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Year 3 > clinical haematology > Flashcards

clinical haematology Flashcards

1
Q

define disseminated intravascular coagulation (DIC)

A

serious illness => disorder of the clotting cascade => depleted platelets and coagulation factors

  • acute overt = EMERGENCY + significant depletion => bleeding
  • chronic overt= slower depletion => hypercoagulable state (increased clotting problems)
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2
Q

Pathophysiology of DIC

A
  1. endothelial damage => releases tissue factor => activates clotting cascade
  2. activates thrombin => depletes platelets/ clotting factors => activates fibrolytic processes
  3. bleeding into subcutaneous tissue, skin, mucosal membranes
  4. fibrin (fibrinolysis) => occludes blood vessels => haemolytic anaemia + ischaemic end organ failure

Chronic DIC - process happens at a slower rate and compensatory processes => less bleeding + hypercoagulable state

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3
Q

Causes of DIC

A
  • INFECTION (gram-ve sepsis)
  • major trauma
  • surgery
  • Obstetric causes (pre-eclampsia, missed misscarriage, placental abruption, amniotic emboli)
  • Malignancy (acute promyelocytic leukaemia => acute DIC, lung/liver/ GI => chronic DIC)
  • haemolytic transfusion reaction
  • severe liver disease
  • aortic aneurysm
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4
Q

Symptoms + signs of DIC

A

Underlying disease: sepsis, preganacy, cancer

Acute DIC: bleeding

  • nose bleeds
  • petechiae, purpura, ecchymoses
  • respiratory distress
  • signs of shock (hypotension, tachycardia)
  • anaemia

Chronic DIC: clottng problems

  • DVT signs (swollen leg, SOB)
  • arterial thrombosis
  • anaemia
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5
Q

Investigations for DIC

A
  1. Bloods
  • FBC- low platelets, low Hb (anaemia)
  • LFTs
  1. Clotting
  • prolonged APTT/PT
  • low fibrinogen
  • high fibrin degradation product/D-dimer (indication of clot formation)
  1. Blood film: schistocytes (damaged RBC- damaged endothelium, haemolytic anaemia)
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6
Q

Management for DIC

A

Acute (use severity score- PT, prothrombin, D-dimer)

  • low bleeding risk => treat underlying condition
  • high bleeding risk (>5) => treat underlying condition +
  1. platelet transfusion
  2. FFP transfusion (replace clotting factors, fibrinogen, plasma proteins, anticoagulant proteins)

Chronic (low bleeding risk)

  • treat underlying cause
  • heparin (anticoagulant)

+/- transexamic acid = antifibrinolytic (for hyperfibrinolysis)

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7
Q

define Haemophillia

A

inherited clotting disorder (deficiency of clotting factors) causing XS deep bleeding and bruising

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8
Q

Types of haemophillia

A
  • A (MOST COMMON)- deficiency of factor 8
  • B - deficiency of factor 9
  • C (RARE)- deficiency of factor 11
  • aquired = autoantibodies against factor 8

A=> usually caused by inversion of factor 8 gene on X-chromosome

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9
Q

Inheritance pattern of haemophillia A/B

A

X-linked recessive

  • Risk factors: FHx of haemophillia, more common in males
  • females are usually carriers (can be symptomatic)
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10
Q

Symptoms + signs of haemophillia

A
  • swollen + painful joints (haemarthrosis- bleeding into joints)
  • haematoma- painful bleeding into. muscle
  • XS bruising
  • haematuria
  • nerve palsies (haemotomas compressing on nerves)
  • pallor (anaemia)
  • symptoms since birth/ after trauma/ after surgery

Females (carriers) => severe bleeding after trauma/ surgery or menorrhagia (heavy period bleeding)

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11
Q

Investigations for haemophillia

A
  • prolonged APTT
  • Plasma factor 8/9/11 assay (diagnostic)

To exclude other causes:

  • FBC - low Hb (if prolonged bleeding) + rule out thrombocytopenia
  • LFTs (look at ALT/AST => liver disfunction => raised PT/APTT)
  • Von willebrand studies => to exlcude VWD
  • PT time - to look at extrinsic pathways + common

Imaging (to look at location of bleeding)

  • Head CT/MRI - intracranial haemorrage
  • Neck CT/MRI- bleeding in airway
  • Abdo CT/ USS - lower GI bleeding
  • Endoscopy - look at bleeding
  • X-rays - to look at bone profile (malignancy)
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12
Q

Management of haemophillia

A

Severe bleeding

  • RESUS (A=>E) IV fluids
  • Replace missing factor (8/9)
  • Anti-fibrinolytics (aminocaproic acid/ transexamic acid)

Can give bypassing agents (recombinant FIIa + FVIII inhibitor bypasssing fraction) => to bypass coagulation cascade and generate thrombin => clotting

Mild Haemophillia A

  • desmopressin (increases FVIII levels)??
  • anti-fibrinolytics
  • FVIII concentrate
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13
Q

define anaemia

A

low Hb concentration due to:

low red cell mass (increased destruction/reduced production)

increased plasma volume

<135g/L M

<115g/L F

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14
Q

symptoms of anaemia

A

fatigue

dyspnoea

faintness

palpitations

headache

tinnitus

anorexia

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15
Q

symptom of anaemia if pre-existing coronary artery disease

A

angina

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16
Q

signs of anaemia

A

may be absent even in severe anaemia

pallor

tachycardia

flow murmurs (ejection-systolic loudest over apex)

cardiac enlargement

retinal haemorrhages

heart failure in later stages

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17
Q

first step in diagnosis of anaemia and type of anaemia

A

assess MCV

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18
Q

causes of microcytic anaemia

A

iron deficiency anaemia

thalassaemia

sideroblastic anaemia

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19
Q

when to suspect thalassaemia

A

if MCV is too low for Hb level and red cell count is raised

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20
Q

in thalassaemia and sideroblastic anaemia, be aware that

A

there is iron accumulation

tests will show increased serum iron/ferritin

low total iron binding capacity

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21
Q

causes of normocytic anaemia

A

acute blood loss

anaemia of chronic disease (reduced MCV)

bone marrow failure

renal failure

hypothyroidism (increased MCV)

haemolysis (increased MCV)

pregnancy

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22
Q

when to suspect marrow failure

A

reduced WCC or platelets in normocytic anaemia

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23
Q

causes of macrocytic anaemia

A

B12/folate deficiency

alcohol excess/liver disease

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24
Q

suspect haemolytic anaemia if

A

reticulocytosis, mild macrocytosis

decreased: haptoglobin
increased: bilirubin, LDH, urobilinogen

mild jaundice

no bilirubin in urine

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25
Q

does anaemia require blood transfusion?

A

no if Hb>70g/L

may be indicated in acute anaemia if Hb<70g/L

vital in anaemia with heart failure

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26
Q

blood transfusion for anaemia with heart failure

A

raise Hb to 60-80g/L slowly

10-40mg furosemide IV/PO

do not mis with blood

check for signs of worsening overload (raised JVP, basal crackles)

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27
Q

causes of iron deficiency anaemia

A

blood loss

malabsorption

hookworm

poor diet/poverty in children/babies

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28
Q

signs of chronic iroin deficiency anaemia

A

koilonychia

atrophic glossitis

angular cheilosis

post-cricoid webs

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29
Q

testing for iron deficiency anaemia

A

blood film

coeliac serology - if negative and no menstruation, urgent gastroscopy and colonoscopy

stool microscopy if revent travel

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30
Q

blood film of iron deficiency anaemia

A

microcytic, hypochromic anaemia

anisocytosis and poikilocytosis

decreased: MCV, MCH, and MCHC

decreased ferritin, increased transferrin

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31
Q

NB ferritin is

A

an acute phase protein and increased with inflammation

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32
Q

treatment of iron deficiency anaemia

A

treat the cause

ferrous sulfate 200mg/8hrs for at least 3 months after Hb normalises

IV iron only if oral route impossible/ineffective

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33
Q

reasons why IDA fails to respond to iron replacement

A

patient compliance

functional iron deficiency in chronic renal failure

malabsorption/GI disturbance

anaemia of chronic disease

misdiagnosis

34
Q

causes of anaemia of chronic disease

A

chronic infection

vasculitis

rheumatoid

malignancy

renal failure

35
Q

pathophysiology of aneamia of chronic disease

A

poor use of iron in erythropoeisis

cytokine induced shortening of RBC survival

decreased production of and response to erythropoeitin

36
Q

tests for anaemia of chronic disease

A

FBC

blood film

B12 and folate

TSH

tests for haemolysis

37
Q

ferritin in anaemia of chronic disease

A

normal

increased in mild normocytic/microcytic anaemia

38
Q

treatment of anaemia of chronic disease

A

treat underlying cause

erythropoeitin

IV iron

hepcidin inhibitors

inflammatory modulators

39
Q

sideroblastic anaemia

A

ineffective erythropoeisis

increased iron absorption

iron laoding in marrow +/- haemosiderosis

40
Q

suspect sideroblastic anaemia if

A

microcytic anaemia not responding to iron

41
Q

define polycythaemia

A

increased RBC concentration and increased Hb concentration than normal

  1. relative= low plasma volume + normal RBC conc.
  2. absolute = increased RBC conc.
42
Q

causes of absolute polycythaemia (increased RBC)

A

Primary

  • polycythaemia rubra vera (increased clonal proliferation of myeloid stem cells => increased RBC => increased Hb conc.) JAK2 mutation

Secondary

  1. appropriate increase in EPO- chronic hypoxia (Chronic lung disease/ high altitudes/ obstructive sleep apnoea)
  2. inappropriate increase in EPO- athletes use illegal EPO, renal/hepatocellular carcinomas, fibroids (benign tumours in uterus)
43
Q

causes of relative polycythaemia (low plasma volume, RBC normal)

A
  • acute- dehydration
  • chronic- obesity, hypertension, smoking, high alcohol
  • Gaisbock’s syndrome - young male smokers + hypertension => low plasma volume
44
Q

Presenting symptoms of polycythaemia

*hyperviscosity (thick blood)

A
  • chest/abdominal pain
  • headaches
  • tiredness
  • red skin
  • blurred vision
  • tinitus
  • gout - joint stiffness/pain/swelling

Polycythaemia vera

  • bruising
  • pruitus after hot bath
  • burning sensation on fingers/toes (erythromelalgia)
  • tummy pain from splenomegaly
  • night sweats
  • Risk factors: >40, PMHx of thrombosis, haemorrhage, Budd-Chiari, FHx of polycythaemia vera,
45
Q

examination findings of polycythaemia

A

Inspection:

  • plethora (red complexion)
  • scratch marks from itching
  • redness of conjuctiva
  • hypertension

Palpation

  • splenomegaly (75%)
  • abdominal masses (renal/hepatic/uterine tumours => secrete EPO)
46
Q

Investigations for polycythaemia

A

General polycythaemia

  • FBC- high Hb, high haematocrit, low MCV
  • LFTs - abnormal = secondary polycythaemia
  • U&Es
  • serum EPO
  • serum ferritin
  • JAK2 mutation
  • abdominal US - splenomegaly

Polycythaemia rubra vera

  • high Hb, high haematocrit, high RBC
  • High WCC
  • high platelets
  • low serum EPO
  • JAK2 mutation
  • bone marrow biopsy -erythoid hyperplasia + raised megakaryocytes

Secondary polycythaemia

  • high serum EPO
  • exclude chronic lung disease/ hypoxia (O2 sats/ CXR)
  • CT scan to check for EPO tumours
47
Q

management for polycythaemia

A

Conservative: (GP)

  • address factors causing low plasma volume (obesity, hypertension, smoking, alcohol)
  • manage CVD risk factors (hyperlipidemia, diabetes, hypertension, smoking)

Medical: (hospital)

  • Polycythaemia vera => VENESECTION (removal of blood- target haemocrit <0.45) + aspirin

*if high platelet/ disease progression => pharmalogical cytoreductive therapy

48
Q

Severe complications of polycythaemia

A
  • DVT- red, swollen, painful legs,
  • PE - breathlessness, haemoptysis, dizzy/lightheaded
49
Q

define pancytopenia

A
  • anaemia- low RBC
  • leucopenia- low WCC
  • thrombocytopenia- low platelets
50
Q

Causes of pancytopenia

A

Central (bone marrow not producing cells)

  • nutritional deficiencies (B12/folate- needed to make new cells)
  • Poor absorption of nutrients (alcohol, malabsorption, tapeworm)
  • Bone marrow failure
  1. viral infections (EBV, HIV, hepatitis C, parvovirus B19)
  2. cancers (lymphoma/leukaemia/multiple myeloma)
  3. chemotherapy (usually for cancer treatment so unless severe symptoms will not be stopped)
  4. genetic - Fanconi’s anaemia

Peripheral (increased destruction of cells)

  • autoimmune (RA, SLE)
  • splenic sequestration due to alcoholic liver cirrhosis, HIV, TB, malaria

*cells pooled and trapped in spleen => destroyed

51
Q

presenting symptoms of pancytopenia

A

symptoms can vary

  • anaemia (low O2)- SOB, tiredness/fatigue, chest pain, pale
  • low WCC- increased infection risk (fever)
  • low platelets - easy bruising, bleeding more easily/difficulty stopping bleeding

Other:

  • dizziness
  • paler than usual
  • rashes
  • ulcers
  • weakness
  • fast HR - palpitations
52
Q

Emergency presentations of pancytopenia (need emergency hospital admisison)

A
  • loss of consciousness
  • seizures
  • confusion
  • significant blood loss
  • SOB
53
Q

Investigations for pancytopenia

A
  1. Bloods:
  • FBC- low RBC/WCC/platelets
  • serum B12/folate
  • LFTs
  • TFTs
  • autoantibodies
  • test for infections (HIV-nucleic acid tests /TB)
  1. Peripheral Blood smear
  2. Bone marrow aspiration + biopsy (diagnose cause)
54
Q

Management of pancytopenia

A

treat cause:

  • nutritional supplements (B12/folate)
  • treat infections (HIV- ART, TB- ABs)
  • stop drugs causing damage
  • immunosuppression (for autoimmune conditions)

More severe symptoms:

  • stem cell transplant
  • bone marrow transplant
  • transfusions (if very low cell counts)
  • broad-spectrum antibiotics (WCC <500/ml)
55
Q

Complications of pancytopenia

A
  • increased infection risk (broad spectrum antibiotics)
  • life threatening anaemia (transfusions)
  • bleeding
  • tumour lysis syndrome (ppl with chemo treatment => causes metabolic disturbance)
56
Q

define leukaemia

A

cancer of the blood/ bone marrow

57
Q

types of leukaemia

A
  • acute myeloid- increase in myeloblasts => reduced RBC/WCC/platelets
  • acute promyeocytic (aggressive AML)
  • acute lymphoid- increase in lymphoblasts => reduced lymphocytes (childhood associated)
  • chronic myeloid- increase in mature cells (translocation -9:22 BCR-ABL fusion gene)
  • chronic lymphoid
58
Q

Risk factors for leukaemia

A
  • AML- increasing age, DOWN’s syndrome, anti-cancer drugs
  • ALL- children <6, irradiation/influenza/genetics
  • CML- genetic philadelpha chromosome (9:22)
  • CLL- genetic, M>F
59
Q

symptoms +signs of acute leukaemia

A

bone marrow failure: anaemia, bleeding, infections (neutropenia)

tissue infiltration:

  • Acute myeloid: swollen gums, mild splenomegaly
  • Acute lymphoid: lymphadenopathy, swollen testes, hepatosplenomegaly (childhood leukaemia)
60
Q

sign + symptoms of chronic leuakaemia

A

*50% asymptomatic

Chronic myeloid (proliferation of mature cells -RBCs, WCC, platelets)

  • massive splenomegaly (time to develop)
  • hyperviscosity (thick blood- thrombotic events)
  • hypermetabolic symptoms (FLAWS)

Chronic lymphoid

  • non-tender lymphadenopathy
  • some bone marrow failure symptoms
61
Q

Investigations for leuakaemia

A
  1. Bloods:
  • FBC - WCC, RBC, platelets
  • LDH - tumour marker
  • Blood smear
  1. Bone marrow biopsy
  • auer rods- AML
  • >20% abnormal lymphoblasts - ALL
  • smudge cells - CLL
  1. Immunophenotyping (chromosomes), CXR
62
Q

Management of leukaemia

A
  1. Chemo (methotrexate)
  2. Bone marrow transplant
63
Q

define lymphoma

A

cancer of lymph nodes/lymphatic tissue

64
Q

Types of lymphoma

A
  1. Hodgkin’s lymphoma
  2. Non-hodgkin’s lymphoma
  • Burkitt’s lymphoma
65
Q

Risk factors for lymphoma

A
  • EBV association
  • non-Hodgkinn’s: increasing age, EBV, HIV, SLE, sjoren’s
66
Q

Symptoms + signs of lymphoma

A
  • painless enlarging neck lump
  • FLAWS - weight loss, fatigue, night sweats
  • Hodgkin’s - painful when drinking alcohol, non-tender lymphadenopathy, >FLAWS
  • Non-Hodgkin’s - organ symptoms (rashes, hepatosplenomegaly, headaches)
67
Q

Investigations for lymphoma

A

Lymph node biopsy: (diagnostic)

  • reed-sternberg cells- Hodgkin’s
  • starry sky apprearance - Burkitt’s
68
Q

What system is used to stage lymphomas?

A

Ann- Arbor

69
Q

management for lymphoma

A
  • chemotherapy (first line)
  • radiotherapy
  • immunotherapy (monoclonal antibodies)
70
Q

define multiple myeloma

A

cancer of plasma cells => increased monoclonal immunoglobulin production

71
Q

risk factors for myeloma

A
  • >70
  • Afro-carribean
  • agricultural work
  • HIV
  • ionising radiation
72
Q

signs + symptoms of multiple myeloma (CRAB)

A
  • Calcium (high)- bones, stones, abdominal groans, psychic moans
  • Renal impariment (worse prognosis)
  • Anaemia- due to overcrowding of plasma cells
  • Bone lesions (back/rib pain)- cell signalling activates osteoclasts
73
Q

Investigations for mutliple myeloma

A
  1. Bloods:
  • FBC
  • Ca2+
  • U&Es
  • ESR/CRP
  • ALP (normal, abnormal in bone cancer)
  1. Blood film:
    * rouleaux formation (protein/immunoglobulin clumped together like a stack of coins)
  2. Electrophoresis
    * bence-jones proteins (monoclonal IgG/ IgA)
  3. Bone marrow aspirate/biopsy
    * increased plasma cells (>10%)
74
Q

management for multiple myeloma

A
  • chemotherapy
  • bone marrow transplant
75
Q

define sickle cell disease

A
  • group of inherited diseases with a mutated beta-globin chain (HbS) which polymerise when deoxygenated so RBC becomes sickle shaped.
  • Gene = Autosomal recessive
  • Sickle cell trait- Hb AS (carriers of the disease)
  • Sickle cell anaemia - Hb SS
76
Q

symptoms of sickle cell disease

A
  • usually diagnosed on newborn screening (heel prick test)
  • signs of haemolysis (jaundice, pallor, tachycardia)
  • splenic sequestration (pallor, tachycardia)
  • in children swollen painful joints, dactylitis (sausage fingers)
  • in adults mainly complications/ crisis
77
Q

symptoms of sickle cell criss

A
  • severe pain
  • infection
  • anaemia
  • stroke/pripaism (prolonged erection)
  • Hx of previous crisis
78
Q

management of sickle cell disease

A
  • refer if signs of crisis
  • patient advice - avoid triggers (exercise, cold), increase fluids, distraction techniques
  • follow care plan/ prescribe paracetomol/ibuprofen/ dihydrocodeine (severe pain)
  • empirical antibiotics if signs of infection/fever

Severe episodes:

  • psychological support
  • elective blood transfusions (for severe anaemia)
  • hydroxycarbamide- treat symptoms
  • surgery
  • stem cell transplant (curative/ rarely done)
79
Q

prevention of complications from sickle cell

A
  • vaccinations - pneumonoccal, influenza, meningitis
  • malaria prophylaxis
  • penicillin prophylaxis
80
Q

advice for sickle cell carriers

A
  • usually asymptomatic
  • still at risk of vaso-occlusion (high altitudes, scuba diving, inform anaesthesist)
  • malaria prophylaxis
  • genetic counselling (kids risk of having it)
81
Q

complications of sickle cell disease

A

Acute

  • sickle cell crisis
  • chest infections
  • osteomyelitis
  • pripaism
  • AKI
  • neurological complications
  • strokes/TIA

Chronic

  • leg ulcers
  • hepatobilliary issues
  • sickle lung
  • pulmonary hypertension
  • gallstones
  • eye complications (most serious)

Year 3 (27 decks)

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