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FACEM Written > Haem/Onc > Flashcards

Haem/Onc Flashcards

1
Q

Haemophilia

Treatment

A
  1. Analgesia + Avoid NSAIDS, aspirin, IM injections
  2. Joints - protect + RICE
  3. Factor replacement is KEY
    a. 30-100units/kg depending to location, severity of bleed
  4. TXA 15mg/kg
  5. Activated PCC (II, VII, IX, X)
    a. If pt has developed inhibitors
  6. Cryo (VIII, XIII and VWF + fibrinogen)
  7. FFP (all factors + fibrinogen)
  8. Factor VII (VII + vWF)
  9. DDAVP - if mild (factor levels > 5%)
    a. Restores Factor VIII + VWF into circulation
    b. 0.3mcg/kg IV
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2
Q

Haemophilia Severity grading

A

Severity grading

Severe <1% factor

  • Frequent bleeding, esp joints + muscles

Mod 1-5% factor

  • can bleed after minor injury, severe bleeds with mod trauma + surgery

Mild >5-40% factor

  • bleeding with major trauma / surgery
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3
Q

Haemorrhage Risk Score

HAS BLED

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4
Q

Orbit Score

Haemorrhage risk whilst taking Anti-coagulants

A

https://www.mdcalc.com/calc/10227/orbit-bleeding-risk-score-atrial-fibrillation

Not statistically superior to HASBLED but validated for DOACs

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5
Q

Acquired Haemophilia

A
  • Malignancy
  • Pregnancy / Post partum
  • Autoimmune
  • Drugs
  • Idiopathic
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6
Q

HUS

A

OVERVIEW
Triad of MAHA + ARF + Thrombycytopaenia
Clinically similar to TTP but ARF > neuro symptoms

TYPES OF HUS
* Diarrhoea-associated (D+ HUS) - >90%
* Preceding infection with a verocytotoxin-producing bacteria, typically E.coli O157:H7
* Non-diarrhoeal/ atypical (D- or aHUS) - rare
* poorer prognosis / familial / complement dysregulation
* Pregnancy (term or post-partum)
* Drugs – quinine, mitomycin, ciclosporin, tacrolimus, ticlopidine, caboplatinum, gemcitabine
* Transplantation – renal, haematological stem cell transplantation
* HIV
* Combined methylmalonic aciduria and homocystinuria (a vitamin B12 metabolism disorder)
* Strep. pneumoniae associated (P-HUS)
* Anti-T immunoglobulin M (IgM) in the serum reacts with the antigen on red blood cells, platelets, and glomeruli causing hemolysis, thrombocytopenia and renal damage

Classic pentad: (FAT R/N)
Common presentation includes bloody diarrhoea +/- crampy AP, fever +/- vomiting

  • Fever
  • Anaemia (microangiopathic haemolytic)
  • Thrombocytopenia
  • Renal problems (88% have renal problems, 15% haematuria) – more likely in HUS
  • Neurological problems (headaches, confusion, seizures) – more likely in TTP

Examination
Shock - hypotension, tachycardia, poor CRT, cool peripheries
Dehydration - fontanelle, mucous membranes, skin turgor
Overload - oedmea, hypertension
CNS - agitation, confusion, ALOC

Ix
MAHA + ARF + thrombocytopaenia
* Bloods
* FBC - anaemia
* Blood film - shistocytes
* Low PLT
* Increased reticulocytes
* Low haptoglobins
* Increased LDH
* High Br (unconj), urinary urobilinogen
* Increased urea and creatinine levels (greater in HUS)
* CMP
* LFTs
* Coombs +ve
* VBG
* Urine and Stool MC+S (E coli)

Management
* Supportive care and monitoring are the mainstays
* Avoid nephrotxins
* ABx may worsen toxin production
* HTN - trial duiretics + vasodilatory agents
* PLT contra-indicated unless requiring surgery
* Plasmapheresis
* may worsen P-HUS
* Rx underlying cause and complications
* No good evidence for steroids, heparin, aspirin, antioxidants, fluid restriction or diuretics, anti-hypertensives
* RRT (acidosis, fluid overload not responding to duiresis, high K or uraemia)

Complications
AKI - oliguria or anuria, hypertension and hyperkalaemia
Cardiac - hypertensive cardiomyopathy / myocarditis, APO
CNS - seizures, coma, cortical blindness
Coagulopathy - bleeding
GI - severe colitis +/- bleeding, bowel necrosis or perforation, peritonitis, intussusception, pancreatitis

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7
Q

Tumour Lysis Syndrome

A

TLS occurs when destruction of malignant cells occurs too quickly for homestatic mechanisms to cope

Spontaneous OR associated with ALL, NHL and Burkitts most commonly

NB as chemo regimes get better, may see more TLS

Hallmarks

  • LOW Ca
  • HIGH uric acid, PO4, K
  • AKI from CaPO4 or uric acid crystals in kidneys

Mx
* Hydration
* 0/9% saline or 1/2 saline
* Aim UO > 1ml/kg/hr
* Alkalinise urine
* If metabolic acidosis present
* Aim pH > 7.0
* Stop when urine pH >8.5
* Hyperuricaemia
* Allopurinol 10mg/kg
* Rasburicase 0.2mg/kg
* CI in G6PD - triggers haemolytic crisis
* Complications: MetHb
* Electrolyte Mx
* Hyperkalaemia Rx
* DO NOT replace Calcium unless cardiac or neuro Sx
* PO4 binders
* Dialysis or RRT if renal failure, uncontrolled electrolytes despite conservative measures

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8
Q

Superior Vena Cava Obstruction

A

SVC susceptiple to compression as thin walls and low pressure

  • Extrinsic
    • Malignancy -Lung, Lymphoma
    • Goitre
  • Intrinsic
    • Thrombus
    • Device related - CVC / PPM
    • Post radiation fibrosis
    • Fungal infections

Signs

  • Life-threatening
    • Tracheal obstruction
    • Cerebral oedema => inc ICP + altered mental status
    • Haemodynamic collapse
  • Other Sx
    • Facial oedema, arm swelling
    • Dyspnoea, voice change, cough
    • Dysphagia
    • Headache worse with bending forward
    • Dizziness / syncope
  • Pemberton’s sign - After lifting hands in the air for one minute => dvpt of facial plethora/ cyanosis +/- inspiratory stridor +/- non-pulsatile elevation of the JVP - indicates thoaric inlet obstruction

Radiology

  • CXR
    • UL lesion - Pancoast tumour
    • Widened mediastinum
    • Promiment right hilar nodes
    • Aortic nipple (enlarged left sup intercostal vein)
  • CT - contrast

Mx

  • Supportive
    • O2
    • Head up 30 degrees
    • Dexamathasone
  • Thrombolysis if indicated
  • DXT
  • SVC Stent
  • Tissue diagnosis + Chemotherapy
  • Surgery
    *
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9
Q

MTP

A

Definition

  • >1 blood volume in 24 hrs
  • > 50% blood volume in 4 hrs
  • Child: >40mL/kg

Adult BV = 70ml/kg and Child BV = 80ml/kg

Goals:

  • Restoration of tissue perfusion and O2 carrying capacity via blood volume and Hb
  • Avoid hypothermia, coagulopathy, acidosis and hypocalcaemia
  • Definitive care - IR or surgery

Parameters

  • Temp > 35.0
  • pH > 7.2, BE <-6, Lactate < 4mmol/l
  • Ion Ca >1.1mmol/l
  • Hb - interpret clinical
  • PLT >50 x 109 and >100 x 109 if HI or ICH
  • PT/aPTT <1.5 x normal
  • Fibrinogen >1.0g/L
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10
Q

Complications of transfusion

A
  1. Febrile Non-haemolytic transfusion reaction
  2. TACO
  3. TRALI
  4. Acute haemolytic transfusion reaction
  5. Anaphylaxis
  6. Transfusion related bacterial infection/sepsis
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11
Q

Haemolytic anaemias

Increased - LDH, Br (unconj), Reticulocytes
Decreased - Haptoglobin
Blood film - Schistocytes
Methhaemalbumin = severe intravascular haemolysis

A

**Classification of causes **
* RBC Defects
* Membrane
* Hereditary spherocytosis, H Elliptocytosis
* Haemoglobinopathy - Sickle, Thalassaemia
* Enzyme Deficiency - G6PD, pyruvate kinase
* Other
* Autoimmune - 95% positive Coomb’s test
* ABx - penicillins, cephalosporins
* ABO incompatibility
* SLE, RA, UC
* Malignancy - lymphoma, CLL
* Mechanical - prosthetic valve, runners

Classification by site of haemolysis
* Intravascular
* MAHA
* March
* Chronic cold agglutinin
* PNH
* Extravascular
* Autoimmune
* Rh Incompatibility
* Chronic haemolytic dsorder

Cx

Pigment gallstones

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12
Q

Haemolytic anaemia - Bloods

A

Inc. Reticultocytes

Inc Br

Inc LDH

Dec Haptoglobin

MetHb if severe

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13
Q

Jaundice DDx

A
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14
Q

Hereditary Spherocytosis

A

Hx - episodic jaundice, neonatal jaundice

FHx - jaunduce, splenectomy, early cholcystectomy

OE - jaundice, splenomegaly

Bloods - dec Hb, inc retic, -ve Coombs, +ve Osmotic fragility (membrane)

Mx

Evaluate for fever, illness

May need blood

Splenectomy if severe

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15
Q

G6PD

A

Intermittent haemolysis + oxidative stress

X-linked (boys)

Risk

Fava bean, moth balls

Drugs - sulfa drugs, dapsone, nitrofurantoin, anti-malarials

Mx

Stop offending agent

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16
Q

Post Splenectomy

A

Encaspulated bacteria

  • HiB, S.pneumo, N. meningitidis

Need pre-op vaccines, post penicillin

Prophylaxis until age 5 - highest risk before 5 yrs old

17
Q

Thrombotic Thrombocytopaenic Purpura

A

One of the MAHA syndromes
Rapidly progressive, 90% mortality without treatment

Deficiency of ADAMTS13 enzyme causes
- abN platelet clumping
- abN formation of large multimers of VWF
- extensive microthrombi + haemolysis + end organ effects

Hereditary vs acquired

Epi
1:100,000-1:500,000
F>M 2:1
Most common betw 30-40yrs, 90% <60 years old

Sx/Signs
* Classic pentad (FATRN)
CNS Sx in 75%
* Headache, AMS, focal deficits, seizures, fluctuating symptoms and signs
Bleeding 45%
* Usually cutaneous
GI in 40%
* N/V/AP
Fever 20%

Bloods
FBC
* Shistocytes, increased reticulocytes
* Anaemia, thrombocytopaenia
Haemolysis
* Coomb’s negative
* Dec haptoglobins
* Inc LDH, Br (unconj), urobilinogen
Coags
- Normal Fibrinogen, D-dimer, coags
UECs
- May have AKI
ADAMST13 antibodies
Decreased ADAMST13 activity
* - < 10% severe deficiency - supports TTP
* 10-20% - maybe
* >20% TTP unlikley

Mx
ICU/Haem referral
Plasmapheresis - removes vWF and ADAMST13 multimers
Plasma exchange with FFP replacement
Corticosteroids 1mg/kg/day
Monoclonal antibody
PLT transfusion - only if life threatening bleeding

Poor prognosis:
GCS < 14
Elevated troponin
High levels of ADAMST13 antibodies
Low levels of ADAMST13 activity

18
Q

Causes MAHA
(DIC, HUS, TTP, malignant HTN, Malignancy, VICC, PET, HELLP)

A

Thrombocytopaenia

Anaemia

Fragmentation of RBCs - Shistocytes

Elevated LDH

Elevated reticulocytes

Elevated Br

True vasculitis absent

19
Q

Febrile Neutropaenia

A

FEBRILE to 38.3 or >38.0 for 1hr +

ABSOLUTE NEUTROPHIL COUNT <0.5

Timeline: 3-14 days post chemo

20
Q

Fever in the cancer patient

A
  • Infection (30%)
  • DVT/PE
  • Chemo / medication
  • Direct tumour burden
  • Transfusion reaction
21
Q

Pericardial Effusion in the Cancer pt

A
  • Malignancy (lung, Breast, Haem, Melanoma)
  • Hypoalbuminaemia
  • Radiation
  • Chemotherapy
22
Q

Hypercalcaemia

A

Serum levels >3mmol/l symptomatic

Calc Correction for low albumin

Corr = Total + 0.02 (40 - albumin)

Sx: Stones, Bones, Moans, Groans

Signs: ECG - short QT interval

Mx

Excretion

  • IVF
  • LOOP diuretic (thiazide makes worse)
  • Dialyse - refractory + CHF/fluid overload

Osteoclast inhibition (dec Ca release)

  • Bisphosphonate - Pamidronate 90mg IV
  • Calcitonin - PRN for cardiac dysrhythmias - takes 24-48 hrs to work
23
Q

Cancer presentations DDx to consider

A

Cancer +

  1. Fever
  2. SOB - effusion, SVC, PE
  3. AMS - raised ICP, hyperviscosity syndrome
  4. ARF - post-renal failure, tumour lysis
  5. BP - spinal mets
  6. AP - necrotising enterocolitis
24
Q

Febrile Neutropaenia Antibiotic Guidelines

A
  • Start antibiotics ASAP
  • Wide spectrum covergage with pip-tazo, meropenem or imipenem
  • Add vancomycin - hypotension, suspected line sepsis, mucositis, a history of MRSA, or recent antibiotic use
  • Add aminoglycoside - septic shock +/- suspected/ proven antibiotic resistance
  • Add IV acyclovir - herpetic infection or encephalopathy
  • Consider anti-fungal - fever >4 days
25
Q

Febrile Neutropaenia Risk Stratification

MASCC vs CISNE

The MASCC Score will identify more patients as low risk, but will have more treatment failures / bounce-backs than the CISNE score

A
26
Q

Neutropaenia causes - general

A
  • Overconsumption
    • Sepsis
    • Autoimmune disease (SLE, rheumatoid arthritis, etc)
  • Underproduction by bone marrow
    • Malnutrition – alcoholism, anorexia, etc
    • Myelodysplastic syndrome
    • Post-viral: varicella, measles, rubella, influenza, hepatitis, Epstein-Barr virus, HIV
    • Drug induced: clozapine, methimazole, sulfasalazine, bactrim, b-lactam antibiotics, NSAIDs, ticlopidine, cephalosporins, chemotherapy
27
Q

Pemberton’s sign

A

After lifting hands in the air for one minute => dvpt of facial plethora/ cyanosis +/- inspiratory stridor +/- non-pulsatile elevation of the JVP - indicates thoracic inlet obstruction

28
Q

Transfusion related-graft vs host diease

A

RISK = haem malignancy + non-irradiated blood products

29
Q

Coag Interpretation

A
30
Q

DIC causes

A
  • Sepsis - Gram -ve, Viral Haemorrhagic fevers
  • Hepatic failure
  • Obstetric - PET, HELLP, Amniotic fluid embolus, IU foetal death
  • Trauma - Massive blood loss, major HI, rhabdo
  • Malignancy - prostate, mucinous, ac promyelocytic leukaemia
  • Immune - T/F rxn, anaphylaxis, transplant rejection
  • Shock
  • Snake envenomation

Other - pancreatitis, large vessel aneurysm, giant haemangiomas, connnective tissue disorders i.e. APL syndrome

31
Q

Sickle Cell Complications

A
32
Q

DVT RF

A
  1. External Compression - pregnancy, masses
  2. Vessel wall - trauma, surgery, vasculilitis
  3. Intra-luminal - FB, lines, prosthetic valves
  4. Blood
    a. Stasis - immobility, travel, POP, obese, AF
    b. Hormones - OCP, pregnancy (up to 12/52 post)
    c. Hyperviscosity
    d. Smoking
    e. DM
    f. Active mlignnacy
    g. Nephrotic syndromes
    f. Defects of haemostasis - Plt, coags, APL, FV Leiden
33
Q

PERC Rule out Criteria

A
34
Q

Wells score for DVT

A

< 1pt - 62% pts - 3-9% have DVT
>2pts - 39% pts - 20-35% have DVT

35
Q

Pancytopaenia

A
36
Q

Von Willebrand Disease

A

Most common inherited bleeding disorder
vWF is a glycoprotein, when activated binds to PLT glycoproteins -> PLT adhesion

Type I - dec production vWF glycoprotein + low FVIII
Type II - dysfunctionsl vWF
Type III - no vWF + low FVIII

Clinical presentation
Easy bruising
Skin and mucosal bleeding

Ix
Normal PLT, coags, INR

Mx
Desmopressin
vWF concentrates

37
Q

Topical Haemostatic agents

A
38
Q

ITP

A
39
Q

Haemolysis Causes

A

FACEM Written (34 decks)

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