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Phase 2 Med > Haematology > Flashcards

Haematology Flashcards

1
Q

Anaemia definition

A

Defined as blood with an insufficient concentration of haemoglobin
Pregnant women over 15 years: Hb <11g/dL
Non-pregnant women over 15 years: Hb <12g/dL
Men over 15 years: Hb <13g/dL

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2
Q

Anaemia general presentation

A

Symptoms: fatigue. Lethargy, dyspnea, palpitations, headache

Signs: pale skin, pale mucous membranes, tachycardia (compensatory to meet demand)

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3
Q

Microcytic/normoytic/macocytic anaemia definition

A

Microcytic anaemia: several types of anaemia characterised by small RBCs (known as microcytes). It is defined as when the mean corpuscular volume (MCV) is less than 80fL. Mean corpuscular volume describes the average size of RBCs.

Normocytic Anaemia: Anaemia with a MCV 80-100fL (normal range). Can be further divided:

  • hyperproliferative (reticulocyte count >2%): the proportion of circulating reticulates increases as a compensatory response to increased destruction or loss of RBCs. The cause is usually acute blood loss or haemolysis
  • hypoproliferative (reticulocyte <2%): primarily disorders of decreased RBC production, and the proportion of circulating reticulocytes remains unchanged

Microcytic Anaemia: where RBCs are larger than their normal volume (MCV > 100fL). Can be further divided as:

  • megaloblastic: deficiency of DNA production or maturation resulting in the appearance of large immature RBCs (megaloblasts) and hyperhsegmented neutrophils in the circulation
  • non-megaloblastic: encompasses all other causes of microcytic anaemia in which DNA synthesis is normal. Megaloblasts and hyperhsegmented neutrophils are absent.
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4
Q

Microcytic/normoytic/macocytic anaemia aetiology

A

Micro:

  • chronic disease (cancer, HF, CKD)
  • thalassaemia
  • Fe deficiency (blood loss, poor diet, malabsorption, hookworm)

Normo:

  • acute blood loss
  • anaemia of chronic disease
  • pregnancy
  • renal failure
  • combined haematinic deficiency (Fe & B12/folate deficiency > needed for haematopeiesis > consider malabsorption as a cause

Macro:

  • B12/folate deficiency
  • excess alcohol/liver disease
  • hypothyroidism
  • bone marrow failure
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5
Q

Iron deficiency anaemia definition

A

Occurs when the body has insufficient Fe to support RBC production.
Most common cause of anaemia worldwide and in pregnancy.
Premenopausal women have a higher presence of IDA because of menstrual blood loss and pregnancy. Is considered a microcytic anaemia and is by far the most common cause of it.

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6
Q

Iron deficiency anaemia aetiology

A

Causes of iron deficiency may be classfied as those due to:
Excessive blood loss:
- blood loss from GI tract is the most common cause of IDA in adult men and postmenopausal women
- blood loss due to menorrhagia is the most common cause in pre-menopausal women

Dietary inadequacy:
- growing children and elderly people with iron-poor diets may become deficient

Failure of iron absorption:

  • some drugs: tetracyclines and quinolones
  • anatacids and PPIs may impair absorption
  • Vitamin C deficiency
  • malabsorption conditions such as coeliacs
  • from gastrectomy
  • H. pylori infection
  • Hookworm

Excessive requirements for iron:

  • times of rapid growth in children
  • pregnancy, especially with twins
  • exfoliative skin disease
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7
Q

Iron deficiency anaemia presentation

A

General signs and symptoms of anaemia
Specific:
- brittle hair and nails
- atrophic glossitis (tongue inflammation with smooth tongue)
- Koilonychia (spoon shaped nails)
- Angular Stomatitis (inflammation fo corners of mouth)

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8
Q

Iron deficiency anaemia diagnosis

A

Drug history - NSAIDs, SSRIs, clopidogrel, corticosteroids
FBC: shows hypochroic microcytic anaemia
- hypochromia means there is a low mean corpuscular haemoglobin (MCH)

Serum ferritin: measured to confirm iron deficiency (except during pregnancy)
- low

Reticulocyte count - reduces
Endoscopy - possible GI bleed related cause
Urinalysis - evaluate blood loss from renal tract

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9
Q

Iron deficiency anaemia management

A

Iron salts should be given by mouth:

- side effects include black stools, constipation, diarrhoea, nausea, GI upset, epigastric pain

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10
Q

Folate deficiency anaemia definition

A

Deficiency of Folate, a B vitamin (B9). CLassically presents as megaloblastic anaemia, without any neurological signs. Unlike B12, reserves of B9 are low and only sufficient for around 4 months.

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11
Q

Iron deficiency anaemia aetiology

A 
Main cause is poor intake 
Dietary deficiency 
- malabsorption (coeliacs etc) 
- anorexia 
Excessive requirements
- pregnancy, lactation, infancy 
- malignancy 
- blood disorders (haemolytic and sick anaemia) 
Antifolate drugs
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12
Q

Iron deficiency anaemia presentation

A

May be completely asymptomatic
Normal anaemia signs and symptoms:
Loss of appetite and weight loss is a symptom of megaloblastic anaemia and the hallmark of folate deficiency
Glossitis (inflammation of the tongue) may be present
Angular stomatitis (ulcers in corners of mouth)
Lemon-yellow skin
Neurological features: mild compared to B12 deficiency

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13
Q

Iron deficiency anaemia diagnosis

A

FBC:
- low haemoglobin with elevated MCV and MCH

Peripheral blood smear:
- macrocytic naemia and hypersegmented neutrophils seen with both folate and B12 deficiency

Erythrocyte folate level

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14
Q

Iron deficiency anaemia differential diagnosis

A

Vitamin B12 deficiency, alcoholic liver disease, hypothyroidism, myelodysplasia and aplastic anaemia

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15
Q

Iron deficiency anaemia management

A

Daily folic acid tablets (build up folate levels)

Dietary advice - good sources of folate include: broccoli, nuts

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16
Q

Iron deficiency anaemia complications

A

Infertility, cardiovascular disease, cancer, problems in childbirth, neural tube defects and folic acid

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17
Q

Haemolytic anaemia defintiion

A

Disorder in which RBCs are destroyed faster than they can be made

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18
Q

Haemolytic anaemia risk factors

A

Sickle cell disorders

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19
Q

Haemolytic anaemia aetiology

A

Genetic: sick cell, thalaseeaemia, G6PD deficiency, pyruvate kinase deficiency
Acquired: autoimmune, infections, secondary to systemic disease

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20
Q

Haemolytic anaemia presentations

A

Symptoms:
- gallstones (excess bilirubin)

Signs:

  • jaundice (increase bilirubin from RBC destructions) - bilirubin stones may cause abdo pain (gallstone pain)
  • leg ulcers
  • splenomegaly
  • signs of underlying disease (SLE malar rash)
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21
Q

Haemolytic anaemia diagnosis

A

FBC - low Hb
Reticulocyte count - increase
Blood film - presence of Schistocytes

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22
Q

Haemolytic anaemia management

A

Folate and iron supplementation
Immunosuppressives
Splenectomy

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23
Q

B12 deficiency anaemia definition

A

Deficiency of Cobalamin, a B vitamin (B12). Classically presents as megaloblastic anaemia, with neurological signs

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24
Q

B12 deficiency anaemia aetiology

A

Pernicious anaemia

  • autoimmune destruction of instrinsic factor due to intrinsic factor targeting antibodies
  • often associated with autoimmune destruction of parietal cells (which produce intrinsic factor)

Dietary insufficiency
- veganism

Gastric causes

  • atrophic gastritis
  • gastrectomy
  • H. pylori infection

Intestinal causes

  • Crohn’s disease
  • malabsorption

Drugs

  • PPIs
  • neomycin
  • metformin
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25
Q

B12 deficiency anaemia key presentations

A

Neurological changes (paraesthesia, numbness, cognitive changes)

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26
Q

B12 deficiency anaemia signs and symptoms

A

Normal anaemia presentation

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27
Q

B12 deficiency anaemia investigations

A
  • blood film
  • autoantibody screen - check for IF antibodies
  • serum B12 (may be normal despite clinical B12 deficiency symptoms - ‘false normal B12 deficiency’)
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28
Q

B12 deficiency anaemia differential diagnosis

A

Folate deficiency

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29
Q

B12 deficiency anaemia management

A
  • IM B12 supplementation (Hydroxocobalamin)
  • care should be taken not to give folic acid (instead of B12) to any patient who is B12-deprived, as this may result in fulminant neurological deficit
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30
Q

B12 deficiency anaemia complications

A
  • severe anaemia causes risk of cardiopulmonary complications
  • predisposes to neural tube defects (such as spina bifida, anencephaly, and encephalocele) in the fetus
  • sterility
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31
Q

Deep vein thrombosis definition

A

Development of a blood clot in a major deep vein in
- leg, thigh, pelvis, or abdomen
May result in impaired venous blood flow/consequent leg swelling and pain.

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32
Q

Deep vein thrombosis epidemiology

A

1-2 per 1000 population, but incidence of up to 37% in those who are critically ill

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33
Q

Deep vein thrombosis aetiology

A

Anything preventing blood flowing/clotting can cause a venous thrombosis. Main causes: damage to vein from surgery or trauma and inflammation from infection or injury

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34
Q

Deep vein thrombosis risk factors

A

Thromboembolic risk factors, such as cancer, trauma, major surgery, hospitalisation, immobilization, pregnancy, or oral contraceptive use.

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35
Q

Deep vein thrombosis pathophysiology

A

See Virchow’s triad (hyper coagulability, venous stasis, damage to vessel wall)
Commonest in lower limb below knee
Starts at low-flow site eg soleal sinus, behind venous valve pockets

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36
Q

Deep vein thrombosis key presentations

A

Calf swelling

Localised pain along deep venous system

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37
Q

Deep vein thrombosis signs and symptoms

A 
Asymptomatic; 
Asymmetrical aedema 
Unilateral leg pain/dilation 
Distention of superficial veins 
Red/discoloured skin
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38
Q

Deep vein thrombosis 1st line investigations

A
  • Wells’ score (point system) (2 or more means DVT is likely)
  • quantitative D-dimer level (if <2 proceed to imaging)
  • proximal duplex ultrasound
  • whole-leg ultrasound (inability to fully compress lumen of vein using ultrasound transducer, reduced or absent spontaneous flow, lack of resp. variation, intraluminal echoes, colour flow patency abnormalities)
  • FBC

Other:

  • Doppler venous flow testing (low flow in veins)
  • CT abdomen and pelvis with contrast (intraluminal filling defect)
  • thrombophilia screen
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39
Q

Deep vein thrombosis gold standard investigations

A

Doppler Ultrasound Scan

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40
Q

Deep vein thrombosis deifferential diagnosis

A
  • cellulitis
  • peripheral oedema
  • vasculitis
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41
Q

Deep vein thrombosis prevention

A
  • stockings
  • mobilisation
  • leg elevation
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42
Q

Deep vein thrombosis management

A 
Anticoagulants such as 
- warfarin
- low molecular weight heparin 
- novel oral anticoagulants (NOACs) - typically apixaban 
IVC filter to prevent PE
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43
Q

Deep vein thrombosis monitoring

A

Oral antocoagulation continued for 3-6 months
In patients with significant thromboembolic risks, careful consideration should be given to maintaining oral anticoagulation indefinitely as long as bleeding risk = lower then risks of recurrent venous thrombosis

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44
Q

Deep vein thrombosis complications

A

Pulmonary embolism is heavily associated with DVT - the umbrella term venous thromboembolism (VTE) is often used to cover both conditions

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45
Q

Deep vein thrombosis prognosis

A

Recurrence is common after one incident of DVT

Without anticoagulation, the risk of recurrence of VTE (DVT or PE) is thought to be 50% within three months of a PE. Risk of recurrence within the first year of a VTE following three months of anticoagulation is thought to be 8%.

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46
Q

Pulmonary embolism definition

A

When a thrombus becomes loged in one of the pulmonary arteries. Usually travelled from another site eg DVT.

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47
Q

Multiple myeloma definition

A

Myloma is the cancer of plasma cells (chief plasma cell dyscrasia).
In myeloma the malignant plasma cell produces an excess of one type of immunoglobulin known as monoclonal paraprotein (mainly IgG 55%, can be IgA 20%, rarely IgM and IgD).

These plasma cell clones accumulate and crowd the bone marrow, preventing the production of normal levels of healthy blood cells -> anaemia, impaired immune function and low platelets.
Monoclonal oaraperotein can also deposit in other organs and cause dysfunction

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48
Q

Multiple myeloma epidemiology

A

Median age 70, more common in Afro-caribbeans and males

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49
Q

Multiple myeloma aetiology

A

Unknown

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50
Q

Multiple myeloma risk factors

A

Chromosomal abnormalities - t(11;14) (most comon) (t(11;14) means reciprocal translocation between chromosome 11 and 14) etc

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51
Q

Multiple myeloma pathophysiology

A

Affects plasma cells

- results in progressive bone marrow failure = excess IgG/IgA

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52
Q

Multiple myeloma key presentations

A

Mneumonic CRAB
C - hypercalcaemia (AKI, thirst, confusion, constipation)

R - renal impairment (nausea/vomiting, weight loss, uraemia)

A - anaemia/neutropenia/thrombocytopenia (dyspnoea, fatigue, pallor, bleeding, infection)

B - bone lesions (bone pain, spinal cord compression)

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53
Q

Multiple myeloma 1st line investigations

A

FBC:

  • anaemia
  • thrombocytopenia
  • neutropaenia
  • persistently high ESR (indicated inflammation)

U&E:

  • hypecalcaemia
  • high alkaline phosphatase

Blood film:
- rouleaux (aggregations of red blood cell stacks caused by increased serum proteins)

Serum and/or urine electrophoresis
Urine:
- bence jones protein (monoclonal globulin protein)

X-rays: lytic ‘punched-out’ lesions, ie

  • pepper-pot skull
  • vertebral collapse
  • fractures
  • osteoporosis

CT:

  • bone lesions
  • cord compression
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54
Q

Multiple myeloma differential diagnosis

A

Monoclonal gammopathy of undetermined significance (MGUS):

  • MIg (monoclonal Ig) <30g/L (but still relatively higher than normal population)
  • no significant increase in bone marrow plasma cells
  • no related organ impairment
  • 1% transform to myeloma per year so eg 20% in 20 years etc
  • there is a paraprotein in the serum but no myeloma, with no bone lesions, no Bence Jones protein
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55
Q

Multiple myeloma management

A

1st line: induction chemotherapy
- thalidomide AND dexamethasone

PLUS:
DVT prophylaxis - aspirin
Stem cell transplant
Bisphosphates for bones - pamidronate disodium

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56
Q

Multiple myeloma monitoring

A

M protein levels in blood/urine determine extent of disease and monitor effectiveness of treatment

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57
Q

Multiple myeloma complications

A
  • hypercalcaemia
  • spinal cord compression
  • hyperviscosity
  • acute renal injury
  • immune deficiency
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58
Q

Multiple myeloma prognosis

A

Although novel treatment strategies have resulted in improved outcomes (including response rates and survival), multiple myeloma (MM) remains an incurable disease. MM patients inevitably relapse 2 to 5 years after treatment.

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59
Q

Sickle cell anaemia definition

A

Disorder of quality; haemoglobinopathy

Autosomal recessive:
A point mutation in the beta globin chain results in haemoglobin S variant instead of HbBv. Polymerisation of the haemoglobin chains dmagaes RBCs, causing sickling. Sickled RBC get stuck easily

Offers protection against falciparum malaria

Heterosygotes may still, however, experience symptomatic sickling hypoxia, eg in unpressurised aircraft or anaethesia (so all those of African descent need a pre-op sickle-cell test)

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60
Q

Sickle cell anaemia epidemiology

A

It is most commonly seen in people of African origin

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61
Q

Sickle cell anaemia signs and symptoms

A

Acute - painful crises, sickle chest syndrome, stroke

Chronic - renal impairment, pulmonary hypertension, joint damage, infections

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62
Q

Sickle cell anaemia gold standard investigations

A

Hb electorphoresis: confirms the diagnosis and distinguishes ss, as states, and other Hb variants

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63
Q

Sickle cell anaemia management

A

Hydroxyurea (increases foetal haemoglobin levels)
Transfusion
Stem cell transplant

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64
Q

Sickle cell anaemia prognosis

A

Reduce life expectancy (around 50 y/o)

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65
Q

Acute myeloid leukaemia definition

A

Neoplastic proliferation of myeloblast cells (which normally gives rise to basophils, neutrophils, eosinophils). Progresses very rapidly, diagnosis is a medical emergency

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66
Q

Acute myeloid leukaemia epidemiology

A

Adults 40+
Most common type of acute leukaemia in adults
Associated with Down’s syndrome and radiation

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67
Q

Acute myeloid leukaemia aetiology

A

Can be a long-term complication of chemo eg for lymphoma
Myelodysplastic states
Radiation
Down’s syndrome (and other syndromes)

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68
Q

Acute myeloid leukaemia signs and symptoms

A

Anaemia, bleeding, infection (marrow failure)
Hepatosplenomegaly
Peripheral lymphadenopathy
GUM HYPERTROPHY

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69
Q

Acute myeloid leukaemia 1st line investigations

A

FBC: anaemia, thrombocytopenia, neutropenia
Blood film: leukamic blast cells
Bone marrow biopsy: increased cellularity and on biopsy, it is differentiated from all by Auer rods,
Immuni-phenotyping
Molecular methods
Cytogenic analysis (eg type of mutation) guides treatment recommendations and prognosis

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70
Q

Acute myeloid leukaemia differential diagnosis

A

Acute promyelocytic anaemia
Sub-type of Acute Myeloid Leukaemia (5-8% of AML in adults)

Abnormal accumulation of immature granulocytes called promyelocytes.
t(15;17) - PML/RARA gene - blocks differentiation of promyelocute to mature granulocyte

Targeted treatment ATRA (APL is unique among leukemias sue to its sensitivity to all-trans retinoic acid (ATRA; tretinoin, the acid form of vitamin A)

71
Q

Acute myeloid leukaemia management

A

Blood and platelet transfusions
Allopurinol to prevent tumour lysis
Chemotherapy - Anthracycline, Cytarabine
Steroids
Sibling matched allogenic bone marrow transplant

Tretinoin (ATRA) if acute promyelocytic leukaemia suspected

72
Q

Acute lymphoblastic leukaemia definition

A

Malignancy of immature lymphoid cells (which give rise to T cells and B cells).
Leads to arrest in the maturation of T and B cells, and promote incontrolled proliferation of lymphoblasts (a blast cell is a precursor cell which is a partially differentiated cell, it is unipotent)

73
Q

Acute lymphoblastic leukaemia epidemiology

A

Commonest form of malignancy in children.

Associated with Down’s syndrome

74
Q

Acute lymphoblastic leukaemia risk factors

A 
Genetic predisposition, ionizing radiation (eg x rays) during pregnancy, and Down's syndrome are important associations, CNS involvement is common
Previous chemo (certain meds eg etoposide)
75
Q

Acute lymphoblastic leukaemia signs and symptoms

A

Anaemia, bleeding, infection (BM failure)
Hepatosplenomegaly
Peripheral lymphadenopathy
CNS involvement ( eg facial palsy)
Organ inflitration
SVC obstruction: red face, dilated superficial chest veins
Bone marrow failure and bone/joint pain

76
Q

Acute lymphoblastic leukaemia 1st line investigations

A

FBC: anaemia,, thrombocytopaenia, neutropenia
Blood film: leukaemia blast cells
Bone marrow: increased cellularity
Lumbar puncture (should be performed to look for cns involvement)
CXR and CT scan to look for mediastinal and abdominal lymphadenopathy

77
Q

Acute lymphoblastic leukaemia management

A 
Chemo 
Steroids 
Intrathecal drugs eg methotrexate 
Blood transfusion 
IV fluids 
Infection control
78
Q

Acute lymphoblastic leukaemia complications

A

Extremely vulnerable to infection

Prone to incontrolled and serious bleeding (lack of platelets)

79
Q

Acute lymphoblastic leukaemia prognosis

A

The younger the patient is at diagnosis, the better the outlook

80
Q

Chronic lymphocytic leukaemia (CLL) definition

A

Most common type of leukaemia

BM malignancy resulting in accumulation of mature B cells that fail to apoptose

81
Q

Chronic lymphocytic leukaemia epidemiology

A

Most common leukaemia (>25%)
Adults 70+
Ppl with low immunity due to HIV/AIDS are three times more likely than the general population to develop leukaemia. Ppl on long term immunosuppression are at risk as well.

82
Q

Chronic lymphocytic leukaemia key presentations

A

Often asymptomatic

Enlarged, subbery non-tender nodes

83
Q

Chronic lymphocytic leukaemia signs and symptoms

A

Dependent on severity. may be:

  • anaemic
  • infection prone
  • weight loss/sweats/anorexia
  • hepatosplenomegaly
  • enlarged render rubbery nodes
84
Q

Chronic lymphocytic leukaemia 1st line investigations

A

FBC: high WCC with high lymphocytes

Blood film: small, mature lymphocytes

85
Q

Chronic lymphocytic leukaemia management

A
  • watch and wait
  • blood transfusions
  • stem cell transplant
  • chemo
  • monoclonal antibodies (eg anti-CD20 - rituximab)
  • targeted therapy (eg bruton kinase inhibitors - ibrutinib)
86
Q

Chronic lymphocytic leukaemia complications

A

Death is often due to infection or transformation to aggressive lymphoma (Richter’s syndrome)

  1. Autoimmune haemolysis, ITP
  2. Raised infection due to hypogammaglobulinaemia (= lower igg), bacterial, viral especially herpes zoster
  3. Marrow failure
  4. Progressive lymphadenopathy/hepatoplenomegaly

If someone comes in with repetitive similar infections (tonsilitis) do a blood count and blood film

87
Q

Chronic myeloid leukaemia definition

A

BM malignancy that creates overproduction of mainly basophils, eosinophils, neutorphils

88
Q

Chronic myeloid leukaemia epidemiology

A

Almost exclusively a disease of adults
Most often in people between 40-60 years old with a slight male predominance

CML is extremely rare (CLL is approx. 10x as common)

89
Q

Chronic myeloid leukaemia aetiology

A

More than 80% patients have a translocation between chromosome 9 and chromosome 22 (‘Philidelphia chromosome’), which creates a ‘fusion gene’ from BCR and ABL kinase, which has tyrosine kinase activity that stimulates cell division

90
Q

Chronic myeloid leukaemia signs and symptoms

A

Mostly chronic and insidious: wight loss, tiredness, fever, sweats
May be features of gout (due to purine breakdown)
Bleeding (platelet dysfunction)
Abdo discomfort - splenomegaly
Anaemia, infection (BM failure)

Signs: massive hepatosplenomegaly

91
Q

Chronic myeloid leukaemia 1st line investigations

A

FBC: anaemia, raised myeloid cells
Bone marrow: increased cellularity
Reverse transcriptase PCR: Philidelphia chromosome t(9;22)

92
Q

Chronic myeloid leukaemia management

A

Chemo
TKI (Tyrosine Kinase Inhibitor eg Imatinib)
Stem cell transplant

93
Q

Chronic myeloid leukaemia complications

A

Can transform to ‘blast phase’ - acute leukaemia

94
Q

Chronic myeloid leukaemia prognosis

A

Depends on treatment response, may be chronic controlled disease

95
Q

Hodgkin lymphoma definition

A

Malignant tumour of the lymphatic system characterised by the presence of Reed-sternberg cells and associated abnormal and smaller mononuclear cells originating from B lymphocytes

96
Q

Hodgkin lymphoma epidemiology

A

Young adults and elderly
Male predominance
Associated with Epstein-Barr virus and lupus

97
Q

Hodgkin lymphoma presentation

A 
Fever and sweating 
Chest discomfort with a cough or dyspnea 
Enlarged rubbery non-tender nodes 
Systemic 'B' symptoms eg fever 
Painful nodes on drinking alcohol
98
Q

Hodgkin lymphoma diagnosis

A

FBC: anaemia, high ESR (erythrocyte sedimentation rate)
CXR: wide mediastinum: mediastinal masses
PET scan
Reed sternerg cells - giant cells usually derived from B cells

Ann Arbor staging system:

  • Stage I: involvement of one lymph-nide region or lymphoid structure (eg spleen, thymus)
  • Stage II: two or more lymph-node regions on the same side of the diaphragm
  • Stage III: lymph nodes on both sides of the diaphragm
  • Stage IV: involvement of extranodal dies
99
Q

Hodgkin lymphoma management

A 
Chemotherapy, ABVD: 
- Adriamycin 
- Bleomycin 
- Vinblastine 
- Dacarbazine 
Autologous marrow transplant
100
Q

Non-Hodgkin lymphoma definition

A

Broad group of all lymphomas (malignancy originating from lymphocytes) except Hodgkins

101
Q

Non-Hodgkin lymphoma epidemiology

A

Aduts 40+
Associated with EBV and Burkitt’s lymphoma
Family history increases risk
Over 5x more common than Hodgkins

102
Q

Non-Hodgkin lymphoma presentation

A

Fever and sweating
Enlarged rubbery non-tender nodes
Systemic ‘B’ symptoms eg fever
GI and skin involvement

103
Q

Non-Hodgkin lymphoma diagnosis

A 
FBC: anaemia, high ESR 
Raised lactose dehydrogenase 
Lymph node biopsy 
Bone marrow biopsy for classification 
Can also use Ann Arbor staging
104
Q

Non-Hodgkin lymphoma management

A 
Steroids 
Monoclonal antibodies to CD20: Rituximad 
CHOP regimen: 
- cyclophosphatase
- hydroxy-daunorubicin 
- Vincristine 
- Prednisolone
105
Q

Malaria definition

A

Parasitic infection carried by mosquitoes - the protozoan parasite invades RBCs = causing an intermittent and remittent fever.

106
Q

Malaria aetiology

A 
Plasmodium parasites - 5 types cause malaria in humans 
Plasmodium falciparum
- Africa 
- most common type of malaria parasite
- causes most malaria deaths worldwide

Plasmodium vivax

  • Asia/South America
  • milder symptoms than PF
  • can stay in liver for up to 3 years > relapses

Plasmodium ovale

  • uncommon
  • West Africa
  • can remain in liver for several years without symptoms

Plasmodium malariae

  • quite rare
  • Africa

Plasmodium knowlesi

  • very rare
  • Southeast Asia
107
Q

Malaria key presentations

A

Initial symptoms of malaria are flu-like and include:

  • a high temp pf 38C or above
  • feeling hot and shivery
  • headaches
  • vomiting
  • muscle pains
  • diarrhoea
  • generally feeling unwell

Symptoms occur in 48-hour cycles

108
Q

Malaria 1st line investigations

A 
Travel history 
Thick and thin blood smears
- negative film can be seen 
- if negative, 2 more films should be sent over the next 48 hours 
Rapid diagnostic tests 
Detect parasitic antigens 
PCR 
FBC, LFTs, U&Es, blood gases, blood culture 
CXR, lumbar puncture
109
Q

Malaria differential diagnosis

A

Pharyngitis, bronchitis, pneumonia, bronchopneumonia, worsening of asthma, pleurisy

110
Q

Malaria management

A

Prevention

  • insect repellents
  • antimalarials

For severe/complicated/P.Falciparum malaria:

  • IV artesunate
  • if IV artesunate not available: IV quinine and doxycycline

For uncomplicated/non-falciparum malaria:

  • iral cholorquinine
  • artemisinin combination therapy (ACT) for mixed infection
  • Primaquine if P. ovale or P vivax (as these species can form hypnozoites in liver which lie dormant - primaquine eliminates these) - contraindicated in pregnancy, breastfeeding, or G6PD deficiency

Ensure that all cases of malaria have been notified to Public Health England

111
Q

Malaria complications

A

Anaemia, cerebreal malaria, liver failure/jaundice, shock - drop n BP, pulmonary oedema, acute respiratory distress syndrome (ARDS), hypoglycaemia, kidney failure, swelling and rupturing of spleen, dehydration

112
Q

Thalassaemia definition

A

Disorder of quantity; haemoglobinopathy

Reduced Hb production

113
Q

Thalassaemia epidemiology

A

African Americans and in people of Mediterranean and Southeast Asian descent

114
Q

Thalassaemia aetiology

A

Mutations in the Hb beta (HBB) gene

115
Q

Thalassaemia risk factors

A

Family history

116
Q

Thalassaemia pathophysiology

A

Globin chain disorders resulting in diminished synthesis of >1/= globin chains = reduced Hb

Hb will be destroyed by spleen for being abnormal
- Also RBCs can’t be made normally because of insufficient erythropoiesis from insufficient globin chains of both types

2 types of BT - alpha and beta

Beta-T occurs from mutational genetic abnormalities
Alpha-T occurs though delusional genetic abnormalities

What affects the phenotype of the disease?

ALPHA
Can be a carrier with just 1/2 abnormal genes.
Varying amounts of abnormal genes can change phenotype of disease:
- 1 abnormality - normal or minimal change to Hb, MCV and MCH (mean corpuscular haemoglobin)
- 2 abnormalities - more marked changes, MHC <25pg (can either be alpha 0 or alpha +
- 3 abnormalities - moderately severe Hb 30-100g/l, MCH 15-20pg (hbH disease)
- 4 abnormalities - hydrops fetalis (Hb Bart’s hydrops fetalis is the most severe form of alpha-thalassemia and is almost always lethal

BETA
Beta thalassemia can be
- thalassemia major (transfusion dependent)
- thalassemia intermedia (less severe anaemia/can survive without regular transfusions)
- thalassemia carrier/heterozygote (asymptomatic)

Gives partial malaria resistance

117
Q

Thalassaemia key presentations

A

Beta Thalassemia Major - Age at presentation: 1st year of life (6-12 months as adult Hb starts to replace foetal).

Clinical presentation with severe symptoms: failure to feed, listless, crying, pale, hypertrophy of bone marrow causes bone deformities and facial structure changes

Hb 40-80g/l but normal ferritin
A-thalassaemia = anaemia symptoms

118
Q

Thalassaemia 1st line investigations

A

Blood film shows large and small cells, pale cells and nucleated cells

  • decreased Hb 30-70 g/l, MCV and MHC very low
  • increased HbF >90%

ALPHA

  • complete blood count (CBC). This test checks the size, number and maturity of different blood cells in a set volume of blood
  • haemoglobin electrophoresis. A lab test that tells what type of haemoglobin is present
  • ferritin. This test is done to ruleout iron-deficiency anaemia
  • DNA testing. This test identifies which alpha globin genes are present, absent, or damaged
119
Q

Thalassaemia gold standard investigations

A

PCR only diagnosis test (Hb electrophoresis which will show variant haemoglobins will not diagnose it)

120
Q

Thalassaemia differential diagnosis

A

Other types of inherited / Fe deficiency anaemia

121
Q

Thalassaemia management

A

Blood transfusions: most will need to have regular blood transfusions to treat anaemia
Chelation therapy: to remove excess iron (desferrioxamine)
Stem cell or bone marrow transplants: the only cute, but rarely done because of risks eg graft vs host
Splenectomy:
- many patients require splenectomy to decrease blood consumption and transfusion requirement with the ultimate goal of reducing iron overload
- it is only done in very severe cases
- vaccinations given as removal of spleen makes you much more at risk of developing severe infections

122
Q

Thalassaemia complications

A

Iron overload due to transfusion therapy is a big problem. Iron deposits in the heart, liver, pituitary, pancreas etc. causing hypothyroidism, hypocalcaemia, and hypogonadism.
Can be mitigated by iron-chelators.
Splenectomy if hypersplenism persists with increasing transfusion requirements - this is best avoided until > 5yrs old due to risk of infections

Abnormal bone growth, osteoporosis etc
Extra medullary hematopoiesis

123
Q

Polycythaemia vera definition

A

A type of philidelphia chromosome negative myeloproliferative neoplams. Characterised by erythrocytosis and often thrombocytosis, leukocytosis and splenomegaly

124
Q

Polycythaemia vera epidemiology

A

PV is rare - approx. 2 cases/100,000 per year

125
Q

Polycythaemia vera aetiology

A

Primary: Polycythaemia rubra vera - ‘overactive bone marrow’
- genetic mutation

Need more RBCs - body compensates by making too much, initiated by ‘secondary causes’

Secondary causes

  • lung disease
  • alcohol
  • ‘apparent erythrocytosis’ - not enough liquid in blood (don’t actually have too many RBCs)
  • EPO producing tumours
126
Q

Polycythaemia vera pathophysiology

A

> 95% have an activating mutation in JAK2 ‘JAK2V617F’, that means EPO signally is constantly ‘switched on’

127
Q

Polycythaemia vera key presentations

A

Elevated haematocrit and haemoglobin, may have splenomegaly. May present with thrombosis

128
Q

Polycythaemia vera signs and symptoms

A

Plethoric appearance (red face)
Erythromelalgia - tenderness or painful burning and/or redness of fingers, palms, heels or toes
Pruritus - classically after contact with warm water
Splenomegaly

129
Q

Polycythaemia vera 1st line investigations

A

Haemoglobin: high
Haematocrit: high (>52% in men, >48% in women)
WBC: usually high
Platelet count: usually high
MCV: usually low and coexisting with iron deficiency
JAK2 gene mutation screen

130
Q

Polycythaemia vera differential diagnosis

A

‘Apparent erythrocytosis’ - not enough liquid in blood (don’t actually have too many RBCs)

131
Q

Polycythaemia vera management

A

Venesection
Aspirin
Hydroxycarbamide - suppresses bone marrow
PLUS manage other CV risk factors
(ie blood pressure, cholesterol, diabetes)

132
Q

Polycythaemia vera complications

A

Arterial thrombosis (stroke, myocardial infarction)
Venous thrombosis (deep vein thrombosis, portal vein thrombosis, splanchnic vein thrombosis)
Haemorrhage
‘Transform’ to acute leukaemia, myelofibrosis

133
Q

Polycythaemia vera prognosis

A

Median 13 years

134
Q

Bleeding - immune thrombocytopenic purpura (ITP) definition

A

Immune disorder in which the blood doesn’t clot normally

135
Q

Bleeding - immune thrombocytopenic purpura epdemiology

A

ITP is more common among young women

Children

136
Q

Bleeding - immune thrombocytopenic purpura aetiology

A

May be triggered by infection with HIV, hepatitis or H. pylori

137
Q

Bleeding - immune thrombocytopenic purpura risk factors

A

Rheumatoid arthritis, lupus and antiphospholipid syndrome

Age <10 or >65

138
Q

Bleeding - immune thrombocytopenic purpura pthophysiology

A

Mostly due to antibody production against platelets. Sometimes T-cells directly attack platelets.
Children may develop ITP after a viral infection and usually recover fully without treatment. In adults, the disorder is often long term.

139
Q

Bleeding - immune thrombocytopenic purpura clinical manifestations

A 
Easy or excessive bruising 
Superficial bleeding into the skin that appears as pinpoint-sized reddish-purples sports (petechiae) that look like a rash, usually on the lower legs 
Bleeding from the gums or nose 
Blood in urine or stools 
Unusually heavy menstrual flow
140
Q

Bleeding - immune thrombocytopenic purpura investigations

A

FBC: low platelet count
- <100 x 10^9/L

Peripheral blood near

  • distinguish true and pseudothrombocytopenia
  • should be no evidence of myelodysplasia or other disorder
  • RBC/WBC = normal

Additional investigations

  • bone marrow biopsy = increased megakaryocytic
  • HIV serology = negative
  • helicobacter pylori breath test or stool antigen test = negative
  • hepatitis C serology = negative
  • thyroid function tests = maybe hyper/hypothyroid
141
Q

Bleeding - immune thrombocytopenic purpura management

A 
Regular monitoring and platelet checks 
If chronic will need treatment 
Combination therapy 
- corticosteroids 
- IV immunoglobulin 
- platelet transfusion

Medications

  • steroids
  • immune globulin
  • drugs that boost platelet production (romiplostim - Nplate; eltrombopag - Promacta)

Splenectomy

142
Q

Bleeding - immune thrombocytopenic purpura complications

A

Anaemia, tiredness, internal bleeding, infection

143
Q

Bleeding - immune thrombocytopenic purpura prognosis

A

Good in children - 80% remission

Increased mortality with age

144
Q

Von Willebrand disease definition

A

Congenital lifelong bleeding disorder in which your blood doesn’t clot well due to low levels of VWF

145
Q

Von Willebrand disease aetiology

A

Autosomal dominant/recessive genetic mutation passed from parents
Autosomal recessive is more severe

146
Q

Von Willebrand disease pathophysiology

A 
There are 3 types 
Type 1: 
- mildest/most common type 
- reduced level of VWF in blood 
- bleeding is only a problem with surgery, injury

Type 2:

  • VWF does not work properly
  • bleeding = more frequent and heavier than in type 1

Type 3:

  • most severe/rarest
  • very low levels of VWF or none at all
  • bleeding from mouth, nose and gut is common, and you can have joint and muscle bleeds after an injury
147
Q

Von Willebrand disease clinical manifestations

A

Excessive bleeding from an injury or after surgery or dental work
Nosebless that don’t stop within 10 mins
Heavy or long menstrual bleeding
Blood in your urine or stool
Easy bruising or lumpy bruises

148
Q

Von Willebrand disease investigations

A

FBC - see which proteins are present in blood
Pt test - test clotting time, factors 1,2,5,7,10
APTT - test clotting time, factors 8,9,11,12
Fibrinogen (CF1) test - ability to form clot

149
Q

Von Willebrand disease diagnostic tests

A

Factor VIII clotting activity
- measure amount of factor VIII in the blood

Von Willebrand factor antigen
- measure the amount of VWF in blood

Ristocetin cofactor or other VWF activity
- to measure how well the VWF works

Von Willebrand factor multimers - to measure th makeup or structure of the VWF

Platelet aggregation tests - to measure how well the platelets are working

150
Q

Von Willebrand disease differential diagnosis

A

Hemophilia A, Hemophilia B, Bernard-Soulier syndrome

151
Q

Von Willebrand disease management

A

Can;t be cured instead managed
Medication to help stop bleeds
- desmopressin - available as a nasal spray or injection
- tranexamic acid - available as tablets, a mouthwash or an injection
- von willebrand factor concentrate - available as an injection
Treating heavy periods (oral contraceptive pill, IUS)

152
Q

Von Willebrand disease complications

A
  • anaemia

- swelling and pain

153
Q

Thrombotic thrombocytopenic purpura definition

A

Blood disorder in which platelet clumps form in small blood vessels. This leads to a low platelet count (thrombocytopenia)

154
Q

Thrombotic thrombocytopenic purpura epidemiology

A

TTP most often occurs after 40 years of age, but congenital forms can occur in children

155
Q

Thrombotic thrombocytopenic purpura aetiology

A

30-50 yos
Black
Female

156
Q

Thrombotic thrombocytopenic purpura risk factors

A

Obesity

Pregnancy

157
Q

Thrombotic thrombocytopenic purpura clinical manifestations

A
  • non-specific prodrome
  • severe neurological symptoms (coma, focal abnormalities, seizures)
  • mild neurological symptoms (headache, confusion)
  • fever
  • digestive symptoms (nausea, vomiting, diarrhoea, abdo pain)
  • weakness
  • bleeding symptoms (purport, ecchymosis, menorrhagia)
158
Q

Thrombotic thrombocytopenic purpura investigations

A
  • platelet count = decreased
  • haemoglobin = <8 g/L
  • haptoglobin = decreased
  • peripheral smear = Schistocytes might be absent from the blood film
159
Q

Thrombotic thrombocytopenic purpura management

A

ACUTE
Acquired (idiopathic) TTP: acute episode

ONGOING
Acquired (idiopathic) TTP: following resolution of acute episode

160
Q

Haemophilia deinfiiton

A

Inherited bleeing disorder, characterised by deficiency of coagulation factor VIII or XI

161
Q

Haemophilia definition

A

Inherited bleeding disorder, characterised by deficiency of coagulation factor VIII or XI

162
Q

Haemophilia epidemiology

A

Almost exclusively in males due to an X-linked pattern of inheritance.
Family history
>60yrs

163
Q

Haemophilia risk factors

A

Autoimmune disorders, inflammatory bowel disease, diabetes, hepatitis, pregnancy, postnatal, or malignancy (acquired haemophilia)

164
Q

Haemophilia pathophysiology

A

Graded as mild, moderate, or severe, based on factor VIII or IX level

165
Q

Haemophilia clinical manifestations

A

Musculoskeletal bleeding is the msot common type of haemorrhage - bleeding into muscles

  • prolonged bleeding following heel prick or cricumcision
  • excessive bruising/haematoma
  • fatigue
  • menorrhagia and bleeding following surgical procedures or childbirth (female carriers)
  • extensive cutaneous purpura (acquired haemophilia)
166
Q

Haemophilia investigations

A

Activated partial thromboplastin time (aPTT)
- usually prolonged

Plasma factor VIII and IX assay
- decreased or absent factor VIII or IX levels;

Mixing study
- aPTT corrected

FBC = usually normal 
PT = normal

Head or neck CT - may show acute bleeding
Head or neck MRI - may show acute bleeding
Abdo ultrasound or abdominopelvic CT scan
- may demonstrate acute intra-abdnominal bleeding

Oseophagogastroduodenoscopy or colonoscopy
- may demonstrate acute gastrointestinal bleeding

167
Q

Haemophilia management

A

Preventitive
Haemophilia A
- regular injection of medicine octocog alfa (Advate) - engineered CF8 (causes rash)

Haemophilia B
- regular injections nonacog alfa (BeneFix) - engineered CF9. (causes headaches, nausea, altered taste)

On demand 
HA = injections of 
- Octocog alfa 
- Desmopressin - stimulates CF8 (causes headaches/nausea) 
HB = injections nonacog alfa
168
Q

Haemophilia complications

A

COmplication of treatment is the development of inhibitory antibodies against infused factor VIII or IX
- joint problems

169
Q

Haemophilia prognosis

A

People haemophilia lead a near normal life expectancy

170
Q

Haemolytic Uraemic Syndrome (HUS) definition

A

Characterised by microangiopathic haemolytic anaemia, thrombocytopenia and acute kidney injury. Around 90% occur in the paediatric population due to shiga toxin -producing E.coli

171
Q

Haemolytic Uraemic Syndrome pathophysiology

A

Common feature is endothelial injury leading to microvascular thrombosis and resulting microangiopathic haemolytic anaemia, thrombocytopenia and AKI

172
Q

Haemolytic Uraemic Syndrome key presentations

A

Diarrhoea, especially bloody diarrhoea in children under the age of 5

173
Q

Haemolytic Uraemic Syndrome 1st line investigations

A

FBC: anaemia, thrombocytopenia
Blood smear: presence of schistocytes
Renal function: raised creatinine, electrolyte abnormalities

Phase 2 Med (9 decks)

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