hematological dis in pregnancy

Question 1

CDC defined anemia in iron-supplemented pregnant women using a cutoff of the 5th percentile — IN 1ST and 3rd trimester, —- in 2nd trimester

Answer 1

—11 g/dL in the first and third trimesters, and 10.5 g/dL in the second trimester

Question 2

Anemia is associated with several adverse pregnancy
outcomes including

Answer 2

preterm birth, Children born to iron-deficient women and without iron supplementation are
reported to have lower mental development scores

Question 3

The two most common causes of anemia during pregnancy and the puerperium are

Answer 3

iron deficiency and acute blood loss.

Question 4

In a typical singleton gestation, the maternal need for iron averages nearly

Answer 4

1000 mg.

Question 5

Routinely in pregnancy, daily oral supplementation btw

Answer 5

30 to 60 mg of elemental iron and 400 μg of folic acid is recommended

Question 6

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.

Answer 6

for parenteral IV iron ferrous sucrose is safer than iron-dextran.

Question 7

Chronic renal insufficiency is the most common disorder that cause anemia of chronic disease during pregnancy.

Answer 7

In pregnancies complicated by chronic renal insufficiency, recombinant erythropoietin is usually considered when the hematocrit approximates 20 percent

Question 8

More folic acid daily 4-mg folic acid supplement is given with

Answer 8

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

Question 9

Those who have undergone total gastrectomy require — μg of vitamin B12 given intramuscularly each month.

Answer 9

1000 μg of vitamin B12 given intramuscularly each month.

Question 10

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.

Answer 10

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

Question 11

Coldagglutinin disease may be induced by

Answer 11

infectious etiologies such as Mycoplasma
pneumoniae or Epstein-Barr viral mononucleosis

Question 12

Hemolysis and positive antiglobulin test results may be the consequence of either (IgM) or (IgG) antierythrocyte antibodies. When thrombocytopenia is comorbid, it is termed

Answer 12

Evans syndrome
Rituximab, along with prednisone, is first-line treatment

Question 13

Drug-induced hemolysis is usually chronic and mild to moderate, but occasionally acute hemolysis is severe.

Answer 13

severe Coombs-positive hemolysis stimulated by cefotetan given as prophylaxis for obstetrical procedures. Alpha-methyldopa can cause similar hemolysis

Question 14

The most fulminant acquired hemolytic anemia encountered during pregnancy is

Answer 14

caused by the exotoxin of Clostridium perfringens or by group A β-hemolytic streptococcus

Question 15

Diamond-Blackfan anemia

Answer 15

pure red-cell hypoplasia. Approximately 40 percent of cases are familial and have autosomal dominant inheritance

Question 16

Gaucher disease

Answer 16

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

Question 17

Sickle-Cell Hemoglobinopathies

Answer 17

(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.

Question 18

. Hemoglobin C originates from a single β-chain substitution of

Answer 18

glutamic acid by lysine, which stems from a T-for-C
substitution at codon 6 of the β-globin gene.

Question 19

Chronic and acute changes from sickling include (complications Happened to sicklers)

Answer 19

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

Question 20

Treatment OF SCD

Answer 20

• 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

Question 21

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.

Answer 21

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.

Question 22

Prophylactic Red Cell Transfusions For sicklers During pregnancy

Answer 22

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

Question 23

Sickle-Cell Trait carriers could have

Answer 23

occasional hematuria, renal papillary necrosis, and hyposthenuria, which is urine of low specific gravity

Question 24

The congenital diseases associated with poor platelet function are divided into four types of dysfunction:

Answer 24

(a) adhesion to collagen,
(b) adhesion to subendothelium, e.g. von Willebrand disease, RF, CML
(c) release reaction defects, and
(d) ADP aggregation defects.

Question 25

The laboratory assessment of the plasma clotting factors

Answer 25

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.

Question 26

SCD had Increase susceptibility to — and — infections

Answer 26

Increase susceptibility to pneumococcal and salmonella infections

Question 27

main factors causing haemolysis in G6PD are:

Answer 27

infections, substances derived from plants, drugs with high oxidation-reduction potential, stress, ketoacidosis in diabetes and surgery operations.

Question 28

Causes of warm Autoimmune haemolytic anaemia (AIHA):

Answer 28

• autoimmune disease: e.g. systemic lupus erythematosus
• neoplasia: e.g. lymphoma, CLL
• drugs: e.g. methyldopa

Question 29

Beta Thalassaemia in Pregnancy , gene mutation, mode of inheritance ?

Answer 29

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

Question 30

Initial Management of Beta Thalassaemia in Pregnancy ( RCOG produced Green-top Guideline No 66 in March 2014)

Answer 30

 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)

Question 31

Haemoglobin is made of of 4 globular protein chains. Which are

Answer 31

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.

Question 32

Alpha-thalassemia major (Hb Bart’s) results in the absence of α-globin (–/–); this is associated with

Answer 32

hydrops fetalis, intrauterine death, and preeclampsia

Question 33

G6PD deficiency in pregnancy may manifest as

Answer 33

increased urinary tract infections, preeclamsia, neonatal jaundice, hydrops fetalis and still birth.

Question 34

Anemia increases risk of

Answer 34

• Preterm delivery
• Postpartum maternal infections
• Maternal iron deficiency anemia in early pregnancy was associated with a 3.60-fold increased risk of abruptio placentae