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Anaemia & other haematological disorders
- 1. ANAEMIA & OTHER HAEMATOLOGICAL DISORDERS Dr.Sneha Jadhav Dr. Milind Shintre Dr. NikitaYedage Dr. Shruti Jaiswal Dr. ArtiWagle
- 2. HAEMATOLOGICAL DISORDERS IN PREGNANCY •Anaemia • HaemoglobinopathiesRBC • Neutrophilias • neutropeniaWBC • Gestational thrombocytopenia PLATELETS
- 4. ■ Erythropoiesis isthe formation of red blood cells. ■ Life span of a normal RBC is 120 days. ■ All blood cells are formed in the bone marrow, which is soft,highly cellular tissue that fills internal cavities of bones. ■ In intrauterine life,erythrocytes are produced by the yolk sac and then by spleen during 3rd month of gestation until the bone marrow is formed in the seventh month which takes over erythrocyte production exclusively.
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- 7. FETAL RBCS ■ 2aand 2y chains ■ More affinity to oxygen ■ Lifespan is comparatively less ■ Present in fetal life ■ HbF ADULT RBCS ■ 2a 2b/ 2a 2d chains ■ Less oxygen affinity ■ Lifespan is 120 days ■ HbA / HbA2
- 8. Iron metabolism.. ■ Consistsof a set of reactions at cellular and systemic level to maintain iron homeostasis. ■ In the human body, iron is present as ferrous and ferric forms. ■ In the ferrous state, iron acts as an electron donor whereas in the ferric state it acts as an acceptor. ■ Iron plays a vital role in the catalysis of enzymatic reactions which involve electron transfer.
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- 10. Iron metabolism atthe cellular level….
- 11. Normal iron storagein human body..
- 12. Investigations in caseof anemia ■ Hemoglobin estimation Mild: 10 to 10.9 gm% Moderate: 7 to 9.9 gm% Severe: <7gm% Very severe: <4gm%
- 13. ■ Total RBCcount <4 million/mm3 Normal 4 to 4.5million/mm3. ■ Packed CellVolume(PCV) MCV × RBC count/10 <30% Normal 32 to 36%
- 19. Serum values ■ Serumiron concentration Normal 65-75µg/dl ■ Serum ferritin Highest sensitive and specific marker of IDA. <12µg/L Normal 15 to 300µg/L
- 20. ■ Total IronBinding Capacity >400µg/dl Normal 300-400µg/dl ■ Transferrin saturation Serum iron÷TIBC <15% Normal 25-50% ■ Serum B12 Normal >300pg/ml
- 21. ■ Serum folate Normal3-8ng/dl ■ Red cell folate Normal >150ng/dl ■ Serum transferrin receptor ■ Free erythrocyte protoporphyrin
- 22. ■ Urine analysis ■Stool examination ■ Investigations for abnormal hemoglobin Electrophoresis HPLC(High Performance Liquid Chromatograghy) HbF% ( normal<2%) (thalassemia >2%) HbA2% (normal 2-3%) (thalassemia >3.5%) ■ Bone marrow analysis
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- 25. Anemia in pregnancy ■Definition: ByWHO ■ Hb. < 11 gm /dl (or haematocrit <32%). ■ Mild anaemia -------- 9 -10.9 gm /dl ■ Moderate anaemia--- 7-8.9 gm /dl 24-37% ■ Severe anaemia-------- < 7gm /dl 13-23% ■ Very sever anaemia-- < 4gm/dl <13%
- 26. Magnitude of Problem ■Globally, is about 30 % In developing countries & ■ India, incidence is around 40 – 90%. ■ Responsible for 40% of maternal deaths in third world countries. ■ Important cause of direct and indirect maternal deaths ■ Vitere FE Adv Exp Med Biol 1994;352:127
- 27. Iron requirement ■ Ironrequired for fetus and placenta ------- 500mg. ■ Iron required for red cell increment ------- 500mg ■ Post partum loss --------- 180mg. ■ Lactation for 6 months - 180mg. ■ Total requirement -------1360mg ■ 350mg subtracted (saved as a result of amenorrhea) ■ So actual extra demand ----------------------1000mg ■ Full iron stores --------------------------------1000mg
- 28. Clinical features ■ Clinicalfeatures depends on the degree of anaemia, ■ Symptoms – 1. Lassitude and fatigue orWeakness 2. Anorexia and indigestion 3. Palpitations 4. Dyspnea 5. Giddiness 6. Swelling over the feet
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- 30. Examination 1. Pallor ofvarying degrees 2. Glossitis and Stomatitis 3. Edema on feet 4.Tachycardia 5. Soft systolic murmur 6. Crepitations at the base of lungs
- 31. Just name thissign
- 32. Just name thissign
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- 35. Type of Anaemia ■Peripheral Blood smear- suggestive of small pale staining cells with variable sizes( Anisocytosis) and shape (Poikilocytosis) suggest Microcytic Hypochromic anaemia.
- 37. Hematological indices A typicaliron deficiency anemia shows the following blood values: ■ Hemoglobin—less than 10 gm%, ■ Red blood cells —less than 4 million/mm3, ■ PCV—less than 30%, ■ MCHC — less than 30%, ■ MCV — less than 75 μ3 ■ MCH—less than 25 pg.
- 38. Reasons for IDA ■Depleted iron stores – dietary lack, chronic renal failure, ■ worm infestation, chronic menorrhagia ■ Chronic infections: ( like malaria) ■ Repeated pregnancies : ■ with interval < 1 year ■ blood loss at time of delivery ■ multiple pregnancy.
- 39. Blood values inIron deficiency anemia ■ Serum iron is usually below 30 μg/100 mL. ■ Total iron binding capacity is elevated > 400 μg/100 ml. ■ Percentage saturation is 10% or less. ■ Serum ferritin below 30 μg/L. (confirms IDA) ■ Serum bilirubin is not raised.
- 40. To find theCause of anemia ■ Appropriate investigations , history and clinical examination ■ Examination of stool:This should be done as a routine specially in tropics, to detect helminthic (particularly hookworm) infestation. ■ The urine is examined for the presence of protein, sugar and pus cells. A “clean catch” mid stream specimen of urine is subjected to culture and colony count. ■ If the counts are over 105/mL, it indicates infection. ■ Rule out pulmonaryTB , achlohydria, hypoproteinemia or other hemoglobinopathies. ■ Bone marrow – in case of not respnding to therapy, hypoplatic anemia, kala azar .
- 41. DIFFERENTIAL DIAGNOSIS ■ Allthe causes of hypochromic anemia are to be differentiated. Apart from iron deficiency, other causes are: ■ (1) Infection ■ (2) Nephritis and pre-eclampsia ■ (3) Hemoglobinopathies
- 42. Complications in pregnancy Duringpregnancy During labour Puerperium Preeclampsia Intercurrent infections Heart failure arnd 30-32 wks Preterm labour Uterine inertia Postpartum hemorrhage Cardiac failure Shock Puerperial sepsis Subinvolution Poor lactation Venous thrombosis Pulmonary embolism Risk periods – At about 30-32 weeks ,during labour , after delivery , puerperium arnd 7- 10days
- 43. Treatment ■ Prophylaxis – Avoidance of frequent childbirth Supplementary iron therapy -- daily supplementation of 200mg of ferrous sulphate iron with 1mg folic acid is quiet effective. Diet – rich in Iron and protein like liver , meat, egg, green vegetables, green peas, figs, beans, whole wheat, green plantains, onion stalks, and jaggery . Iron utensils are preferred for cooking. Eradicate infections – hookworm, dysentery, malaria, bleeding piles, and UTI Hemoglobin estimation done at 1st neonatal visit, 30th week and at 36th week of gestation
- 45. Curative Accurate diagnosisof the cause of anaemia Hospitalisation when hb <7.5gm% can be considered General treatment- diet rich in iron , eradicate septic focus , cure disease contributing to anemia . Specific therapy – to raise the hemoglobin to near normal and restore iron stores. Choice of therapy – 1. severity of anemia 2. duration of pregnancy 3. associated complicaticating factors.
- 46. Iron therapy ■ Oraltherapy – ■ Best absorbed in ferrous form ■ Forms like Ferrous fumarate , Ferrous succinate, Ferrous gluconate are available ■ Ferrous sulphate ( fersolate ) contains traces of copper and manganese contains 325 mg ferrous sulphate i.e. 60mg elemental iron can be given 3 times a day and dose can be increased to 6 tabs ■ Drawbacks – ■ Intolerance – epigastric pain, nausea vomiting , diarrhea and constipation ■ Unpredictable absorption rate – Antacids, phosphates and oxalates hamper absorption. ■ Difficulty in replenishing stores
- 47. Response to therapy (1)Senseof well being (2) Increased appetite (3) Improved outlook of the patient (4) Hematological examination: (a) Rise in hemoglobin level (b) Hematocrit value returning to normal (c) Reticulocytosis within 7–10 days. If no significant improvement is evident clinically and hematologically within 3 weeks, diagnostic re-evaluation is needed. • Rate of improvement: The improvement should be evident within 3 weeks of the therapy. After a lapse of few days, the hemoglobin concentration is expected to rise at the rate of about 0.7 gm/100 mL per week.
- 48. Faiure of therapy (1)Improper typing of anemia ■ (2) Defective absorption due to associated gastrointestinal disorders ■ (3)The patient fails to take iron ■ (4) Concurrent blood loss as in hookworm infestation or bleeding piles ■ (5) Inhibition of erythropoiesis by infection ■ (6) Co-existent folate deficiency. ■ • Contraindications of oral therapy: ■ (1) Intolerance to oral iron. ■ (2) Severe anemia in advanced pregnancy. Considering the unpredictable absorption and utilization following oral therapy, parenteral therapy is the preferred choice.
- 49. Paentral iron therapy Insufficient or no response to oral iron Severe anaemia Insufficient absorption of oral iron due intestinal Disease Need for rapid efficacy Intolerance of oral iron Poor compliance Advantage isThe expected rise in hemoglobin concentration after parenteral therapy is 0.7 to 1 g/100 mL per week.
- 50. Parenteral iron ■ Totaldose infusion (TDI):The deficit of iron is first calculated and the total amount of iron required to correct the deficit is administered by a single sitting intravenous infusion. ■ The compound used is iron dextran compound, or iron (ferrous) sucrose. ■ Iron sucrose is safe, effective and has less side effects (ACOG–2008). ■ Advantages: ■ (1) It eliminates repeated and painful intramuscular injections. ■ (2)The treatment is completed in a day and the patient may be discharged much earlier from the hospital. ■ (3) It is less costly compared to the repeated intramuscular therapy.
- 51. Types of parenteraliron ■ Iron dextran ■ Preparation: Iron Dextran (Imferon) - Intramuscularly ■ Dose: Elemental iron needed (mg) = 0.66 x(Desired HB- Patient’s Hb) xWeight (kg) + 500 ■ Disadvatages- persistent pain, skin discoloration ■ Iron sucrose ■ Preparation: venofer/ orofer - Intravenously ■ Dose: Elemental iron (mg)= (Normal Hb - patient’s Hb) x weight (kg) x 0.24 + 500 ■ Less side effect and better tolerated, improved ID in shorter period
- 52. Limitations ■ (1) Asthe maximum hemoglobin response does not appear before 4 to 9 weeks, the method is unsuitable if at least 4 weeks time is not available, to raise the hemoglobin to a safe level of 10 g% before delivery. ■ Thus, it is mostly suitable during 30–36 weeks of pregnancy where the patient is unwilling or unable to complete the course of intramuscular injections ■ (2) Previous history of reaction to parenteral therapy is contraindicated for its use.
- 53. Intramuscular therapy ■ Thecompounds used are • Iron sucrose • Iron-dextran (Imferon) • Iron-sorbitol citric acid complex in dextrin (Iron sorbitol complex—Jectofer) ■ Both the preparations contain 50 mg of elemental iron in one milliliter. ■ Total dose to be administered is calculated as that previously mentioned in intravenous therapy.
- 54. Drawbacks ■ (1)The injectionsare painful although less with Jectofer ■ (2) Chance of abscess formation and a discoloration of the skin over the injection sites are real problems specially with Imferon . ■ (3) Reactions are far and few—pyrexia, lymphadenopathy, headache, nausea, vomiting and allergic reactions are infrequently met with.
- 55. Blood transfusion ■ (1)Tocorrect anemia due to blood loss and to combat postpartum hemorrhage. ■ (2) Patient with severe anemia seen in later months of pregnancy (beyond 36 weeks)— to improve the anemic state and oxygen carrying capacity of blood before the patient goes into labor. ■ The primary concern is not only to correct anemia but also to make the patient to withstand the strain of labor and blood loss following delivery. ■ (3)Refractory anemia:Anemia not responding to either oral or parenteral therapy inspite of correct typing. ■ (4) Associated infection
- 56. During labor ■ Firststage: • The patient should be in bed and should lie in a position comfortable to her. • Arrangements for oxygen inhalation is to be kept ready to increase the oxygenation of the maternal blood and thus diminish the risk of fetal hypoxia. • Strict asepsis is to be maintained to minimize puerperal infection. ■ Second stage: • Asepsis is maintained. Prophylactic low forceps or vacuum delivery may be done to shorten the duration of second stage. Intravenous methergin 0.2 mg should be given following the delivery of anterior shoulder. ■ Third stage: • Significant amount of blood loss should be replenished by fresh packed cell transfusion after taking the usual precautions mentioned earlier. • The danger of postpartum overloading of the heart should be avoided.
- 57. Megaloblastic anemia • Itis derangement in red cell maturation with the production in the bone marrow of abnormal precursors known as megaloblasts due to impaired DNA synthesis. • Thus, it may be regarded as a deficiency disease caused by lack of either vitamin B12 or folate or both. • Folic acid deficiency is most common cause • At cellular level Folic acid reduced to Dihydrofolicacid thenTetrahydro-folicacid . (THF) c is required for cell growth & division. • So more active tissue reproduction & growth more dependant on supply of folic acid.
- 58. Diagnosis ■ Increased MCV( > 100 fl) ■ Peripheral smear: - Macrocytosis, hypochromia ■ Hyper segmented neutrophils (> 5 lobes) ■ Neutropenia ■ Thrombocytopenia ■ Low Serum folate level. ■ Low RBC folate.
- 59. Daily folate requirementfor ■ Non pregnant women - 50 -100 microgram ■ Pregnant woman – 300-400 microgram ■ Usually folic acid present in diets like fresh fruits and vegetables and destroyed by cooking. ■ Folate deficiency: ■ 0.5-1.0mg folic acid/day ■ If history of neural tube defect ■ - 4mg folic acid/day.
- 60. treatment ■ PROPHYLACTICTHERAPY: Allwoman of reproductive age should be given 400 μg of folic acid daily. ■ Additional amount (4 mg) should be given in situations where the demand is high. Such conditions are : ■ multiple pregnancy, ■ patient having anticonvulsant therapy, ■ hemoglobinopathies or associated chronic infection or disease. ■ Women, who have infants with neural tube defects, should be given 4 mg of folic acid daily beginning 1 month before conception to about 12 weeks of pregnancy. ■ .
- 61. CURATIVE ■ Specific therapyincludes—daily administration of folic acid 4 mg orally which should be continued for at least 4 weeks following delivery. ■ Supplementation of 1 mg of folic acid daily along with iron and nutritious diet can improve pregnancy induced megaloblastic anemia by 7 to 10 days. ■ Response is evidenced by ■ (i) sense of well being and increased appetite ■ (ii) increase in reticulocyte, leukocyte and thrombocyte count ■ (iii) rise in hemoglobin level. ■ Folic acid should never be given without supplemental iron. ■ Supplementary intramuscular vitamin B12 100 μg daily or on alternate days may be added when response to folic acid alone is not adequate. ■ Ascorbic acid 100 mg tablet thrice daily enhances the action of folic acid by converting it into folinic acid
- 62. Sickle Cell Haemoglobinopathy -Innormal individual Hb consists of 96% HBA (aabb), 3% HbA2 (aadd), and 1%HbF (aayy). -Sickle hemoglobin(HBS) originates from single beta chain substitution of glutamic acid by valine at codon 6 of beta globin chain -Include 1)Sickle cell anaemia-HbSS 2)Sickle cell Hemoglobin C disease-HbSC 3)Sickle cell BetaThalassemia-HbSB 4)Sickle cell E disease-HbSE
- 63. ■ Abnormalities ofHemoglobinA- ■ 1)Quantitative-Beta thalassemia ■ 2)Qualitative-Sickle cell disease ■ Homozygous –Sickle cell gene inherited from both parents ■ Heterozygous-Sickle cell gene from one parent ■ HbA-70%
- 64. ■ Pathophysiology: – RBCswith HbS undergo sickling on deoxygenetion. – Constant sickling and desickling causes membrane damage and persistant sickling. – Presence of HbS increases stickiness of RBCs.
- 65. ■ Consequence ofsickling: 1 endothelial cell adhesion,erythrocytic dehydration,vasomotor dysregulation Ischaemia and infarction of organs-resulting in PAIN Sickle cell crisis
- 66. Complications ■ -Femoral andhumeral osteonecrosis ■ -Nephropathy ■ -Autosplenectomy ■ -Splenomegaly ■ -Hepatomegaly ■ -Pulmonary infarctions ■ Leg ulcers ■ Acute chest syndrome
- 67. Pregnancy complications ■ -Cerebralvein thrombosis ■ -Pneumonia ■ -Pyelonephritis ■ -Pulmonary embolism ■ -DVT
- 68. Delivery complications ■ -GestationalHypertension and pre-eclampsia ■ -Eclampsia ■ -Placental abruption ■ -Preterm delivery ■ -IUGR
- 69. Management during pregnancy Pre-pregnancycouncelling -Women should be informed that hypoxia,cold ,dehydration,stress,overexertion may precipitate crisis. -Partners Hb electrophoresis and couple should be counselled about the baby being affected . -Complete evaluation of status of the disease and chronic complications like cardiac,renal to be done. -Folic acid 5mg/day and penicillin prophylaxis’ -Vaccination-Pneumococcal,meningococcal,H.influenza B
- 70. ■ Antenatal ■ -Jointmanagement by hematologist and obstetrician ■ Sickling test,Routine lab investigations to be done. ■ Iron suppliments if ferritin is low. ■ Low dose aspirin 75 mg ■ Regular BP monitoring and urine analysis to detect pre-eclampsia
- 71. ■ Prophylactic bloodtransfusion-controversial ■ Fetal surveillance to detect IUGR and fetal wellbeing
- 72. ■ Labour andcontraception- ■ -Maintain hydration,avoid infections ■ -Prevent supine hypotension ■ -monitor oxygen saturation by pulse oximeter. ■ -ABG analysis ■ -Opoids given,Pethidine avoided ■ -Cesaren section only for obstetric indication. ■ Epidural anaesthisia preferred. ■ Puerperal care- ■ -LMWH –Vaginal delivery -7 days ■ -Caesarean section-6 weeks ■ Contraception-POP,DMPA,LNG IUD
- 73. Treatment ■ 1)Hemoglobin Finduction- ■ -Mechanism-Gamma chain synthesis-Increases Hemoglobin F which inhibits Hemoglobin S polymerization. ■ -Agent-Hydroxyurea ■ 2)Hematopoetic stem cell transplantation-including cord blood stem cell transplantation ■ 3)Bone marrow transplantation
- 74. Thalassaemia ■ Classified accordingto globin chain that is deficient ■ Types of beta thalassaemia ■ 1)Minor-one locus of globin chain lacking ■ 2)Major-Both beta chains absent ■ 3)Intermediate-Both beta chains absent plus reduction in alpha chains.
- 75. Management ■ Thalassaemia major ■-Preconceptional counselling ■ -Maternal cardiac function,thyroid function,blood glucose measured. ■ -Serum ferritin maintained between 1000-2000 ng/ml.Desferroxamine given only before pregnancy ■ -Husbands electrophoresis. ■ -Indirect coombs test.
- 76. ■ Maintain hemoglobinlevel of 10 gm/dl. ■ Blood transfusion. ■ Vitamin C stopped. ■ Folic acid supplementation. ■ Fetal well being assessment
- 77.
- 78. ■ Platelet count- < 150000 ■ Mild – 1,50,000 to 1,00,000 ■ Moderate – 1,00,000 to 50,000 ■ Severe – 50,000
- 79. ■ Thrombocytopenia inpregnancy may be due to – 1. Defective production 2. Sequestration ( enlarged spleen ) 3. Accelerated destruction : 1 Non – immunological : pre eclampsia HELLP syndrome abruptio placenta DIC 2 Immunological : thrombocytopenic purpura lupus anticoagulant SLE antiphospholipid antibody
- 80. ■ A benigncommon disorder ■ Etiology is unknown ■ Appears around 8% pregnancy ■ Women are asymptomatic and have completely negative history ■ Treatment : if platelet < 20,000 – RDP , SDP steroids are given to raise platelets Gestational thrombocytopenia
- 81. ■ Incidence –1-2 in 10,000 pregnacy ■ Characterized by an autoantibody – mediated destruction of maternal platelets ■ Thrombocytopenia in first half of pregnancy is suggestive of possibility of ITP as a diagnosis. ■ When ITP appears during pregnancy , both mother and foetus affected as IgG crosses placenta ImmuneThombocytopaenia
- 82. ■ ITP inpregnancy monitored on monthly basis and then fortnightly in the last trimester ■ Treatment : Corticosteroids: first-line therapy in pregnancy: lower doses (20–30 mg/day), which are safe and effective. Splenectomy should be avoided in pregnancy if possible may be necessary in extreme cases. Ideally it should be performed in the second trimester Women with ITP who have previously been treated with splenectomy should continue penicillin prophylaxis throughout pregnancy
- 83. ■ Platelet transfusionsas a last resort for bleeding or prior to surgery will increase antibody titres and do not result in a sustained increase in platelet counts.
- 84.