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Osha 3a > Haematology (paeds + GP) > Flashcards

Haematology (paeds + GP) Flashcards

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1
Q

What is sickle cell anaemia?

A
  • mutation in β global gene > HbS
  • Autosomal recessive
  • HbS polymerises when deoxygenated
  • Blocks blood vessels > ischaemia, sequestration (away from organs)
  • chronic haemolysis > low baseline Hb
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2
Q

How is sickle cell anaemia diagnosed?

A
  • 1st line: sickle solubility test: cloudy looking
  • Gold: HPLC, capillary electrophoresis (Hb separated based on size and charge)
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3
Q

What are some complications of sickle cell disease?

A
  • sequestration in liver/spleen
  • thrombosis (DVT, PE)
  • acute chest syndrome
  • aplastic crisis
  • vaso-occlusive crisis
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4
Q

What is lymphoproliferative disease?

A
  • Cancer of the white blood cells
  • 2 categories: Hodgkin’s and non-Hodgkin’s (aggressive or indolent)
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5
Q

How does lymphoma present?

A
  • lymphadenopathy > neck, armpit, groin
  • non-tender, rubbery nodes
  • b symptoms: fever, night sweats, weight loss
  • pain after drinking alcohol (Hodgkin’s)
  • recurrent infection
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6
Q

How is lymphoma investigated?

A
  • lymph node biopsy: core needle or excision node
  • raised LDH
  • Reed-Stenberg test: abnormally large B cells with multiple nuclei: Hodgkin’s (owl eyes)
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7
Q

How is lymphoma treated?

A
  • Hodgkin’s: DBVD: doxorubicin, bleomycin, vinblastine, dacarbazine
  • NH: R-CHOP: rituximab, cyclophosphamide, doxorubicin, vincristine, prednisolone
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8
Q

What is pernicious anaemia and the pathophysiology?

A
  • B12 deficiency
  • parietal cells produce intrinsic factor for B12 absorption in ileum
  • antibodies form against parietal cells or intrinsic factor
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9
Q

How does pernicious anaemia present?

A
  • peripheral neuropathy, paraesthesia
  • loss of proprioception
  • visual changes
  • mood/cognitive changes
  • lemon yellow skin
  • angular chelitis and glossitis
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10
Q

How is pernicious anaemia diagnosed?

A
  • testing for auto-antibodies
  • 1st line: intrinsic factor antibody
  • can also test for gastric parietal cell antibodies
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11
Q

How is pernicious anaemia treated?

A
  • dietary: oral replacement with cyanocobalamin
  • 1mg of IM hydroxycobalamin every other day/3x week for 2 weeks then 3 monthly
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12
Q

What is mean corpuscular volume (mcv) and what are the reference ranges?

A
  • microcytic: <80
  • normocytic: 80-95
  • macrocytic: >95
  • normal range: 120-165g/l in women or 130-180g/l in men
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13
Q

What are the causes of microcytic anaemia?

A
  • T – Thalassaemia
  • A – Anaemia of chronic disease
  • I – Iron deficiency anaemia
  • L – Lead poisoning
  • S – Sideroblastic anaemia
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14
Q

What are the causes of normocytic anaemia?

A
  • A – Acute blood loss
  • A – Anaemia of Chronic Disease
  • A – Aplastic Anaemia
  • H – Haemolytic Anaemia
  • H – Hypothyroidism
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15
Q

What is megaloblastic anaemia?

A
  • megaloblastic: results from impaired DNA synthesis. Rather than dividing, the cell continues to grow becoming abnormally large.
  • B12 deficiency
  • folate deficiency
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16
Q

What are the causes of normoblastic macrocytic anaemia?

A
  • hypothyroidism
  • alcohol excess
  • liver disease
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17
Q

What are the causes of iron deficiency anaemia?

A
  • blood loss: menorrhagia, IBD, GI bleeding
  • dietary insufficiency in children
  • poor iron absorption
  • increased requirements during pregnancy
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18
Q

How is iron absorbed in the GI tract?

A
  • mainly absorbed in duodenum and jejunum
  • stomach acid needed to keep iron in soluble Fe2+ form
  • changes to Fe3+ when acid drops so PPIs interfere
  • travels around the blood as Fe3+ bound to transferrin
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19
Q

What is seen on investigation of iron deficiency anaemia?

A
  • low transferrin saturation and ferritin
  • high total iron binding capacity: space for transferrin molecules to bind
  • FBC
  • blood film: Howell Jolly bodies
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20
Q

How is iron deficiency anaemia managed?

A
  • OGD/colonoscopy if no clear cause
  • blood/iron infusion
  • oral iron (ferrous sulphate): take for 3 months after corrected for replenishment
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21
Q

How does iron deficiency anaemia present?

A
  • koilonychia (spoon shaped nails)
  • angular chelitis
  • atrophic glossitis
  • brittle hair and nails
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22
Q

What is thalassaemia?

A
  • genetic defect in protein chains making up Hb
  • autosomal recessive
  • defects in α chains > α thalassaemia
  • defects in β chains > β thalassaemia
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23
Q

What is the presentation of thalassaemia?

A
  • fatigue
  • pallor
  • jaundice
  • gallstones
  • splenomegaly: RBC fragile so more damaged collected by spleen
  • pronounced forehead and cheekbones: bone marrow expands
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24
Q

How is thalassaemia diagnosed?

A
  • FBC
  • Hb electrophoresis
  • DNA testing
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25
How is α thalassaemia managed?
- monitoring FBC - blood transfusions - splenectomy - bone marrow transplant
26
How is β thalassaemia managed?
- monitoring - transfusions - iron chelation to prevent overload
27
What is haemolytic anaemia and how does it present?
- destruction of RBC leading to anaemia - splenomegaly - jaundice: bilirubin released during destruction
28
How is haemolytic anaemia investigated?
- FBC: normocytic anaemia - blood film shows schistocytes (fragments) - direct Coombs test: +ve in autoimmune haemolysis
29
What are the complications of sickle cell anaemia?
- infection - avascular necrosis - pulmonary hypertension - CKD - acute chest syndrome
30
How is sickle cell anaemia managed?
- Abx prophylaxis for infection - hydroxycarbamide stimulates HbF - blood transfusion - bone marrow transplant
31
What are the different types of sickle cell crises?
- vaso-occlusive: sickle shaped cell clogs capillaries - aplastic: temporary loss of creation of new RBC - splenic sequestration: RBC block flow into spleen > splenomegaly > hypovolaemic shock
32
What is acute chest syndrome?
- results from sickle cell anaemia - fever/resp symptoms + new infiltrates on X-Ray - can be due to infection or emboli - may need antibiotics, transfusion, ventilation
33
What is leukaemia?
- cancer of stem cells in bone marrow - classified as acute or chronic - myeloid or lymphoid depending on cell line affected
34
What is the pathophysiology behind leukaemia?
- genetic mutation in precursor cells leads to excessive production of single type of abnormal WBC - leads to suppression of other cell lines
35
What is the epidemiology of leukaemia?
- ALL: 2-5 years - AML: <2 years
36
How does leukaemia present?
- fatigue - fever - failure to thrive (kids) - lymphadenopathy - abnormal bleeding/bruising
37
How is leukaemia diagnosed?
- FBC - blood film >20% blast cells - LDH - GOLD: bone marrow biopsy
38
How is leukaemia managed?
- chemotherapy and steroids - radiotherapy
39
Describe acute lymphoblastic leukaemia
- acute proliferation of B-lymphocytes - associated with Downs - blood film shows blast cells
40
How does ALL present?
- anaemia: lethargy and pallor - thrombocytopenia: easy bruising - neutropenia: frequent infection - bone pain - hepato/splenomegaly
41
Describe acute myeloid leukaemia
- blood film: high proportion of blast cells - may have Auer rods in cytoplasm
42
What is PT?
- prothrombin time: the time taken for blood to clot via the extrinsic pathway - factor VII is the only factor in this pathway and rarely deficient so this measures overall clothing factor synthesis - affected by liver disease, vit K deficiency, warfarin levels
43
What is INR?
- international normalised ratio - calculated from PT: 1.0 represents the global average - used to monitor patients on warfarin - higher - takes longer to clot
44
What is APTT?
- activated partial thromboplastin time - time taken for blood to clot via intrinsic pathway - can indicate issues with factor VIII, vWF, IX and XI - heparin can cause prolonged APTT
45
What is Ann Arbor staging for lymphoma?
- if affected nodes are above or below diaphragm - Stage 1: confined to one region - Stage 2: in more than one region on same side of diaphragm - Stage 3: affects nodes above and below diaphragm - Stage 4: widespread involvement inc non-lymphatic organs
46
What are some types of Non-Hodgkin's lymphoma?
- Burkitt: associated w EBV, malaria, HIV - MALT: affects mucosa-associated lymphoid tissue (H. pylori) - Diffuse large B cell
47
What are risk factors for Non-Hodgkin's lymphoma?
- HIV, EBV, malaria, H. pylori - Hep B/C infection - exposure to pesticides and trichloroethylene - family history
48
What is von Willebrand's disease?
- type 1: reduced amount of vWF - type 2: defective vWF - type 3: little/no vWF - important in platelet adhesion and binds to factor VIII - autosomal dominant
49
How does von Willebrand disease present?
- bleeding gums with brushing - epistaxis (nose bleeds) - menorrhagia - heavy bleeding - family history
50
How is von Willebrand disease investigated?
- APTT prolonged - Low factor VIII - immunoassay of vWF
51
How is von Willebrand disease managed?
- desmopressin: stimulates release of vWF - infused vWF and factor VIII - tranexamic acid
52
What is the presentation of haemophilia?
- spontaneous haemorrhage, bleeding into joints and muscles - intracranial haemorrhage and cord bleeding in newborns - bleeding: gums, GI, urinary tract, retroperitoneal space
53
How is haemophilia diagnosed?
- bleeding scores: prolonged APTT - coagulation factor assays (factor VIII/IX) - genetic testing
54
How is haemophilia managed?
- prophylaxis: infusions of factor VIII/IX - avoid NSAIDs and contact sport - desmopressin and tranexamic acid (antifibrinolytic)
55
What is haemophilia?
- X linked recessive inherited bleeding disorders - almost exclusively affect males, women would need 2 affected X copies - A: deficiency in factor VIII - B: deficiency in factor IX
56
What are the normal reference ranges for neutrophils?
- neutrophilia: high - acute bacterial infection - neutropenia: low - myeloma, lymphoma
57
What are the normal reference ranges for platelets?
- thrombocytosis: high - myeloproliferative disorders - thrombopenia: low - 150x10^9 - ITP
58
What are the normal reference ranges for lymphocytes?
- lymphocytosis: high - leukaemia - lymphocytopenia: low - infection
59
What is immune thrombocytopenic purpura (ITP) and who does it affect?
- autoimmune platelet destruction - in children 2-6 (post viral infection)
60
How does ITP present, how is it diagnosed and how is it treated?
- symptoms: purpuric rash, easy bleeds - diagnosis: thrombocytopenia, inc megakaryoblasts - prednisolone and IV immunoglobulins
61
What is thrombotic thryombcytopenic purpura and how does it present?
- inherited - deficiency in enzyme clearing vWF so aggregation at endothelial injury sites - causes platelets to clump in vessels
62
How does TTP present?
- purpuric rash - menorrhagia - AKI - anaemia - neuro symptoms
63
How is TTP diagnosed and treated?
- diagnosis: thrombocytopenia, schistocytes, dec ADAMTS13 - treatment: plasmapheresis, prednisolone, rituximab
64
How does malaria present and how is it diagnosed and treated?
- anaemia, blackwater fever, hepatosplenomegaly - blood film - treatment: quinine and doxycycline
65
What is aplastic anaemia?
- pancytopenia where the bone marrow fails - deficiency in red cells, white cells and platelets
66
What is sideroblastic anaemia?
- ineffective erythropoesis due to defective Hb synthesis - leads to inc iron absorption, iron loading in bone marrow - cardiac, endocrine and liver damage due to iron deposition
67
What is tumour lysis syndrome?
- rapid cell death when starting chemo for rapidly proliferating blood cancers - causes rise in urate, K, PO4 > renal failure - low calcium - prevention with hydration and allopurinol
68
What is disseminated intravascular coagulopathy?
- widespread activation of coagulation due to release of procoagulant agents - clotting factors and platelets consumed > inc risk of bleeding
69
What is hereditary spherocytosis?
- sphere shaped RBC - easily destroyed
70
What is the epidemiology and inheritance of hereditary spherocytosis?
- MC inherited haemolytic anaemia in northern Europeans - autosomal dominant
71
How does hereditary spherocytosis present?
- jaundice - anaemia - gallstones - splenomegaly - crises
72
What is aplastic crisis?
- increased anaemia, haemolysis and jaundice - BM doesn't create new reticulocytes - triggered by parvovirus
73
How is hereditary spherocytosis diagnosed?
- family history and clinical - spherocytes on film - mean corpuscular haemoglobin conc is raised - reticulocytes raised
74
How is hereditary spherocytosis managed?
- folate supplementation - splenectomy - transfusion in crises

Osha 3a (15 decks)

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