Haematology Flashcards

Question

prognosis AML

Answer 1

5 year survival of 66% if poor cytogenic features or response to therapy - <20% cured

Answer 2

x linked recessive more common in males

Answer 3

A - factor VIII (more common) B - factor IX

Answer 4

factor VIII is one of main clotting factors that contibutes to production of thrombin via intrinsic pathway. The intrinsic pathway is activated in response to endothelial collagen exposure...therefore lack of favot VIII...deficient in thrombin...results in insufficient production of fibrin for clot stabilisation

Answer 5

depends on plasma conc of factor VIII if severe, may have no measurable factor in blood

Answer 6

male family hx

Answer 7

prolonged, unexplained bleeding recurrent episodes spontaneous bleeding joint pain, swelling, reduced ROM - haemarthrosis

Answer 8

active bleeding pallor bruising haemarthrosis

Answer 9

non accidental injury (social care) von willebrand disease vit K deficiency ALL

Answer 10

FBC - anaemia coagulation screen - prolonged APTT, normal PT, factor will be low CT head, joint aspiration - additional

Answer 11

treating bleeds - in: one off infusion of factor prophylaxis - regular IV infusion of factor education avoid contact sports good dental hygeine

Answer 12

chronic arthopathy development of factor VIII or IX inhibitors transfusion related complications

Answer 13

hodgkin and non hodgkin lymphoma

Answer 14

over 10% of childhood cancers on boys older children

Answer 15

multifactorial adults - obesity, smoking, alcohol

Answer 16

EMV immunosuppresed - transplant, previous cancers weight loss, night sweats, fevers

Answer 17

non tender lymphadenopathy if mediastinus - wheeze, difficulty breathing SVC obstruction

Answer 18

reactive lymphadenopathy leukaemia met malignancy

Answer 19

FBC UE - tumour lysis syndrome can cocur before tx begins LDH - elevated USS of area and nodes and biopsy CXR - mediastinal nodes full body CT lymph node biopsy

Answer 20

1 - single group of nodes or organ 2 - 2 or more groups of nodes or organs on same side of diaphragm 3 - in lymph nodes or organs on both sides 4 - diffuse involvement of nodes and organs such as liver and bones B added to stage if B symptoms present - weight loss, night swats, fevers

Answer 21

airway compromise high dose steroids SVCO may require stenting of veins hyperhydration in tumour lysis syndrome

Answer 22

chemo and possible radiotherapy, depending on stage

Answer 23

majority recover hodgkin's more favourable than non

Answer 24

tumour lysis syndrome, which is seen when rapid lysis of tumour cells causes release of large amounts of phosphorus, potassium and calcium leading to potential kidney damage. This is most likely to occur when chemotherapy is first commenced, but may occur beforehand neurotropenia alopecia sub fertility

Answer 25

a mutation in the gene encoding the haemoglobin subunit β, encoded by the beta globin gene. It is inherited in an autosomal recessive

Answer 26

1 in 2,449 in the UK with a carrier risk of 1 in 89 marked variation by region malarial hypothesis - survival advantage against malaria increasing prevalence of HbS allele in high income countries

Answer 27

The HbS allele results from a single nucleic acid substitution from GAG to GTG in the beta globin gene. This causes glutamic acid to be substituted with valine. Individuals with one HbS allele (HbAS) are carriers while those who are homozygous for the HbS allele (HbSS) have sickle cell disease. This is the most common type of sickle cell disease, however it is also possible to have one HbS allele and a different faulty beta globin allele other than HbS (such as β-thalassaemia, written as HbSβ-thalassaemia This makes it prone to sickling where it becomes rigid and distorts into crescent shape. in the deoxygenated state, HbS tetramers bind to each other and polymerise...vaso-occlusion and chronic haemolysis

Answer 28

black african, black caribbean - 1 in 200 affected, 1 in 10 carriers next common - asian lowest - white

Answer 29

intermittent vaso-occlusive crises - severely painful, precipitated by hypoxia, infection, exercise, dehydration, acidosis dactylitis family history

Answer 30

Pallor, lethargy – due to anaemia Jaundice – due to haemolysis Fever Tachycardia, tachypnoea Digital redness, swelling and pain

Answer 31

gout acute abdomen

Answer 32

consider osteomyelitis or avascular necrosis

Answer 33

haemoglobin electrophoresis - HbA absence high performance liquid chromatography blood film - nucleated red cells, howell jolly bodies, sickle shaped FBC and reticulocyte count iron studies X ray of long bones CXR - acute chest syndrome

Answer 34

antenatal screening - heel prick test

Answer 35

analgesia fluid oxygen abx if infection

Answer 36

Hydroxycarbamide – reduces frequency of painful crises Novel and experimental treatments – including L-glutamine, crizanlizumab and Voxelotor Regular blood transfusions – targeted to maintain HbS <30%

Answer 37

increased susceptibility to infection - pneumococcal and salmonellavac

Answer 38

seasonal influenza vaccines – annual pneumococcal vaccines – every 5 years hepatitis B vaccine – if receiving regular blood transfusions other vaccines, such as meningococcal C or haemophilus influenza type B vaccine – may be advisable if they haven’t had them before

Answer 39

pulmonary hypertension leg ulcers priapism chronic kidney disease large-artery ischemic stroke

Answer 40

result from infection with parvovirus B19

Answer 41

median life expectancy is 58 years for HbSS and 66 years for HbSC and HbSbeta-thalassaemia life expectancy increasing infection or stroke - most common mortality acute chest syndrome - adults

Answer 42

earlier first presentation high frequency of vaso-occlusive crises

Haematology Flashcards

(68 cards)