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Systems > Haematology and Immunology > Flashcards

Haematology and Immunology Flashcards

1
Q

Polycythaemia

A

Increased rbc

- JAK2 gene (myeloid malignancies)

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2
Q

Thrombocytosis

A

Increased platelets

  • Infection
  • Trauma
  • Myeloid malignancy (myeloproliferative disorders)
  • Iron deficiency
  • Inflammation
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3
Q

Thrombocytopenia

A

Reduced platelets: decreased production (Tx: thrombopoietin analogues), increased consumption

  • Acquired: Marrow failure, DIC
  • Autoimmune (high dose steroids)
  • Hypersplenism (splenectomy)
  • Normal blood except reduced platelet count
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4
Q

Hormones controlling blood cell production

A
  • Thrombopoietin (liver) - platelets
  • Erythropoietin (kidneys)
  • Cytokine granulocyte colony-stimulating factor
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5
Q

Normal Hb (male)

A

140-180 g/L

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6
Q

Normal Hb (female)

A

120-160 g/L

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7
Q

Normal platelets

A

150-400 x10^9/L

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8
Q

Normal WBC

A

4-10 x10^9/L

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9
Q

Donor/recipient compatibility

A
  • ABO group
  • Rhesus
  • Serum alloantibodies
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10
Q

Anaemia

- and morphological description

A

Reduced levels of rbc’s or Hb

  • Bone marrow (cellularity, stroma, nutrients)
  • Red cell (membrane, Hb, enzymes)
  • Destruction/loss (haemorrhage, haemolysis, hypersplenism)
  • Hypochromic, microcytic
  • Normochromic, normocytic
  • Macrocytic
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11
Q

Anaemia presentation

A
  • Tiredness/fatigue
  • Pallor
  • Peripheral oedema
  • Dizziness
  • Chest pain

Symptoms relating to underlying cause

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12
Q

Hypochromic microcytic anaemia

A

Do serum ferritin

  • Iron deficiency (low)
  • Thalassaemia (normal)
  • Secondary anaemia - infection, inflammation, malignancy
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13
Q

Normochromic normocytic anaemia

A

Do reticulocyte count

  • Blood loss/haemolysis (increased)
  • Secondary anaemia (normal)
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14
Q

Macrocytic anaemia

A

Do B12/folate

  • Megaloblastic (low) -> pernicious anaemia
  • Normal -> non-megaloblastic anaemia (alcohol, drugs, liver)
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15
Q

Iron deficiency anaemia

A

Increased iron loss (bleeding, diet, malabsorption) or requirement (pregnancy)

Physiology: Ferroportin (Hepcidin inhibits - inflammation) -> Transferrin (plasma) -> Ferritin

Examination: koilonychia, atrophic tongue, angular cheilitis

Investigation: blood film (hypochromic, microcytic), low serum ferritin

Management: iron supplementation, blood transfusion, correct cause

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16
Q

Haemolytic anaemia

A

Accelerated red cell destruction

  • Congenital: HS, G6PD deficiency, SSD
  • Autoimmune (positive DGAT)
  • Non-immune: mechanical, infection (negative DGAT)

Investigation:

  • Reticulocyte count (increased)
  • Low haptoglobin (free Hb)
  • DGAT
  • Increased conjugated bilirubin

Management:

  • Folic acid
  • Correct cause - immunosuppression, splenectomy etc
  • Transfusion
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17
Q

Macrocytic anaemia

A

Large red blood cells.

Megaloblastic:

  • B12 deficiency (pernicious anaemia)
  • Folate deficiency
  • Neurological symptoms
  • Yellow tinge

Non-megaloblastic: alcohol, marrow infiltration, drugs, disordered liver, hypothyroidism

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18
Q

Pernicious anaemia

A

Autoimmune disease

  • Low B12 and macrocytic blood film
  • Anti-intrinsic factor antibodies
  • IM vitamin B12
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19
Q

Hereditary spherocytosis

A

Spherical RBCs

  • Autosomal dominant
  • 5 structural protein defects
  • Anaemia, jaundice, splenomegaly, pigment gallstones
  • Negative DAGT
  • Folic acid, transfusion, splenectomy
20
Q

G6PD deficiency

A

Cells vulnerable to oxidative damage

  • X-linked and precipitated by infection, acute illness, drugs
  • Anaemia, jaundice, splenomegaly, pigment gallstones
  • Haemoglobinuria and low haptoglobin
21
Q

Thalassaemia

A

Reduced/absent globin chain production

  • X-linked
  • Hypochromic, microcytic blood film
  • Normal ferritin
  • Severe anaemia, bone deformities, splenomegaly
22
Q

Sickle cell disease

A

Structurally abnormal globin chain

  • X-linked
  • Symptoms occur due to vaso-occlusion: bone crisis, chest crisis, stroke, infection, chronic haemolytic anaemia, sequestration crisis
  • Reticulocyte count, bilirubin, LDH, haemoglobinopathy screen
  • Lifelong prophylaxis - folic acid, vaccines, penicillin
23
Q

Haemophilia A and B

A

A: CFVIII deficiency
B: CFIX deficiency

  • X-linked
  • Coagulation factor pattern of bleeding: haemarthrosis, muscle haematoma, CNS bleeding, retroperitoneal bleeding
  • Coagulation tests and assays: prolonged aptt, normal pt
  • Coagulation factor replacement: recombinant, transfision, DDAVP, tranexamic acid, emicizumab
24
Q

Von Willebrand disease

A

vWF
Type 1: quantitative deficiency
Type 2: qualitative deficiency
Type 3: severe deficiency

  • Autosomal dominant
  • Platelet-type pattern of bleeding: epistaxis, purpura, menorrhagia, GI
  • Coagulation tests
  • vWF concentrate, DDAVP, tranexamic acid, combined OCP
25
Q

Causes of bleeding

A
  • Haemophilia (FVIII, IX)
  • vWD
  • Thrombocytopenia
  • Liver failure (prolonged PT, APTT, reduced fibrinogen)
  • Vitamin K deficiency (haemorrhagic disease of newborn)
26
Q

Leukaemia

A

Progressive, malignant disease of the blood-forming organs (leukocytes and precursors in blood and bone marrow)

  • Myeloid or lymphoid
  • Acute or chronic

Acquired genetic and environmental

27
Q

Leukaemia presentation

A
  • Fatigue
  • Weight loss
  • Fever
  • Pallor
  • Bruising
  • Petechiae
  • Sweats
  • Anaemia: SOB, dizziness, palpitations
28
Q

Leukaemia investigations

A

Blood analysis

  • FBC, blood film
  • Coagulation screen
  • B12, folate, ferritin
  • U&Es, LFTs, CRP, ESR
  • Reticulocyte count

Bone marrow biopsy

29
Q

Acute myeloid leukaemia (AML)

A

Blast myeloid cells have ability to proliferate but cannot differentiate.
- Myelodysplastic syndrome can progress to AML.

Presentation: bone marrow failure

  • Anaemia
  • Thrombocytopenic bleeding
  • Infection
  • Gum hypertrophy

Investigation

  • Bone marrow biopsy
  • Reduced erythrocytes

Management

  • Supportive
  • Anti-leukaemic chemotherapy
30
Q

Chronic myeloid leukaemia (CML)

A

Myeloid cells have ability to proliferate and differentiate
- Philadelphia chromosome T(9;22)

Presentation

  • Anaemia of chronic disease
  • Splenomegaly
  • Weight loss
  • Hyperleukostasis
  • Gout
  • Thrombocytopenia

Investigation

  • High WCC/platelets, film
  • Bone marrow biopsy (hypercellular)

Management

  • Tyrosine Kinase Inhibitors
  • BCR-ABL inhibitors
  • Allogeneic transplantation
  • Folic acid
  • Steroids
31
Q

Myeloproliferative neoplasms (MPN)

A 
Polycythaemia vera (PV)
Essential thrombocythemia (ET)
- JAK2 V617F/CALR mutation

Presentation

  • Headaches
  • Itch
  • Vascular occlusion
  • Thrombosis
  • TIA, stroke
  • Splenomegaly
  • Plethora
  • Myelofibrosis: extensive scarring in bone marrow leading to severe anaemia

Investigations
- Mutation screening

32
Q

Polycythaemia vera (PV)

A

Myeloproliferative neoplasms (MPN)

  • Raised Hb and haematocrit
  • Raised uric acid
  • Low (iron-deficient PV)

Management:

  • Venesection
  • Aspirin
  • Cytoreduction
  • JAK2 inhibitor
33
Q

Essential thrombocythemia (ET)

A 
Myeloproliferative neoplasms (MPN)
- Raised platelets

Management:

  • Aspirin
  • Cytoreduction
34
Q

Acute lymphoblastic leukaemia (ALL)

A

Lymphoid (blast) cells can proliferate but not differentiate

Presentation:

  • Bone marrow failure - anaemia, thrombocytopenia
  • Bone/joint pain
  • Infection
  • Sweats

Investigation:

  • Low Hb, platelets
  • High WBC
  • Spherocytes
  • High reticulocyte count

Bone marrow biopsy

  • CD-20: mature
  • CD-34, TDT: immature

Management:
- Chemotherapy

35
Q

Chronic lymphocytic leukaemia (CLL)

A

Lymphoid (blast) cells have ability to proliferate and differentiate.

Presentation

  • Asymptomatic
  • Bone marrow failure
  • Lymphadenopathy
  • Splenomegaly
  • Systemic features
  • Hepatomegaly
  • Infections

Investigation

  • High WBC
  • Flow cytometry

Staging (Binet)

a) <3 lymph nodes
b) 3+ nodes
c) 3+ nodes + bone marrow failure

Management

  • Observe
  • Chemotherapy
  • Monoclonal antibodies
  • Novel agents
36
Q

Lymphoma

A

Cancer originating in lymphoid tissue (lymphocytic differentiation in germinal centre of lymph nodes)
- Lymph nodes, spleen, thymus, bone marrow

Presentation

  • Lymphadenopathy (painless)
  • Hepato/splenomegaly
  • Systemic (B) symptoms
  • Bone marrow involvement

Investigations

  • High WBC
  • Lymph node biopsy
  • PET-CT scan
  • Bone marrow aspirate
  • Virology and FNA (rule out)

Staging (look at table)

Combination chemotherapy

37
Q

Non-Hodgkin lymphoma

A

Lineage (B or T-cell)
Grade
- High: diffuse large B-cell (aggressive), curable
- Low: follicular, marginal zone, often asymptomatic, incurable

38
Q

Hodgkin lymphoma

A

Presence of Reed-Sternberg cells

Associations:

  • Epstein Barr virus
  • Familial
  • Geographical
  • Young

Management

  • +/- radiotherapy
  • Monoclonal antibodies (anti-30)
  • Immunotherapy
39
Q

IgM paraproteins present

A

Lymphoma

40
Q

IgG, IgA paraproteins present

A

Myeloma

41
Q

Myeloma

A

Neoplastic disorder of plasma cells
- IgG, IgA paraprotein present

Paraproteins or plasma cells -> symptoms:
CRAB
- Hypercalcaemia
- Renal failure
- Anaemia 
- Bone disease/failure
  • Infections: leukopenia

Serum total electrophoresis and protein, ESR

Management:
Asymptomatic -> don't treat
- Chemotherapy 
- Biphosphonate therapy
- Radiotherapy 
- Steroids
- Surgery (stabilise bones)
- SCT
42
Q

Infection in neutropenia

A

Bacterial

Fungal

43
Q

Infection in monocytopenia

A

Fungal (deep-seated)

  • Candida
  • Aspergillus
44
Q

Infection in low eosinophils (Cushing’s)

A

Parasitic

45
Q

Infection in lymphopenia

A

T-cell: fungal and viral infection, PJP (pneumocystis jirovecii)

B-cell: bacterial

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