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Finals!!! > Haematology > Flashcards

Haematology Flashcards

1
Q

What is pancytopoenia?

A
  • Anaemia
  • Neutropoenia (infections)
  • Thrombocytopoenia (clotting)
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2
Q

Key features of aplastic anaemia

A
  • = Rare stem cell disorder
  • Causes- Idiopathic (60%), inherited, drugs (chemo, gold, penicillamine), viruses (hepatitis, parvovirus), SLE
  • Presentation- pancytopoenia
  • Ix- BM= hypocellular
  • Tx:
    • Supportive- transfusion, Tx sepsis
    • Immunosuppression
    • Allogenic BMT
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3
Q

Causes of thrombocytopoenia

A
  • Reduced production- liver disease, BM failur e
  • Increased destruction- hypersplenism, liver disease, AI, HIV, Hep C, ITP
  • Increased consumption- HELLP, DIC, TTP
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4
Q

Key features of AML

A
  • Adults
  • Genetic- 8,21
  • RF: Down’s, male, chemo/radio to BM, myelodypslastic syndrome
  • Presentation:
    • BM failure, cytopoenias
    • Infiltrates- gum, skin, bones, hepatosplenomegaly
  • Ix:
    • Bloods- anaemia, thrombocytopeonia, high WCC (blasts)
    • BM aspirate- >20% blasts, Auer Rods
  • Tx: supportive, chemo, BMT
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5
Q

Key features of ALL

A
  • Childen
  • RF: Genetic (12,21 translocation), radiation, Down’s
  • Presentation:
    • Pancytopoenia
    • Infiltration- lymphadenopathy, orchidomegaly, thymic englargerment, CNS palsies/ meningism, bone pain
  • Ix:
    • Bloods- ++ WCC (lymphoblasts), low RBC, low platelets
    • Imaging- CXR/ CT- mediastinal + abdo LN
    • BM aspirate >20% blasts
    • LP- CNS involvement
  • Tx:
    • Supportive- Blood products, allopurinol, Tx infections (gent, taz)
    • Chemo
    • BMT
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6
Q

Key features of CML

A
  • Usually 40-60y.
  • Philidelphia chromosome- translocation of 9,22 –> BCR-ABL. Tx= Imatinib
  • Features:
    • Systemic- weight loss, fever, night seats, lethargy
    • Massive HEPATOSPLENOMEGALY
    • Bruising/ bleeding
    • Gout
    • Hyperviscosity
  • 3 phases: chronic, accelerated, blast crisis
  • Ix:
    • Bloods- ++WCC, high urate, +/- low Hb/platelets
    • BM cytogenic analysis
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7
Q

Key featuresof CLL

A
  • V insidious. May be incidental finding in elderly.
  • B memory cells.
  • Presentation:
    • Often Asx
    • Symmetrical painless lymphadenopathy
    • Hepatosplenomegaly
    • Anaemia
    • ?B Sx- weight loss, fever, night sweats
  • Ix:
    • Bloods- ++ WCC, low serum Ig.
    • Smear cells on blood film.
  • Complications:
    • AI haemolysis
    • Infection
    • BM failure
    • Richter transformation- CLL –> large B cell lymphoma
  • Tx- usually supportive –> chemo/ radio. Rituximab, cyclophosphamide
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8
Q

Key Features of Hodgkin’s Lymphoma

A
  • M:F 2:1. Bimodal age: 20-29y, >60y
  • May be associated with EBV
  • Presentation:
    • Lymphadenopathy- Painless (painful w/ alcohol), asymmetrical, spread to adjacent nodes, cervical 70%, mediastinal –> masse effect
    • BSx- Fever, weight loss (>10% 6m), night sweats
    • Itch
    • Hepatosplenomegaly
  • Ix:
    • Bloods- FBC, film, ESR, LFTs, LDH, calcium
    • LN biopsy- Reed-sternberg cells (owl eye)
    • Staging- CT/MRI chest-abdo-pelvis
    • BM biopsy if B Sx or stage 3/4
  • Tx- chemo/ radio –> BMT
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9
Q

Ann Arbor Staging

A
  1. Single LN region
  2. >1 LN region on one side of diaphragm
  3. Nodes on both sides of diaphragm
  4. Spread beyond nodes eg liver, BM
  • A= if no B Sx, B if B Sx
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10
Q

Key Features of Non-Hodgkins Lymphoma

A
  • Presentation:
    • Lymphadenopathy - symmetrical, painless, spreads discontinually, multiple sites
    • Splenomegaly
    • BSx
    • Oropharynx
    • CNS
    • Skin - T cell lymphoma
  • Ix:
    • Bloods- pancytopoenia, hyperviscosity, U+Es, LFTs, LDH, film
    • LN + BM biopsy
    • Staging- CT/MRI. Ann Arbor
  • Classification:
    • B/T Cell
    • Low grade- follicular, small cell lymphocytic, marginal zone
    • High grade- aggressive, diffuse large B cell, Burkitts (jaw/abdo)- stary sky appearance
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11
Q

When to refer a LN

A
  • 1 LN >1cm for >6w
  • Widespread LN >2 non-contiguous areas
  • <6w but with B Sx
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12
Q

What is Multiple Myeloma and how might it present?

A
  • Neoplasm of plasma cells- make Abs –> ++ IgG/A
  • –> may produce free light chains (renal failure) and IL-6 (activate osteoclasts)
  • Features= CRAB
    • Calcium - high
    • Renal impairment - light chains/ casts
    • Anaemia - normocytic, normochromic
    • Bony lytic lesions- bone pain, fracture, hypercalcaemia, high alk phos
  • Pre-existing MGUS- high total protein, low albumin
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13
Q

Ix and Tx of multiple myeloma

A
  • Ix:
    • Bed- urine bence jones protein
    • Bloods- FBC, film (rouleaux, ++ Plasma cells), ++ESR, U+Es, calcium, normal ALP, serum electrophoresis
    • Imaging- skeletal survery- ?lytic lesions - punched ou, fractures
  • Tx:
    • Supportive- bone pain (analgesia, bisphosphonates), anaemia, renal, Infection
    • Complications- Ca2+, cord compression, hyperviscosity (plasmapheresis)
    • Specific- Chemo, BMT
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14
Q

What are the myeloproliferative neoplasms and their key features?

A
  • BM produces ++ Hb, WCC and platelets –> THROMBOSIS RISK
  • Polycythaemia Vera- all cell lines. 95% JAK2 mut
    • Primary or secondary - smoking, alcohol, high altitude, EP secreting tumour
    • Ix- blood film, FBC, USS
    • Tx- aspirin, venesection, hydroxycarbamide
  • Essential thrombocytosis- ++ platelets only. Blood film. Tx <60y= aspirin >60y= hydroxycarbamide
  • Myelofibrosis= proliferation of whole BM –> fibrotic and ineffective –> splenomegaly. Presentation- sweats, splenomegaly, cytopoenias. Blood film= RBCs tear shaped. Tx- no cure. Supportive
  • CML
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15
Q

Measurement of intrinsic and extrinsic clotting pathways and their clotting factors?

Vit K dependent clotting factors

A
  • Intrinsic pathway= APTT. Factors 4, 8, 11, (12)
    • APTT corrects= intrinsic
    • APTT doesn’t correct= coagulant inhibitor eg lupus anticoagulant
  • Extrinsic pathway= PT. Factor 7
  • Vit K dependent clotting factors - 2,7,9,10
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16
Q

Key features of Haemophilia A

A
  • VIII deficiency. <1%= severe, 1-5%= Mod, >5%= mild
  • Presentation:
    • Swollen joints –> joint damage
    • Bruise easily
  • Tx:
    • Cons- education, ice, physio, immobilisation
    • Med- injections of recombinant VIII, DDAVP, analgesia, desmopressin
    • Avoid aspirin, NSAIDs, heparin
17
Q

Key features of haemophilia B

A
  • IX deficiency. ‘Christmas Disease’
  • X-linked
  • Presentation- less severe and later than Haemophilia A
  • Tx= IX concentrate
18
Q

Key features of acquired haemophilia

A
  • AutoAb against factor VIII suddenly appears –> big mucosal bleeds
  • Ix: raised APTT and AutoAb against VIII
  • Tx: Steroids
19
Q

Key features of Von Willebrand Disease

A
  • VWF carries VIII around –> raised APTT
  • Presentation= mild bleeding disorder- bruising, mucocutaneous bleeding, bleeding from mild wounds, menorrhagia, PPH, bleeding after surgery.
  • Classification- mild (dominant), qualitative, severe (recessive)
  • Tx:
    • Desmopressin
    • DDAVP
    • Plasma containing VWF
20
Q

Aquired bleeding disorders

A
  • Liver disease - less production of coag factors
  • Renal disease - abnormal platelet function
  • Vitamin K def - haemorrhagic disease of newborn
  • DIC - sepsis, malig, trauma, obstetric
21
Q

DIC - causes, presentation, Ix and Tx

A
  • = Dissemintate intravascular coagulation.
  • Massive coagulation signal –> coagulation and consumption of clotting factors and platelets –> bleeding an thrombosis in differnt parts of circulation
  • Causes- sepsis, malignancy, trauam, obstetric
  • Dx- low platelets, low fibrinogen, high PT/ APTT and d-dimer
  • Tx- underlying cause! resus. Blood replacement. ?heparin
22
Q

Risk factors for thrombosis

A
  • Arterial- smoking, HTN, hyperlipidaemia, DM
  • Venous- surgery, trauma, immobility, pregnancy, OCP, HRT, age, obesity, varicose veins, diseases - HF, malignancy, IBD, nephrotic syndrome
  • Inherited:
    • Factor V leiden (protein C resistance)
    • Prothrombin gene mutation
    • Protein C and S deficiency
    • Antithrombin deficiency
23
Q

Symptoms of anaemia

A
  • Fatigue
  • SOB
  • Faintness
  • Palpitations
  • Tinnitus
24
Q

Ix of anaemia

A
  • FBC - Hb, MCV
  • Haematinics - B12, folate, ferratin
  • Reticulocytes - increased if anaemic + normal BM. Low in cancer/ B12/folate deficiency
  • Iron:
    • Ferratin- low in iron deficiency. high in acute phase reaction
    • Total iron binding capacity- ability to bind blod with transferrin. Raised in iron deficiency
    • Transferrin sats
    • Serum iron - ?haemachromatosis
25
Q

Causes of microcytic anaemia

A
  • Loss - Menorrhagia, GI bleeding, thallassaemia
  • Reduced intake - diet
  • Malabsorption - Crohn’s, coeliac
  • Ix –> FBC, haematinics, upper and lower endoscopy
26
Q

Key features of thallassaemia

A
  • Haemolysis whilst still in BM –> removed by spleen
  • Beta worse than alpha
  • Presentation:
    • Severe anaemia
    • Jaundice
    • FTT
    • Hepatoslenomegaly
  • Tx- transusions, SC desferroxamine, BM transplant
27
Q

Causes of Normocytic anaemia

A
  • Poor diet
  • Inflammation/ chronic disease
  • Multiple Myeloma
  • Renal failure
28
Q

Causes of macrocytic anaemia + Ix

A
  • B12/ folate deficiency
  • Alcohol excess
  • Pernicious anaemia - AI gastritis. Increased risk of gastric Ca
  • Thyroid dysfunction
  • Pregnancy
  • Cytotoxics eg trimethoprim
  • Ix- blood film (alc= target cells, B12/folate= hypersegmented/ oval macrocytes), LFTs, TFTs, serum B12, red cell folate. ?BM biopsy
29
Q

Key features of folate deficiency

A
  • Causes:
    • Reduced intake - diet: green veg, nuts, liver
    • Increased demand - Pregnancy, haemolysis, malignancy
    • Malabsorption - coeliac, crohn’s,
    • Drugs- alcohol, phenytoin, methotrexate
  • Tx:
    • Tx cauase
    • B12 replacement 1st (SACD)
    • Folate replacement
30
Q

Key features of B12 deficiency

A
  • Causes:
    • Reduced intake - diet. vegans.
    • Reduced intrinsic factor- pernicious anaemia, post-gastrectomy
    • Terminal ileum- Crohn’s, ileal resection, bacterial overgrowth
  • Features:
    • General- Anaemia Sx, pallow, glossitis
    • Neuro- paraesthesia, peripheral neuropathy, optic atrophy, severe combined demyelination
  • Ix- Bloods- low WCC, intrinsic factor/ parietal cell Ab
  • Tx- malabsorption –> IM B12. Dietary- PO B12
31
Q

Key features of severe combined demyelination

A
  • Complication on low B12/ pernicious anaemia
  • Combined symmetrical loss of dorsal collumn and corticospinal tract
  • Distal sensory loss (proprioception and vibration) ataxia, wide gait, romberg’s +ve
32
Q

Causes of Haemolysis

A
  • Direct Coomb’s test looks for Abs
  • Causes:
    • AI - idiopathic, RA, SLE. Tx: Immunosuppresion, splenectomy
    • HUS - E.coli 0157 in kids
    • TTP= Thrombotic thrombocytopoenic purpura. Adult females. Fever, CNS, haemolytic anaemia, renal failure, thrombocytopoenia. Tx: plasmapharesis, immunosuppression, splenectomy.
33
Q

Key features of sickle cell anaemia

A
  • African, Caribbean, Middle-East
  • Point mutation in beta globin –> sickling, haemolysis, thrombosis
  • Ix- Hb, high retics, high bili, film (sickling), Hb electrophoresis
  • Triggers- cold, inf, hypoxia, dehydration
  • Features= SICKLED
    • Splenomegaly
    • Infarction eg stroke
    • Crisis- pulm, mesenteric, pain
    • Kidney disease
    • Liver/ Lung
    • Erection
    • Dactylisis
  • Tx:
    • Chronic- imms, folate, hydroxycarbamide
    • Acute- analgesia, o2, hydration, warm. Ceftriaxone, exchange transfusion
34
Q

Early and late complications of blood transfusion

A
  • Early:
    • Hameolytic reaction (–> renal damage)
    • Allergic reaction
    • Clotting abrnomalities
    • Fever
    • TRALI (tranfusion associated lung injury)- no HTN
    • TACO (transfusion associated circulatory overload)- RF= HF, chronic anaemia, +ve fluid balance –> Acute resp distress, tachycardia, HTN, oedema CXR. Acute HF Tx
  • Late:
    • BBV
    • GVHD
    • Iron overload

Finals!!! (19 decks)

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