NEW HAEM 2 Flashcards
Multiple myeloma aetiology
Malignant proliferation of plasma cells
Increased production of Ig - Paraproteins
Accumulation in the renal system - Bence-Jones proteins
Multiple myeloma clinical features
CRAB-E
Calcium - Hypercalcaemia - Increased osteoclast activity
- GROANS
- STONES
- MOANS
- THRONES
- BONES
Renal (AKI) - Light chain deposition within renal tubules - Bence-Jones protein
- Dehydration
- Increasing thirst
Anaemia - Pancytopenia
Bones - BM infiltration by plasma cells + Cytokine mediated osteoclasts
- Lytic bone lesions
- Back pain
- Fragility fractures
- Raindrop skull
ESR ^ - Increased risk of VTE
Multiple myeloma investigations
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
Multiple myeloma diagnosis
- Monoclonal plasma cells in BM
- Monoclonal proteins within serum/urine on electrophoresis
- Evidence of end-organ damage - Hypercalcaemia, AKI, etc.
Multiple myeloma management
Induction therapy
BM transplant
Manage symptoms
- Hypercalcaemia - Hydrate + Bisphosphonates
- Hyperviscosity - VTE prophylaxis (Aspirin)
- Renal - Hydrate
- Infections - Vaccinations + Ig
- Fatigue - EPO
- Pain - Analgesia
Hodgkin’s lymphoma aetiology
Malignant proliferation of lymphocytes
20-30
60-70
Risk factors
- Family history
- T-cell disorders
- EBV
- H.Pylori
- Ionising radiation
Hodgkin’s lymphoma clinical features and investigations
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
Hodgkin’s lymphoma staging and management
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
Non-Hodgkin’s lymphoma aetiology
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
NHL clinical features
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
NHL investigations and management
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
AML
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
ALL
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
CML aetiology and clinical features
Philadelphia chromosome!
Malignant clonal disorder of haematopoietic stem cells
Clinical features - 60-70
- Anaemia
- Weight loss
- Diaphoresis
- Splenomegaly - Abdo discomfort
CML investigations and management
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
CLL
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
DVT clinical features
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
Wells score
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)
DVT investigations and management
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
Antiplatelets
Always assess HASBLED!
ACS/PCI
- Aspirin - Lifelong
- Ticagrelor - 12 months
- Clopidogrel - Lifelong
Ischaemic stroke / TIA
- Clopidogrel - Lifelong
- Aspirin - Lifelong
- Dipyridamole - Lifelong
PAD
- Clopidogrel - Lifelong
- Aspirin - Lifelong
DIC aetiology
Activation of coagulation pathways
Intravascular thrombi
Platelet and clotting factors depleted
Thrombi = 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
DIC clinical features
Systemic signs of circulatory collapse
- Oliguria
- Tachycardia
- Hypotension
Purpura fulminans, gangrene, acral cyanosis
Delirium / Coma
Petechiae, ecchymosis, oozing, haematuria
DIC investigations and management
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
High INR
Major bleeding
- Stop Warfarin
- Vitamin K - 5mg IV
- Prothrombin complex concentrate
INR > 8
- Stop Warfarin
- Vitamin K - 1-5mg (PO if stable, IV if bleeding)
- Check INR after 24 hours
- 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
Overdose management
Paracetamol Salicylate Opioid Benzos Tricyclics Lithium Warfarin Heparin BBs Digoxin
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
Poisoning management
Ethylene glycol Organophosphate Iron Lead CO Cyanide
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
ITP
Evan’s syndrome
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
- Children - Platelet transfusion
Evan’s syndrome - ITP + AIHA
TTP aetiology and clinical features
Thrombotic thrombocytopenic purpura - Unwell patient with thrombocytopenia
- Antibodies against enzyme that cleaves vWF
- Large vWF multimers leading to platelet aggregation
Clinical features - PENTAD
- Microangiopathic haemolytic anaemia
- Thrombocytopenic purpura
- Neuro dysfunction
- Renal dysfunction
- Fever
TTP investigations and management
FBC/LFT - Haemolytic anaemia Platelets - Low Blood film - Shistocytes Urinalysis - Proteinuria Urea and creatinine ^
Management
- Plasma exchange
- Prednisolone PO
Lymphadenopathy DDx
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
EBV clinical features
HHV4
- Fever
- Pharyngitis
- Lymphadenopathy
Malaise Palatal petechiae Splenomegaly - Risk of rupture Haemolytic anaemia Maculopapular rash in patients who take amoxicillin!
EBV investigations and management
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
CMV aetiology
B-Herpes virus
Asymptomatic if normal immune function
Infected cells have “owls eye” appearance
Risk factors - Immunocompromise
CMV manifestations
Congenital CMV
- Growth retardation
- Pinpoint petechial “blueberry muffin” skin lesions
- Microcephaly
- SN deafness
- Encephalitis - Seizures
- Hepatosplenomegaly
CMV mononucleosis
CMV retinitis
- HIV patients with low CD4 < 50
- Blurred vision
- Fundoscopy - Retinal haemorrhages and necrosis - Pizza retina
- Treatment - IV Ganciclovir
CMV encephalopathy - HIV patients with low CD4 < 50
CMV pneumonitis
CMV colitis
CMV
Other clinical features / investigations / management
Fever
Malaise
N/V
Diarrhoea
FBC
- Immunocompetent - Lymphocytosis
- Immunocompromised - Pancytopenia
LFTs ^
CMV serology
CD4 count
CXR
Management
- Immunocompetent - Supportive
- Immunocompromised - IV Ganciclovir
HIV aetiology
Retrovirus
Destruction of CD4 cells
AIDS - Develops over 10-15 years
IVDU
Unprotected sex
Needle-stick injury
Vertical transmission
HIV investigations and management
HIV PCR
P24 antigen tests
Antiretroviral therapy
Counselling
Manage comorbidities
Prophylaxis - Vaccinations - Live vaccines CI
- Pneumococcal
- Meningococcal
- Influenza
- Hep B
- HPV
- DTP
HIV vertical transmission
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
Malaria aetiology and protective factors
- Plasmodium falciparum
- Plasmodium vivax - Benign
- Plasmodium ovale - Benign
- Plasmodium malariae - Benign
Protective factors
- Sickle-cell trait
- G6PD deficiency
- HLA-B53
- Absence of duffy antigens
Risk factors - Travel to endemic areas - Tropics
Malaria clinical features and investigations
Fever > 39 Headache Weakness Myalgia Arthralgia Anorexia Diarrhoea
Blood film - Schizonts Rapid diagnostic tests - RDTs FBC - Anaemia and thrombocytopenia BM - Hypoglycaemia ABG - Acidosis
Malaria management and complications
Chloroquine
Complications
- Cerebral malaria - Seizures/coma
- Acute renal failure
- ARDS
- Hypoglycaemia
- DIC
Haemophilia aetiology and presentation
X-linked recessive
A - Factor 8 deficiency
B - Factor 9 deficiency
Neonates
- IC haemorrhage
- Bleeding post-circumcision
- Heel-prick oozing
Bleeding
- Haemarthrosis
- Post-dental
- Post-surgery
- Haematomas
Haemophilia investigations and management
aPTT ^
Everything else = Normal
Management
- A - Factor 8
- B - Factor 9
- FFP
Avoid NSAIDS
VWD aetiology and pathophysiology
AD
VWF
- Promotes platelet adhesion to damaged endothelium
- Factor 8 CARRIER
- Partial reduction
- Abnormal form
- Total lack - AR inheritance
VWD presentation / investigations / management
Bleeding
Bruising
Epistaxis
Bleeding time ^
APTT ^
Factor 8 - LOW
Desmopressin
Tranexamic acid if bleeding
Factor 8
HIV clinical features
Seroconversion
- Sore throat
- Lymphadenopathy
- Malaise, myalgia, arthralgia
- Diarrhoea
- MP rash
- Mouth ulcers
HIV complications
Oral thrush
Shingles
Hairy leukopenia
Kaposi’s sarcoma
Cryptosporidosis Toxoplasmosis Progressive multifocal leukoencephalopathy Pneumonia - Pneumocystitis jiroveci HIV dementia
Aspergillosis
Oesophageal candidiasis
Cryptococcal meningitis
Primary CNS lymphoma
CMV retinitis
