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Haem Flashcards

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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:

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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:

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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:

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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:

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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
Haematological malignancies: infections
EBV: Hodgkin’s and Burkitt\s lymphoma, nasopharyngeal carcinoma Bacteria: H.Pylori: MALT lymphoma Protozoa: Burkitt’s lymphoma
26
Haemophilia
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
B-symptoms:
Fever >38 Night sweats Weight loss >10% in the last 6 months
28
Hodgkin’s lymphoma: presentation
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
Non-Hodgkin lymphoma:
Examples: Burkitt, MALT and diffuse large B cell lymphoma. Management: Observation, chemo, monoclonal antibodies e.g., rituximab, radiotherapy, stem cell transplant
30
Immune thrombocytopenia in adults (ITP)
Presentation: petechiae, purpura, epistaxis Investigations: FBC (isolated thrombocytopenia), blood film Management: oral prednisolone (first line) IV immunoglobulins Immunosuppressive drugs e.g., cyclophosphamide
31
Iron deficiency anaemia vs anaemia of chronic disease
Iron deficiency - TIBC high, ferritin low Anaemia of chronic disease - TIBC low, ferritin high
32
MGUS:
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
Myelodysplastic syndrome:
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
Multiple myeloma:
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
Neutropenic sepsis:
Most common cause: staphylococcus epidermidis Prophylaxis: patients with neutrophil count <0.5*109 should have fluroquinolone as prophylaxis Management: Tazobactam and piperacillin
36
Non-Hodgkin’s lymphoma:
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
Paroxysmal nocturnal haemoglobinuria:
Features: dark coloured urine in the morning, haemolytic anaemia Management: blood product replacement, anticoagulation, stem cell transplantation
38
Polycythaemia rubra vera:
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
Sickle cell anaemia:
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
Oncological emergencies:
Tumour lysis syndrome Spinal cord compression/metastasis Neutropenic sepsis SVCO
41
Management of hypercalcaemia in multiple myeloma
IV fluids and zolendronic acid
42
Complication of multiple myeloma
Renal impairement Anaemia Spinal cord compression Hypercalcaemia Infection Peripheral neuropathy Hyperviscosity
43
Consequences of sickle cell crisis
MI VTE Priapism Acute chest syndrome
44
Precipitants of sickle cell crisis
Dehydration Cold Infection Pregnancy Hypoxia
45
Acute chest syndrome:
Vaso-occlusion within the pulmonary microvasculature Features: dyspnoea, chest pain, pulmonary infiltrates on chest x-ray. Management: pain relief, oxygen, antibiotics
46
Aplastic crisis:
Caused by infection with parvovirus Sudden fall in haemoglobin Bone marrow suppression causes a reduced reticulocyte count.
47
Sequestration crisis:
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
Von Willebrand Disease:
Most common inherited bleeding disorder. Autosomal dominant Investigations: prolonged bleeding time – APTT Management: tranexamic acid, desmopressin (raises levels of vWF)
49
B12 and folate deficiency:
Treat the B12 first via IM injections and then start oral folate supplementation when B12 levels are stable and within normal range.
50
Haemochromatosis:
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
Diagnosing haemolytic anaemia:
- FBC – Hb - Serum bilirubin - Serum LDH - Direct coomb’s test
52
Causes of haemolytic anaemia
G6PD Hereditary spherocytosis Sickle cell disease Metallic heart valves Autoimmune haemolytic anaemia Haemolytic transfusion reactions

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