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HAEMOTOLOGICAL DISORDERS IN PREGNANCY. Dr. RAMYA MODERATOR : Dr.PALLAVEE. HAEMOTOLOGICAL DISORDERS IN PREGNANCY ANAEMIA PLATELET DISORDERS HAEMOGLOBINOPATHIES INHERITED COAGULATION DEFECTS. ANAEMIA Commonest haematological disorder occur in preg. Prevalance in pregnant women –
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HAEMOTOLOGICAL DISORDERS IN PREGNANCY Dr. RAMYA MODERATOR : Dr.PALLAVEE
HAEMOTOLOGICAL DISORDERS IN PREGNANCY ANAEMIA PLATELET DISORDERS HAEMOGLOBINOPATHIES INHERITED COAGULATION DEFECTS
ANAEMIA Commonest haematological disorder occur in preg. Prevalance in pregnant women – 14 % - Developed 51% - Developing countries 65-75% - India 80 % leading to maternal deaths
DEFINITION Reduction in circulating Hb mass < 12g/dl in non-pregnant women <10 g/dl in pregnant women CDC Anaemia in iron supplemented preg. Woman Hct 33% & Hb 11g/dl – 1st & 3rd trimester Hct 32% & Hb 10.5 g / dl - 2nd trimester
WHO grading of anemia • Mild 10g/dl • Moderate 7- 10 g/dl • Severe < 7 g/dl
Physiological Anemia of pregnancy Plasma volume s 40-50% RBC mass s 30 % As a result Hb concentration decreases by 2g/dl Decreased Hb concn. Is due to haemodilution Criteria of Physiological Anemia 1) Hb 10 gm % 2)RBC 3.2 million cells / cu mm 3)PCV 32% 4)Peripheral Smear – Normal morphology
IRON Requirements during Pregnancy • Maternal req. Of total Iron -1000mg • 500 mg Mat. Hb. Mass expansion • 300 mg Fetus & Placenta • 200mg Shed through gut urine, skin
DEVELOPMENT OF Iron def. anemia Iron Deficiency Anemia – 3 stages • a)Depletion of Iron stores • b)Iron deficient erythropoiesis • c)Frank Iron deficiency Anemia
Symptoms of IRON DEFICIENCY ANEMIA • Fatigue • Weakness • Headache • Loss of appetite • Dysphagia • Palpitations • Dyspnea on exertion • Ankle swelling • Paresthesias • Leukoplakia
Physical examination • Pallor of varying degrees (Mucous membranes , nail beds – Koilonychia or Platynychia • Glossitis • Stomatitis • Heart murmurs • Increased JVP • Tachycardia • Tachypnea • Postural hypotension • Crepitations- due to lung congestion
Depletion of Iron stores • Ferritin <20 ng/ml • Hb / Hct. Normal • RBC INDICES normal Iron deficient erythropoiesis • Ferritin <20 ng/ml • Transferrin saturation<25% • Hb –Normal • Serum Iron < 60mg/dl
c)Frank Iron deficiency Anemia • ferritin <20 ng/ml • Transferrin saturation<25 % • Serum iron <60 mg/dl • Hb <10g/dl, Hct.<28%
PROPHYLAXIX • WHO - 60 mg Elemental iron + 400 micro gram Folic acid / day * 6 months & 3 months postpartum • National Nutritional Anemia Control Programme of India - 60 mg elemental Iron + 500 mcg Folic acid & Prophylactic supplementation * 100 days in 2nd trimester
Ferrous sulphate 300mg Tid orally daily after meals • To be contd for 12 months after anemia is corrected • Indicators of iron therapy response • Increase in Reticulocyte count (Increases 3-5 days after initiation of therapy ) • Increase in Hb levels. Hb increases 0.3 to 1 g/ week 3 .Epithelial changes (esp tongue & nail ) revert to normal • Hb concn. Is normal after 6 wks of therapy
PARENTERAL ADMINISTRATION • INDICATIONS • Intolerance to oral iron • Non compliance pt. • Inflammatory bowel disease • Pt. unable to absorb iron orally • Patients near term
TDI – Total Dose Infusion Amount of iron needed to restore Hb conc to normal & additional allowance to provide adequate replenishment of iron stores • Formulae 1 Total Dose ( mg ) = ( normal Hb – Pts Hb ) * (body wt. in kg ) * 2.21 2 Total Iron Dose (mg ) = 2.3 * wt. kg before preg * D (Target Hb) + 500 mg for body store
MEGALOBLASTIC ANAEMIA • Incidence – 0.2 – 5 % • Caused by folic acid deficiency & Vit B12 deficiency
Folic Acid Defciency Pathophysiology • Preg. Causes 20 -30 fold increase in Folate requirement (150-450 microgram / day ) to meet needs of fetus & placenta. • Placenta transports folate actively to fetus even if the mother is deficient. • This cause decreased plasma folate levels.
Causes of Folic acid deficiency 1.Diet- Poor intake, prolonged cooking. 2. Malabsorption – Coeliac disease. 3.Increased demand – Pregnancy, cell proliferation (hemolysis ) 4.Drugs – anticonvulsants, contraceptive pill, cytotoxic drugs (Methotrexate ) 5.Diminished storage – Hepatic disorders & Vit C deficiency
Diagnostic features of Folic acid deficiency 1.Serum Folate levels – Low <3 ng/ml 2.Erythrocyte Folate levels - <20 ng/ml 3.Peripheral smear – Hypersegmented neutrophils,Oval macrocytes,Pancytopenia
Treatment • Pregnancy induced megaloblastic anaemia- Folic acid, nutritious diet & Iron . • Supplementation of 1mg of folic acid daily can improve MA by 7 to 10 days • Folic acid should be given with iron • Ascorbic acid 100mg Tid enhances action • In other conditions • Recommended folic acid dose – 5mg /day orally daily • Prophylaxis • WHO – 400 micrograms folic acid daily to prevent neural tube defects
Pathophysiology • Vit B12 absorption is unaltered during pregnancy • Tissue uptake is increased Decreased serum B12 • Recommended B12 intake – 3 microgram /day. CAUSES of Vit B12 def. • Strict Veg. diet • Use of proton pump inhibitors • Metformin. • Gastritis • Gastrectomy • Ileal bypass • Crohn’s • H. Pylori infection
Pathogenisis of PERNICIOUS ANEMIA Gastric juice IF Antibody
Clinical manifestations • Macrocytic Megaloblastic Anemia • Glossitis • Peripheral neuropathy • Subacute combined degeneration of the Spinal cord
DIAGNOSIS Ser.Vit B12 levels ,100 pg /ml Radio active Vit B12 absorption test . ( Schilling Test ) Treatment 1000 microgram parenteral cyanocobalamin every wk * 6 weeks Pernicious Anaemia – Oral Vit B12 Total Gastrectomy – 1000 microgram Vit B12 im every month. Partial gastrectomy – Ser. Vit B12 levels measured.
ANAEMIA ASSOC. WITH CHRONIC INFECTIONS / DISEASE • Common in developing countries • Poor response to Haematinics unless primary cause is treated • Worm infestations is common ( Diagnosed by stool examination ) • Urinary tract inf, & asymptomatic bacteriuria in preg. Is assoc. with refractory anaemia • Chronic renal disorders = due to erythropoietin def. • Treated with recombinant Erythropoitin
Anaemia from acute blood loss • In preg. Abortion , ectopic preg, hydatidform mole, PPH Treatment. • Blood transfusion • Indicated patient – symptomatic • Not indicated – If hemodynamically stable, Hb < 7 g/dl, able to ambulate without adverse symptoms & not septic.
Acquired hemolytic anemia • AUTOIMMUNE HEMOLYTIC ANEMIA • AUTOANTI-BODIES OF iGg OR WARM ANTIBODIES AGAINST Red cell antigens, causes premature destruction of RBC”s • ETIOLOGY • Lymphomas,Leukemias , Connective tissue diseases, Infections , Chronic. Inflammatory diseases & drug induced antibodies
Diagnosis Direct Coomb’s Test Blood smear – Spherocytosis & Reticulocytosis TREATMENT Prednisone 1mg / kg / day orally Azathioprine Splenectomy
2)Preg. Induced hemolytic anemia • Unexplained hemolytic anemia uring pregnancy is rare • Severe hemolysis occurs early in pregnancy & resolves within months after delivery • No evidence of immune mechanism or defects in RBCs • Prednisone given untill delivery • 3) Paroxysmal Nocturnal Hemoglobinuria • Acquired hemolytic anemia • Arises from one abnormal clone of cells like neoplasm • Anemia is insiduous in onset & hemoglobinuria develoes at irregular intervals
Hemolysis may be initiated by transfusion , infections or surgery • 40% suffer venous thrombosis, renal failure , HTN & Budd Chiari syndrome. • Prophylactic anticoagulation is required • Bone marrow transplantation – Definitive treatment Effect on pregnancy • Serious & unpredictable • Maternal mortality 10 – 20% • Venous thrombosis occurs during post partum
APLASTIC ANAEMIA • Rarely seen in preg. • Marked decrease in marrow stem cels ETIOLOGY • Infections • Irradiation • Leukemia • Immunological disorders
May be Immunological mediated or autosomal recessive inheritance 30% cases Anaemia improves once pregnancy is terminated. Complications Infection Haemorrhage
Diagnosis • Blood Values – • Anemia • Leucopenia • Thrombocytopenia • Bone Marrow - Hypocellular
Management • Supportive care – Cont. Infection surveillance & anti microbial therapy • Red cell transfusions to maintain Hct. > 20 • Granulocyte transfusion given only during Infections • Platelet transfusion to control haemorrhage. • Glucocorticoid therapy may be helpful IN SEVERE cases • Bone marrow or Stem Cell Transplantation • Vaginal delivery is preferred