Anemia in pregnancy
Basically the most common medical ds in pregnancy
In India prevalance is more than 47%
So it becomes a necessity to diagnose and treat anemia as soon as u make diagnosis
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Added: Sep 25, 2024
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ANEMIA IN PREGNANCY Presented by : Dr Ankita
DEFINITION WHO defines anemia in pregnant women < 11 gm / dL and hematocrit <33 % CDC recommends that Hb in pregnant women should not be allowed to fall < 10.5 gm/ dL in 2 nd trimester and 11 gm/ dL in 1 st and 3 rd trimester Acc to ICMR : MILD : 10 to 10.9 Moderate: 7 to 10 Severe : <7 Very severe: <4 Most common medical disorders in pregnancy Acc to NFHS 4 : prevalance in india : 57-96 % Postpartum anaemia is defined as Hb <10 g/ dL (NICE)
Acc to WHO : High prevalance : > 40 % Medium prevalance : > 15-39 % Low prevalance : 5-14.9 % Not a problem : < 5 % PHYSIOLOGY : RBC mass : 30% increment Plasma volume : 40 to 50 % increment causes : 1. erythrocyte dilution by : 15 % 2. Hb fall by 2 gm/ dL 3. PBF : normocytic normochromic anemia THIS IS* PHYSIOLOGICAL ANEMIA OF PREGNANCY * First apparent in 7-8 weeks of pregnancy
Advantages of physiological anemia in pregnancy Decrease in blood viscosity : reduced work load on heart and facilitate blood flow through placenta Increase blood volume acts as a buffer against blood loss in 3 rd stage of labour not beneficial in CARDIAC PATIENT
Total iron demand in pregnancy : 1200mg I ron required for Uterus and its content - ------ 500mg. Iron required for red cell increment ------- 500mg Post partum loss --------- 150-200 mg . 225 mg subtracted (saved as a result of amennorrhoea) So actual extra demand ---------------------- 600 to 700mg REQUIREMENT : 2 nd trimester : 4-6mg/day 3 rd trimester : 6-8mg/day Full iron stores --------------------------------1000mg
EFFECTS OF ANEMIA ON MOTHER Cardiac failure : in severe anemia Infection susceptibility Antepartum complications aggravated by anemia : preeclampsia and antepartum haemorrhage During labour :PPH/maternal exhausation Puerperium : subinvolution /lactation failure/delayed wound healing
EFFECTS ON FETUS Low birth weight IUGR Maternal anemia during 1 st trimester have greatest adverse effect on fetus Preterm labour and PROM Fetal iron stores inadequate Folate deficiency leads to : abortion/fetal malformation/APH/prematurity/LBW
HISTORY AND CLINICAL EXAMINATION HISTORY Worms in stool/ haematuria / hyperemesis / malabsorption TB/Malaria/ bleeding diasthesis / menorrhagia Dietary habits/Family history SYMPTOMS Weakness/light-headedness/ anorexia Skin and nail changes/ankle swelling Palpitation/ worsening of angina CLINICAL FEATURES Pallor/ koilonychia / platynychia / cheilosis / glossitis Tachycardia/ tachypnoea /heart murmurs/ankle edema
LABORATORY INVESTIGATIONS CBC/PBF/packed cell volume Reticulocyte count RBC indices: MCV (80-95fL)/MCH(27-32 pg)/MCHC (34-37 g/ dL ). Stool examination RFT/LFT NESTROFT test Urine routine and microscopy IRON STUDIES Bone marrow study Test for Malaria Sickling test MCV not a good indicator in distinguishing type of anemia in pregnancy : 1.Physiological macrocytosis during pregnancy 2.Reduced size of RBC in iron deficiency anemia masked by macrocystosis caused by folic acid deficiency(in combined iron and folic acid deficiency)
Types of anaemia Iron deficiency Megaloblastic Aplastic varieties Haemoglobinopathies Haemolytic Secondary (repeated bleeding,chronic infection,hodgkin’s disease) Bone marrow insufficiency Anemia of infection (malaria , TB,hookworm )
Pathophysiology of Iron
Dietary iron is found in two forms : Heme (meat): most bioavailable source of iron Non heme (cereals) Factors enhancing absorption of iron : Ascorbic acid Fermented food items and alcohol Gastric acidity Low iron stores Increased erythropoietic activity Factors inhibiting absorption : Phytates c. Tea and coffee Calcium d. High iron stores
IRON DEFICIENCY ANEMIA Stages of development of iron deficiency Storage iron depletion : iron supply to bonemarrow reduced but still no anemia Iron deficient erythropoiesis Iron Deficiency anaemia : Microcytic normochromic anemia MCV/MCHC reduced Anisocytosis and polychromasia
Causes of Iron deficiency Increased demand Dietary deficiency Impaired absorption : due to various malabsorption,chronic diarrhoea . Increased blood loss : Hookworm infestation : blood loss 0.2mL/worm/day Multiple pregnancies Lactation : loss of iron :0.5 to 1gm/day
PARAMETERS NORMAL IRON DEFICIENCY Serum Iron 60-120 <60 TIBC 325-400 Normal/increased Transferrin saturation 20-50 % decrease Free erythrocyte protoporphyrin (substrate used for heme synthesis) increase Erythrocyte zinc protoporphyrin 40-70 >70 Serum transferrin receptor( TFr ) 5.5 8.8(2-3 times increased) Serum transferrin receptor: ferritin ratio increase Ferritin (marker of storage iron ) 50-150 decrease
Iron deficiency anemia Thalassemia RBC count <5.5 million >5.5 million anisopokilocytosis marked Mild RCDW increased Normal Ferritin decreased Normal S.Iron /TIBC Decreased/increased Normal/normal Transferrin saturation <15 % 30-40 % HbA2 level Normal or reduced <3.5 % Increased
DEGREE Hb SERUM Ferritin Iron deficient but not anemic >11 g/ dL <12 ng / mL Iron deficiency anemia <11 g/ dL <12 ng / mL Anemia not due to Iron deficiency <11 g/ dL >12 ng / mL Prophylactic supplementation: Acc to WHO : 60mg elemental Fe+400 mcg folic acid per day for 6 months antenatal and 3 months postpartum(if prevalance >40 %) If prevalance <40 % only 6 months antenatal Fe and folic acid given
PREGNANCY POSTPARTUM PROPHYLAXIS TREATMENT WHO Daily 60mg iron +400 mcg folic acid till term Daily 120mg +400 mcg folic acid till term Daily 60mg iron + 400 mcg folic acid for 3 months MoHFW (2016) Daily 100mg iron + 500 mcg folic acid for 6 months Mild anemia : 2 IFA tablets/day for 100 days Moderate anemia : IM iron therapy + oral folic acid Severe anemia : IV sucrose Daily 100mg iron + 500 mcg folic acid for 6 months
TREATMENT OF IRON DEFICIENCY ANAEMIA Oral Iron : Ferrous sulphate :cheapest and suitable for all. Side effects GIT intolerance a.Take empty stomach b. A intermediary approach to take iron midway between 2 meals c.Take iron solution : but causes staining of teeth Ferrous Fumarate : similar efficacy and GIT symptoms similar to ferrous sulphate Carbonyl and Iron polymatose complex : Advantages: 1.less GIT symptoms 2. Lethal dose of FeSO4:200mg/Kg and carbonyl iron : 50,000mg/Kg Iron hydroxide carbohydrate comple x : GIT symptoms very less Ferrous ascorbate : High proportion of elemental iron Converts ferric to ferrous forms Inhibits formation of insoluble iron complexes
IRON SALT DOSE ELEMENTAL IRON Ferrous Fumarate 200 mg 65 mg Ferrous Gluconate 300 mg 35 mg Ferrous Sulphate (dried) 200mg 65 mg Ferrous Sulphate 300mg 60 mg This should be on an empty stomach, 1 h before meals, with a source of vitamin C (ascorbic acid) such as orange juice to maximize absorption. Other medications or antacids should not be taken at the same time
LABORATORY parameters : 5-7 days : increase in reticulocyte counts upto 5% 2-3 weeks : Increase in Hb levels @ 0.8 -1 gm/ dL /week Improvement in RBC indices :MCV/MCH/MCHC 6-8 weeks : Hb level comes to normal level Peripheral smear : normocytic normochromic anemia Increase in ferritin level
Compliance of oral iron can be checked by Repeated questioning about the intake Colour of the stool which should be black Associated symptoms like constipation and gastritis Return of empty blister packs if she follow regularly
Indication of parenteral preparations Poor tolerance to iron therapy Poor absorption of iron like in chronic diarrhoea,ulcerative colitis,coeliac disease Non compliance to oral iron When oral iron ineffective Women near term with severe anemia Presence of concurrent diseases Hb <7gm/ dL and pregnancy >30 weeks
PARENTERAL IRON: Total iron dose requirement : 1.Total iron dose : 250x(target Hb -present Hb ) +500* (*: for iron stores) 2.Total Iron doses : Body weight (kg) (target Hb -present Hb ) x 2.4+1000 Target Hb : gm/ dL INTRAMUSCULAR IRON ; 1. Iron Dextran : IM and IV (not much in use ) each amp: 2ml solution containing 50mg iron / mL 2. Iron Sorbitol citric acid complex in dextrin : IM
SIDE EFFECTS : Anaphylactic reaction Arthalgia,backache,chills,dizziness,fever,headache,malaise , nausea,vomiting Should used with caution in women with impaired liver function,known allergies and asthma and should not be administered during acute kidney infection Discoloration of overlying skin DOSE: 1.5 mg /Kg daily or on alternate days. 200mg elemental iron raise Hb by 1 gm/ dL a.A test dose of 25mg should be given to all patients before giving test dose b. Full dose should be given at least after 1 hour c. Given in upper outer quadrant of buttock. d.Injected deeply with a 2 or 3 inch 20-22 guage needle with Z track technique
Intravenous administration IRON DEXTRAN (1 st generation) IRON SUCROSE(2 nd generation) Ferric carboxymaltose,ferumoxytol and iron isomaltoside (3 rd generation )
IroN HYDROXIDE DEXTRAN COMPLEX (COSMOFER) IRON hydroxyide sucrose complex (VENOFER) IRON carboxymaltose (FER-inject) IRON isomaltoside dose 50 mg/ mL 20 mg/ mL 50 mg/ mL 100mg/ mL Test dose Yes,before every IV dose and once before IM dose First dose to new patients only no No methods Slow IV inj IV infusion of total dose IM 1.Slow IV inj 2. IV infusion 1.Slow IV inj 2. IV infusion 1.Slow IV inj 2. IV infusion
dosage 1.100-200 mg per IV inj upto 3 times a week 2. Total dose infusion upto 20 mg/Kg over 4-6 hrs 3.100 mg IM into alternate buttocks daily in active patients and in bed ridden upto 3 times a week Total IV inj dose no more than 200 mg ,can be repeated upto 3 times in a week 1000 mg by IV inj upto 15 mg/Kg. Total dose infusion upto 20 mg/Kg Max weekly dose of 1000mg which can be adminstrered over 15 mins 100-200 mg per IV inj upto 3 times a week Total dose infusion upto 20 mg/Kg BW per week Doses upto 10mg/Kg bodyweight can be given Use in pregnancy Not adequate use in pregancy Not in first trimester Avoid in 1 st trimester * NOT FDA APPROVED* No adequate data for use in pregnancy
Disadvantages of IM vs IV : causes staining of skin Painful Abscess Multiple injections are required as max 2 mL containing 100 mg iron can be given at a time Absorption is irregular
Patient with severe anaemia in late pregnancy : PRC transfusion with a diuretic to avoid vol overload Recombinant human erythropoietin injection helpful in treatment of anemia with end stage renal disease with or without pregnancy. Given in patient end stage renal failure or severe anemia.
Indications of PRC transfusion antepartum intrapartum Postpartum Indications Pregnancy <34 weeks a.Hb <5gm/ dL with or without signs of cardiac failure or hypoxia b. 5-7 gm/ dL in presence of impending heart failure Hb <7 gm /dl 2. Decision of blood transfusion depends on medical history or symptoms Anemia with signs of shock /acute haemorrhage with signs of haemodynamic instability Pregnancy >34 weeks Hb <7 gm/ dL without signs of cardiac failure Severe anemia with decompensation 2. Hb <7gm % (postpartum )
3. Anemia not due to hematinic deficiency: Hemoglobinopathy or bone marrow failure syndome Hematologist should always be consulted 4. Acute haemorrhage : Hb <6 gm/ dL If patient is haemodynamic unstable due to ongoing haemorrhage
Deworming necessary : Albendazole 400mg single dose Mebendazole 500mg single dose or 100mg twice daily for 3 days Levamisole 2.5 mg/Kg single dose ,best if 2 nd dose is repeated on next 2 consecutive dose Pyrantel 10mg/Kg single dose ,best if dose is repeated on next 2 consecutive days
8 GUIDELINES FOR MANAGEMENT OF MATERNAL ANAEMIA FLOW CHART (ICMR 2016) I. AT 14-16 WEEKS OF GESTATION:- Deworming with one 400 mg. of Tablet Albendozole after meals at 14-16 weeks First estimation of Blood Haemoglobin at 14-16 weeks of gestation by cyanmeth-haemoglobin method using semi- auto analyser or photocalorimeter If Blood Hemoglobin level more than 11 gm/dl. If Blood Hemoglobin level between 7.1-10 .9gm/dl. If Blood Hemoglobin level less than 7 gm./dl. Refer to Higher Institutions (CEmONC centres) for Blood transfusion and further management Theraputic dose of Tablet Ferrous sulphate 100 mg. of elemental iron 1 bd. with 0.5 mg. of folic acid 1 Tablet of Vitamin B12 15mcg. And Vitamin C 100 mg./od. to be supplemented . Preventive dose of Tablet Ferrous sulphate 100 mg. of elemental iron 1 od. 0.5mg of folic acid 1 Tablet of Vitamin B12 15mcg. And Vitamin C 100 mg./od. to be supplemented .
9 * If the AN registration done earlier than 14 weeks then oral iron to be started from 12 weeks onwards. II. AT 20- 24 th WEEK OF GESTATION:- Second estimation of Blood Hemoglobin at 20-24 weeks of gestation after the consumption of preventive/therapeutic dose of iron If Blood Hemoglobin level is 9-11 gm/dl. If Blood Hemoglobin level between 7.1-8.9 gm./dl. ** If Blood Hemoglobin level less than 7 gm./dl. Refer to Higher Institutions (CEmONC centres) for Blood transfusion and further management Injection iron sucrose infusion *** intra venous – 4 doses of 100 mg. for 4 days over a period of 2 weeks with 2-4 days interval between each dose. Continue with therapeutic dose of Tablet Ferrous sulphate 100 mg. of elemental iron 1 bd. 1 Tablet of Vitamin B12 15mcg. And Vitamin C 100 mg./od. – 1 st d o se 100 mg . of injection iron sucrose in 100 ml. of Normal saline infusion for 30 min. only. 2 nd, 3 rd and 4 th dose (each 100 mg) over a period of 2 weeks, at 2-4 days interval - 100mg . of injection iron sucrose in 100 ml. of Normal saline infusion for 30 min. only If Blood Hemoglobin level is more than 11 gm/dl. Continue with preventive dose of Tablet Ferrous sulphate 100 mg. of elemental iron 1 od. 1 Tablet of Vitamin B12 15mcg. And Vitamin C 100 mg./od.
11 III. AT 26-30 WEEKS OF GESTATION:- Third estimation of Blood Hemoglobin after 1 month of the above 4 doses (not later than 30 weeks ) If Blood Hemoglobin level is 9-11 gm./dl. If Blood Hemoglobin level is 7.1-8.9 gm./dl. If Blood Hem o g l obin level less than 7 gm./dl. Refer to Higher In st it u tions (CEmONC centres) for Blood tr a n sfusi o n and further management Two top up doses of Injection iron sucrose infusion intra venous – 100 mg(each) in 100 ml. of normal saline for 30 min. only (with 2-4 days interval between each dose) Assure and counsel the mother for the further improvement of Blood Hemoglobin level and to continue **** ora li r on suppl e m e nt a tion till delivery If Blood Hemoglobin level is more than 11 gm./dl Continue with pre v e n tive dose of Tablet Ferrous sulphate 100 mg. of elemental iron 1 od. 1 Tablet of Vitamin B12 15mcg. And Vitamin C 100 mg./od. Received Inj. Iron sucrose earlier in the current pregnancy Not Received Inj. Iron sucrose earlier in the current pregnancy Injection iron sucrose infusion *** intra venous – 4 doses of 100 mg. over a period of 2 weeks with 2-4 days interval.
12 ****Continue preventive dose of iron (100 mg. of elemental iron) + 0.5 mg. of folic acid till delivery. IV. AT 30-34 WEEKS OF GESTATIONS:- **** Continue preventive dose of iron (100 mg. of elemental iron) + 0.5 mg. of folic acid till delivery. Estimation of Blood Hemoglobin at 30-34 weeks of gestation If Blood Hemoglobin level is 9 -11 gm/dl. If Blood Hemoglobin level is 7.1-8.9 gm/dl If Blood He m o g l o b i n level less than 7 gm/dl. Refer to Higher Instituti ons (CEmONC Centres) for Blood transfusion and further manag e m e nt Refer to Higher Institutions (CEmONC Centres) for Blood transfusion And further manag e m e nt Assure and counsel the mother for the further improvement of Blood Hemoglobin level and to continue **** ora l i r o n supple m entation till delivery If Blood Hemoglobin level is more than 11 gm/dl Continue with pre v e n tive dose of Tablet Ferrous sulphate 100 mg. of elemental iron 1 od. 1 Tablet of Vitamin B12 15mcg. And Vitamin C 100 mg./od.
Management in labour 1 st stage : bed rest,oxygen inhalation,aseptic precautions 2 nd stage :cut short 2 nd stage of labour by prophylactic vaccum or forceps 10 units oxytocin or ergometrine Iv with delivery of baby 3 rd stage : oxytocin to continue Bladder emptying. watch for signs and symptoms of heart failure
Programs related to iron deficiency anemia : National nutritional anemia prophylaxis program 1970 National anemia control program 1991 12/12 initiative 2007(12 Hb by age of 12 years) National Iron plus initiative 2017 Weekly iron folic acid supplementation program (WIFS) (RAJASTHAN ) Acc to food fortification : Double fortified salt provides 93 mg of iron within 6 months of supplementation
LATEST GUIDELINES POINTS Full blood count (FBC) should be assessed at booking and at 28 weeks.(NICE 2008) Compulsory Haemoglobin estimation by Cyanmeth-haemoglobin method at 14-16 weeks, 20-24 weeks, 26-30 weeks and 30-34 weeks of pregnancy for all pregnant mothers (minimum four Hb estimations). The interval between one haemoglobin estimation and another should have a minimum of four weeks. (In INDIA) For anemic women, a trial of oral iron should be considered as the first line diagnostic test, whereby an increment demonstrated at 2 weeks is a positive result. Serum ferritin should be checked prior to starting iron in patients with known haemoglobinopathy The serum ferritin level is the most useful and easily available parameter for assessing iron deficiency. Levels below 15 μg /l are diagnostic of established iron deficiency. A level below 30 μg /l in pregnancy should prompt treatment
All pregnant women at 14-16th week during the second trimester should be given one tablet of Albendazole 400mg – single dose.
MEGALOBLASTIC ANAEMIA Deficiency of vit B12 and folic acid Incidence : 0.2-5 % Usual time of onset of megaloblastic anaemia : 5 th -7 th month of pregnancy Clinical features : Severe anaemia not responding to parenteral iron therapy Atrophic glossitis Hepatospleenomegaly,Congestive cardiac failure may occur in severe cases
LAB DIAGNOSIS PBF :hypersegmented neutrophil,macrocytes , Pancytopenia Serum folate and erythrocyte folate : folate <3ng/ mL,erythrocyte folate activity <150ng/ mL Increased LDH and homocysteine TREATMENT : For prophylaxis : 400mcg in antenatal period and 600 mcg for lactation(acc to WHO ) For anaemia : 5mg of folic acid should be given RESPONSE : a.decrease LDH in 3-4 days b. Increase in reticulocyte count in 6-8 days 3.Blood transfusion in case of antepartum haemorrhage
Anaemia associated with haemoglobinopaties and thallasemia aggravates folic acid deficiency : daily treatment requires 5mg/day Folate supplements should be given to all epileptic women taking anticonvulsants in pregnancy as well as from conception. FETUS and FOLATE deficiency : Neonate of folate deficient mother at increased risk of megaloblastic deficiency. Folate requirement of newborn :20-50 mcg/day Neural tube defects in fetus associated with periconceptional folate deficiency. Periconceptional folate administration(400mcg) recommended to all pregnant female during 1 st trimester . Women who have previous child with neural tube defect should receive 4mg of folic acid to prevent recurrence of neural tube defect It is recommended that all women in reproductive age group should receive 5mg folic acid daily
VITAMIN B12 deficiency : Addisonian pernicious anaemia Non pregnant level of B12: 205-1025 mcg/ dL In pregnant : 20-510mcg/ dL Treatment : Parenteral cyanocobalmin 1000 mcg given every month in moderate cases.In Severe cases 1000mcg/day for 1 week following by weekly for 1 month Injection containing folate and cyanocobalmin is given for treatment of megaloblastic anaemia A PLASTIC ANAEMIA : Termination of pregnancy has been recommended when condition occurs in early pregnancy Injection containing folic acid and cyanocobalmin given for treatment of megaloblastic anaemia
HAEMOGLOBINOPATHIES Haemoglobin : globin +4 haem Defect in haem part lead to PORPHYRIA defect in globin part leads to haemoglobinpathies Structurally abnormal globin chains lead to haemoglobinopathies Reduced number of globin chain lead to thalassemia
Β THALASSAEMIA Impaired synthesis of beta chains results in low concentration of normal HbA ( α 2 β 2) with compensatory increase in HbF and HbA2 Prevalance in India : 3.5 to 15 % Β thalassemia mutuation disorder and α thalassemia deletion disorder
α thalassemia trait HbH disease Hb Barts Asymptomatic,microcytic hypochromic anaemia Tetramer of β 4 ppt in red cells causing premature erythrocyte destruction γ 4 ineffective for oxygen transport Maternal complications : preeclmapsia , poly or oligo hydraminos α to β globin chain 0.7 to 0.8 0.2 to 0.6 Fetal complications : severe anemia,HSM , edema,cardiac failure diagnosis γ 4 ( HbBarts ) 3 to 8 % HbBarts 25 % HbH ( β 4) :1-40 % Hb : 7 -10 treatment Genetic counselling Folic acid supplementation (5gm/day) Blood transfusion Assess for iron overload Termination of pregnancy
Obstetric complications of b thalassemmia : Early pregnancy loss/IUGR/prematurity CPD Teratogenic effects of iron chelating agent : retardation of bone ossification,vertebral aplasia,abnormalities in ribs
B thalassemia Lab diagnosis Normal adult B Thalassemia minor B thalassemia major HbA HbA2 HbF 95 2-3 <2 80-95 4-10 1-5 4-10 90-96% Hb >11 9-11 5-8 PBF Microcytosis , hypochromic , anisopoikilocytosis Aniopoikilocytosis,target cells,drop cells,nucleated red cells
MANAGEMENT CF : 1.Pallor 2. Bone deformities : a. Frontal and posterior bossing b. Prominent zygomatic bone with base of nose deformed c. ribs prominent 3. Osteoporosis(51%) Gall stone disease(4-23%) Thrombotic disease (4-10%) Preconceptional evaluation: a.Assess cardiac/thyroid/blood glucose level and ferritin (to assess iron overload) b.Chelation therapy with desferrioxime and need for blood transfusion c. Maintain ferritin level : 1000-2000 ng / mL d. Screen for transfusion related infection : Hep B/ Hep C/HIV / Syphillis e. Test for isoimmunization by ICT f. Preconceptional genetic counselling
Treatment High folic acid preconceptionally and during pregnancy Parenteral iron therapy C/I Blood transfusion if Hb <8 gm/ dL at term Screening of partner should be screened out with peripheral smear,Nestroft and Hb electrophoresis
ANC care: Iron supplementation C/I Blood transfusion to maintain Hb 9.5-10%.Complete CBC done every 2 weeks. High dose folic acid (5gm/day ).because folic acid depletion is common due to marrow overactivity Iron chelation with desforrioxime should be stopped Factors that precipitate hemolytic crisis include fever,infection,ingestion of oxidative compounds.Avoid these factors. Postpartum management Lactation is not C/ I.Iron chelation is started 3 weeks after 1 st blood transfusion which is given in postpartum period Cardiac,hepatic ,endocrine functions reevaluated 4-6 months postpartum OCPs C/I in splenectomized women due to risk of thrombosis
Screening of thalassemia NESTROFT (naked eye single tube osmotic fragility test ) using 0.36 % buffered saline solution Procedure: RBCs in thalassemia patients hemolyse in hypotonic saline resulting solution hazy Principle :normocytic normochromic cells when put in hypotonic solution will undergo lysis whereas in thalassemia trait ,cells are microcytic hypochromic which are resistant to hemolysis due to decreased fragility. If nestroft positive : Hb electrophoresis (HPLC ) and CBC done Raised HbA2 with or without HbA2 gives a diagnosis of thalassemia minor α thalasemia ruled out by DNA testing
HbS is a beta chain mutuation,manifests after 6 month of age,when HbA becomes significant CF: Effects of chronic haemolysis : anemia,jaundice,cholelithiasis Effects of vasoocclusion : Dactylitis Pain in any part damaged by vaso -occlusive crisis Splenic enlargement followed by infarction Renal papillary necrosis Leg ulcers Retinopathy and stroke Acute chest syndrome LAB DIAGNOSIS: Hb : 6-10 gm/ dL PBF : target cells,sicke cells,cigar shaped cells,ovalocytes,basophillic stipplings ESR :low LDH and alkaline phosphatase increase HbElectrophoresis : HbS dominant (85-95%)
Complications in pregnancy Anemia severe Pyelonephritis,preeclampsia,hematuria , Thrombophelbitis During 3 rd trimester and postpartum sicking crisis and its complications like CHF and pulmonary embolism increase Abortion,preterm birth,IUGR
Antepartum management Prevent dehydration,infection,acidosis because of risk of sickling crisis Foli acid 1 gm/day Prophylactic antibodies : penicillin 250mg twice daily Fundal examination to rule out proliferative retinopathy Asymptomatic bacteuria should be tested and treated Fetal monitoring from 32 weeks and doppler to find out IUGR Close watch to detect preeclampsia/IUGR and abruption Sickling episodes treated by IV fluids and oxygen Timely blood transfusion to maintain HbA 60-70 % of total
Transfusion therapy in pregnancy Indications: Acute anemia : due to blood loss or splenic sequestration Acute chest syndrome : exchange transfusion done if Hb normal. Aim of transfusion to maintain : hematocrit : 30% and HbA 70-80 % Multiorgan failure :exchange transfusion to maintain HbS level to 30 % and hematocrit 30 %
Intrapartum : avoid dehydration , hypoxia,sepsis,acidosis Postpartum : increased risk of infection,thromboembolism,vasoconstrictive diseases.early ambulation,proper hydration and if required heparin therapy Contraception : IUCD C/I due to risk of infection DMPA or implant Low dose OCPs can be given (though risk of thromboembolism present)