Medical conditions pregnancy Flashcards
What is thrombotic thrombocytopenic purpura?
Condition that causes microvascular platelet aggregation. Systemic and extensive. Often involves CNS. (HUS less extensive consumption of platelets)
When is TTP mostly seen and what symptoms does the patient have?
PP
Haemolytic anaemia
Thrombocytopenia
Fever
Neurological manifestations (headache, irritability, drowsiness, seizures, coma)
AKI
How do you diagnose TTP?
Bilirubin and LDH raised
AKI
Thrombocytopenia
Coags normal
ADAMSTS-13 activity is <10% (inhibitor of vWF - cleaving protease - ADAMSTS-13) (Diffuse vascular endothelial insult, endoethelial cells secrete unsually large forms of vWF - these large multimers aggregate platelets)
How do you diagnose aHUS?
In HUS, platelet aggregation is relatively less extensive, with predominantly renal involvement.
Diagnosis of exclusion, more likely if:
ADAMST-13 >10%
Serum Cr >177
LDH >1000
Hb <80
Feature persistent more than 72h PPw
How should you manage TTS / HUS?
No effect on fetus
No evidence that delivery improves course of disease
Need to differentiate from HELLP
Aggressive treatment with FFP and plasmaphoresis can limit vascular injury and improve prognosis
Supportive therapy for AKI (sometimes dialysis)
Platelet transfusions contraindicated.
What is the genetics of sickle cell anaemia?
Autosomal recessive - sickle gene affects the haemoglobin structure
HbSS = sickle cell anaemia
(Sickle cell trait asymptomatic apart from increased risk UTIs and microscopic haematuria)
Clinical features of sickle cell disease
Anaemia - haemolytic
Painful vaso-occlusive crises
Infections (Loss of splenic function)
Acute chest syndrome - fever, tachypnoea, pleuritic chest pain, leukocytosis, anaemia, pumonary infiltrates)
Splenic sequestration - sickled red cells get stuck in spleen and damage it
Gallstones
Retinopathy
Leg ulcers
Aseptic necrosis of bone
AKI / kidney disease
Stroke
Pulm HTN
What precipitates sickle crisis?
hypoxia
cold
acidosis
dehydration
Can cause
- Vaso-occlusive symptoms (tissue infarction, pain!)
- Splenic sequestration
- Aplastic (BM stops producing red cells - often ass with parvovirus)
How do you diagnose sickle cell anaemia?
Electrophoresis
What is the effect of sickle cells disease on pregnancy?
perinatal mortality 4-6 fold increase
Increased
- Miscarriage
- FGR (sickling infarcts, + anaemia)
- PTB
- APH and placental abruption
- Fetal distress
- CS
- Infection (UTIs, pneumonia, peurperal sepsis) (encapsulated organisms in hyposplenic individuals)
- Thromboembolic events
What is the effect of pregnancy on sickle cell disease?
Complications more common
35% will have a crisis in pregnancy
How should you manage sickle cell disease pre-pregnancy, antenatally, intrapartum, postpartum?
Pre pregnancy:
- Counsel re risks
- 5mg folic acid
- MDT: haematologists / obs
- Test partner - consider PGD if partner carrier
- ECHO to exclude pulm HTN
- Renal and LFTs annually
- Retinal screening
- Ferritin - to screen for iron overload - aggresive iron cehlation prior to concetion
- Alters meds - hydroxyurea is teratogenic - discontinue 3/12 prior to pregnancy. If become pregnant on it - structural abnormality scan but TOP not indicated as some cases no adverse effects.
- sometimes patients on ACEI for renal dysfunction - switch
- Only given iron if iron deficient
- Vaccinations: menigitis, pneumoniae, hemophilius influenzae
Pregnancy
- Penicillin prophylaxis throughout pregnancy - oral twice / day
Low dose aspirin
Hb and MSU every visit
Uterine artery dopplers, regular growth USS (2-4 weekly)
If hospital admission for LMWH
Manage crises aggressively: admission, treat pain (IV / SC morphine), adequate rehydration, early use of abx if infection suspected
Oxygen if sats <95% - pulse oximetry mandatory.
NSAIDs can be used between 12 and 28 weeks
(WCC raised in SCD so doesn’t necessarily indicate infection)
CXR: looking for acute chest syndrome - infiltrates, hypoxia - treat with blood transfusion or exchange transfuion, abx, resp support . LMWH until PE excluded.
Brain imaging if concerned re stroke
If acute anaemia - do reticulocyte count - if low could be parvovirus
intrapartum
- IOL 38-40/40 (no RCTs to support this)
- Avoid dehydration, hypoxia, sepsis, acidosis
- encourage epidural analgesia
NO is safe
CTG advisable
continuous sats monitoring - ABD and O2 replacement if <94%)
PP
- LMWH 7/7 for NVB, 6/52 CS
Contraception
What will happen to a fetus that is alpha thalassaemia major?
Hydrops - no functional alpha genes so severely anaemic. Not compatible with life
What is beta thalassaemia major?
Defective beta genes, inefficient carrying of oxygen, haemolysis because the alpha chains will hold onto the oxygen and this will cause damage to the red cells - haemolysis in BM or in spleen. These people only survive into 2nd or 3rd decade of life. Present wtih anaemia, hepatsplenomegaly, growth retardation, jaundice, haemochromotosis (arrhytmias, pericarditis, cirrhosis etc…). Initially HbF hangs around for first 6 months and then patients present.
Enlarged forehead and cheekbones because oof increased bone marrow production.
diagnose with electrophoresis
Beta thalassaemia major
- Pre conception
- Antenatal
- Intrapartum
- PP
Most are subfertile. (can have damage to pituitary)
Periconceptual:
* Counsel risks - inheritance, worsening anaemia, cardiomyopathy, new development endocrinopathies secondary to no iron chelation in pregnancy (DM, hypothyroid), IUGR, PTB, CS for CPD
* Test partner, consider PIGD or gamete donation
* Aggressive Fe chelation, stop 3m prior to pregnancy
* Screen re Fe overload and end organ damage - TFT, echo, ECG, cardiac MRI, LFTs, liver USS, DEXA scan, fructossamine,
* RBC antibody (alloimmunity in 16.5%) and hepatitis screen
* High dose folate
Antenatal:
* MDT - high risk obs, MFM, haematology, anaesthetics, paediatrics, genetics
* High dose folate
* Fe only if low
* Aspirin if splenectomy or plt count >600, add LMWH if both (prothrombotic tendency due to abnormal red cell fragments)
* Fetal genotype if at risk - NIPT or CVS/amniocentesis
* Growth surveillance from 24/40
* Fructossamine (monthly) to assess for diabetes, or BSL monitoring - HbA1c less reliable if transfusion dependent
* Consider chelation after 20weeks - desferrioxamine
* Blood transfusions to aim >100g/L, 2-3 weekly Hb
Delivery:
* Aim VB
* CTG
* Cross match
* Active 3rd stage
* IV desferrioxamine (excess iron can cuase free radical damage and cardiac dysrhtymia in labour)
Postnatal:
* Breastfeeding support - OK to breastfeed on desferroxiamine)
* VTE prophylaxis (7/7 NVB, 6/52 CS)
* Contraception
What are the causes of thrombocytopenia in pregnancy?
- Spurious result
- Gestational thrombocytopenia
- Immune thrombocytopenic purpura (ITP)
- HELLP
- DIC
- Sepsis
- Haemolytic uraemic syndrome (HUS) / thrombotic thrombytopenic purpura (TTP)
- HIV, drugs, infection
- SLE and APS
- Bone marrow suppression and folate deficiency
Immune thrombocytopenia
- When to suspect
- Effect of pregnancy on ITP
- Effect of ITP on pregnancy
Diagnosis of exclusion, consider if diagnosed in early pregnancy (destruction by spleen after antibodies tag platelets)
Effect of pregnancy on ITP - doesn’t effect
Effect of ITP on pregnancy
- Capillary bleeding and purpura more common with counts <50, spontaneous mucous membrane bleeding <20
Antiplatelet IgG crosses placenta - causes fetal thrombocytopenia
0 - 1.5% risk fetal or neonatal ICH
- If a previous child is affected - high risk
How should you manage ITP
- Maternal considerations
- Fetal considerations
Maternal considerations
- Exclude SLE and APLS
- Monthly plt count
- Treatment required in 1st and 2nd trimester if symptomatic with bleeding, count <20, interventional procedures required e.g. CVS
Aim counts >50 prior to delivery, >80 if want regional anaesthesia
Corticosteriods first line therapy 20-30mg prednisolone / day, then wean
IVIG in resistant cases - delays clearance of IgG coated platelets from maternal circulation (more rapid response but expensive) (lasts 2-3weeks)
Splenectomy in severe cases - if has had splenectomy - penicillin prophylaxis
If IVIG not succesful - methylprednisone, azathioprine, cyclosporin. plt transfusion last resort - increases Ab titres.
Fetal considerations
- Baby not at risk of bleeding before labour and delivery (Transfer increases towards end of pregnancy)
- ICH rates not reduced with CS
Cord platelet count immediately after delivery (nadir reached 2-5 days later though so observe over that time, IVIG if plt <20)
Avoid FSE, FBS, if plt count <80 in mother
avoid ventouse
Causes of DIC
Haemorrhage e.g. abruption
PET / HELLP
AFE
Massive infection, particularly intrauterine infection
retention of dead fetus
Pathogenesis of DIC
Endothelial injury
consumption of clotting factors and platelets
Fibrinolysis stimulated
Bleeding
Diagnosis of DIC
Fibrinogen <2g/L
Thrombocytopenia
Prolonged clotting times - thrombin time, APTT, PT
Increased fibrinogen degradation products
Blood looks watery
How should you manage DIC?
Treat underlying cause
Activate MTP
Coagulopathy treated with
- FFP (all coagulation factors)
Red cells
- Plt if plt <80 and ongoing bleeding
Cryoprecipitate (clotting factors)
Recombinant fibrinogen (if <1g/L and ongoing haemorrrhage)
What is the inheritance of vWD?
Autosomal dominant
(vWF - adhesive protein, important role in plt function and stability of FVIII. Is required for binding of platelets to subendothelium after vessel injury)
How is vWD diagnosed?
APTT prolonged
vWF reduced
VIII reduced