to die sooner than normal. And your bone marrow cannot make new RBCs fast enough to replace the ones that have died. • Further the disease complications [jaundice, hemogobinuria, arrhythmias or cardiomyopathy ] also have to be explained.
- 40. ABOUT MEDICATIONS • Tab.GEMFOS -Should be taken with 1 full of glass of water, don’t eat, lie down, bend down, for 2hrs. Can walk and sit. • Tab.THYRONORM-should be taken morning only and a 30mins- 1hr time gap have to be maintained even to drink water. • There should be time gap of 30mins between CALICIUM and RISEDRONATE. • Parameters specific for each drug must be monitored • Follow the medication as per the prescription • Don’t miss the dose and double the dose if missed • If any ADRs or SE seen report it to physician immediately.
- 41. LIFESTYLE MODIFICATIONS • NO squatting/strains/walking on uneven surface. • Reduce weight • Physiotherapy should be continued • Staying away from people who are sick and avoid large crows of people • Stay away from cold temperatures • During cold weather wear a hat, scarf, & warm coat • Take adequate amount of rest • Avoid infections by maintain good hygiene conditions
- 42. DIET FOODS TO BE TAKEN • Leafy greens – [spinach, kale, broccoli] – Helps to prevent relapse of anemia • Meats- [chicken, beef ,ham] –useful in treating anemia • Honey, amla juice, jack fruit & custard apple increases Hb content in body. • Beetroot is good remedy for treating anemia • Banana and figs helps to stimulate production of RBC • Enriched cereals or pastas helps to treat anemia • Spices- including mustard seeds and ginger
- 44. FOODS TO BE AVOIDED • Tea and coffee • Foods that contain tannins, such as grapes, corn causes nausea and impairs absorption of iron • Foods rich in gluten like wheat, barley, oats – it triggers autoimmune response and causes further complications. • Foods that contain phytic acid like brown rice impairs absorption of vitamins and minerals. • Foods that contain oxalic acid such as peanuts parsely and chocolates impair mineral absorption
- 45. REFERENCES • Harshamohan – textbook of pathophysiology • Drugs.com • Medscape.com • Healthline.com • British journal of hematology • The new england journal of autoimmune hemolytic anemia