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3b > Haematology > Flashcards

Haematology Flashcards

1
Q

Microcytic anaemia

A

TAILS

Thalassaemia
AoCD
Iron deficiency
Lead poisoning
Sideroblastic
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2
Q

Normocytic anaemia

A 
AoCD
Acute bleed
Pregnancy
CKD
Aplastic anaemia
Bone marrow infiltration

Haemolytic anaemia

  • G6PD
  • Hereditary spherocytosis
  • Sickle cell
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3
Q

Macrocytic anaemia

A

Folate
B12

Hypothyroid
Alcohol

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4
Q

Iron deficiency anaemia aetiology

A

Uptake in the duodenum

Excessive blood loss

  • Menorrhagia
  • GI bleed - Colorectal cancer in men, peptic ulcers

Inadequate dietary intake

  • Meat and leafy greens
  • Veggie diet is RF

Poor intestinal absorption

  • Coeliac / Crohn’s
  • Gastrectomy

Increased iron requirements

  • Children
  • Pregnant ladies

NSAIDs
Haemodialysis

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5
Q

Iron deficiency anaemia clinical features

A

Fatigue
SOBOE
Weakness

PICA - Abnormal craving for non-food substances
Restless leg syndrome
Kolionychia - Spoon nails

Glossitis
Angular stomatitis

Gastric difficulties
Hair loss

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6
Q

IDA investigations

A

History

  • Change in diet
  • Medication history
  • Menstrual history
  • Weight loss
  • Change in bowel habit

FBC - Microcytic hypochromic + Low reticulocytes
Blood film - Anisopoikilocytosis - Red cells of different shapes and sizes

Iron studies

  • MCH - Low
  • Serum ferritin - Low
  • TIBC - High
  • Transferrin saturation - Low

Coeliac seriology

OGD if > 55 or red flags

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7
Q

IDA management

A

Treat cause

Ferrous sulphate

  • Nausea
  • Abdo pain
  • Constipation
  • Diarrhoea
  • Black stools

Iron-rich diet

  • Leafy greens
  • Meat
  • Iron-fortified bread
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8
Q

Pernicious anaemia pathophysiology

A

B12 deficiency

Normal absorption

  • Oily fish, meat, dairy
  • Parietal cells produce IF
  • IF binds to B12
  • Absorption in terminal ileum

Pathology

  • AI disorder affecting gastric mucosa
  • Antibodies to IF and/or gastric parietal cells
  • Antibodies bind to IF - Block B12 binding site
  • Gastric parietal cell antibodies - Reduce IF production

B12 important for…

  • Erythropoiesis - Megaloblastic anaemia
  • Nerve myelination - Neuropathy
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9
Q

Pernicious anaemia RFs and clinical features

A

Females > Males
AI disorders - Thyroid, DM, Addison’s, RA, etc.

Symptomatic anaemia

Peripheral neuropathy - Paraesthesia

Subacute combined spinal cord degeneration

  • Progressive weakness
  • Ataxia
  • Paraesthesia
  • Spasticity
  • Paraplegia

Neuropsychiatric features

  • Memory loss
  • Poor concentration
  • Confusion
  • Depression
  • Irritability
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10
Q

Pernicious anaemia investigations

A

FBC - Macrocytic anaemia

  • WCC - Low
  • Platelets - Low

Blood film - Hypersegmented polymorphs

B12 < 200

Antibodies

  • Anti-IF antibodies
  • Anti-GP cell antibodies

Schilling test

  • Radiolabelled B12 administered
  • Once without IF + Once with oral IF
  • Urine B12 measured
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11
Q

Pernicious anaemia management and complications

A

B12 replacement therapy - IM

  • 3 injections per week for 2 weeks
  • Then 3 monthly
  • More frequently if neurological symptoms

Folic acid supplementation

Complications - Increased risk of gastric cancer

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12
Q

Folate deficiency aetiology

A

Leafy green vegetables
Converted to tetrahydrofolate
Involved with DNA/RNA synthesis

Dietary deficiency
Malabsorption - Coeliac/IBD
Pregnancy and breast-feeding
Prematurity
Alcohol excess

Iatrogenic

  • Phenytoin / Phenobarbitol
  • Trimethoprim
  • Methotrexate
  • Sulfasalazine
  • Pyrimethamine
  • Anticonvulsants
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13
Q

Folate deficiency clinical features and investigations

A

Symptomatic anaemia
Exfoliative dermatitis
Weight loss / LOA
Headaches

Serum folate - Low
Blood film - Hypersegmented neutrophils
Reticulocytes - Low

FBC - Macrocytic anaemia

  • Hb - Low
  • MCV ^
  • MCH ^
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14
Q

Folate deficiency management

A

Folic acid - 5mg

Preventing NTD during pregnancy

  • 400mcg folic acid until 12th week of pregnancy
  • Higher risk of NTD - 5mg folic acid before conception to 12th week

Increased risk of NTD

  • Either partner has NTD
  • Previous pregnancy affected by NTD
  • Family history of NTD
  • Anti-epileptic drugs
  • Coeliac disease
  • DM
  • Thalassaemia
  • Obesity
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15
Q

Haemolytic anaemia clinical features

A

Jaundice - Unconjugated bilirubin ^

Pallor
Dark urine

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16
Q

G6PD deficiency

A

Haemolytic anaemia - X-linked

Clinical features - Haemolytic anaemia following a precipitant

  • Abx - Nitro
  • Anti-malarial - Chloroquine
  • Infection
  • Fava beans

Investigations - Normocytic anaemia with high reticulocytes

Blood film

  • Heinz bodies
  • Bite cells
  • Hemi-ghosts

Management - Supportive

  • Transfusion if severe
  • Avoid precipitants
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17
Q

Hereditary spherocytosis

A

Haemolytic anaemia - AD

Defect in RBC membrane synthesis
Spherical RBCs removed by spleen
Reduced RBC lifespan

Patients from Northern Europe - Finland, etc.

Clinical features = Haemolytic anaemia + Splenomegaly

Investigations - Normocytic anaemia with high reticulocytes

  • Blood film - Spherocytes
  • EMA binding test

Management

  • Acute crisis - Supportive + Transfusion
  • Long-term - Folic acid + Splenectomy
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18
Q

Sickle cell pathophysiology

A

Haemolytic anaemia - Autosomal recessive

Glutamate to Valine
Abnormal HbS
HbS turns sickle shaped when deoxygenated
Results in haemolysis and capillary obstruction

Precipitants

  • Hypoxia
  • Cold
  • Exercise
  • Dehydration
  • Infection
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19
Q

Sickle cell crises

A

Thrombotic - Painful / vaso-occlusive crises

  • AVN femoral head
  • Hand-foot syndrome in children
  • Lungs
  • Spleen
  • Brain - CVA
  • Mesenteric ischaemia

Sequestration - Sickling with organs

  • Spleen or lungs
  • Pooling of blood with worsening anaemia

Acute chest syndrome

  • Dyspnoea
  • Chest pain
  • Pulmonary infiltrates
  • Low pO2

Aplastic - Infection with parvovirus
- Sudden fall in Hb

Haemolytic - Fall in Hb due to increased haemolysis

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20
Q

Sickle cell investigations and crisis management

A

Guthrie heel-prick test
FBC - Normocytic anaemia with high reticulocytes

Protein electrophoresis

  • HbAS/HbSS
  • HbF ^

Blood film - Howell jolly bodies

Crisis management - Treat cause

  • O2
  • IV fluids
  • Analgesia
  • Transfusion
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21
Q

Sickle cell long-term management and complications

A 
Avoid triggers!
Hydroxyurea - Stimulates HbF production
Vaccinations - Influenza and pneumococcal
Prophylactic abx - Penicillin
Repeated transfusions
Bone marrow transplant

Complications

  • CVA
  • Stunted growth
  • Developmental delay
  • Crises
  • Osteomyelitis - Salmonella
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22
Q

Aplastic anaemia aetiology

A

Peak incidence 30 years old

Idiopathic
Congenital - Fanconi or dyskeratosis congenita
Toxins - Benzene
Infection - Parvovirus and Hepatitis
Radiation

Iatrogenic

  • Cytotoxics
  • Chloramphenicol
  • Sulphonamides
  • Phenytoin
  • Gold
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23
Q

Aplastic anaemia clinical features / investigations / management

A

Symptomatic anaemia
Neutropenia - Infections
Thrombocytopenia - Bleeding and bruising

Investigations

  • Normocytic hypochromic anaemia
  • Hypoplastic bone marrow
  • Pancytopenia
  • Reticulocytes - Low

Management - Monitoring

  • Immunosuppressants
  • Transfusions
  • Prophylactic abx
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24
Q

Autoimmune haemolytic anaemia

A

Warm vs Cold - According to what temperature the antibodies cause haemolysis

Investigations

  • Direct antiglobulin test - Coombs’ test - Positive
  • FBC - Haemolytic anaemia

Management

  • Corticosteroids
  • Immunosuppression
  • Splenectomy
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25
Q

Warm AIHA - IgG

A

Haemolysis best caused at body temperature
Haemolysis at extravascular sites - E.g. spleen

Aetiology

  • AI disease - SLE
  • Neoplasia - Lymphoma / CLL
  • Iatrogenic - Methyldopa

Clinical features - Haemolytic anaemia

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26
Q

Cold AIHA

A

Haemolysis best caused at 4c
Haemolysis mediated by complement
More commonly intravascular

Aetiology

  • Neoplasia - Lymphoma
  • Infections - Mycoplasma / EBV

Clinical features

  • Haemolytic anaemia
  • Raynaud’s
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27
Q

AoCD

A

Inflammation mediated reduction in RBC production

Aetiology

  • IBD
  • RA
  • JIA
  • Malignancy

Clinical features - Symptomatic anaemia

Investigations - Normocytic anaemia with low reticulocytes

  • Iron - Low
  • TIBC - Low
  • Transferrin saturation - Low
  • Ferritin - High

Management - Treat cause

  • Transfusion
  • ESAs - Epoetin Alfa
  • Iron supplementation - Sodium ferric gluconate complex
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28
Q

Sideroblastic anaemia aetiology

A

Microcytic - X-linked

Defect in synthesis of protoporphyrin ring
Iron can’t bind haem
Free iron circulating
Leads to iron overload picture

Aetiology - X-linked

  • Alcohol
  • Lead
  • Myelodysplasia
  • TB medication
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29
Q

Sideroblastic anaemia clinical features / investigations / management

A

Symptomatic anaemia + Iron overload

  • Heart
  • Liver
  • Kidneys

Investigations

  • FBC - Hypochromic microcytic anaemia
  • Ferritin ^
  • TIBC - Low
  • Iron ^
  • Bone marrow biopsy - Sideroblasts

Management - Treat cause

  • Desferoxamine
  • Pyridoxine
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30
Q

Alpha thalassaemia

A

Hypochromic microcytic

SE Asia
Deficiency of alpha chains in Hb
2 separate alpha-globulin genes located on each Ch16

1 or 2 alleles affected - Normal Hb

3 alleles affected

  • HbH disease
  • Splenomegaly

All 4 alleles affected

  • Death in utero
  • Hydrops fetalis
  • Bart’s hydrops
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31
Q

Beta thalassaemia aetiology

A

Trait - Hypochromic microcytic - Mediterranean

Major - Hypochromic microcytic

  • Absence of beta chains
  • Ch11
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32
Q

Thalassaemia clinical features / investigations / management

A

Haemolytic anaemia

Present in first year of life

  • FTT
  • Hepatosplenomegaly

Skull bossing
Major - Hydrops fetalis

Investigations - Unconjugated bilirubin ^
Hb electrophoresis 
- HbA2 ^
- HbF ^
- HbA - Absent in major

Management

  • Transfusion
  • Desferoxamine
33
Q

Polycythaemia aetiology

A

Myeloproliferative disorder
Clonal proliferation of a marrow stem cell
Increased red cell volume
Overproduction of neutrophils and platelets

Peak incidence aged 60-70

Congenital - JAK2 mutation

Acquired - Chronic hypoxia

  • OSA
  • COPD
  • Altitude

Associated with Budd-Chiari syndrome

34
Q

Polycythaemia clinical features

A 
Ruddy appearance - Flushed
Pruritus - Palms and soles - Worse after a bath
Headaches
Tinnitus
Angina
Dizziness
Splenomegaly
HTN - 1/3 patients
VTE risk
Haemorrhage - Secondary to abnormal platelet function
35
Q

Polycythaemia investigations / management / prognosis

A

FBC

  • Hb ^
  • Crit ^
  • Neutrophils ^
  • Basophils ^
  • Platelets ^

Mutation screen - JAK2

Management - Venesection

  • Hydroxyurea
  • Phosphorous-32 therapy
  • VTE prophylaxis - Low-dose Aspirin

Prognosis

  • Thrombotic events cause morbidity
  • Myelofibrosis
  • Acute leukaemia
36
Q

Multiple myeloma aetiology

A

Malignant proliferation or plasma cells
Increased production of Ig - Paraproteins
Accumulation in the renal system - Bence-Jones proteins

Median age 70 years old

37
Q

Multiple myeloma clinical features

A

CRAB-E

Calcium - Hypercalcaemia - Increased osteoclast activity

  • Constipation
  • Nausea
  • Anorexia
  • Confusion

Renal (AKI) - Light chain deposition within renal tubules - Bence-Jones protein

  • Dehydration
  • Increasing thirst

Anaemia - Pancytopenia

  • Symptomatic anaemia
  • Infections
  • Bleeding/bruising

Bones - Bone marrow infiltration by plasma cells + Cytokine mediated osteoclasts

  • Lytic bone lesions
  • Back pain
  • Fragility fractures
  • Raindrop skull

ESR ^ - Increased risk of VTE

38
Q

Multiple myeloma investigations and diagnosis

A 
BM biopsy - Monoclonal plasma cells
Serum/urine electrophoresis - Monoclonal paraprotein
Calcium profile - Isolated calcium ^
U&E - Urea/creatinine ^
FBC - Pancytopenia
ESR
Blood film - Rouleaux formation

Skeletal survey - WBCT / MRI

  • Raindrop skull - Numerous dark spots seen on XR
  • Osteopenia
  • Osteolytic lesions
  • Pathological fractures

Diagnosis

  1. Monoclonal plasma cells in BM
  2. Monoclonal proteins within serum/urine on electrophoresis
  3. Evidence of end-organ damage - Hypercalcaemia, AKI, etc.
39
Q

Multiple myeloma management and complications

A

Chemo - Induction therapy

  • Stem-cell transplant suitable - Bortexomib + Dex
  • Stem-cell transplant NOT suitable - Thalidomide + Alkylating agent + Dex

Manage symptoms

  • Hypercalcaemia - Hydrate + Bisphosphonates
  • Renal - Hydrate
  • Hyperviscosity - VTE prophylaxis

Complications

  • Pain - Analgesia
  • Pathological fractures - Zoledronic acid
  • Infection - Vaccinations + Prophylactic Ig
  • VTE - Aspirin
  • Fatigue
40
Q

Hodgkin’s lymphoma aetiology

A

Malignant proliferation of lymphocytes
20-30
60-70

Risk factors

  • EBV
  • H.Pylori
  • Ionising radiation
  • Family history
  • T-cell disorders
41
Q

Hodgkin’s lymphoma clinical features

A 
Painless lymphadenopathy - More painful after drinking alcohol
SOB
Cough + Haemoptysis 
Hepatosplenomegaly 
B-symptoms

Investigations

  • FBC - Hb and Pt - Low
  • ESR ^
  • CXR - Mediastinal mass
  • PET-CT - Staging
  • USS + Lymph node biopsy - Reed Sternberg cells
42
Q

Hodgkin’s lymphoma staging and management

A 
Ann-Arbor classification
1. Single lymph node
2. 2 or more lymph nodes on the same side of the diaphragm
3. Nodes on either side of the diaphragm
4. Metastatic spread beyond lymph nodes
A. No systemic symptoms
B. Systemic symptoms

Management - Chemo/Radio - ABVD

  • Doxorubicin - Adriamycin
  • Bleomycin
  • Vinblastine
  • Dacarbazine
43
Q

Non-Hodgkin’s lymphoma aetiology

A

Malignant proliferation of lymphocytes
B-cells or T-cells
1/3 cases over the age of 75

Risk factors

  • Caucasian
  • History of viral infection - EBV
  • Family history
  • Certain chemical agents - Pesticides / solvents
  • Chemo/Radio
  • Immunodeficiency - Transplant, HIV, DM
  • AI disease - Sjogren’s, SLE, Coeliac
44
Q

NHL clinical features

A

Painless lymphadenopathy
Hepatosplenomegaly
Testicular mass

B-symptoms

Extranodal disease - More common in NHL

  • Gastric - Dyspepsia, dysphagia, weight loss, abdo pain
  • Bone marrow - Pancytopenia, bone pain
  • Lungs
  • Skin
  • CNS - Nerve palsies
45
Q

NHL investigations and management

A

FBC - Pancytopenia
Blood film - Nucleated RBCs + Left shift
USS + Lymph node biopsy
LDH ^

Staging - Ann-Arbor

Management - RCHOP 21 days

  • Rituximab
  • Cyclophosphamide
  • H - Doxorubicin
  • O - Vincristine
  • Prednisolone
46
Q

AML

A

Acute leukaemia in adults

Clinical features - BM failure

  • Anaemia
  • Neutropenia
  • Thrombocytopenia
  • Hepatosplenomegaly
  • Bone pain
  • Lymphadenopathy

Investigations

  • FBC - Macrocytic pancytopenia
  • Blood film - Blasts + Auer rods
  • BM biopsy - Blasts + Auer rods

Classification - French-American-British - FAB

Management - Chemo/Radio

47
Q

ALL

A

Acute leukaemia in children

Clinical features

  • Pancytopenia
  • Hepatosplenomegaly
  • Bone pain
  • Fever
  • Testicular swelling

Types

  • Common - 75%
  • T-cell only - 20%
  • B-cell only - 5%

Investigations

  • FBC - Pancytopenia
  • Blood film - Leukaemic lymphoblasts
  • BM biopsy - Lymphoblast infiltration

Management - Chemo/Radio

48
Q

CML aetiology and clinical features

A

Philadelphia chromosome!
Malignant clonal disorder of haematopoietic stem cells

Clinical features - 60-70

  • Anaemia
  • Weight loss
  • Diaphoresis
  • Splenomegaly - Abdo discomfort
49
Q

CML investigations

A

FBC

  • Anaemia
  • WCC ^
  • Thrombocytopenia

Blood film

  • Maturing myeloid cells
  • Basophils
  • Eosinophils

BM biopsy - Granulating hyperplasia

Genetic testing - Philadelphia chromosome

Management - Chemo/Radio

  • Imatinib - Tyrosine kinase inhibitor
  • Hydroxyurea
  • Interferon-Alpha
  • BM transplant
50
Q

CLL

A

Monoclonal proliferation of well-differentiated lymphocytes
Almost always B cells

Clinical features - Asymptomatic

  • Anaemia
  • Lymphadenopathy
  • Splenomegaly

Investigations

  • FBC - Anaemia + WCC ^
  • Blood film - Smudge/smear cells
  • Immunophenotyping

Management - Chemo/Radio

51
Q

DVT aetiology and clinical features

A

Clot in a major deep vein of leg, thigh, pelvis, abdomen
Impaired venous flow = Leg swelling and pain

May occur in upper extremities

  • Portal vein
  • Mesenteric vein
  • Ovarian vein
  • Retinal vein
  • Cerebral venous sinus

Clinical features

  • Calf swelling
  • Localised pain along deep venous system
  • Asymmetric oedema
  • Prominent superficial veins
52
Q

Wells score

A

Wells score

  • Active cancer - 1
  • Calf swelling > 3cm - 1
  • Prominent superficial veins - 1
  • Pitting oedema - 1
  • Swelling of entire leg - 1
  • Localised pain - 1
  • Immobilisation - 1
  • Bed rest > 3 days or surgery with GA in last 12 weeks - 1
  • Previous DVT/PE - 1
  • Alternative diagnosis more likely (-2)
53
Q

DVT investigations and management

A

Wells score
D-dimer if Wells < 2
USS if Wells > 2 or D-dimer +ve

USS not available within 4 hours - Anticoagulate - Rivaroxaban

Management - Anticoagulate - 3 months if provoked, 6 months if unprovoked

  • DOAC - Rivaroxaban
  • Renal impairment - LMWH
54
Q

Antiplatelets

A

Always assess HASBLED!

ACS/PCI

  1. Aspirin - Lifelong
  2. Ticagrelor - 12 months
  3. Clopidogrel - Lifelong

Ischaemic stroke / TIA

  1. Clopidogrel - Lifelong
  2. Aspirin - Lifelong
  3. Dipyridamole - Lifelong

PAD

  1. Clopidogrel - Lifelong
  2. Aspirin - Lifelong
55
Q

Clopidogrel

A

P2Y12 inhibitor

Prasugrel
Ticagrelor
Ticlopidine

Interactions - PPIs may alter efficacy

56
Q

DOACs

A

Dabigatran - Thrombin inhibitor - Renal excretion
Rivaroxaban - Factor Xa inhibitor
Apixaban - Factor Xa inhibitor

NICE indications

  • Prevention of VTE following hip/knee surgery
  • Treatment of DVT/PE
  • Prevention of stroke in non-valvular AF
57
Q

DIC aetiology

A

Activation of coagulation pathways
Formation of intravascular thrombi
Depletion of platelets and coagulation factors
Thrombi lead to vascular obstruction and multi-organ failure
Spontaneous bleeding may occur

Triggers

  • Sepsis
  • Major trauma
  • Obstetric complications - Amniotic fluid embolism, haemolysis, HELLP
  • Malignancy
  • Major vascular disorders
58
Q

DIC clinical features

A

Systemic signs of circulatory collapse

  • Oliguria
  • Tachycardia
  • Hypotension

Purpura fulminans, gangrene, acral cyanosis
Delirium / Coma
Petechiae, ecchymosis, oozing, haematuria

59
Q

DIC investigations

A 
Platelets - Low
PT ^
aPTT ^ 
Bleeding time ^
Fibrinogen - Low
D-dimer ^
Blood film - Shistocytes - Microangiopathic haemolytic anaemia

Management - Treat cause + Transfusion

  • Platelets
  • FFP
  • Cryoprecipitates
  • Fibrinogen concentrates
60
Q

Clotting studies interpretation

A

Warfarin

  • PT - Prolonged
  • aPTT - Normal
  • Bleeding time - Normal
  • Platelet count - Normal

Aspirin

  • PT - Normal
  • aPTT - Normal
  • Bleeding time - Prolonged
  • Platelet count - Normal

Heparin

  • PT - Prolonged
  • aPTT - Prolonged
  • Bleeding time - Normal
  • Platelet count - Normal

DIC

  • PT - Prolonged
  • aPTT - Prolonged
  • Bleeding time - Prolonged
  • Platelet count - Low
61
Q

High INR

A

Major bleeding

  • Stop Warfarin
  • Vitamin K - 5mg IV
  • Prothrombin complex concentrate
  • It not available - FFP

INR > 8

  • Stop Warfarin
  • Vitamin K - 1-5mg (PO if stable, IV if bleeding)
  • Check INR after 24 hours
  • Repeat vitamin K if necessary
  • Restart Warfarin when INR < 5

INR 5-8 + Minor bleeding

  • Stop Warfarin
  • Vitamin K - 1-3mg IV
  • Restart Warfarin when INR < 5

INR 5-8 - No bleeding

  • Withhold 1 or 2 doses of Warfarin
  • Reduce subsequent maintenance dose
62
Q

Overdose management

A

Paracetamol

  • Activated charcoal < 1 hour
  • NAC

Salicylate

  • Urinary alkalisation - IV bicarb
  • Haemodialysis

Opioid - Naloxone

Benzos - Flumazenil (seizure risk)

Tricyclics - IV bicarb

Lithium

  • Volume resus - NaCl
  • Haemodialysis

Warfarin - Vitamin K + Prothrombin

Heparin - Protamine sulphate

BBs

  • Bradycardia - Atropine
  • Resistant - Glucagon

Digoxin - Digoxin-specific antibody fragments

63
Q

Poisoning management

A

Ethylene glycol / Methanol

  • Fomepizole
  • Ethanol
  • Haemodialysis

Organophosphate insecticides - Atropine

Iron - Desferroxamine

Lead

  • Dimercaprol
  • Calcium edetate

CO - Hyperbaric 100% O2

Cyanide

  • Hydroxycobalamin
  • Amyl nitrate / Sodium nitrate / Sodium thiosulphate
64
Q

ITP

A

Immune thrombocytopenia - Well patient with thrombocytopenia

  • Autoimmine
  • Adults - Chronic
  • Children - Acute following infection or vaccination

Clinical features - Incidental finding?

  • Petichae / Purpura
  • Bleeding - Epistaxis

Investigations - Isolated platelet count < 100

Management

  • Pred PO
  • IVIG
  • Splenectomy
  • Rhesus positive - Anti-G Ig
  • Children - Platelet transfusion

Evan’s syndrome - ITP + AIHA

65
Q

TTP aetiology and clinical features

A

Thrombotic thrombocytopenic purpura - Unwell patient with thrombocytopenia

  • Antibodies against enzyme that cleaves vWF
  • Large vWF multimers leading to platelet aggregation

Clinical features - PENTAD

  1. Microangiopathic haemolytic anaemia
  2. Thrombocytopenic purpura
  3. Neuro dysfunction
  4. Renal dysfunction
  5. fever
66
Q

TTP investigations and management

A 
FBC/LFT - Haemolytic anaemia
Platelets - Low
Blood film - Shistocytes
Urinalysis - Proteinuria
Urea and creatinine ^

Management

  • Plasma exchange
  • Prednisolone PO
67
Q

Lymphadenopathy DDx

A

Infective

  • Infectious mononucleosis - EBV
  • HIV
  • Eczema
  • Rubella
  • Toxoplasmosis
  • CMV
  • TB
  • Roseola infantum

Neoplastic - Leukaemia / Lymphoma

Others

  • AI - SLE/RA
  • Graft vs host disease
  • Sarcoidosis

Drugs

  • Phenytoin
  • Allopurinol
  • Isoniazid
68
Q

EBV clinical features

A

HHV4

  1. Fever
  2. Pharyngitis
  3. Lymphadenopathy
Malaise
Palatal petechiae
Splenomegaly - Risk of rupture
Haemolytic anaemia
Maculopapular rash in patients who take amoxicillin!
69
Q

EBV investigations and management

A

FBC - Lymphocytosis + Haemolytic anaemia
EBV-specific antibodies
Monospot test - Heterophile antibodies - Non-specific
LFTs ^

Management - Supportive - Resolves in 2-4 weeks

  • Avoid sport for 8 weeks - Risk of splenic rupture
  • Airway obstruction - Pred
  • Thrombocytopenia - Pred or IVIG
70
Q

CMV aetiology

A

Beta-Herpes virus
Asymptomatic in individuals with normal immune function
Infected cells have “owls eye” appearance

Risk factors - Immunocompromise

71
Q

CMV manifestations

A

Congenital CMV

  • Growth retardation
  • Pinpoint petechial “blueberry muffin” skin lesions
  • Microcephaly
  • SN deafness
  • Encephalitis - Seizures
  • Hepatosplenomegaly

CMV mononucleosis

  • Similar to infectious mononucleosis
  • May develop in immunocompetent individuals

CMV retinitis

  • Common in HIV patients with low CD4 < 50
  • Visual impairment - Blurred vision
  • Fundoscopy shows retinal haemorrhages and necrosis - Pizza retina
  • Treatment - IV Ganciclovir

CMV encephalopathy - HIV patients with low CD4 < 50

CMV pneumonitis
CMV colitis

72
Q

CMV

Other clinical features / investigations / management

A

Fever
Malaise
N/V
Diarrhoea

FBC

  • Immunocompetent - Lymphocytosis
  • Immunocompromised - Pancytopenia

LFTs ^
CMV serology
CD4 count
CXR

Management

  • Immunocompetent - Supportive
  • Immunocompromised - IV Ganciclovir
73
Q

HIV aetiology

A 
Retrovirus
Destruction of CD4 cells
HIV1 - Global epidemic
HIV2 - Less pathogenic - Restricted to West Africa
AIDS - Develops over 10-15 years

IVDU
Unprotected sex
Needle-stick injury
Vertical transmission

74
Q

HIV investigations and management

A

HIV PCR
P24 antigen tests

Antiretroviral therapy
Counselling
Manage comorbidities

Prophylaxis - Vaccinations - Live vaccines CI

  • Pneumococcal
  • Meningococcal
  • Influenza
  • Hep B
  • HPV
  • DTP
75
Q

HIV vertical transmission

A

25-30%

Factors to reduce risk of transmission…

  • Maternal ART
  • Bottle feeding

Method of delivery

  • Viral load < 50 - Vaginal
  • Viral load > 50 - CS + Zidovudine infusion 4 hours prior

Neonatal ART

  • Maternal viral load < 50 - Zidovudine PO
  • Maternal viral load > 50 - Triple ART for 4-6 weeks
76
Q

Malaria aetiology and protective factors

A
  1. Plasmodium falciparum
  2. Plasmodium vivax - Benign
  3. Plasmodium ovale - Benign
  4. Plasmodium malariae - Benign

Protective factors

  • Sickle-cell trait
  • G6PD deficiency
  • HLA-B53
  • Absence of duffy antigens

Risk factors - Travel to endemic areas - Tropics

77
Q

Malaria clinical features and investigations

A 
Fever > 39
Headache
Weakness
Myalgia
Arthralgia
Anorexia
Diarrhoea
Blood film - Schizonts
Rapid diagnostic tests - RDTs
FBC - Anaemia and thrombocytopenia
BM - Hypoglycaemia
ABG - Acidosis
78
Q

Malaria management and complications

A

Chloroquine

Complications

  • Cerebral malaria - Seizures/coma
  • Acute renal failure
  • ARDS
  • Hypoglycaemia
  • DIC

3b (23 decks)

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