Haem Flashcards

(60 cards)

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Transfusion: non-haemolytic febrile reaction

A

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

= fever, chills

-> slow / stop, paracetamol

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Transfusion: minor allergic reaction

A

Foreign plasma proteins

= itchy, urticaria

-> temp stop, antihistamine

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Transfusion: anaphylaxis

A

IgA deficiency patients who have anti IgA Ab

= hypotension, wheezing, SOB, angioedema

-> stop, IM adrenaline

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Situation with CMV negative blood

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

FFP

A

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

Also prophylaxis before risky surgery

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

Cryoprecipitate

A

Factor 8 , VwF, fibrinogen, factor 13 and fibronectin

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

Prothrombin Complex Concentrate

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Factor V Leiden
Most common inherited thrombophillia, activated protein C resistance Factor V is inactivated much more slowly = VTE
26
Fanconi Anaemia
AR = aplastic anaemia, ^risk of AML, neuro issues, short stature, thumb/ radius abnormalities, cafe au lait spots
27
G vs H disease
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
IV vs EV causes of haemolysis
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
Haemophilia
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
Hodgkin Lymphoma
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
Hodgkin - types and staging
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
Hyposplenism
Causes - splenectomy, graves, sickle cell, coeliac, SLE, amyloid Inv - Howell-jolly bodies, siderocytes
33
Lead poisoning
= abdo pain, peripheral neuropathy, neuropsychiatric features, fatigue, constipation, blue gums Inv - blood lead level, FBC (microcytic anaemia), film (basophilic stippling, clover leaf)
34
Lymph drainage
Ovaries - Para-aortic lymphatics Uterine fundus - para aortic and inguinal nodes Uterine Body - iliac nodes Cervix - external iliac, presacral and internal iliac
35
Causes of Normocytic Anaemia
Chronic disease CKD Aplastic Haemolytic Blood loss acutely
36
Causes of Macrocytic Anaemia
Megaloblastic - Vit b12 and folate lack Normoblastic - alcohol, liver disease, hypothyroid, myelodysplasia and reticulocytosis
37
Multiple Myeloma
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
MGUS
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
Waldenstrom's Macroglobulinaemia
Lymphoplasmacytoid malignancy, secretion of monoclonal IgM paraprotein = older men, weight loss, lethargy, hyperviscosity syndrome (v vision), hepatosplenomegaly, nodes, cryoglobulinaemia (Raynaud) -> chemo
40
Polycythaemia Vera
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
Myleofibrosis
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
Neutropenic sepsis
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
Paroxsymal Nocturnal Haemoglobinuria
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
Platelet transfusion cut offs
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
Sickle cell
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
Sickle Cell Crisis
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
Thymoma
Most common tumour of ant mediastinum, 60yrs Link - myasthenia gravis, dermatomyositis, SLE, red cell aplasia Comp - cardiac tamponade, airway compression
48
Von Willebrand's
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
Tumour Lysis Syndrome
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
Tranexamic acid
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
Superficial Thrombophlebitis
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
Smouldering Myeloma
Progression of MGUS with higher levels of the antibody, higher chance of becoming Myeloma
53
Pernicious Anaemia
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
Post-thrombotic syndrome
Due to venous outflow obstruction and venous insufficiency = painful heavy calves, itching, swelling, varicose veins and ulcers -> compression stockings
55
Calcium disease Myeloma
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
INR Targets
AF - 2.5 VTE - 2.5 Recurrent VTE - 3.5 As a rule prosthetic mitral valves need higher INR then aortic
57
Anaemic of chronic disease vs iron def
TIBC in iron def - high Low or normal in chronic disease
58
Varicose veins
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
Non-Hodgkins Lymphoma
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
Red Cell Transfusion
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