dr mere kende, -...
TRANSCRIPT
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Dr Mere Kende, MBBS (UPNG), MMED (Path), MAACB (AUS), MACTM (AUS), MACRRM (AUS)
Lecturer-SMHS
UPNG
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Outline
Hemopoiesis in Normal Pregnancy
Anaemia
Iron Deficiency
Folate Deficiency
Aplastic Anemia
Hemolysis
Drug-induced/G6PD
Thalassaemia
Sickle Cell
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Hemopoesis in Normal Pregnancy
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Hemopoesis in Normal Pregnancy Blood Volume
Increases 20-100% (av . 45-50%)
Rapid 1st tri------plateaus at 30th wks
Large women >small
Singlet>multiple
?Mechanism- Hormonal/aldosterone, increased fetal demand
increased kidney perfusion
prepare for maternal loss e.g., 500-600mls vaginal --1000mls C/s
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Changes in RBC/iron Red Cells
Increased 33% ~450mls RBC
Increase is greater with iron supplements
Plasma increase>RBC until end of 2nd trimester
Iron Increase RBC....> increased requirement for iron
No supplement = iron deficiency
Fetus extracts iron from mother
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Changes in WBC/PLT White Blood cells Normal 400-5000/mcl
Increased to 2-3x normal or even up to 5-6x
?Mechanism – reduced chemotaxis/adherence in 3rd trimester
–prone to infection
Platelets
Increased production and consumption
Increases in both PGI2 (aggregation inhibitor) & TXA2 (aggregation inducer),
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Changes in coagulation factors Clotting factors
Increased viz. Fibrinogen (I) and factor VIII (+++) and Factors (VII, IX, X, XII) +
Factor XIII (fibrin-stabilising factor) decreased 50% towards term
Depressed fibrinolysis
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Anemia Significant problem in Pregnancy
Investigate if Hb<11g/L or HCT <33%
Anaemia will worsen from blood loss during birth of child (vaginal/caesarean Section).
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Causes Nutritional iron & folate deficiency (common)
Aplastic Anaemia (parvo virus B19)
Drug-induced hemolysis
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Iron Deficiency Accounts for 95% of anaemia in pregnancy
Iron requirement increases in pregnancy
Hb (contains 70% of body iron)
Storage iron (ferritin, haemosiderin, RES) ~300mg
Diet supply must meet increased demand by growing foetus
Decreased BM iron (hemosiderin) is early sign of iron deficiency
If severe, can endanger foetal life
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Causes of iron deficiency. Deficient diet (children on milk)
Decreased absorption
Increased requirements
Pregnancy
Lactation
Blood loss (common)
Gastrointestinal (PUD/aspirin)
Menstrual
Blood donation
Hemoglobinuria
Iron sequestration
Pulmonary hemosiderosis
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Absorption: stomach, duodenum, and upper jejunum.
Diet iron: heme is efficiently absorbed (10–20%) , Nonheme iron less so (1–5%)
Loss ---approximately 1 mg/d—are normally lost through exfoliation of skin and mucosal cells.
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Iron requirement in Pregnancy Requirement for iron increases in 2nd trimester
Mother: 500mg (increased RBC & Hb)
Baby: 300mg (growth)
Total over course of pregnancy 800mg
Requires 3.4mg/day or elementary iron >40mg/day
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Clinical Findings Pallor
Tachycardia/Palpiations
Tiredness/Easy fatigue
Low Hb (hypochromic microcytic)
If severe Glossitis, stomatitis, koilonykia
may endanger baby
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Laboratory Findings Iron Studies:
low iron,
low ferritin,
Elevated TIBC & transferrin
decreased % saturation,
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Iron Parameters
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Red Cell indices: low MCV, low retics
WbC normal
Elevated soluble transferrin receptor
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Blood Film: hypochromic Microcytic
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Differential Diagnosis Thalassaemia Trait (normal iron studies, elevated
HbA2)
Chronic Inflammation (can see hypochromia, microcytosis)
Sideroblastic Anaemia
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Complications Heart: Angina/CHF
Plummer-Vinson syndrome
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Prevention Iron supplements in pregnancy (at least 60-80mg/day
elemental iron daily
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Treatment Oral iron –Ferrous Sulphate 300mg (60mg
elementary iron -> 10% absorbed) tds
Hb increased by 3-5g/L/week if responding
Continue 3 months after Hb is normal to replenish stores
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Iron salt Amount Contents of Ferrous iron
Ferrous fumarate 200mg 65
Ferrous gluconate 300mg 35
Ferrous succinate 100mg 35
Ferrous sulphate 300mg 60
Ferrous sulphate, dried 200mg 65
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Only marginal differences between Fe++ salts
Choices depend on cost and side effects
Treatment dose is t.d.s versus daily prophylaxis dose
Some combined with vitamin C or folate (fefol)- cost?
Fefol 150mg ferrous sulphate (47mg iron) / 500mcg folate
Fefol is inadequate to treat megaloblastic anaemia
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Parental Iron Different Forms available
Iron dextran (ferric-OH + dextran)—IV/IM injection
Iron sorbitol (ferric- OH +sorbitol)-IV/IM injection
Iron sucrose (ferric –OH + sucrose)-deep IM injection
Reserved for unsuccessful oral iron treatment
Response similar to oral dose
Risk of Anaphylaxis (small test dose initially)
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Folate in Pregnancy/Megaloblastic Anaemia
Folate (+ vit B12) is required for DNA synthesis in Red cell maturation
It is essential for the growth and division of all body cells for healing processes.
It aids protein metabolism
Helps prevent premature greying
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Lack of folate is associated with LBW and Neural Tube Defect.
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Valuable sources Deep FGLV such as spinach, lettuce, brewers yeast,
mushrooms , nuts, peanuts and
liver.
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Incidence on BM studies 25-60% (depends on population studied)
Inadequate dietary folate in pregnancy
Demand for folate increased in Pregnancy
Minimum requirement: 50mcg/day
Requirement increases to 800mcg in pregnancy
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Folic Acid Deficiency Anaemia Demand is greater with multiple pregnancy and
multigravid
Megalobalstic picture in pregnancy always implies folate deficiency
Megaloblastic picture may be concealed by iron deficiency or thalassaemia
May recur in subsequent pregnancy
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Other causes of folate deficiency Impaired absorption
OCP
Antibiotics (bactrim/septrin)
Anticonvulsants (phenytoin/barbiturates)
Malabsorption syndromes
Increased requirement
Hemolysis
Hemoglobinopathies
Malaria infection
Alcohol use
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Clinical Findings Non specific
Anorexia
Nausea & vomiting
Diarrhoea
UTI is common
Purpura (occasionally)
Sore mouth & tongue is rare
Pallor is not marked
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Other symptoms serious skin disorders
Loss of hair
impaired circulation
Fatigue
Mental depression.
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Laboratory tests Blood Film
hypersegmented neutrophils (5 lobes)
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Bone Marrow - megaloblastic change
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Ovalo- macrocytosis Anisocytosis Poikilocytosis Low reticulocytes, +/-nucleated red cels Pancytopenia (only if severe)
High MCV >100fl
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A red blood cell folate level of less than 150 ng/mL is diagnostic
Serum vitamin B12 (deficiency extremely rare in pregnancy)
Iron studies: normal.
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Therapy Prevention: folate supplements in pregnancy
Treatment: 1-5mg/day continued several weeks post-partum to replace body stores
Prognosis-good with folate replacement
70% of patients with megaloblastic anaemia also need iron replacement.
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Hemolysis
Intrinsic
Membrane
Hereditary
Sperecytosis/ Ovalocytosis
Enzyme
G6PD Deficiency
PK Deficiency
Hemoglobinopathies
Thalassemia
Unstable Hb
Sickle Cell
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Hemolysis Extrinsic
Ab -mediated
Coomb’s +ve autoimmune, ABO/Rh-related, Drug Induced,
Cold Agglutinins,
T-antigen activation
Microangiopathy
HUS
TTP
DIC
Post-heart valve
Infections
Malaria
Severe Sepsis
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General Features of Hemolysis General exam: Jaundice, Pallor, dark urine
Other physical findings Spleen may be
enlarged; bossing of skull in severe congenital cases
Hemoglobin: From normal to severely reduced
MCV, MCH Usually increased
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Reticulocytes Increased (% & absolute nos.)
Bilirubin Increased (mostly unconjugated)
LDH Increased (up to 10X normal with intravascular hemolysis)
Haptoglobin Reduced to absent
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Other laboratory abnormalities; increased AST
Increased urine/stool urobilinogen
Increased Urine Haemoglobin (Hemoglobinuria)
No bilirubinuria
Increased serum Hb (haemoglobinaemia)
Increased Bone Marrow Activity Macrocytes (retics & sometimes nucleated RBCs)
Polychromasia
erythroid hyperplasia.
Once a HA is suspected, specific tests will usually be required for a definitive diagnosis of the specific type of HA.
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Autoimmune Hemolytic Disease
Mechanism of antibody-mediated immune destruction of red cells
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Used to diagnose AIHA.
Coomb's reagent Is a rabbit IgM antibody raised against human IgG or human complement.
Direct Coomb's test -
mix the patient's red blood cells with the Coomb's reagent Positive Agglutination Presence of antibody on the red blood cell surface.
Indirect Coomb's test
Mixing the patient's serum with a panel of type O red blood cells. Incubate test serum and panel O red blood cells, Add Coomb's reagent Positive Agglutination Presence of free antibody in the patient's serum.
Coomb’s antiglobulin test
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Drug-Induced Hemolysis Decreased G6PD activity in 1/3 of patients in 3rd
trimester
Increased risk of hemolysis
Over-exposure of G6PD deficiency fetus to sulphonamides commonly prescribed for UTI – risk of fetal hemolysis
Pregnant mother can be screened for G6PD status
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G6PD Deficient Hemolytic Anemia
Hereditary enzyme defect
Decreased ability of RBC to withstand oxidative stress-----episodic hemolytic anemia
Oxidized hemoglobin ----denatures -----Heinz bodies (precipitants).
Heinz bodies ----------membrane damage --------- removal of these cells by the spleen.
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Hexose Mono phosphate Pathway
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Genetic Considerations G6PD gene is X-linked,
Males have only one G6PD gene (i.e., they are hemizygous for this gene),
Females, having two G6PD genes, can be normal, deficient (homozygous), or intermediate (heterozygous).
As a result of the phenomenon of X-chromosome inactivation, heterozygous females are genetic mosaics, with a highly variable ratio of G6PD-normal to G6PD-deficient cells and an equally variable degree of clinical expression;
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Some heterozygotes can be just as affected as hemizygous males.
Gene defect: -mutations in the coding region of the G6PD gene.
Almost all of the 140 different mutations known are single (missense )point mutations, entailing single amino acid replacements in the 514 amino acid G6PD protein.
G6PD activity decreases with age of RBC so aging cells are more susceptible hemolysis
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Clinical Findings G6PD deficiency is an X-linked recessive disorder –
mainly affects males
USA: 10–15% of American black males.
Female: carriers are rarely affected—only when an unusually high percentage of cells producing the normal enzyme is inactivated
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Symptoms and Signs Asymptomatic .
Hemolysis occurs as a result of oxidative stress on the red blood cells, generated either by infection or exposure to certain drugs.
Common drugs initiating hemolysis include dapsone, primaquine, quinidine, quinine, sulfonamides, and nitrofurantoin.
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Typically, a hemolytic attack starts with malaise, weakness, and abdominal or lumbar pain.
After an interval of several hours to 2–3 days,
patient develops jaundice
often dark urine (hemoglobinuria)
Most serious threat is acute renal failure (exceedingly rare in children).
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Laboratory Test Peripheral blood film
Bite cells/immature re cells/henz bodies
Reticulocyte count; increased
Haptoglobulin/LDH/AST/bilirubin
Low Haptoglobulin and increased LDH/AST/unconj Bil
Urinalysis: urobilinogen/bilirubin/Hb
High urobilinogen & hemoglobin; no bilirubin
G6PD assay: qualitative /quantitative test
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Heinz Bodies:
A classic test, is supravital staining with methyl violet (rarely done now)
Reveals the presence of Heinz bodies,
precipitates of denatured hemoglobin
Regarded as a signature of oxidative damage to red cells
Once the threat of acute anemia is over, and in the absence of comorbidity, full recovery from acute HA associated with G6PD deficiency is the rule.
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Peripheral Blood Smear
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Treatment Treat Anemia/Infection
Avoid offending drug/oxidant
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Thalassemia/Sickle Cell Anaemia Hemoglobinopathies
Genetic mutation in alpha or beta -globin chains
Family history present
Clinically:
Anaemia/chronic hemolysis
Symptomatic Trait
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Hypochromic microcytosis film
Risk of enhanced sickling of red cells during hypoventilation eg during General anaesthesia
Multi-organ symptoms with SC disease
Eg MSS pain and lung, CNS, renal or heart infarct or retinal vein thrombus
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Effects on Pregnancy Effects on Mother
Anaemia
Iron/folate deficiency
Risk of infection
Heart failure
Effects on Foetus
Stilbirth
LBW/IUGR/preterm
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Laboratory Diagnosis Blood Film-hypochromic, microcytosis, bizzare cell
FBE-Anaemia
Normal/increased iron status
Hb Electrophoresis
DNA studies
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Beta Thalassemia syndromes.
-Globin Genes Hb A Hb A2 Hb F
Normal Homozygous 97–99% 1–3% < 1%
T. Major Homozygous 0 0-10% 4–10% 90–96%
T. Minor Heterozygous 80–95% 4–8% 1–5%
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Hemoglobin distribution in sickle cell syndromes.
Genotype Clinical DX Hb A Hb S Hb A2 Hb F
AA Normal 97–99% 0 % 1–2% < 1%
AS Sickle Trait 60% 40% 1–2% < 1%
SS SC Anemia 0% 86–98% 1–3% 5–15% S-T SC-Thalassemia 0-20% 70–80% 3–5% 10–20% AS, -Th Sickle trait 70–75% 25–30% 1–2% < 1%
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Management Symptomatic
Transfusion
Prenatal Diagnosis (DNA studies) and counselling
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Aplastic Anaemia Rapid Development of anaemia
Bone Marrow failure (idiopathic/infection)
Pancytopenia and hypocellular BM
Increased risk of infection
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Pregnancy Complications:
FDIU/stillbirths
Prematurity
maternal death
Spontaneusly resolve post-patum
May recur in subsequent pregnancies
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Pancytopenia.
No abnormal cells seen.
Hypocellular bone marrow
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Causes "Idiopathic" (probably autoimmune) -MAJORITY
Chemotherapy
Radiotherapy
Toxins: benzene, toluene, insecticides
Drugs: chloramphenicol, phenylbutazone, gold salts, sulfonamides, phenytoin, carbamazepine, quinacrine, tolbutamide
Posthepatitis Pregnancy
Paroxysmal nocturnal hemoglobinuria
Congenital (rare)
Systemic lupus erythematosus (rare)
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Clinical Findings
Anemia (low RBCs)
weakness and fatigue,
Neutropenia (low neurtrophils)
vulnerability to bacterial infections
Thromtocytopenia (low platelets)
mucosal and skin bleeding.
Pallor, purpura, and petechiae .
hepatosplenomegaly & lymphadenopathy
No bone tenderness
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Laboratory Findings
Pancytopenia.
one or two cell lines may be reduced.
always associated with decreased reticulocytes.
Anemia may be severe
Normal Red blood cell morphology.
Neutrophils and platelets are reduced in number, and no immature or abnormal forms are seen.
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Bone Marrow Biopsy Hypocellular BM
Only scant amounts of normal hematopoietic progenitors.
No abnormal cells are seen
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Laboratory Features Pancytopenia
No abnormal cells seen
Hypocellular BM
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References 1. Emedecine.medscape.com
2. Current Obstetrics & Gynaecology, Diagnosis & Treatment, International 9th Edition