hematological dis in pregnancy Flashcards
CDC defined anemia in iron-supplemented pregnant women using a cutoff of the 5th percentile — IN 1ST and 3rd trimester, —- in 2nd trimester
—11 g/dL in the first and third trimesters, and 10.5 g/dL in the second trimester
Anemia is associated with several adverse pregnancy
outcomes including
preterm birth, Children born to iron-deficient women and without iron supplementation are
reported to have lower mental development scores
The two most common causes of anemia during pregnancy and the puerperium are
iron deficiency and acute blood loss.
In a typical singleton gestation, the maternal need for iron averages nearly
1000 mg.
Routinely in pregnancy, daily oral supplementation btw
30 to 60 mg of elemental iron and 400 μg of folic acid is recommended
For iron-deficiency anemia, resolution and restitution of iron stores can be accomplished with simple iron salts that provide approximately 200 mg daily of elemental iron.
for parenteral IV iron ferrous sucrose is safer than iron-dextran.
Chronic renal insufficiency is the most common disorder that cause anemia of chronic disease during pregnancy.
In pregnancies complicated by chronic renal insufficiency, recombinant erythropoietin is usually considered when the hematocrit approximates 20 percent
More folic acid daily 4-mg folic acid supplement is given with
Women who previously have had infants with neural-tube defects, multifetal pregnancy, hemolytic anemia, Crohn disease, alcoholism, and inflammatory skin disorders. Women with a family history of congenital heart disease may also benefit from higher doses
Those who have undergone total gastrectomy require — μg of vitamin B12 given intramuscularly each month.
1000 μg of vitamin B12 given intramuscularly each month.
Autoimmune Hemolysis: The cause of aberrant antibody production is unknown. Typically, both the direct and indirect antiglobulin (Coombs) tests are positive. Anemias caused by these factors may be due to warm-active autoantibodies (80 to 90 percent), cold-active antibodies, or a combination.
also may be classified as primary (idiopathic) or secondary due to underlying diseases or other factors. Examples of the latter include lymphomas and leukemias, connective-tissue diseases, infections,
chronic inflammatory diseases, and drug-induced antibodies
Coldagglutinin disease may be induced by
infectious etiologies such as Mycoplasma
pneumoniae or Epstein-Barr viral mononucleosis
Hemolysis and positive antiglobulin test results may be the consequence of either (IgM) or (IgG) antierythrocyte antibodies. When thrombocytopenia is comorbid, it is termed
Evans syndrome
Rituximab, along with prednisone, is first-line treatment
Drug-induced hemolysis is usually chronic and mild to moderate, but occasionally acute hemolysis is severe.
severe Coombs-positive hemolysis stimulated by cefotetan given as prophylaxis for obstetrical procedures. Alpha-methyldopa can cause similar hemolysis
The most fulminant acquired hemolytic anemia encountered during pregnancy is
caused by the exotoxin of Clostridium perfringens or by group A β-hemolytic streptococcus
Diamond-Blackfan anemia
pure red-cell hypoplasia. Approximately 40 percent of cases are familial and have autosomal dominant inheritance
Gaucher disease
autosomally recessive lysosomal enzyme deficiency
characterized by deficient activity of acid β-glucosidase. Affected women have anemia and thrombocytopenia that is usually worsened by pregnancy
Sickle-Cell Hemoglobinopathies
(hemoglobin S) originates from a single β-chain substitution of glutamic acid by valine, which stems from an A-forT substitution at codon 6 of the β-globin gene.
. Hemoglobin C originates from a single β-chain substitution of
glutamic acid by lysine, which stems from a T-for-C
substitution at codon 6 of the β-globin gene.
Chronic and acute changes from sickling include (complications Happened to sicklers)
bony abnormalities such as osteonecrosis of femoral and humeral heads, renal medullary damage, autosplenectomy in homozygous SS patients and splenomegaly in other variants, hepatomegaly, ventricular hypertrophy, pulmonary infarctions, pulmonary HTN, cerebrovascular accidents, leg ulcers, and a propensity for infection and sepsis, cerebrovascular aneurysms and sickle-cell vasculopathy
Treatment OF SCD
- hydroxyurea: which augments Hgb F production and reduces the number of sickling episodes
- crizanlizumab, an antibody against P-selectin, significantly lowered the incidence of adverse events.
Bone marrow transplantation has 5-year survival rates
that exceed 90%. Cord-blood stem-cell transplantation from related donors also shows great promise. Finally, successful gene therapy has been accomplished by lentiviral vector-mediated addition of a B-globin gene into stem cells
in PREGNANT women with sickle-cell syndromes they had a –fold greater incidence of hypertensive disorders— percent; a —–fold higher rate of fetal‑growth restriction— percent; and a –fold increased cesarean delivery rate— percent.
a 1.8-fold greater incidence of hypertensive disorders—19 percent; a 2.9-fold higher rate of FGR —6 percent; and a 1.7-fold increased cesarean delivery rate—45 percent.
Prophylactic Red Cell Transfusions For sicklers During pregnancy
the most dramatic benefit of prophylactic transfusions has been on maternal morbidity rates, Transfusions were given throughout pregnancy to maintain the
hematocrit above 25 volumes percent and the portion of hemoglobin S <60 percent, Maternal morbidity was minimal, and erythropoiesis suppression was not problematic. Their outcomes were compared with historical controls who were not routinely transfused. Overall, morbidity and hospitalization rates were significantly reduced in the transfused group, Still, adverse perinatal outcomes are prevalent
Sickle-Cell Trait carriers could have
occasional hematuria, renal papillary necrosis, and hyposthenuria, which is urine of low specific gravity
The congenital diseases associated with poor platelet function are divided into four types of dysfunction:
(a) adhesion to collagen,
(b) adhesion to subendothelium, e.g. von Willebrand disease, RF, CML
(c) release reaction defects, and
(d) ADP aggregation defects.
The laboratory assessment of the plasma clotting factors
prothrombin time(PT;factors V,VII,and X,prothrombin,and fibrinogen)and the activated partial thromboplastin time (APTT;factorsVIII,IX,XI,andXII).
Specific factor assays also can identify the exact deficiencies. One must remember that a factor deficiency a slow as 30% can generate a normal PTand APTT.
This relation is important in investigating minimal prolongations of the PT or APTT that appear insignificant but could be hiding a moderately severe deficiency.
SCD had Increase susceptibility to — and — infections
Increase susceptibility to pneumococcal and salmonella infections
main factors causing haemolysis in G6PD are:
infections, substances derived from plants, drugs with high oxidation-reduction potential, stress, ketoacidosis in diabetes and surgery operations.
Causes of warm Autoimmune haemolytic anaemia (AIHA):
- autoimmune disease: e.g. systemic lupus erythematosus
- neoplasia: e.g. lymphoma, CLL
- drugs: e.g. methyldopa
Beta Thalassaemia in Pregnancy , gene mutation, mode of inheritance ?
Caused by mutation HBB gene Chromosome 11
Autosomal Recessive Inheritance
Homozygous b-thalassaemia (Major) produces severe transfusion dependent anaemia
Heterozygote b-thalassamia (Minor/trait/carrier) produces mild microcytic anaemia
Initial Management of Beta Thalassaemia in Pregnancy ( RCOG produced Green-top Guideline No 66 in March 2014)
Diabetes should be screened for and well controlled. If detected refer diabetologist
Thyroid function should be screened and patients maintained as euthyroid
Patients should be assessed by a cardiologist and have ECG, echo and T2 cardiac MRI
All patients should have Biliary ultrasound & have FerriScan or Liver T2
All women should be offered bone density scan Folic acid 5 mg daily should be commenced 3 months prior to conception in these patients
All women with thalassaemia major should be receiving blood transfusions on a regular basis aiming for a pretransfusion haemoglobin of 100 g/l
Women should be offered an early scan at 7 to 9 weeks of gestation
In addition to the routine first trimester scan (11-14 weeks of gestation) and a detailed anomaly scan at 18-20+6 weeks of gestation, women should be offered serial fetal biometry scans every 4 weeks from 24 weeks of gestation
Women with thalassaemia who have undergone splenectomy and have a platelet count >600 should be offered LMWH & Aspirin (75 mg/day)
Haemoglobin is made of of 4 globular protein chains. Which are
Adult haemoglobin (HbA) is made of 2 alpha globulin chains and 2 beta globulin chains and accounts for 97% of total haemoglobin in a normal adult. HbA2 is a normal variant of hemoglobin A that consists of two alpha and two delta chains and makes up 1.5-3% of total haemoglobin. Fetal haemagobin (HBF) is the main haemoglobin type in the fetus and persists after birth for around 6 months. Fetal hemoglobin is composed of two alpha and two gamma subunits The alpha globulin chain is coded for by genes on Chromosome 16. Alpha Thalassemias are therefore associated with Chromosome16 defects The beta and delta globulin chains are coded for by genes on Chromosome 11. Beta and Delta Thalassemias are therefore associated with Chromosome 11 Defects.
Alpha-thalassemia major (Hb Bart’s) results in the absence of α-globin (–/–); this is associated with
hydrops fetalis, intrauterine death, and preeclampsia
G6PD deficiency in pregnancy may manifest as
increased urinary tract infections, preeclamsia, neonatal jaundice, hydrops fetalis and still birth.
Anemia increases risk of
- Preterm delivery
- Postpartum maternal infections
- Maternal iron deficiency anemia in early pregnancy was associated with a 3.60-fold increased risk of abruptio placentae
