Haem Flashcards

1
Q

Acute myeloid leukaemia:

A

Presents with letheragy, recurrent infections, splenomegaly
Most common form of acute leukaemia in adults.
May occur as primary disease or following secondary transformation from a myeloproliferative disorder.
Features: Anaemia, neutropenia, thrombocytopaenia
Associated with Auer rods

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2
Q

Antiphospholipid syndrome: pregnancy management

A

Low dose aspirin and LMWH (discontinued at 34 weeks)

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3
Q

Aplastic anaemia:

A

When your body stops producing enough red blood cells.
Characterised by pancytopenia and hypoplastic bone marrow.
Features: normocytic anaemia, thrombocytopenia
Causes: idiopathic, congenital (Fanconi anaemia), infections (parvovirus, hepatitis), radiation

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4
Q

Autoimmune haemolytic anaemia:

A

Divided into warm and cold type according to what temperature the antibodies best cause haemolysis.
Investigations: general features of haemolytic anaemia i.e., anaemia, reticulocytotic, raised LDH.
Specific tests: positive Coomb’s test
Warm AIHA: causes haemolysis at body temperature
Cold AIHA: haemolysis at 4 degrees C.
Causes: EBV
Management: treat underlying disorder +steroids +/- rituximab (first-line)

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5
Q

Alpha Thalassaemia:

A

Caused by defects in alpha-globin chains.
Management:
* Blood transfusions
* Splenectomy may be performed
* Bone marrow transplant can be curative

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6
Q

Beta Thalassaemia:

A

Types: thalassaemia minor, intermedia and major

Major: homozygous for the deletion of genes/. Severe microcytic anaemia, splenomegaly, and bone deformities
Management: regular transfusions, iron chelation (to prevent overload) and splenectomy.

Intermedia: two defective genes or one defective gene and one deletion.
Patients may require monitoring and occasional blood transfusions. May require iron chelation to prevent overload.

Minor: One normal gene and one abnormal gene.
Causes mild microcytic anaemia.
Usually, patients only require monitoring and no active treatment.

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7
Q

‘Tear-drop’ poikilocytes’

A

Myelofibrosis

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8
Q

Howell-Jolly bodies:

A

Hyposplenism

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9
Q

Schistocytes:

A

Intravascular haemolysis
Mechanical heart valve
Disseminated intravascular coagulation

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10
Q

‘Pencil’ Poikilocytes:

A

Iron deficiency anaemia

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11
Q

Spherocytes:

A

Autoimmune haemolytic anaemia
Hereditary spherocytosis

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12
Q

Acute haemolytic reaction:

A

ABO incompatible blood e.g., secondary to human error.
Features: Fever, abdominal pain, and hypotension
Management: stop transfusion, high flow oxygen, fluid resuscitation, move to HDU/ICU, consider intubation, haemodialysis

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13
Q

Transfusion associated circulatory overload:

A

Excessive rate of transfusion, pre-existing heart failure.
Features: Pulmonary oedema, and hypertension
Management: Slow or stop transfusion. Consider IV loop diuretics e.g., furosemide and oxygen

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14
Q

Transfusion-related acute lung injury:

A

non-cardiogenic pulmonary oedema thought to be secondary to increased vascular permeability caused by activation of host neutrophils.
Features: Hypoxia, pulmonary infiltrates on chest x-ray, fever, and hypotension
Management: stop transfusion, oxygen, and supportive care.

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15
Q

Burkitt’s lymphoma:

A

High grade B-cell neoplasm
Usually involves maxilla or mandible.
‘Starry night’ on bone marrow histology
Management: chemotherapy
Complication: tumour lysis syndrome

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16
Q

Tumour lysis syndrome:

A

Hyperkalaemia
Hyperphosphatemia
Hypocalcaemia
Hyperuricaemia
Acute renal failure
Prophylaxis – allopurinol oral or IV

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17
Q

Chronic lymphocytic leukaemia: complications

A

Warm autoimmune haemolytic anaemia
Transformation to high grade lymphoma (Richter’s transformation)

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18
Q

Richter’s transformation:

A

Richter’s transformation:
Lymph node swelling
Fever without infection
Weight loss
Night sweats
Nausea

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19
Q

Chronic lymphocytic leukaemia: features and investigations

A

Monoclonal proliferation of well-differentiated B lymphocytes.
Most common form of leukaemia in adults
Features: anorexia, weight loss, bleeding, infections
Investigations: FBC, Blood film: smudge cells, immunophenotyping

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20
Q

Chronic myeloid leukaemia

A

Associated with Philadelphian chromosome
Presentation: anaemia, weight loss, splenomegaly, increase in granulocytes at different stages of maturation
Management: imatinib (first line) (tyrosine kinase inhibitor)
Hydroxyurea
Interferon alpha
Bone marrow transplant

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21
Q

G6PD deficiency:

A

X-linked recessive
African + Mediterranean descent
Features: neonatal jaundice, gallstones, Heinz bodies on blood film
Diagnosis: G6PD enzyme assay.
Management: Avoid triggers

22
Q

Hereditary spherocytosis:

A

Autosomal dominant
Northern European
Features: Neonatal jaundice, gallstones, splenomegaly
Blood film: spherocytes
Diagnosis: EMA binding
Management:
Acute: supportive
Chronic: folate replacement, splenectomy

23
Q

Acute GVHD:

A

Classically within 100 days of transplantation
Usually affects the skin, liver, and GI tract
Features: Painful maculopapular rash, jaundice, watery or bloody diarrhoea, persistent nausea, and vomiting
Multiple organ involvement carries worse prognosis

24
Q

Chronic GVHD:

A

May occur following acute disease or arise de novo
Classically after 100 days following transplantation
Skin: vitiligo, scleroderma
Eye: conjunctivitis sicca, corneal ulcers, scleritis
GI: dysphagia, oral ulcers
Lung: obstructive or restrictive lung disease

Management: immunosuppression and supportive measures.

25
Q

Haematological malignancies: infections

A

EBV: Hodgkin’s and Burkitt\s lymphoma, nasopharyngeal carcinoma

Bacteria: H.Pylori: MALT lymphoma

Protozoa: Burkitt’s lymphoma

26
Q

Haemophilia

A

X-linked recessive
Haemophilia A: clotting factor VIII
Haemophilia B: clotting factor IX
Signs and symptoms: spontaneous haemorrhage, intracranial haemorrhage, cord bleeding in neonates, HEMARTHROSIS.
Diagnosis: bleeding scores, genetic testing
Management: IV infusions of clotting factors VII and IX
Desmopressin to stimulate release of von Willebrand factor
Antifibrinolytics: tranexamic acid

27
Q

B-symptoms:

A

Fever >38
Night sweats
Weight loss >10% in the last 6 months

28
Q

Hodgkin’s lymphoma: presentation

A

Risk factors: HIV, EBC, family history
Presentation: non-tender, rubbery lymph nodes. Lymph node pain when drinking alcohol
Investigations: Reed-Sternberg cells- abnormally large B cells. CT, MRI and PET for staging and grading
Management:
Chemotherapy and radiotherapy.
ABVD
BEACOPP
Stem cell transplantation

29
Q

Non-Hodgkin lymphoma:

A

Examples: Burkitt, MALT and diffuse large B cell lymphoma.
Management: Observation, chemo, monoclonal antibodies e.g., rituximab, radiotherapy, stem cell transplant

30
Q

Immune thrombocytopenia in adults (ITP)

A

Presentation: petechiae, purpura, epistaxis
Investigations: FBC (isolated thrombocytopenia), blood film
Management: oral prednisolone (first line)
IV immunoglobulins
Immunosuppressive drugs e.g., cyclophosphamide

31
Q

Iron deficiency anaemia vs anaemia of chronic disease

A

Iron deficiency - TIBC high, ferritin low
Anaemia of chronic disease - TIBC low, ferritin high

32
Q

MGUS:

A

Monoclonal gammopathy of unknown significance. A common condition that causes a paraprotein and is often mistaken for myeloma.
Features: usually asymptomatic, no bone pain or increased risk of infections.
Stable level of paraprotein

33
Q

Myelodysplastic syndrome:

A

Caused by myeloid bone marrow cells not maturing properly.
Causes anaemia, neutropoenia and thrombocytopaenia.
Increased risk of transforming into acute myeloid leukaemia.
Diagnosis: FBC, blood film – blast cells. Bone marrow aspiration and biopsy to confirm diagnosis.
Management: supportive, chemo and stem cell transplant

34
Q

Multiple myeloma:

A

Features: CRABBI
Calcium (raised)
Renal impairment
Anaemia
Bone lesions/bone pain
Bleeding
Infection
Investigations:
Whole body MRI (first-line)
Peripheral blood film – rouleaux formation.
Protein electrophoresis: raised concentration of monoclonal IgA/IgG. Bone marrow aspiration: plasma cells raised.
X-ray – rain-drop skull
NSAIDs can precipitate renal failure in patients with multiple myeloma
Management: Chemo, stem cell transplantation, VTE prophylaxis with aspirin or LMWH whilst on certain chemotherapy regimes.

35
Q

Neutropenic sepsis:

A

Most common cause: staphylococcus epidermidis
Prophylaxis: patients with neutrophil count <0.5*109 should have fluroquinolone as prophylaxis
Management: Tazobactam and piperacillin

36
Q

Non-Hodgkin’s lymphoma:

A

Symptoms: B-symptoms, painless lymphadenopathy, testicular mass, fever
Management: watchful waiting, chemo, or radio
Prognosis:
High grade – progresses faster but more curable and responsive to chemotherapy
Low grade – progresses slower but less curable and not responsive to chemotherapy

37
Q

Paroxysmal nocturnal haemoglobinuria:

A

Features: dark coloured urine in the morning, haemolytic anaemia
Management: blood product replacement, anticoagulation, stem cell transplantation

38
Q

Polycythaemia rubra vera:

A

A myeloproliferative disorder causing increase in red blood cell volume.
JAK2 mutation.
Features: pruritis (typically after a hot bath), splenomegaly, hypertension, hyper viscosity (leading to arterial and venous thromboses)
Management: aspirin, venesections, and chemotherapy (hydroxyurea)

39
Q

Sickle cell anaemia:

A

Investigations: haemoglobin electrophoresis
Crisis management:
Analgesia
Fluids
Oxygen
Blood transfusion
Exchange transfusion

Long term management:
Hydroxyurea/hydroxyurea (increases the HbF levels)
Pneumococcal vaccine every 5 years.

40
Q

Oncological emergencies:

A

Tumour lysis syndrome
Spinal cord compression/metastasis
Neutropenic sepsis
SVCO

41
Q

Management of hypercalcaemia in multiple myeloma

A

IV fluids and zolendronic acid

42
Q

Complication of multiple myeloma

A

Renal impairement
Anaemia
Spinal cord compression
Hypercalcaemia
Infection
Peripheral neuropathy
Hyperviscosity

43
Q

Consequences of sickle cell crisis

A

MI
VTE
Priapism
Acute chest syndrome

44
Q

Precipitants of sickle cell crisis

A

Dehydration
Cold
Infection
Pregnancy
Hypoxia

45
Q

Acute chest syndrome:

A

Vaso-occlusion within the pulmonary microvasculature
Features: dyspnoea, chest pain, pulmonary infiltrates on chest x-ray.
Management: pain relief, oxygen, antibiotics

46
Q

Aplastic crisis:

A

Caused by infection with parvovirus
Sudden fall in haemoglobin
Bone marrow suppression causes a reduced reticulocyte count.

47
Q

Sequestration crisis:

A

Sickling within organs such as the spleen and lungs causes pooling of blood with worsening of the anaemia
Associated with an increased reticulocyte count.

48
Q

Von Willebrand Disease:

A

Most common inherited bleeding disorder.
Autosomal dominant
Investigations: prolonged bleeding time – APTT
Management: tranexamic acid, desmopressin (raises levels of vWF)

49
Q

B12 and folate deficiency:

A

Treat the B12 first via IM injections and then start oral folate supplementation when B12 levels are stable and within normal range.

50
Q

Haemochromatosis:

A

Autosomal recessive disorder of iron absorption and metabolism. Mutation in HFE gene.
Features: fatigue, erectile dysfunction, and arthralgia. Bronze pigmented skin, diabetes, liver disease.
Investigations: transferrin saturation and genetic testing for HFE mutation
Raised ferritin and low TIBC.
Management: venesections (first-line), desferrioxamine.

51
Q

Diagnosing haemolytic anaemia:

A
  • FBC – Hb
  • Serum bilirubin
  • Serum LDH
  • Direct coomb’s test
52
Q

Causes of haemolytic anaemia

A

G6PD
Hereditary spherocytosis
Sickle cell disease
Metallic heart valves
Autoimmune haemolytic anaemia
Haemolytic transfusion reactions