HAEMOTOLOGICAL DISORDERS IN PREGNANCY
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Transcript of 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 % - Developed51% - Developing countries65-75% - India80 % leading to maternal deaths
DEFINITIONReduction in circulating Hb mass < 12g/dl in non-pregnant women<10 g/dl in pregnant womenCDCAnaemia in iron supplemented preg. WomanHct 33% & Hb 11g/dl – 1st & 3rd trimesterHct 32% & Hb 10.5 g / dl - 2nd trimester
WHO grading of anemia
Mild 10g/dlModerate 7- 10 g/dlSevere < 7 g/dl
ICMR GRADING Range in g/dl
MILD 10 – 10.9
MODERATE 7 – 9.9
SEVERE < 7
VERY SEVERE < 4
Hemotological Changes in preg.
Physiological Anemia of pregnancyPlasma volume s 40-50%RBC mass s 30 %As a result Hb concentration decreases by 2g/dlDecreased Hb concn. Is due to haemodilution
Criteria of Physiological Anemia1) Hb 10 gm %2)RBC 3.2 million cells / cu mm3)PCV 32%4)Peripheral Smear – Normal morphology
Classification of Anaemia
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ERYTHROPOISES
IRON METABOLISM
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. anemiaIron Deficiency Anemia – 3 stagesa)Depletion of Iron storesb)Iron deficient erythropoiesisc)Frank Iron deficiency Anemia
Symptoms of IRON DEFICIENCY ANEMIAFatigueWeaknessHeadacheLoss of appetiteDysphagiaPalpitationsDyspnea on exertionAnkle swellingParesthesiasLeukoplakia
Physical examinationPallor of varying degrees (Mucous membranes
, nail beds – Koilonychia or PlatynychiaGlossitisStomatitisHeart murmursIncreased JVPTachycardiaTachypneaPostural hypotension Crepitations- due to lung congestion
Depletion of Iron storesFerritin <20 ng/mlHb / Hct. NormalRBC INDICES normal
Iron deficient erythropoiesisFerritin <20 ng/mlTransferrin saturation<25%Hb –NormalSerum Iron < 60mg/dl
c)Frank Iron deficiency Anemiaferritin <20 ng/mlTransferrin saturation<25 %Serum iron <60 mg/dlHb <10g/dl, Hct.<28%
Microcytic Hypochromic
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
Iron Supplements
Ferrous sulphate 300mg Tid orally daily after meals
To be contd for 12 months after anemia is corrected
Indicators of iron therapy response1.Increase in Reticulocyte count (Increases 3-5 days
after initiation of therapy )2.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 ADMINISTRATIONINDICATIONS1.Intolerance to oral iron2.Non compliance pt.3.Inflammatory bowel disease4.Pt. unable to absorb iron orally5.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 DefciencyPathophysiologyPreg. 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/ml2.Erythrocyte Folate levels - <20 ng/ml3.Peripheral smear – Hypersegmented
neutrophils,Oval macrocytes,Pancytopenia
TreatmentPregnancy induced megaloblastic anaemia- Folic acid, nutritious diet & Iron .Supplementation of 1mg of folic acid daily can
improve MA by 7 to 10 daysFolic acid should be given with ironAscorbic acid 100mg Tid enhances actionIn other conditionsRecommended folic acid dose – 5mg /day
orally dailyProphylaxisWHO – 400 micrograms folic acid daily to
prevent neural tube defects
Vit – B12 Deficiency
PathophysiologyVit B12 absorption is unaltered during pregnancyTissue uptake is increased Decreased serum
B12Recommended B12 intake – 3 microgram /day.
CAUSES of Vit B12 def.Strict Veg. dietUse of proton pump inhibitorsMetformin.GastritisGastrectomyIleal bypass Crohn’sH. Pylori infection
Gastric juice IF Antibody
Pathogenisis of
PERNICIOUS
ANEMIA
Clinical manifestations
Macrocytic Megaloblastic AnemiaGlossitisPeripheral neuropathySubacute combined degeneration of the
Spinal cord
DIAGNOSISSer.Vit B12 levels ,100 pg /mlRadio active Vit B12 absorption test .
( Schilling Test )Treatment1000 microgram parenteral
cyanocobalamin every wk * 6 weeksPernicious Anaemia – Oral Vit B12Total Gastrectomy – 1000 microgram Vit
B12 im every month.Partial gastrectomy – Ser. Vit B12 levels
measured.
ANAEMIA ASSOC. WITH CHRONIC INFECTIONS / DISEASE
Common in developing countriesPoor response to Haematinics unless primary
cause is treatedWorm infestations is common ( Diagnosed by
stool examination )Urinary tract inf, & asymptomatic bacteriuria
in preg. Is assoc. with refractory anaemiaChronic renal disorders = due to
erythropoietin def.Treated with recombinant Erythropoitin
Anaemia from acute blood lossIn 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 anemiaAUTOIMMUNE HEMOLYTIC ANEMIAAUTOANTI-BODIES OF iGg OR WARM
ANTIBODIES AGAINST Red cell antigens, causes premature destruction of RBC”s
ETIOLOGYLymphomas,Leukemias , Connective tissue
diseases, Infections , Chronic. Inflammatory diseases & drug induced antibodies
DiagnosisDirect Coomb’s TestBlood smear – Spherocytosis &
Reticulocytosis
TREATMENT Prednisone 1mg / kg / day
orally Azathioprine Splenectomy
2)Preg. Induced hemolytic anemiaUnexplained hemolytic anemia uring pregnancy is rareSevere hemolysis occurs early in pregnancy & resolves
within months after deliveryNo evidence of immune mechanism or defects in RBCsPrednisone given untill delivery
3) Paroxysmal Nocturnal HemoglobinuriaAcquired hemolytic anemiaArises from one abnormal clone of cells like neoplasmAnemia 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 requiredBone marrow transplantation – Definitive
treatment
Effect on pregnancySerious & unpredictableMaternal mortality 10 – 20%Venous thrombosis occurs during post partum
APLASTIC ANAEMIARarely seen in preg.Marked decrease in marrow stem cels
ETIOLOGYInfectionsIrradiationLeukemiaImmunological disorders
May be Immunological mediated or autosomal recessive inheritance
30% cases Anaemia improves once pregnancy is terminated.
ComplicationsInfectionHaemorrhage
DiagnosisBlood Values – Anemia Leucopenia Thrombocytopenia
Bone Marrow - Hypocellular
ManagementSupportive care – Cont. Infection surveillance &
anti microbial therapyRed cell transfusions to maintain Hct. > 20Granulocyte transfusion given only during
InfectionsPlatelet transfusion to control haemorrhage.Glucocorticoid therapy may be helpful
IN SEVERE casesBone marrow or Stem Cell TransplantationVaginal delivery is preferred
Effect of anaemia in preg.In MOTHERDuring preg. Pre eclampsia Infectuion Heart failure Pretem labour
Labour Uterine inertia Postpartum Haemorrhage Cardiac failure Shock
Puerperium Puerperal sepsis Subinovulation Failure of lactation Puerperal venous thrombosis Pulmonary embolism
FetusAmount of iron transferred to fetus is
unaffected even if mother is in iron deficient state
PrematurityLow birth weight babiesIntra uterine deaths due to severe
maternal anoxemia
Effect of pregnancy in anaemia
Pt. Mildly anemic progresses to marked Anaemia
Pt. Who is severely anemic becomes symptomatic by the end of 2nd trimester
DIAGNOSIS OF ANEMIA
DURING PREGNANCY
PLATELET DISORDERSThrombocytopenia - Gestational - Immune mediated Mild – 1,50,000 – 1,00,000 Moderate – 1,00,000 – 50,000 Severe - < 50,000Abnormalities of Platelet function
Causes of Thrombocytopenia during PregnancyCOMMON CAUSES1.Gestational Thrombocytopenia2.Severe Pre-eclampsia3.HELLP syndrome4.Immune thrombocytopenic Purpura5.Disseminated Intravascular coagulation
RARE CAUSES1.Lupus anticoagulant/APA syndrome2.SLE3.Hemolytic Uremic Syndrome4.Type 2b Von- Willebrand’s syndrome5.Folic acid def.6.HIV infections7.Hemotoligical malignancies8.May – Hegglin syndrome – Congenital
Thrombocytopenia
Gestational ThrombocytopeniaBenign Common disorderAppears in 8% of all preg.Unknown causeRarely drops < 70,000 /mm3
FEATURESDiagnosis of Exclusion – No specific test availableMild Thrombocytopenia , Count > 70,000 – 1 lakhNo maternal bleedingNo past history of thrombocytopeniaOccurs in 3rd trimester No assoc. fetal thrombocytopeniaSpont. Resolution after deliveryMay reccur in subsequent pregnancies
ManagementMajority cases treated as normalIn mod. To severe cases – Reluctance of
Anaesthesiologists to give spinal or epidural if Platelets = < 80,000 /cu mm
Treatment with steroids & IgG or platelet transfusion before delivery
Cord sample should be takenSamples taken on day 1 & 4CS reseved for obstetric indications
Immune ThrombocytopeniaChronic condition , Incidence 1 in 1000 to 1
in 10000 pregnanciesCharecterised by autoantibody mediated
destruction of maternal platelets
MECHANISMAutoanti bodies react with platelet
Glycoprotein complex & antibody coated platelets are phagocytosed by Macrophages
SYMPTOMSUsually asymptomatic , sometimes
Bleeding , Petechiae
DIAGNOSISPlat count < 50,000 / cu mm with
past h/o bleeding disordersNo specific diagnostic test
Prepregnancy counselling for ITP ( RCOG 2009 )May relapse or worsenIf treatment reqd, it will carry for both
maternal & fetal risksIncreased risk of Hemorrhage at deliveryEpidural Anaesthesia is not possibleRisk prediction in neonate is not possible.
High risk if sibling has thrombocytopenia or mother has undergone splenectomy
Maternal deaths / serious outcome – RARERisk of Intracranial Haemorrhage in fetus
is very low.
ManagementAdequate Plat. Count should be maintainedCounts monitored throughout pregnancyIf > 30,000 – No treatmentIf< 30,000 - 1 ) Prednisolone 1 -2 mg / kg oral daily, 2) IV IG – 2g/kg over 2 to 5 days , If no
response then 3) Splenectomy 4) Immunosuppressive drugs like a) azathioprine b) Cyclophosphamide ,
Cyclosporine
Management Of deliveryPlatelet > 50,000 / cu mm – Vaginal or
operative deliveryPlatelet 50,000 Platelets standbyCS not routinely recommendeMeasures should be taken to avoid trauma
to baby headCord sample taken, If low confirmed by
capillary sampleIf count low, further day 1 & 4 is collectedInj im Vit K avoided till count is known
Fetal & Neonatal EffectsPA IgG antibodies crosss placenta causes
fetal & neonatal ThrombocytopeniaMaternal treatment do not have effect o fetal
countRole of Intracaranial hemorrhage < 1 %
MICROANGIOPATHIESThrombotic thrombocytopenic purpuraRare – life threateningSigns & symptoms ( PENTAD )1.Microangiopathic hemolytic anemia2.Thrombocytopenia3.Neurological symptoms4.Renal dysfunction5.Fever
ETIOLOGYSevere def. of VON WILLIBRAND FACTOR
( cleaving protein ( ADAM TS13)Acqd autoantibodyCongenital Genetic defect
Incidence 1 in 25000 pregnanciesTime of onset of TTP is variable 1st trimester to several wks post partumMaternal mortality is high
MANAGEMENTACQUIREDPlasma Exchange Fresh frozen Plasma infused daily until Platelets
turn to NormalRituximab, Monoclonal antibodies against CD20
CONGENITALFFPPlatelet transfusion contraindicated
HEMOLYTIC UREMIC SYNDROMEMicroangiopathic hemolytic anaemia &
thrombocytopenia with predominant Renal involvement
Due to endothelial damage by bacterial or viral infections
In Preg. Response is poor for plasma exchange
THROMBOCYTOSISDefined as persistant Platelet count >4.5 lakhs /
cummCAUSES1 )Secondary or Reactive ( > 80000) a)Iron def. b) Infections c) Splenectomy d) Surgery & Trauma ( bone fractures ) e) Malignancy 2) Essential Thrombocytosis > I million a) Idiopathic b) Myelodysplastic syndromes
SIGNS & SYMPTOMSUsually asymptomaticArterial & venous ThrombosisHepatomegalyBone marrow – Hyperplastic with gross
increase in megakaryocytesBlood picture ->1 million Leucocytosis Anemia or mild
polycythemia Anisocytosis &
Poikilocytosis
In Pregnancy – Spont. Abortion , fetal demise & preeclampsia.
TREATMENTAspirin , Dipyramidole, Heparin,
Plateletpheresis
PROGNOSIS depends on underlying diseaseDeath due to either thrombosis /
hemorrhage / comp of Myeloproliferative disorders/ marrow failure.