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ALS > Haem > Flashcards

Haem Flashcards

1
Q

ITP

A

Ab against gp2b/3a, chronic in adults vs acute post-infection/ vacc in kids

= older F, petechiae, bleeding

Inv - FBC (v PLT), blood film

-> oral pred, IVIg (^PLT quicker)

Evans syndrome: AIHA + ITP

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2
Q

Transfusion: non-haemolytic febrile reaction

A

Ab reacting with white cell fragments, more likely in platelet transfusion

= fever, chills

-> slow / stop, paracetamol

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3
Q

Transfusion: minor allergic reaction

A

Foreign plasma proteins

= itchy, urticaria

-> temp stop, antihistamine

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4
Q

Transfusion: anaphylaxis

A

IgA deficiency patients who have anti IgA Ab

= hypotension, wheezing, SOB, angioedema

-> stop, IM adrenaline

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5
Q

Transfusion: acute haemolytic reaction

A

ABO incompatibility e.g., human error

= fever, abdo pain, hypotension

-> stop, fluids, check identity, direct Coombs/ type/ XM

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6
Q

Transfusion: TACO vs TRALI

A

TACO
Rate too fast, pre-existing HF
= pulmonary oedema, HYPERtension
-> slow or stop, IV loop, oxygen

TRALI
Host neutrophils activated by donor blood
= non-cardio pulm oedema, fever, HYPOtension
-> stop, oxygen

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7
Q

DIC

A

Normally coagulation and fibrinolysis are balanced, unregulated in DIC due to tissue factor release

Causes - sepsis, trauma, malignancy, obstetric issues

Inv - v platelets, v fibrinogen, ^ fibrinogen degradation products, ^PT / APTT, schistocytes (microangiopathic HA)

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8
Q

Iron Deficiency Anaemia

A

Causes - blood loss, diet (meat, green leafy veg), poor absorption, ^iron requirements

= fatigue, SOB on exertion, palpitations, pallor, spoon nails, hair loss, glossitis, angular stomatitis

Inv - FBC, v ferritin (correlates to iron stores but APR), v transferrin sat, ^TIBC, ^transferrin, film (diff sizes and shapes, pencils)

-> oral ferrous sulphate (for 3m after corrected), if no tolerated and need surgery then use IV iron

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9
Q

Hereditary Spherocytosis

A

AD, extravascular haemolytic anaemia, defect of cytoskeleton so destroyed by spleen

= FTT, jaundice, gallstones, splenomegaly, aplastic crisis (parvovirus)

Inv - ^reticulocytes, ^MCHC, film (sphere), EMA binding test

-> support or transfuse acute, longer term splenectomy and folate replacement

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10
Q

G6PD deficiency

A

X linked recessive, most common RBC defect, v glutathione so more prone to oxidative stress,

RF - Meditaranean/ African descent

Triggers - Fava beans, sulpha drugs, anti-malarials, ciprofloxacin

= neonatal Jaundice, gallstones, splenomegaly,
IV haemolysis, Heinz bodies, bite and blister cells.

Inv - diagnose with G6PD enzyme levels 3m after attack

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11
Q

Acute Intermittent Prophyria

A

AD, rare, issue in the biosynthesis of haem, leads to toxic accumulation of porphobilinogen and aminolaevulinic acid

RF - F, 20-40yrs

= abdo pain, vomiting, motor neuropathy, depression, HTN, ^HR, red urine on standing

-> avoid triggers, IV haem arginate (or IV glucose) for acute attacks

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12
Q

Antiphospholipid syndrome

A

Acquired disorder, can be 2nd to SLE

= arterial/ venous thrombi, recurrent fetal loss, thrombocytopenia, livedo reticularis, pre-eclampsia

Inv - paradoxical ^APTT, v PLT, anticardiolipin Ab

-> low dose aspirin for primary prevention, lifelong warfarin for 2nd prevention (target 2-3, 3-4 if more)

*In pregnancy + LMWH from when fetal heart beat is seen until 34 weeks

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13
Q

Aplastic Anaemia

A

Pancytopenia and hypoplastic bone marrow

Causes - idiopathic, may be presenting feature of AML/ ALL, Fanconi anaemia, parvovirus, hepatitis, chloramphenicol, phenytoin, sulpha, benzenes, radiation

= 30yrs, normo normo anaemia, leukopenia, thrombocytopenia

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14
Q

Autoimmune Haemolytic Anaemia

A

Causes - idio, 2nd to SLE, lymphoma, EBV, methyldopa

= anaemia, ^reticulocytes, ^LDH, ^BR, +ve Coombs, film (sphero, reticulo)

Warm
Most common, IgG causes haemolysis at room temp (extravascular)
-> steroids +/- rituximab

Cold
IgM causes haemolysis at 4 degree (intravascular)

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15
Q

Beta Thalassaemia

A

AR

Major (no beta globulin chains, chr 11)
= 1yr, FTT, hepatosplenomegaly, microcytic anaemia
^HbA2, ^HbF, no HbA
-> repeated transfusions, iron chelation to avoid overload

Trait
= no symptoms, mild hypochromic microcytic anaemia
^HbA2

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16
Q

Situation with CMV negative blood

A

Needed for:
- granulocyte transfusions
- intra uterine transfusions
- neonates <28 days post-expected delivery
- pregnancy

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17
Q

Irradiated Blood

A

Depleted of T cells to prevents GvH disease

Needed for:
- granulocyte transfusions
- intra uterine transfusions
- neonates <28 days post-expected delivery
- bone marrow/ stem cell transplant
- Hodgkin lymphonma

(NOT pregnancy and HIV)

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18
Q

FFP

A

Used for clinically significant but not major bleeds where PT / APTT >1.5

Also prophylaxis before risky surgery

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19
Q

Cryoprecipitate

A

Factor 8 , VwF, fibrinogen, factor 13 and fibronectin

Used for clinically significant but not major bleeds where fibrinogen (<1.5)

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20
Q

Prothrombin Complex Concentrate

A

Used for emergency reversal of AC in those with severe bleed or IC bleed

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21
Q

Burkitts Lymphoma

A

High-grade B cell cancer caused by c-myc t(8:14)

Endemic form (jaw, Africa, EBV) vs sporadic (abdo tumour, HIV)

Inv - microscopy (starry sky)

-> chemo, rasburicase (prevents tumour lysis, ^phos/K/uric, v Ca, renal failure)

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22
Q

AML

A

Most common acute leakamia in adults

Causes - primary or 2nd to myeloproliferative

= anaemia (pale, tired), neutropenia (frequent infections), thrombocytopenia (bleeds), splenomegaly, bone pain

Promyeloctyic M3
- Translocation 15:17, 25yrs, Auer rods, DIC, vPLT

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23
Q

CML

A

95% Philadelphia chromosome - BCR-ABL t(9:22)

= 65yrs, anaemia, weight loss, sweating, massive splenomegaly

Inv - ^granulocytes at diff maturations, thrombocytosis, v ALP

-> imatinib (TK inhibitor)

Comp - blast transformation (80% AML)

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24
Q

CLL

A

Monoclonal proliferation of B cells, most common adult leukaemia

= asymp, lymph, anorexia, weight loss, bleeding, recurrent infections

Inv - FBC (^WCC, v Hb, v PLT), film (smear cells: crushed little lymphocytes), immunophenotyping

Comp - warm AIHA, Richter’s (high grade NH lymphoma, suddenly unwell), hypogammaglobulinemia

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25
Q

Factor V Leiden

A

Most common inherited thrombophillia, activated protein C resistance

Factor V is inactivated much more slowly

= VTE

26
Q

Fanconi Anaemia

A

AR
= aplastic anaemia, ^risk of AML, neuro issues, short stature, thumb/ radius abnormalities, cafe au lait spots

27
Q

G vs H disease

A

Multi-system complication of bone marrow transplantation (also solid organs), poor prog

T cells in donor tissue mount an immune response against host cells

Acute (<100 days)
= painful maculopapular rash, TENS-like, jaundice, watery/ bloody diarrhoea, n+v, fever

Chronic (>100 days)
= scleroderma, viitiligo, lichen planus, keratoconjunctivitis sicca, corneal ulcer, scleritis, dysphagia, oral ulcers, ileus, lung disease

-> IV steroids

28
Q

IV vs EV causes of haemolysis

A

In IV free haem binds to haptoglobin (-> saturated)

IV
- mismatched blood transfusion
- G6PD def
- Heart valves, TTP, DIC, HUS
- paroxysmal nocturnal haemoglobinuria
- Cold AIHA

EV
- Sickle cell, thalassemia
- Hereditary spherocytosis
- hemolytic disease of newborn
- Warm AIHA

29
Q

Haemophilia

A

X linked recessive disorder of coagulation, A (factor 8) vs B (factor 9)

= bleeding into joints, prolonged bleeding

Inv - ^APTT but other tests normal (bleeding time, PT, thrombin time)

30
Q

Hodgkin Lymphoma

A

Malignant proliferation of lymphocytes

RF - 20 and 75yrs, HIV, EBV, AI, FHx

= non tender asymmetrical nodes, pain on drinking alcohol (10%), weight loss, night sweats, itchy, fever

Inv - v Hb, ^eos, ^LDH, CXR (MS mass), node biopsy (Reed-Sternberg cells, multiple nuclei)

-> chemo

31
Q

Hodgkin - types and staging

A

Subtypes
Nodular sclerosing - most common, good prog, F
Mixed - lots of RS cells, good prog
Lymphocyte predominant - best prognosis
Lymphocyte depleted - worst prognosis

Poor prognosis if; B symptoms, >45yrs, v Hb, v lymphocytes, v albumin, ^^WCC, male

Ann arbor
1 - single node
2 - 2 or more on same side diaphragm
2 - both sides of diaphragm
4 - past nodes

32
Q

Hyposplenism

A

Causes - splenectomy, graves, sickle cell, coeliac, SLE, amyloid

Inv - Howell-jolly bodies, siderocytes

33
Q

Lead poisoning

A

= abdo pain, peripheral neuropathy, neuropsychiatric features, fatigue, constipation, blue gums

Inv - blood lead level, FBC (microcytic anaemia), film (basophilic stippling, clover leaf)

34
Q

Lymph drainage

A

Ovaries - Para-aortic lymphatics

Uterine fundus - para aortic and inguinal nodes

Uterine Body - iliac nodes

Cervix - external iliac, presacral and internal iliac

35
Q

Causes of Normocytic Anaemia

A

Chronic disease
CKD
Aplastic
Haemolytic
Blood loss acutely

36
Q

Causes of Macrocytic Anaemia

A

Megaloblastic - Vit b12 and folate lack
Normoblastic - alcohol, liver disease, hypothyroid, myelodysplasia and reticulocytosis

37
Q

Multiple Myeloma

A

Haem cancer, plasma cell proliferation, large number of one type of antibody (Monoclonal paraprotein, IgG), ^70yrs

CRABBI
hyperCalcaemia (osteoclastic bone resorption, kidneys, ^PTH)
= constipation, nausea, anorexia, confusion
Renal (BJ light chain deposit in tubules, amyloid, calcinosis, stones)
= polydipsia, polyuria
Anaemia (v erythropoesis)
= fatigue, pallor
Bones (^osteoclasts, lytic lesions)
= back pain, fractures
Bleeding (marrow crowding)
= thrombocytopenia, hyperviscosity
Infection

Inv - FBC, blood film (rouleaux formation), U&Es, bone profile, protein electrophoresis (serum ^IgA/G, urine BJ light chain), bone marrow asp (^^plasma c), skull XR (rain drop)
*whole-body MRI 1st line

BLIP

38
Q

MGUS

A

Monoclonal gammopathy of undetermined significance

Benign paraproteinaemia (mistaken for myeloma, 10% develop this)

= normal immune function, no lytic lesions or renal issues, no bone pain

39
Q

Waldenstrom’s Macroglobulinaemia

A

Lymphoplasmacytoid malignancy, secretion of monoclonal IgM paraprotein

= older men, weight loss, lethargy, hyperviscosity syndrome (v vision), hepatosplenomegaly, nodes, cryoglobulinaemia (Raynaud)

-> chemo

40
Q

Polycythaemia Vera

A

Myeloproliferative disorder

Clonal proliferation of marrow stem cell leading to inc red cell volume, 95% mutation in JAK2

= 60yrs, itchy after bath, splenomegaly, HTN, hypervisous (thrombosis), bleeds

Inv - ^HCT, ^neut/baso, ^PLT, ^ALP, v ESR, JAK2 mutation, ferritin, renal/ liver function

-> aspirin, venesection, chemo

Comp - 10% progress to myelofibrosis, 10% AML

41
Q

Myleofibrosis

A

Myeloproliferative disorder

Hyperplasia of abnormal megakaryocytes leading to ^platelet derived growth factor, stimulates fibroblast formation, bone marrow replaced by scar tissue.

= old person, fatigue, massive splenomegaly

Inv - v Hb, ^WBC, ^PLT, film (tear drop poikilocytes), biopsy (dry tap), ^urate, ^LDH

42
Q

Neutropenic sepsis

A

Complication 1-2 weeks post-chem, ^coag-neg gram +ve staph epidermidis (lines)

= neutrophils <0.5 + temp >38 or sepsis symptoms

-> immediate Abx (pip taz), may add meropenem at 48hrs, look for fungus at 4 days

Fluoroquinolone proph if risk

43
Q

Paroxsymal Nocturnal Haemoglobinuria

A

Acquired disorder leading to haemolysis and ^VTE
- v GPI, activation of complement cascade, v CD59

= hemolytic anaemia, pancytopenia, dark urine in morning, prone to thrombosis

Inv - flow cytometry of CD59

44
Q

Platelet transfusion cut offs

A

Active bleed:
<30 if clinically significant bleeding
<100 if severe bleeding or CNS

No bleeding and no better treatment:
<10

Prophylactic:
Aim for >50
50-75 if high risk of bleeding
>100 if surgery to critical site

Platelet transfusions carry the highest risk of bacterial contamination

45
Q

Sickle cell

A

AR, formation of HbA, chr11 (same as b-thal)

Heterozygous trait = HbAS, v malaria risk
Homozygous disease = HbSS

Inv - Hb electrophoresis

-> 5 yearly pneum, hydroxyurea (^HbF, proph)

Crisis
-> opiate analgesia, rehydration, oxygen, blood transfusion

46
Q

Sickle Cell Crisis

A

Thrombotic: sickled cells clog capillaries leading to distal ischaemia
Causes - infection, dehydration, high altitude
Inv - clinical diagnosis

Acute chest: vaso-occlusion in pulm vasculature
= SOB, chest pain
Inv - v pO2, CXR (pulm infiltrates)
-> pain relief, oxygen, transfuse, Abx if infective

Aplastic
Causes - parvovirus infection
= sudden fall in Hb, LOW reticulocytes

Sequestration: sickling in organs, pooling of blood
= worsening anaemia, ^reticulocytes

47
Q

Thymoma

A

Most common tumour of ant mediastinum, 60yrs

Link - myasthenia gravis, dermatomyositis, SLE, red cell aplasia

Comp - cardiac tamponade, airway compression

48
Q

Von Willebrand’s

A

AD, most common inherited bleeding disorder

Type 1 - partial reduction in VwF
Type 2 - abnormal form of VwF
Type 3 - total lack of it (AR)

= epistaxis, menorrhagia

Inv - ^bleeding time, mild ^APTT, v F8

-> tranexamic acid for mild bleeding, desmopressin (^ vWF), factor 8 conc

49
Q

Tumour Lysis Syndrome

A

Breakdown of tumour cells leads to ^K, ^phosphate, ^uric acid, v Ca

-> IV fluids, IV allopurinol or rasburicase before chemo if high risk, allopurinol during chemo if lower risk

Clinical syndrome if electrolyte labs + any of;
serum creatinine 1.5x upper limit, cardiac arrhythmia, death, seizure

50
Q

Tranexamic acid

A

Antifibrinolytic, reversibly binds to receptor sites on plasminogen or plasmin, can’t bind to fibrin

In trauma given as IV bolus then slow IV infusion

51
Q

Superficial Thrombophlebitis

A

Inflammation of superficial veins, ^long saphenous

20% have an underlying DVT at presentation, 4% progress to one

Inv - US to excl. DVT if prox saphenous

-> NSAIDs (top if mild), top heparinoids, LMWH, compression stockings (ABPI first)

52
Q

Smouldering Myeloma

A

Progression of MGUS with higher levels of the antibody, higher chance of becoming Myeloma

53
Q

Pernicious Anaemia

A

Ab against parietal factor or IF causing B12 deficiency

Parietal cells produce intrinsic factor (enables B12 absorption in ileum)

-> IM Hydroxycobalamin (3x per week for 2wks, then 3 monthly)

**If low folate too, TREAT B12 FIRST (risk of subacute degeneration of the cord)

54
Q

Post-thrombotic syndrome

A

Due to venous outflow obstruction and venous insufficiency

= painful heavy calves, itching, swelling, varicose veins and ulcers

-> compression stockings

55
Q

Calcium disease Myeloma

A

The cytokines released by myeloma cells cause increased osteoclastic bone resorption.

This causes a high calcium with normal or high phosphate (the kidney disease in myeloma means it is not excreted). Normal ALP

56
Q

INR Targets

A

AF - 2.5
VTE - 2.5
Recurrent VTE - 3.5
As a rule prosthetic mitral valves need higher INR then aortic

57
Q

Anaemic of chronic disease vs iron def

A

TIBC in iron def - high
Low or normal in chronic disease

58
Q

Varicose veins

A

Dilated tortuous superficial veins due to incompetent valves which allow backflow

= hyperpigmentation, hard tight skin, atrophie blanche (hypopigment), eczema

Inv - venous US

-> compression stockings

Surgical referral if:
Sig symptoms - swelling or pain
Bleeding
Skin changes
Thrombophlebitis
Active or healed venous leg ulcer

59
Q

Non-Hodgkins Lymphoma

A

Most common lymphoma, B or T cell, high or low grade

RF - white, old, HIV

= later b symptoms, extra-nodal disease e.g., gastric, bone pain, nerve palsies

Inv - excisional node biopsy, CT TAP, HIV, ESR/ LDH for prognosis

-> wait, chemo (CHOP + rituximab), radio

Comp - marrow infiltration, SVC obstruction, mets, spinal cord comp

60
Q

Red Cell Transfusion

A

without ACS vs with ACS
Transfusion threshold 70 g/L 80 g/L
Target after transfusion 70-90 g/L 80-100 g/L

Non-urgent scenario - unit over over 90-120 minutes

ALS (22 decks)

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