Haematology & Electrolytes Flashcards

1
Q

ECG findings hypocalacemia

A

QT interval prolongation secondary to a prolonged ST segment

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2
Q

ECG findings hypercalacemia

A

QT interval shortened due to a shortened ST segment

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3
Q

Pathophysiology of sickle cell disease

A

Autosomal recessive
Mutation in Beta-globin gene - chromosome 11
Produces an abnormal HbS (rather than HbA)
Causes Hb to be “sickle” shaped –> decreased lifespan & get stuck and occlude vessels

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4
Q

Sickle cell and its relation to malaria

A

More common in pt from areas traditionally affected by malaria (Africa, India, middle east, Caribbean)

One copy of gene (sickle cell trait) reduces severity of malaria –> selective advantage of having the gene

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5
Q

What is Sickle cell Trait?

Sickle cell anaemia (HbSS), HbSC disease, Sickle beta-thalassaemia (HbSA), sickle cell trait

A

1 Hb gene + 1 normal HbA gene
Carrier
Asymptomatic

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6
Q

What is sickle cell anaemia HbSS?

Sickle cell anaemia (HbSS), HbSC disease, Sickle beta-thalassaemia (HbSA), sickle cell trait

A

Homozygous for abnormal HbS
Symptomatic - most severe form

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7
Q

With relation to sickle cell

What is HbSC disease?

Sickle cell anaemia (HbSS), HbSC disease, Sickle beta-thalassaemia (HbSA), sickle cell trait

A

1 HbS gene + 1 abnormal HbC gene
Symptomatic (but less severe than HbSS)
“mild” form of sickle cell anemia

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8
Q

What is sickle beta-thalassaemia ?

Sickle cell anaemia (HbSS), HbSC disease, Sickle beta-thalassaemia (HbSA), sickle cell trait

A

1 HbS gene + Beta-thalasaemia trait
Symptomatic - more mild form of sickle cell anemia

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9
Q

Why does sickel cell cause hyposplenism?

A
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10
Q

Sickle cell

What preciptates sickling?

A

Dehydration, infection cold, acidosis, hypoxia

Causes polymerisation of the HbS, initally reversible but with repeated sickling becomes irriversible

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11
Q

Diagnosis of sickle cell disease

A
  1. Screening pregnant women who are carriers
  2. Newborn screening heel prick test @ day 5 of age
  3. Blood film - sickle cells and target cells
  4. FBC - Hb and iron studies
  5. LFTs & Bilirubin raised
  6. Hb electrophoresis by HPLC
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12
Q

General managment of sickle cell disease

A
  • Avoid dehydrayion and other triggers of crises
  • Ensure vaccines up to date
  • Antibiotic prophylaxis with penicillin V daily lifelong (because of hypospenism)
  • Daily folate supplementation
  • Hydroxycarbamide - used to stimualte production of fetal haemoglobin, used if repeat sickle cell crises / acute chest syndrome
  • Blood transfusion for severe anaemia
  • bone marrow transplant is the only cure
  • New treatments: crizanlizumab antibody that stops RBCs sickling
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13
Q

Management of vaso-occlusive crises

A
  1. A-E assessment
  2. Analgesia - IV/SC morphine/paracetamol/ibuprofen
  3. Septic screen
  4. Cross-match & transfusion
  5. FBC, reticulocytes
  6. rehydrate, keep warm
  7. Oxygen
  8. +/- antibiotics
  9. exhcange transfusion - severe cases
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14
Q

Sickle cell crises

What is a vaso-occlusive ‘painful’ crisis?

A

Common, due to microvascular occlusion, causing distal ischaemia
Sx: Pain, fever, and sx of what has caused crisis

Affected areas:
* Often affects the marrow, causing severe pain
* Hands/feet affected in young children –> dactylitis
* Mesenteric ischaemia
* CNS –> stroke, seizures, congitive deficits
* Avascular necoriss (e.g. femoral head)
* Priapism (urological emergency)
* Acute chest syndrome (emergency)

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15
Q

Types of sickle cell crises

A

Vaso-occlusive crises
Acute chest syndrome
Alpastic crisis - due to parvovirus
Sequestration crisis - in children

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16
Q

What is sequestration crisis and why does it only affect children?

A

= emergency
* Caused by rbc blocking blood flow within the sleep –> acutely enlarged and painful spleen
* Pooling of blood in spleen can lead to severe anaemia and hypovolaemia shock
* Only children because in adult the spleen becomes atrophic

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17
Q

Sickle cell disease

What is acute chest syndrome?

A

= Fever or respiratory symptoms with mew infiltrates seen on a chest xray

Causes:
* infection (pneumonia, bronchiolitis)
* fat embolism (from bone marrow)
* pulmonary vaso-occlusion

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18
Q

Sickle cell disease

What is an Aplastic crisis?

A

Caused by parvovirus B19
Sudden reduction in marrow production, especially RBCs
+/- hepatosplenomegaly +/- abdo pain

Usually self limiting

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19
Q

Sickle cell: what is seen on blood film?

A

Sickle cells and target cells

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20
Q

FAT RBC

Causes of macrocytic anaemia

A

F - folate deficiency
A - Autoimmune
T - Thyrotoxicosis
R - Reticulocytosis - compentatory from blood loss
B - B12 deficiency
C - Chemotherapy/cytotoxics

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21
Q

AAAHH

Causes of normocytic anaemia

A

Acute blood loss
Anaemia of chronic disease
Aplastic anaemia
Haemolytic anaemia
Hypothyroidism

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22
Q

TAILS

Causes of microcytic anaemia

A

T - Thalasaemia
A - Anaemia of chronic disease
I - Iron deficiency anaemia
L - Lead poisoning
A - Sideroblastic anaemia

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23
Q

Intravascular haemolytic anaemia - blood test results

A

Decreased Hb
Increased Reticulocytes (haemolysis stimulate erythopoeisis)
Increased unconjugated bilirubin (From Hb Breakdown)
Increased LDH (eznyme in RBC)
Increased Iron (from Hb breakdown)
Decreased haptoglobin (mop up free Hb - used up)

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24
Q

Extravascular haemolytic anaemia - blood test results

A
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25
Intrinsic (to RBC) causes of haemolytic anaemia - causes
Thalassaemias Sickle cell disease Hereditary spherocytosis Enzyme defectis (G6PD, Pyruvate kinase)
26
Extrinstic (to RBC) haemolytic anaemia - causes
Immune mediated (cold & warm autoimmune haemolytic anaemia) Drugs (nitrofurantoin, penicillin, quinine) Infection Microangiopathic
27
Causes of Intravascular haemolytic anaemia
Microangiopathic haemolysis (= physical trauma to RBC in circulation) Osmotic lysis (from IV fluids) Acute tranfusion reactions (ABO incompatability)
28
Causes of extravascular haemolytic anaemia
abnormal RBC cleared in reticuloendothelial system (phagocytosis in the spleen) * Warm automimmune haemolytic anaemia * Hypersplenism * Malaria * Haemoglobinopathies (Thalassaemia, sickle cell) * Hereditary spherocytosis * Enzyme deficiency (G6PD, pyruvate kinase)
29
What type of haemolytic anaemia causes brown urine (haemosiderin)
Intravascular haemolysis - due to increased iron from breakdown of Haem
30
Causes of Microangiopathic haemolysis
* Haemolytic uraemic syndrome * Thrombotic thrombocytopenia purpura (TTP) * Prosthetic heart valves * Pre-eclampsia / Eclampsia * maligant HTN * DIC * Vasculitis
31
What is cold autoimmune haemolytic anaemia
Less common IgM antibodies predominate Mainly intravascular haemolysis Spherocytosis is present as a result of red cell damage. Haemolysis occurs at <4 degrees May experience raynauds pnenomenon Causes: Idiopathic, Infections (mycoplasma, EBV), Lymphoproliferative disorders
32
What is warm autoimmune haemolytic anaemia
IgG antibodies predominate Mainly extravascular haemolysis Spherocytosis is present as a result of red cell damage. Haemolyisis occurs at body temperature Causes: Primary (idiopathic), Secondary (SLE, lymphoma, or chronic lymphocytic leukemia)
33
Causes of Drug-induced immune haemolytic anaemia
34
Management of warm autoimmune haemolytic anaemia
1. Folate 2. Steroids +/- mycophenolate 3. Rituximab or splenectomy
35
Management of cold autoimmune haemolytic anaemia
1. Folate and cold avoidance! mainly KEEP WARM 2. Severe anaemia - give warmed blood 2. Rituximab +/- chemotherapy
36
# Sickle cell Blood test to differentiate between aplastic crisis and sequestration crisis
Reticulocytes - Reduced in aplastic crisis - Increased in sequestration crisis
37
Types of thalasaemia
Beta thalassaemia - major - minior Alpha thalassaemia - Major (death in utero) - HbH disease - Alpha thalassaemia trait
38
Which conditions show target cells on blood film
thalassemia hepatic disease with jaundice hemoglobin C disorders postsplenectomy state Lesser numbers in: sickle cell anemia iron deficiency
39
What is Thalassaemia ?
Autosomal recessive Disorders of Alpha-(x4) or beta- globin (x2) genes Causing decreased HbA
40
What is Beta-thalasaemia major
- Both genes abnormal - no HbA * presents in the first year of life with failure to thrive and hepatosplenomegaly * microcytic anaemia * Severe anaemia when HbF runs out --> tranfusion dependent
41
What is beta thalasaemia minor
* 1 gene abnormal - almost normal Hb with low MCV (in the 60s) * mild anaemia with a disproportionate microcytosis and a raised haemoglobin A2
42
What is alpha thalasaemia
43
Management of beta-thalasaemia major
1. Life long monthly **blood transfusions** 2. **Iron chelation** (to prevent iron overload from repeated transfusion) (e.g. **desferrioxamine**) 3. Bone marrow transplant is the only cure
44
Blood film of chronic lymphocytic leukaemia
Smudge cells
45
Blood film of acute lymphoblastic leukaemia
Blast cells
46
Blood film of acute myeloid leukaemia
Blast cells + Auer rods
47
Differenitals for petechiae
* Leukaemia * Meningococcal septicaemia * Vasculitis * Henoch-Schonlein Purpura (HSP) * Idiopathic Thrombocytopenia Purpura (ITP) * Non-accidental injury
48
What condition can myelodysplastic disorder transform into?
Acute myeloid Leukaemia
49
# CRABBI Features of Myeloma
C - Hyper**C**alcaemia R - **Renal dysfunction** (light chain deposition within the renal tubules) A - **Anaemia** (Decreased erythropoeisis) B - **Bleeding** (decreased platelets) B - **Bone pain**/lesions (osteoclast overactivity creates lytic bone lesions) I - **Infection** (reduced production of normal immunoglobulins)
50
What are Howell-Jolly bodies
nuclear remnants that are found in the RBCs of patients with reduced or absent splenic function
51
Universal donor
O neg
52
Universal receptient
AB positive
53
Key difference between TACO and TRALI
Trali --> hypotension Taco --> hypertension
54
Components of haemolysis screen
LDH Unconjugated bilirubin Haptoglobin
55
# Chronic Lymphocytic leukaemia What is Richter's transformation?
CLL can transform into high-grade lymphoma - making patients suddenly unwell When leukaemia cells enter lymph nodes and change into a high-grade fast growing **Non-Hodgkin's Lymphoma**
56
Which coagulation disorders is desmopressin used for and why?
Haemophilia Von Willebrands disease It is an antidiuretic that causes secretion of factor VIII and vWF into plasma
56
Which coagulation disorders is desmopressin used for and why?
Haemophilia Von Willebrands disease It is an antidiuretic that causes secretion of factor VIII and vWF into plasma
57
# Lymphoma Where are reed-sternberg cells seen?
Hodgkin lymphoma They are mutated B cells lymph node biopsy
58
Staging system for lymphoma
Ann Arbor staging system Stage 1: Confined to one region of lymph nodes. Stage 2: In more than one region but on the same side of the diaphragm (either above or below). Stage 3: Affects lymph nodes both above and below the diaphragm. Stage 4: Widespread involvement including non-lymphatic organs such as the lungs or liver.
59
Blood transfusion threshold for a patient with ACS
80 g/l | 70 g/L in most people
60
What are myeloproliferative disorders?
Overproduction of cells in bone marrow - but maturation still occurs Differenitated myeloid cell subtype expasion Maturation arrest can occur --> acute myeloid leukaemia (all of the different types can become AML)
61
Types of myeloproliferative disorders
Chronic myeloid Leukaemia (High WBCs) Polycythaemia rubra vera (High RBC +/- platelets) Essential thrombocythaemia (High platelets) Primary myelofibrosis (fibrosis of bone marrow)
62
JAK2 associated myeloproliferative disorders
Polycythaemia rubra vera (High RBC +/- platelets) Essential thrombocythaemia (high platelets)
63
What bllod cancer is associated with BCR-ABL
Chronic myeloid leukaemia