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Haematology Flashcards

1
Q

IRON DEFICIENCY ANAEMIA

What is the physiology of iron?

A
  • Bone marrow requires iron to produce Hb which is mainly absorbed in the duodenum + proximal jejunum enhanced by vitamin C + inhibited by tannin (tea)
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2
Q

IRON DEFICIENCY ANAEMIA

What are some causes of iron deficiency anaemia?

A
  • Increased loss = menorrhagia, GI bleeding (?colorectal cancer), hookworm
  • Inadequate intake/increased needs = poor diet, puberty growth, pregnancy
  • Malabsorption = IBD, coeliac disease
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3
Q

IRON DEFICIENCY ANAEMIA

What is the clinical presentation of iron deficiency anaemia?

A
  • Symptoms = fatigue, SOB, headaches, dizziness, palpitations
  • Generic signs = (conjunctival) pallor, tachycardia + tachypnoea
  • Pica = consumption of non-food materials
  • Koilonychia, angular stomatitis, atrophic glossitis + brittle hair/nails
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4
Q

IRON DEFICIENCY ANAEMIA

What investigations would you do in iron deficiency anaemia?

A
  • FBC = low Hb, low MCV + normal reticulocytes
  • Blood film = hypochromic microcytic RBCs, target cells + pencil poikilocytes
  • Iron studies
  • ?2ww suspected colorectal cancer for colonoscopy if ≥60
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5
Q

IRON DEFICIENCY ANAEMIA

What would iron studies show in iron deficiency anaemia?

A
  • Low = ferritin, iron + transferrin saturation (proportion of transferrin bound to iron)
  • High = total iron binding capacity (total space on transferrin for iron to bind)
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6
Q

IRON DEFICIENCY ANAEMIA
What is the management of iron deficiency anaemia?
Side effects of the treatment?

A
  • Diet = red meat, oily fish, green veg (broccoli, spinach), dried fruit (raisins)
  • PO iron supplementation (ferrous sulfate/fumarate)
  • Note SE: constipation, black coloured stools, nausea + abdo pain
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7
Q

THALASSAEMIA
What is thalassaemia?
What is the epidemiology?

A
  • Autosomal recessive disorders caused by ≥1 gene defect, resulting in a reduced rate of production of ≥1 alpha/beta globin chains making RBCs more fragile
  • Common in Mediterranean, Middle East + East Africa
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8
Q

THALASSAEMIA

How is alpha thalassaemia classified?

A
  • 1–2 alpha globin alleles affected = hypochromic + microcytic but Hb often ok
  • 3 = hypochromic microcytic anaemia with splenomegaly (Hb H disease)
  • 4 = death in utero due to foetal hydrops
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9
Q

THALASSAEMIA

How is beta thalassaemia classified?

A
  • Minor = 1 abnormal and 1 normal gene
  • Intermedia = 2 defective or 1 defective + 1 deletion
  • Major = homozygous for deletion genes
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10
Q

THALASSAEMIA
What is the clinical presentation of beta thalassaemia…

i) minor?
ii) intermedia?

A

i) Mild microcytic anaemia which only needs monitoring

ii) Jaundice + hepatosplenomegaly, monitoring ± blood transfusions

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11
Q

THALASSAEMIA

What is the clinical presentation of beta thalassaemia major?

A
  • Presents in first year with failure to thrive, hepatosplenomegaly, jaundice, microcytic anaemia
  • Extramedullary haemopoiesis = hepatosplenomegaly, bone marrow expansion (maxillary overgrowth + frontal bossing)
  • HbA2 + HbF raised but HbA absent
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12
Q

THALASSAEMIA
What investigations would you do in thalassaemia?
How do you differentiate from iron deficiency anaemia?
What investigation is diagnostic?

A
  • FBC = low Hb + MCV
  • Film = hypochromic + microcytic RBCs
  • Serum ferritin NORMAL
  • Hb electrophoresis = diagnostic
  • DNA testing via CVS
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13
Q

THALASSAEMIA
What is the management of thalassaemia?
What is a complication of this and how is it managed?

A
  • Blood transfusions (regular in beta thalassaemia major)
  • Require iron chelation with desferrioxamine to avoid overload + organ failure (cardiomyopathy, cirrhosis, DM, arthritis)
  • Splenectomy ± curative bone marrow transplant
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14
Q

B12 DEFICIENCY ANAEMIA

What is the physiology of vitamin B12?

A
  • Absorption occurs in the terminal ileum + is intrinsic factor (secreted by gastric parietal cells) dependent for transport across the intestinal mucosa
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15
Q

B12 DEFICIENCY ANAEMIA

What are some causes of B12 deficiency anaemia?

A
  • Malabsorption = Crohn’s, coeliac, ileal resection
  • Dietary (vegans) as B12 high in fish, meat, dairy
  • Autoimmune (pernicious) = atrophic gastritis leads to destruction of parietal cells > intrinsic factor deficient
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16
Q

B12 DEFICIENCY ANAEMIA

What is the clinical presentation of B12 deficiency anaemia?

A
  • Anaemia Sx = fatigue, SOB, headaches, dizziness, palpitations
  • B12 deficiency = dorsal column affected (proprioception, loss of vibration, peripheral neuropathy paraesthesia, neuropsych mood disturbance), glossitis (beefy-red sore tongue)
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17
Q

B12 DEFICIENCY ANAEMIA

What investigations would you do in B12 deficiency anaemia?

A
  • FBC = low Hb, high MCV (macrocytic, megaloblastic)
  • Haematinics = low B12
  • Blood film = hypersegmented neutrophil nuclei
  • Intrinsic factor Ab specific for pernicious, may have gastric parietal cell Ab
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18
Q

B12 DEFICIENCY ANAEMIA

What are some differentials of macrocytic anaemia which are normoblastic?

A
  • Alcohol
  • Liver disease
  • Hypothyroidism
  • Myelodysplasia
  • Reticulocytosis
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19
Q

B12 DEFICIENCY ANAEMIA
What should you check before managing B12 deficiency anaemia?
What is the management of B12 deficiency anaemia?

A
  • Folate > do not give folic acid as can cause subacute combined degeneration of the cord = distal sensory loss, ataxia + mixed U/LMN signs
  • Initially IM hydroxocobalamin then maintenance with PO cyanocobalamin if diet-related or IM hydroxocobalamin every 2–3m
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20
Q

FOLATE DEFICIENCY ANAEMIA

What is the pathophysiology of folate deficiency anaemia?

A
  • Develops over 4m of deficiency due to bodily reserves

- Folate is absorbed in the proximal jejunum

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21
Q

FOLATE DEFICIENCY ANAEMIA

What are some causes of folate deficiency anaemia?

A
  • Increased demand = pregnancy, malignancy, haemolysis
  • Decreased intake = poor diet (green vegetables)
  • Decreased absorption = coeliac, Crohn’s, jejunal resection
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22
Q

FOLATE DEFICIENCY ANAEMIA

What is the clinical presentation of folate deficiency anaemia?

A
  • Anaemia = fatigue, SOB, headaches, dizziness, palpitations

- NO neuropathy

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23
Q

FOLATE DEFICIENCY ANAEMIA
What investigations would you do in folate deficiency anaemia?
What’s the management?

A
  • FBC = low Hb, high MCV, macrocytic megaloblastic
  • Haematinics = low folate
  • PO folic acid 5mg OD
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24
Q

HEREDITARY SPHEROCYTOSIS

What is the pathophysiology of hereditary spherocytosis?

A
  • Autosomal dominant mutation of structural RBC membrane proteins leading to removal of abnormal membrane as it passes by spleen resulting in reduced SA:V + becoming spheroidal leading to extravascular haemolysis + destroyed by spleen
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25
HEREDITARY SPHEROCYTOSIS | What is the epidemiology and clinical presentation of hereditary spherocytosis?
- Most common inherited haemolytic anaemia in Northern Europeans - Anaemia, jaundice, splenomegaly + gallstones (due to increased bilirubin excretion)
26
HEREDITARY SPHEROCYTOSIS | What are the investigations for hereditary spherocytosis?
- FBC = raised MCHC, raised reticulocytes - Film = spherocytes (no central pallor) + schistocytes (haemolysis) - EMA binding test = diagnostic
27
HEREDITARY SPHEROCYTOSIS What complication can occur in hereditary spherocytosis and why? How does it present? What is the management?
- Aplastic crisis due to parvovirus B19 infection - Increased anaemia, extravascular haemolysis + jaundice without normal bone marrow (reticulocyte) response to create new RBCs - Supportive, ?transfusion
28
HEREDITARY SPHEROCYTOSIS | What is the management of hereditary spherocytosis?
- PO folate replacement | - Splenectomy before 5y is curative > Hib, Men A + C, annual influenza, PCV 5 yearly, PO pen V for at least 2y
29
G6PD DEFICIENCY | What is G6PD deficiency?
- X-linked recessive haemolytic anaemia so predominantly affects males, more common in Mediterranean, Middle Eastern + African background
30
G6PD DEFICIENCY | What is the clinical presentation of G6PD deficiency?
- Neonatal jaundice often within 3d | - Anaemia, intermittent jaundice, splenomegaly + gallstones
31
G6PD DEFICIENCY What investigations would you do in G6PD deficiency? What is diagnostic? What considerations would you take?
- Blood film = Heinz bodies + bite & blister cells - Diagnostic = G6PD enzyme assay - 3m after acute episode of haemolysis as RBCs with most severely reduced G6PD activity will have haemolysed so false negative results
32
G6PD DEFICIENCY What is a complication of G6PD deficiency? What can trigger it? How does it present?
- Acute (intravascular) haemolysis - Infection, fava bean, henna + drugs (antimalarials primaquine, sulfa-drugs, ciprofloxacin) - Fever, malaise, dark urine (Hb + urobilinogen)
33
G6PD DEFICIENCY | What is the management of G6PD deficiency?
- Avoid precipitants | - ?Transfusion if needed
34
SICKLE CELL DISEASE | What is the pathophysiology of sickle cell disease?
- Autosomal recessive condition leads to amino acid substitution of glutamine > valine causing abnormal HbS variant - This polymerises when deoxygenated into sickle shape making them more fragile (haemolysis) + prone to getting trapped in microvasculature (thrombosis) leading to ischaemia/infarction
35
SICKLE CELL DISEASE What are the different phenotypes in sickle cell disease? What is the epidemiology and why is this significant?
- Heterozygous = trait, homozygous = disease - More common in African descent in areas traditionally affected by malaria as having sickle-cell trait reduces severity of falciparum malaria making them more likely to survive + pass the gene on
36
SICKLE CELL DISEASE | What is the general clinical presentation of sickle cell disease?
- HbF unaffected so usually manifests when HbF decreases around 6m - All have moderate anaemia with detectable jaundice from chronic haemolysis
37
SICKLE CELL DISEASE | What are the various acute presentations of sickle cell disease?
- Vaso-occlusive painful crises - Sequestration crises - Aplastic crises - Acute chest syndrome
38
SICKLE CELL DISEASE What is the clinical presentation of... i) sequestration crises? ii) aplastic crises?
i) Pooling of blood in spleen leads to severe anaemia + shock, if >2x = splenectomy ii) Parvovirus B19 = acute sudden anaemia
39
SICKLE CELL DISEASE What can cause vaso-occlusive painful crises? How do they present?
- Triggers = cold, infection, dehydration - Affects bones of limbs + spine, can lead to avascular necrosis - Hand-foot syndrome = dactylitis
40
SICKLE CELL DISEASE What is the clinical presentation of acute chest syndrome? What are some potential causes?
- Pleuritic chest pain, fever, SOB, CXR = pulmonary infiltrates - Infective or non-infective (pulmonary vaso-occlusion, fat emboli)
41
SICKLE CELL DISEASE What are the investigations for sickle cell disease? What is the definitive diagnostic investigation?
- Prenatal Dx with CVS and detected on neonatal heel prick test at 5d - FBC = normocytic anaemia, high reticulocytes - Blood film = sickled RBCs, Howell-Jolly bodies + schistocytes - Definitive = Hb electrophoresis showing high HbSS + absent HbA
42
SICKLE CELL DISEASE | What are some long-term complications of sickle cell disease?
- Short stature + delayed puberty - Hyposplenism due to sequestration + infarction so increased infection risk - Stroke - Chronic renal failure
43
SICKLE CELL DISEASE | What is the management of acute crises in sickle cell disease?
- PO/IV analgesia titrate to effect, IV fluids, oxygen, ?NIV - Infection = Abx - Blood transfusion if severe anaemia - Exchange transfusion if severe (e.g., neuro complications)
44
SICKLE CELL DISEASE | What is the chronic management of sickle cell disease?
- PO hydroxyurea to increase HbF levels as prophylaxis for painful crises - PCV vaccine every 5y, may have prophylactic pen V - Bone marrow transplantation curative + offered if failed response
45
HAEMOLYTIC ANAEMIA | What is the pathophysiology of haemolytic anaemias?
- Extravascular = RBC destruction within reticuloendothelial system (spleen + liver) > sickle cell, hereditary spherocytosis - Intravascular = RBC destruction within blood stream so release of contents into circulation > G6PD, AIHA, paroxysmal nocturnal haemoglobinuria
46
HAEMOLYTIC ANAEMIA | How are the causes of haemolytic anaemias split?
Inherited – - Hereditary spherocytosis, G6PD, sickle cell disease Acquired – - Immune coombs +ve = autoimmune or alloimmune (transfusion reactions) - Non-immune = paroxysmal nocturnal haemoglobinuria, microangiopathic haemolysis
47
HAEMOLYTIC ANAEMIA What is the typical biochemical picture seen in haemolysis? How does this differ between intravascular and extravascular?
- Both = increased unconjugated bilirubin, LDH + reticulocytes - Intravascular = haemoglobinuria + decreased haptoglobin which binds to free Hb in blood (not seen in extravascular)
48
HAEMOLYTIC ANAEMIA | What are the 2 types of autoimmune haemolytic anaemia?
- Warm = IgG mediated extravascular haemolysis where spleen tags cells for splenic phagocytosis occurring at body temp - Cold = IgM mediated where IgM fixes complement causing direct intravascular haemolysis (cold agglutinins) occurring in cold <4 degrees
49
HAEMOLYTIC ANAEMIA | What are the causes of warm and cold autoimmune haemolytic anaemia?
- Warm = idiopathic, lymphoproliferative neoplasms (CLL, lymphoma), SLE - Cold = idiopathic, mycoplasma, EBV
50
HAEMOLYTIC ANAEMIA | What is the clinical presentation of warm and cold autoimmune haemolytic anaemia?
- Warm = jaundice, splenomegaly | - Cold = acrocyanosis, Raynaud's
51
HAEMOLYTIC ANAEMIA | What is the management of warm and cold autoimmune haemolytic anaemia?
- Warm = steroids, rituximab or splenectomy | - Cold = keep warm (?blood warmers)
52
HAEMOLYTIC ANAEMIA What are the features of paroxysmal nocturnal haemoglobinuria? What is the management?
- Early adulthood nocturnal episodes of intravascular haemolysis > anaemia, dark urine (haemoglobinuria) in morning - Flow cytometry diagnostic, eculizumab
53
``` HAEMOLYTIC ANAEMIA What is microangiopathic haemolytic anaemia? What causes it? How does it present? What's the management? ```
- Mechanical destruction of RBCs - TTP, HUS + DIC - Fever, renal failure, abdo pain, schistocytes on blood film - Plasma exchange to remove vWF multimers
54
ALL What is acute lymphoblastic leukaemia (ALL)? What is the epidemiology? What are some risk factors?
- Abnormal proliferation of lymphoid progenitor cells (usually B-lymphocytes) - Accounts for 80% of leukaemia in children + peaks at 2–5y - Trisomy 21 + immunocompromised
55
ALL What is the general clinical presentation of ALL? What causes the various clinical presentations of ALL?
- Fever, weight loss, night sweats, lymphadenopathy | - Infiltration with leukaemic blast cells > bone marrow (pancytopaenia), reticuloendothelial, other organs
56
ALL How does ALL present in terms of... i) bone marrow infiltration? ii) reticuloendothelial infiltration? iii) other organ infiltration?
i) Anaemia (pallor, lethargy, SOB), neutropaenia (infection) + thrombocytopaenia (bruising, petechiae, epistaxis) ii) Hepatosplenomegaly iii) CNS (CN palsies), bone pain, testicular swelling
57
ALL What investigations would you do in ALL? What is diagnostic?
- FBC + blood film = pancytopaenia, blast cells - CXR if ?mediastinal mass, LP if ?CNS involvement - Bone marrow examination via ASPIRATION = diagnostic
58
ALL | What are some complications of ALL?
- CNS development - Growth impact - Infertility - Cardiac + renal toxicity
59
ALL What is the management of ALL? What are good prognostic factors in ALL?
- Supportive = IV fluids, transfusions, allopurinol to protect kidneys from tumour lysis syndrome - Chemo = remission, consolidation & CNS protection + maintenance - Ages 2–10, female, WCC <20, no CNS disease, Caucasian
60
AML | What is acute myeloid leukaemia (AML)?
- Neoplastic proliferations of myeloid precursor cells | - Commonest acute leukaemia in adults, over 75y affected
61
AML | What are some causes of AML?
- Myelodysplastic + myeloproliferative syndromes | - Radiation + chemotherapy
62
AML | What is the clinical presentation of AML?
- Bone marrow failure (pancytopaenia) = anaemia, neutropaenia + thrombocytopaenia - Infiltration = lymphadenopathy, hepatosplenomegaly + gum hypertrophy
63
AML What are the investigations for AML? What is diagnostic?
- FBC = pancytopaenia, may present in DIC - Blood film = blast cells + auer rods - Bone marrow examination via ASPIRATION diagnostic
64
AML | What is the management of AML?
- Supportive = IV fluids, transfusions, allopurinol | - Chemotherapy or bone marrow transplant but generally poor prognosis
65
CLL What is CLL? What is the epidemiology?
- Monoclonal proliferation of functionally incompetent malignant B cells - Most common in those >60y
66
CLL | What is the clinical presentation of CLL?
- Often none but incidental lymphocytosis on FBC | - Anaemia, hepatosplenomegaly + B symptoms (weight loss, fever, night sweats)
67
CLL | What are the investigations for CLL?
- FBC = anaemia + lymphocytosis - Blood film = smudge/smear cells - Immunophenotyping (flow cytometry) diagnostic
68
CLL | What are the complications of CLL?
- Richter transformation to high grade lymphoma = CLL > large B cell lymphoma - Hypogammaglobulinaemia - Warm autoimmune haemolytic anaemia
69
CLL | What is the management of CLL?
- Chemotherapy
70
CML What is chronic myeloid leukaemia (CML)? What are the phases? What is the epidemiology?
- Uncontrolled clonal proliferation of myeloid cells - 3 phases = chronic (5y asymptomatic), accelerated + blast phase - Ages >65
71
CML | What is the clinical presentation of CML?
- Anaemia + thrombocytopaenia - Systemic = fever, weight loss + night sweats - Massive splenomegaly which can cause abdominal pain
72
CML | What are the investigations in CML?
- FBC = anaemia, thrombocytopaenia + leukocytosis of neutrophils, monocytes, basophils + eosinophils - Philadelphia chromosome t(9:22) in almost all leading to BCR-ABL gene coding for fusion protein with excess tyrosine kinase activity - Bone marrow aspiration + biopsy = diagnostic
73
CML | What is the management of CML?
- Imatinib = tyrosine kinase inhibitor
74
LYMPHOMA What is Hodgkin's lymphoma? What is the epidemiology? What are some risk factors?
- Proliferation of lymphocytes - Bimodal age distribution with peaks around 20 + 75 years - HIV, EBV, autoimmune conditions like RA, sarcoidosis
75
LYMPHOMA | What is the clinical presentation of Hodgkin's lymphoma?
- Non-tender, rubbery lymphadenopathy at neck with alcohol-induced pain - Pruritus - Compression Sx = SOB, abdominal pain - B symptoms (night sweats, fever, weight loss) imply poorer prognosis
76
LYMPHOMA What are some investigations for lymphoma? What is diagnostic? What is the characteristic finding of Hodgkin's lymphoma?
- Bloods = normocytic anaemia, LDH raised - CT CAP/PET for staging - Lymph node biopsy = diagnostic for Reed-Sternberg cells (mirror-image nuclei, often multinucleate malignant lymphocytes)
77
LYMPHOMA What staging system is used in both lymphomas? How is it categorised?
Ann Arbor – - I = confined to single LN region - II = ≥2 nodal areas on same side of diaphragm - III = nodal areas on both sides of diaphragm - IV = spread beyond LN - A = no systemic Sx, B = systemic B symptoms
78
LYMPHOMA | What is the management of Hodgkin's lymphoma?
- Chemotherapy ± radiotherapy
79
LYMPHOMA What is non-Hodgkin's lymphoma? Give some key types
- All lymphomas without Reed-Sternberg cells - Diffuse large B cell lymphoma most common = rapidly growing mass in >65y + hepatitis C - Burkitt = associated with EBV, malaria + HIV - Mucosa-associated lymphoid tissue (MALT) = associated with H. pylori
80
LYMPHOMA | What is the clinical presentation of non-Hodgkin lymphoma?
- Non-tender, firm lymphadenopathy often symmetrical at multiple sites - B symptoms (poor prognostic marker)
81
LYMPHOMA What are the investigations for non-Hodgkin's lymphoma? What is the management of non-Hodgkin's lymphoma?
- Bloods = normocytic anaemia, elevated LDH - LN biopsy diagnostic + Ann Arbor staging with CT CAP/PET - Chemotherapy
82
TUMOUR LYSIS SYNDROME What is tumour lysis syndrome? What is it related to?
- Breakdown of tumour cells + subsequent release of chemicals from the cell - Treatment of high-grade lymphomas + leukaemias
83
TUMOUR LYSIS SYNDROME | What is the clinical presentation of tumour lysis syndrome?
- Often 2 days post chemo with dysuria/oliguria, abdo pain + weakness
84
TUMOUR LYSIS SYNDROME What investigations would you do for tumour lysis syndrome? What laboratory findings would you expect?
- U&Es + serum uric acid - AKI - High K+, phosphate, uric acid (25% increase) - Low calcium (25% decrease)
85
TUMOUR LYSIS SYNDROME | How do you diagnose tumour lysis syndrome?
- Laboratory confirmation | - PLUS 1 of increased serum creatinine (1.5x upper limit of normal), cardiac arrhythmias/sudden death, seizure
86
TUMOUR LYSIS SYNDROME | What is the management of tumour lysis syndrome?
- High risk = IV allopurinol OR rasburicase immediately prior to + during first days of chemo for prophylaxis - Low risk = PO allopurinol during chemo cycles to prevent
87
TUMOUR LYSIS SYNDROME | What is the mechanism of action of rasburicase?
- Recombinant urate oxidase an enzyme that metabolises uric acid to more water soluble allantoin so excreted more easily via kidneys
88
MYELOMA What is the pathophysiology of myeloma? What are the risk factors?
- B cell malignancy causing clonal proliferation of plasma cells > increased monoclonal Igs (paraprotein), mostly IgG = hypogammaglobulinaemia - Older age, male, Black African ethnicity
89
MYELOMA | What condition may be seen before developing myeloma?
- Monoclonal gammopathy of undetermined significance (MGUS) = excess of a single type of antibody (components) without other features of myeloma
90
MYELOMA | What is the clinical presentation of myeloma?
OLD CRAB – - Old age (peak incidence 60–70) - Calcium high - Renal failure (light chain deposition within renal tubules) - Anaemia (classically pancytopaenia) - Bone lesions = pathological #, pain
91
MYELOMA What are some investigations of myeloma? What is diagnostic
- Bloods = FBC (normocytic anaemia), U&Es, Ca2+ & ESR high, normal ALP + phosphate - Blood film = rouleaux formation (RBCs stacked) - Monoclonal proteins in serum/urine electrophoresis = Bence Jones protein - Imaging for bone lesions (whole body MRI/CT or skeletal XR survey) - Bone marrow biopsy = diagnostic
92
MYELOMA | What are some complications of myeloma?
- Renal failure - Hypercalcaemia - Peripheral neuropathy - Spinal cord compression
93
MYELOMA | What is the management of myeloma?
- Bone pain = bisphosphonates or radiotherapy for localised pain - Influenza + PCV vaccination for infection prevention - Chemo - Stem cell transplant
94
NEUTROPENIC SEPSIS What is neutropenic sepsis? Why does it commonly occur?
- Neutrophils <0.5x10^9/L in a patient on anticancer treatment with 1 of: temp >38 or other signs/symptoms of clinical sepsis - Complication of cancer therapy often chemo, often occurring 7–14d after
95
NEUTROPENIC SEPSIS | What are the investigations for neutropenic sepsis?
- Blood gas - Blood cultures - FBC, CRP, U&E, LFT, clotting - Urine MC&S - CXR
96
NEUTROPENIC SEPSIS | What is the management of neutropenic sepsis?
- SEPSIS 6 = BUFALO - Piperacillin + tazobactam (Tazocin) within 1h (do NOT wait for FBC) - If febrile + unwell after 48h meropenem ± vancomycin
97
NEUTROPENIC SEPSIS | What is the prophylaxis of neutropenic sepsis?
- If anticipated to have neutrophils of <0.5 due to their treatment then offer fluoroquinolone
98
HAEMOPHILIA What is haemophilia? What are the 2 types?
- X-linked recessive deficiency of one of the clotting factors (more common in males) - Haemophilia A (more common) = factor VIII, haemophilia B = factor IX
99
HAEMOPHILIA | What is the clinical presentation of haemophilia?
- Large bleeds into joints (haemoarthrosis) or muscles (haematoma) - Prolonged bleeding after surgery/trauma - Neonates = intracranial haemorrhage + cord bleeding
100
HAEMOPHILIA | What are the investigations for haemophilia?
- PT (F2/5/7/10, extrinsic) = normal - APTT (intrinsic) = greatly elevated - Diagnosis with factor VIII/IX assay - Prenatal Dx with CVS DNA testing
101
HAEMOPHILIA | What is the management of haemophilia?
- Avoid NSAIDs + IM injections - Minor bleeds = desmopressin which stimulates vWF release - Major bleeds = IV infusion of recombinant FVIII/FIX concentrate, TXA
102
HAEMOPHILIA | What prophylactic treatment can you give in haemophilia but what is a potential risk of this?
- Recombinant factor VIII/IX concentrate to reduce risk of arthropathy - Risk of antibodies forming against the factor + making treatment ineffective
103
VON WILLEBRAND DISEASE | What is the role of von Willebrand factor?
- Facilitates platelet adhesion to damaged endothelium | - Acts as carrier protein for FVIII:C, protecting it from inactivation + clearance
104
VON WILLEBRAND DISEASE What is the pathophysiology of von Willebrand disease (vWD)? What causes it?
- Defective platelet plug formation + deficient FVIII:C > bleeding - Most common inherited bleeding disorder, mostly autosomal dominant
105
VON WILLEBRAND DISEASE What is the clinical presentation of vWD? How does this differ to haemophilia?
- Mucosal bleeding (epistaxis, menorrhagia, bleeding gums) + bruising - Spontaneous large soft tissue bleeding is uncommon
106
VON WILLEBRAND DISEASE | What are the investigations for vWD?
- Prolonged bleeding time as primary haemostasis affected | - PT normal, APTT elevated
107
VON WILLEBRAND DISEASE | What is the management of vWD?
- Avoid NSAIDs + IM injections - Desmopressin causes release of vWF + FVIII antigen - TXA for mild bleeding - Severe vWD may require plasma derived FVIII concentrate or vWF infusion
108
MYELODYSPLASIA | What is the pathophysiology of myelodysplastic syndromes?
- Hypercellular bone marrow with abnormal maturation of all three cell lineages - Decreased peripheral blood cells as cells can't get out > bone marrow failure
109
MYELODYSPLASIA What causes myelodysplastic syndromes? How do they present and what's a key complication?
- Secondary to radio or chemotherapy - Bone marrow failure (pancytopaenia) Sx - May progress to AML
110
MYELODYSPLASIA | What investigations would you do in myelodysplastic syndromes?
- FBC = pancytopaenia, decreased reticulocytes - Blood film = ring sideroblasts - Bone marrow aspiration + biopsy diagnostic = hypercellular bone marrow
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MYELODYSPLASIA | What is the management of myelodysplastic syndromes?
- Supportive = transfusions, EPO + G-CSF | - Stem cell transplant
112
MYELOPROLIFERATIVE What are the 3 myeloproliferative disorders to know? What are their similarities?
- Polycythaemia vera, essential thrombocytosis + myelofibrosis - All linked to JAK2 mutation which can be targeted by JAK2 inhibitors ruxolitinib - All have potential to progress + transform to AML
113
MYELOPROLIFERATIVE What is polycythaemia vera? What are some causes?
- Proliferation of erythroid cell line = RBCs | - Primary or secondary to hypoxia, increased EPO
114
MYELOPROLIFERATIVE | What is the clinical presentation of polycythaemia vera?
- Hyperviscosity = thrombotic complications (DVT, PE, TIA) - Plethoric complexion - Splenomegaly + pruritus (esp. after hot bath)
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MYELOPROLIFERATIVE | What investigations would you do in polycythaemia vera?
- Bloods = raised Hb, haematocrit + ALP - 95% JAK2 +ve - Bone marrow = hypercellular
116
MYELOPROLIFERATIVE | What is the management of polycythaemia vera?
- First-line = venesection to aim for haematocrit <0.45 - Aspirin 75mg OD to reduce risk of thrombotic events - Hydroxyurea as chemo
117
MYELOPROLIFERATIVE What is essential thrombocytosis? How does it present?
- Proliferation of the megakaryocyte cell line > platelets - Both thrombosis + bleeding - Platelet count >600
118
MYELOPROLIFERATIVE | What is the management of essential thrombocytosis?
- Aspirin 75mg OD to reduce risk of thrombotic events | - Hydroxyurea to reduce platelet count
119
MYELOPROLIFERATIVE | What is myelofibrosis?
- Megakaryocyte hyperplasia producing increased platelet derived growth factor = intense bone marrow fibrosis
120
MYELOPROLIFERATIVE | What is the clinical presentation of myelofibrosis?
- Most commonly elderly presenting with anaemia (fatigue) - Systemic = fever, weight loss + night sweats - Massive splenomegaly
121
MYELOPROLIFERATIVE | What are the investigations in myelofibrosis?
- Bloods = anaemia, high urate + LDH due to increased cell turnover - Blood film = teardrop poikilocytes - Bone marrow aspirate difficult resulting in 'dry tap' so biopsy needed
122
MYELOPROLIFERATIVE | What is the management of myelofibrosis?
- Stem cell transplantation - Hydroxyurea + folic acid - ?Splenectomy
123
DIC What is disseminated intravascular coagulation (DIC)? What causes it?
- Dysregulated coagulation + fibrinolysis leading to widespread clotting with resultant bleeding - Sepsis, trauma, malignancy, obstetrics (HELLP syndrome)
124
DIC | What is the clinical presentation of DIC?
- Bleeding from unrelated sites (epistaxis, cannula, haematuria) - Petechiae/purpura, confusion + shock
125
DIC | What are the classic investigation findings in DIC?
FBC + clotting crucial – - Raised = PT, APTT, bleeding time, fibrinogen degradation products - Decreased = platelets, fibrinogen Schistocytes may be seen due to microangiopathic haemolytic anaemia
126
DIC | What is the management of DIC?
- Treat underlying | - Supportive = FFP + platelets
127
ITP | What is the pathophysiology of immune thrombocytopaenic purpura (ITP)?
- Type 2 hypersensitivity reaction with platelet destruction by anti-platelet IgGs
128
ITP | What is the clinical presentation of ITP in paeds/adults and in general?
- Paeds = acute 1-2w post-viral infection or vaccine - Adults = more chronic condition, commoner in older females - Petechiae/purpuric rash, bruising, bleeding (less common)
129
ITP | What investigations would you do for ITP?
- FBC = isolated thrombocytopaenia - Blood film - Bone marrow examination only required if atypical features
130
ITP | What is the management of ITP in paeds/adults + in both?
- Paeds = first-line often no treatment - Adults = first-line PO pred - IVIg or platelet transfusions may be required based on platelet count or bleeding
131
TTP | What is the pathophysiology of thrombotic thrombocytopaenic purpura (TTP)?
- Deficiency of ADAMTS13 protease which usually inactivates vWF to reduce platelet adhesion to vessel walls + clot formation so vWF overactivity + sticky multimers > clots in small vessels which haemolyses RBCs = microangiopathic haemolytic anaemia
132
TTP | What are some causes of TTP?
- Post-infection (urinary, GI), pregnancy + SLE | - Congenital absence of ADAMTS13 or autoimmune disease with Ab against it
133
TTP | What is the clinical presentation of TTP?
- Fever - Fluctuating neuro signs from microemboli - Renal failure - Abdominal pain (Overlap with HUS)
134
TTP | What is the investigations and management of TTP?
- Bloods = raised LDH, film = schistocytes from haemolysis | - Plasma exchange, steroids + rituximab (monoclonal Ab against CD20 B cells)
135
THROMBOPHILIA | What are some causes of thrombophilia?
Inherited - Factor V leiden = activated protein C resistance #1 cause - Protein C & S deficiency = usually inactivates factors V + VIII respectively - Antithrombin III deficiency = usually inactivates factors IIa, IXa, Xa + XIa Acquired - Antiphospholipid syndrome - COCP
136
THROMBOPHILIA What are some investigations you would do in thrombophilias? What is the management?
- FBC, clotting + fibrinogen levels - Assays = protein C & S, antithrombin - Factor V Leiden mutation analysis - Anticoagulated so INR 2-3 or 3-4 if recurrence on warfarin
137
TRANSFUSIONS What are the RBC transfusion thresholds? What are the Hb targets post-transfusion? How much does 1 unit raise Hb and how quickly do you transfuse it?
- 70g/L or 80g/L if ACS - 70–90g/L or 80–100g/L if ACS - 10g/L + if non-urgent transfuse over 90-120m
138
TRANSFUSIONS | What are the platelet transfusion thresholds in active bleeding?
- Clinically significant (haematemesis, melaena, prolonged epistaxis) + platelets <30 - Severe bleeding/critical site (CNS) + platelets <100
139
TRANSFUSIONS What are the platelet transfusion thresholds for... i) pre-invasive procedure prophylaxis? ii) no active bleeding or planned invasive procedure?
i) >100 if surgery at critical site, 50–75 if high bleeding risk or >50 for most ii) 10
140
TRANSFUSIONS What is an important risk of platelet transfusions? How quickly do you transfuse it? When would you not perform a platelet transfusion?
- Highest risk of bacterial contamination - 1 unit/30m - Chronic bone marrow failure, autoimmune thrombocytopaenia, HIT or TTP
141
TRANSFUSIONS What is present in FFP and the indications? What is present in cryoprecipitate and the indications? How quickly do you transfuse them both?
- All clotting factors > major bleeding, DIC, liver disease, TTP - Factors 8, vWF + fibrinogen > major bleeding, DIC - 1 unit/30m
142
TRANSFUSIONS | What are the 6 main acute and 2 late transfusion reactions that can occur?
Acute – - Minor allergic reaction - Anaphylaxis - Acute haemolytic transfusion reaction - Febrile non-haemolytic transfusion reaction - Transfusion-related acute lung injury (TRALI) - Transfusion-associated circulatory overload (TACO) Late – - Delayed haemolytic transfusion reaction - Transfusion-associated graft versus host disease
143
TRANSFUSIONS | What are the features and management of minor allergic reactions?
- Pruritus, urticaria | - Temporarily stop, antihistamine + monitor
144
TRANSFUSIONS | What are the risk factors, features and management of anaphylaxis?
- Increased risk in patients with IgA deficiency with anti-IgA Ab - Hypotension, SOB, wheeze, angioedema - Stop transfusion, usual Mx (ABC, IM adrenaline)
145
TRANSFUSIONS | What are the causes, features and management of acute haemolytic transfusion reaction?
- ABO-incompatibility due to RBC destruction by IgM Ab - Fever, hypotension minutes after, later DIC - Stop transfusion, fluid resus, send blood for direct Coombs
146
TRANSFUSIONS | What are the features and management of febrile non-haemolytic transfusion reactions?
- Fever, rigors but otherwise well | - Slow transfusion, antipyretic (paracetamol), monitor
147
TRANSFUSIONS | What are the features and management of TRALI?
- Hypoxia, fever, HYPOtension, CXR = pulmonary infiltrates | - Stop transfusion, oxygen + supportive care
148
TRANSFUSIONS | What are the features and management of TACO?
- Pulmonary oedema, raised JVP, HYPERtension | - Slow transfusion, ?furosemide
149
TRANSFUSIONS What are the features of... i) delayed haemolytic transfusion reaction? ii) transfusion-associated graft versus host disease?
i) Jaundice, anaemia + fever often 5d post-transfusion ii) Donor blood lymphocytes attack the recipient's body (irradiated blood products are depleted of T-lymphocytes to avoid this)
150
ANTICOAGULATION What is the mechanism of action of warfarin? What monitoring is required?
- Vitamin K antagonist so decreases vitamin K dependent factors 2, 7, 9 + 10 (1972) - INR (patient's PT ÷ normal PT)
151
ANTICOAGULATION What are some indications for warfarin? What are the target INRs?
- First line (+ aspirin) for mechanical heart valves (mitral usually higher INR than aortic) - 2nd line after DOACs for VTE + AF - Usually 2.5 but 3.5 if recurrent VTE
152
ANTICOAGULATION What are some side effects of warfarin? What can lower INR? What can increase INR?
- Haemorrhage, teratogenic (safe in breastfeeding) - P450 inducers = PC BRAS (Phenytoin, Carbamazepine, Barbiturates, Rifampicin, Alcohol chronic, sulfonylureas & vitamin K food (green leafy veg) - P450 inhibitors = AO DEVICES (allopurinol, omeprazole, disulfiram, erythromycin, valproate, isoniazid, ciprofloxacin, ethanol acute, sulfonamides
153
ANTICOAGULATION A patient on warfarin presents with an INR between 5.0–8.0 and... i) no bleeding ii) minor bleeding what is the management?
i) Withhold 1–2 doses, reduce subsequent maintenance dose | ii) Stop warfarin (restart when INR <5.0), IV vitamin K 1–3mg (phytomenadione)
154
ANTICOAGULATION A patient on warfarin presents with an INR >8 and... i) no bleeding ii) minor bleeding what is the management? What about any INR and major bleeding?
i) Stop warfarin (restart when INR <5.0), PO vitamin K 1–5mg (IV prep PO), repeat after 24h if required ii) Stop warfarin (restart when INR <5.0), IV vitamin K 1–3mg, repeat after 24h if required - IV vitamin K 5mg and prothrombin complex concentrate (Beriplex) or FFP
155
ANTICOAGULATION What is the mechanism of action of DOACs? What is the reversal agent? What would you use in renal impairment?
- Apixaban, rivaroxaban = direct Xa inhibitors reverse with andexanet alfa - Dabigatran = direct thrombin (IIa) inhibitor reverse with idarucizumab - Apixaban
156
ANTICOAGULATION What is the mechanism of action of heparin? How would you monitor it? What are some side effects?
- Activate antithrombin III - Unfractionated = APTT, LMWH = factor Xa - Bleeding, osteoporosis hyperkalaemia + HIT
157
ANTICOAGULATION What is heparin-induced thrombocytopaenia (HIT)? How is it managed?
- Low platelets BUT prothrombotic due to increased activation of platelets - Stop heparin, monitor platelets, reversal with protamine sulfate
158
BONE MARROW FAILURE What are some causes of bone marrow failure? What anaemia pattern is seen?
``` Pancytopaenia – - Inherited = Fanconi's anaemia - Acquired = aplastic anaemia, leukaemia Single cell cytopaenia – - Acquired = red cell aplasia - Normochromic, normocytic ```
159
BONE MARROW FAILURE What is Fanconi anaemia? How does it present? What is the management?
- AR condition with pancytopaenia leading to aplastic anaemia - Skin = café-au-lait spots, skeletal = absent thumbs, GU = horseshoe kidney - Bone marrow transplant as high mortality from failure or acute leukaemia
160
BONE MARROW FAILURE What is aplastic anaemia? What causes it?
- Hypocellularity of bone marrow + pancytopaenia | - Fanconi's anaemia, parvovirus B19
161
BONE MARROW FAILURE What investigations would you do in aplastic anaemia? What is the management?
- FBC, blood film, bone marrow biopsy diagnostic (hypocellular marrow) - Supportive (transfusions), immunosuppression, stem cell transplant
162
BONE MARROW FAILURE What is red cell aplasia? How does it present? How is it managed?
- Parvovirus B19 infection destroys erythroid precursor cells + erythropoiesis stops for 5–10d - Slapped cheek syndrome - PCR/serology, supportive or transfusion
163
ANAEMIA OF CHRONIC DISEASE What are some causes of anaemia of chronic disease? What would investigations show? What is the management?
- Autoimmune/connective tissue disease (SLE), inflammation (RA, Crohn's), cancer - Normocytic anaemia with reduced TIBC, high ferritin - Treat underlying + ?EPO
164
MALARIA | What is malaria?
- Disease caused by plasmodium protozoa which is transmitted by the female anopheles mosquito
165
MALARIA | What is the pathophysiology of malaria?
- Sporozoites in saliva > enter hepatocytes and mature > rupture to release merozoites into blood + enter RBCs > sporozoites a mosquito can pick up
166
MALARIA | What are the causes of malaria?
- Plasmodium falciparum = causes almost all severe malaria cases, #1 cause overall - Plasmodium vivax = #1 non-falciparum malaria, more benign - Plasmodium ovale - Plasmodium malariae
167
MALARIA | What are some protective factors from malaria?
- Sickle cell trait - G6PD deficiency - HLA-B53
168
MALARIA | What is the clinical presentation of malaria?
- Flu-like prodrome = headache, myalgia, malaise - Fever paroxysms (vivax/ovale is cyclical every 48h, malariae every 72h) - Anaemia + splenomegaly - Hx of foreign travel = Central America, Indian subcontinent, Africa
169
MALARIA | How do you investigate malaria?
- Thick + thin blood films with Giemsa stain | - FBC = anaemia, blood gas may show acidosis
170
MALARIA | What are some complications with malaria?
- Cerebral malaria = confusion, seizures - Hypoglycaemia - AKI - DIC
171
MALARIA | What is the management of non-falciparum malaria?
- Artemisinin-based combination therapy (ACT) or chloroquine (ACT if chloroquine-resistance or pregnancy) - Primaquine following acute treatment if vivax or ovale to prevent relapse
172
MALARIA | What is the management of falciparum malaria?
- Uncomplicated = ACT first line | - Severe = IV artesunate

FINALS (20 decks)

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