Haemotology Flashcards

Question

Red cells in blood donations 1. shelf life? 2. storage? 3. how much time once out of fridge 4. uses

Answer 1

1. 35 day shelf life 2. at 4 degrees 3. transfuse < 4 h after removing from fridge 4. Uses - Blood loss - Anaemia

Answer 2

``` 1. I. Pool 4-6 donors blood II. Apheresis - single donor 2. max for 7 days 3. Storage: 22oC 4. Uses: - Thrombocytopenia -Bleeding with low plt -Preventing bleeding -very low plt -before procedures ```

Answer 3

1. Plasma frozen to -30oC 2. 2 yr storage 3. For clotting factor replacement if bleeding 4. I.Viral inactivation -Methylene Blue II. Solvent detergent treatment (Octaplas)

Answer 4

1. Precipitate that forms when thawing FFP 2. Fibrinogen replacement

Answer 5

If below Hb < 7g/dL

Answer 6

1. at any time up to 24 hours following a transfusion 2. I. haemolytic reactions, II. transfusion-related acute lung injury (TRALI), III. transfusion-associated circulatory overload (TACO), IV. transfusion-associated dyspnoea (TAD) V. those due to bacterial contamination of the component 3. Fever, renal failure

Answer 7

A receives from A or AB B receives from B or AB O receives from any AB receives from AB

Answer 8

1. Collagen and tissue factor exposed 2. Von Willebrand Factor binds collagen 3. Platelets adhere to vWF-collagen 4. Platelets activate and aggregate 5. TF initiates rapid thrombin generation on activated platelets (by causing release of Proteases (FVII, FX, FIX, FXI) and Co-factors (FVIII and FV)) 6. Thrombin converts fibrinogen to fibrin and completes platelet activation 7. Stable fibrin-platelet clot is formed

Answer 9

1. vWF activity is regulated by ADAMTS 13 2. Thrombin is regulated by Antithrombin and Activated protein C 2. Fibrin cleaved by Plasmin into fibrin degradation products (D-dimer)

Answer 10

1. Primary haemostasis disorders - defect: Platelets, VWF, vessel wall - mucocutaneous bleeding eg epistaxis, purpura - immediate bleeding 2. Coagulation pathway disorders - defect: Coagulation factors and fibrinogen - deep tissue bleeding (joints) - delayed bleeding (weak clot forms and breaks, followed by secondary bleeding) Both can cause GI/CNS bleeds

Answer 11

``` 1. Platelet count + blood film Indicates platelet number (not function) 2. Coagulation screen I. Prothrombin time (PT) II. Activated partial thromboplastin time (aPTT) Indicate function of coagulation pathway ```

Answer 12

1. Venous blood anticoagulated in 0.9% citrate to remove Calcium and stop clotting 2. centrifuge plasma 3. Add coagulation ‘activator’: includes calcium 4. Incubate at 37 C 5. Time to fibrin clot formation PT: • ‘Thromboplastin’ activator • Detects abnormal FVII, (extrinsic pathway) FX, FV, FII, Fibrinogen (common pathway) aPTT • ‘Contact activator’ • Detects abnormal FVIII, FIX, FXI (intrinsic factors) FX, FV, FII, Fibrinogen (common pathway)

Answer 13

1. A & B 2. Defect in F8 gene causing reduced FVIII (haemophilia A) Defect in F9 gene causing reduced FIX (haemophilia B) 3. Sex-linked recessive inheritance (on X chromosome) 4. Coagulation pathway disorder I. Mild or severe bleeding depending on factor level II. Soft tissue and joint bleeds III. Life-threatening CNS or GI tract bleeds IV. Chronic arthropathy (bleeding in joint leads to inflammation) V. Treatment acquired HCV and HIV 5. - Recombinant factor concentrate (synthetic factor 8 & 9) - prophylaxis every 2-3 weeks

Answer 14

1. - VWF mediates PLT adhesion to collagen - stabilises coagulation Factor VIII in the plasma 2. MILD VWD: - Epistaxis, easy bruising and traumatic skin bleeding - Defective primary haemostasis SEVERE VWD: - Additional coagulation pathway - defect due to low FVIII 3. I. First line tests • Platelet count normal • Long aPTT in moderate or severe VWD (when FVIII level is low) II. Second line tests • reduced plasma VWF activity • reduced plasma FVIII activity 4. I. Tranexamic acid Reduces clot break-down (antifibrinolytic) II. DDAVP/Desmopressin - Releases endogenous FVIII and VWF - only works once, temporary release III. VWF/FVIII concentrate

Answer 15

``` 1. Abnormal synthesis • Liver disease* • Vitamin K deficiency (coagulation factors II, VII, IX, X) • Warfarin* 2. Abnormal function • Heparin and direct acting oral anticoagulants* • Renal failure (platelet dysfunction) • Anti-platelet drugs 3. Dilution • Massive transfusion • Cardiopulmonary bypass 4. Consumption • Disseminated intravascular coagulation* • Thrombocytopenia in sepsis ```

Answer 16

1. I. ↓Synthesis of most clotting factors, fibrinogen and coagulation regulators II. ↓PLT number (hypersplenism) and PLT function III. Biliary obstruction gives vit K malabsorption (↓synthesis of Factors II, VII, IX and X) IV. Hyperfibrinolysis 2. • increased PT and APTT, reduced PLT count • reduced Fibrinogen and all clotting factors ``` 3. • Local measures (compress) • Endoscopy for variceal bleeding • Tranexamic acid • Vitamin K • Vitamin K factor concentrate • Fresh frozen plasma (if everything is missing) • Fibrinogen concentrate • Platelet transfusion ```

Answer 17

* Acquired disorders of haemostasis * May have bleeding and thrombotic features * Multisystem disorders requiring emergency care 1. Disseminated intravascular coagulation (DIC) 2. Thrombotic thrombocytopenic purpura (TTP)

Answer 18

``` 1. I. Sepsis II. Major trauma III. Obstetric emergencies (pre-eclampsia, amniotic fluid embolism, retained POC) IV. Advanced malignancy ``` ``` 2. I. Pro-coagulant stimulus + loss of regulatory pathways --> Widespread activation of coagulation pathway II. Fibrin/platelet rich microvascular thrombi and Multiorgan failure III. Platelet, fibrinogen and coag factor consumption and bleeding 3. • PT and APTT increased • Fibrinogen and PLT count decreased • D dimer increased (marker of clots) ``` ``` 4. • Fresh frozen plasma • Platelet transfusion • Fibrinogen concentrate or cryoprecipitate ```

Answer 19

``` 1. Often insidious onset fever, malaise, arthralgia • Bleeding (from thrombocytopenia) • Variable neurological features  Headache  Reduced consciousness  Stroke  Visual disturbance • Chest pain • Jaundice • Pallor and fatigue • Abdominal pain 2. Caused by anti-ADAMTS13 antibodies 3. anti-ADAMTS13 antibodies-> Reduced proteolysis of VWF -> Circulating ultra-high molecular weight VWF multimers -> Platelet agglutination in ‘high shear’ vessels, microvascular, thrombosis, platelet consumption, mechanical haemolysis ``` ``` 4. I. FBC: PLT reduced and Hb reduced II. Blood film is diagnostic (smashed in half) III. LDH increased IV. PT and APTT- N ``` (ADAMTS 13 level reduced) ``` 5. I. PLASMA EXCHANGE II. Supportive care • RBC transfusion • Aspirin and low molecular weight heparin • Immunosuppression • Iv corticosteroids Rituximab ```

Answer 20

1. Wells score for DVT diagnosis 2. I. Score >1= - DVT likely - Doppler USS II. Score 1 or less: - DVT unlikely - D dimer test - Doppler USS if high 3. • Unilateral pain, swelling, tenderness, redness. • Dilated superficial veins, venous gangrene very rare 4. Post-thrombotic syndrome - Chronic swelling and aching Venous eczema and ulcers - managed by Elevation, compression stockings, skin care

Answer 21

``` 1. • Dyspnoea, pleuritic chest pain, haemoptysis • Syncope, palpitations, collapse (massive or sub-massive PE) 2. I. ECG- tachycardia, R heart strain II. Blood gasses/sats- low O2 III. CXR- normal or wedge infarcts IV. D-dimer- increased V. CT pulmonary angiogram filling defect 3. I. death II. Chronic thromboembolic pulmonary hypertension (CTPH) • 0.5-5% of treated PEs • Clots are replaced by fibrous tissue • Progressive pulmonary hypertension and R heart failure ```

Answer 22

1. Antithrombin deficiency - MANAGEMENT ---Resistance to heparin anticoagulation ---Longterm anticoagulation, short term AT concentrate 2. Factor V Leiden - Reduces inactivation of Factor V by activated protein C 3. antiphospholipid syndrome - Some APL antibodies ‘activate’ vascular endothelial cells and platelets and are potently pro-thrombotic - MANAGEMENT: - If thrombotic, longterm anticoagulation (plus antiplatelet agent if arterial thrombosis) - Thromboprophylaxis improves pregnancy outcomes

Answer 23

I. Immediate anticoagulation - Fast acting anticoagulant minimum 3 months - (Rivaroxaban or LMW heparin) II. Long term anticoagulation - Depends on individualised risk vs benefit - Longterm if recurrent or on-going risk factor - (Rivaroxaban, warfarin. LMWH in cancer or pregnancy)

Answer 24

1. Anti-platelet drugs • Inhibit platelet activation or aggregation • eg. Aspirin, clopidogrel, abxiximab • Treatment/prevention arterial thrombosis (ACS, PVD, CVD) 2. Anti-coagulant drugs • Inhibit coagulation pathway • Treatment/prevention venous thrombosis or low pressure vessel thrombosis (DVT, PE, CVA in AF and mechanical heart valves, Cardiopulmonary bypass, dialysis)

Answer 25

1. All patients are assessed for risk of VTE and bleeding 2. All patients at risk of VTE should: • Mobilise early • Receive pharmaceutical thromboprophylaxis (eg enoxaparin 40 mg once daily)

Answer 26

1. • Increases activity of natural anticoagulant antithrombin • Inhibits active clotting factors esp. Factors IIa (thrombin) and Xa

Answer 27

1. IV vs SC 2. Complex, renal vs Predictable, renal 3. 1-2 hours vs 4-6 hours 4. Bleeding 5. 5000 iu loading then 20 iu/kg/hr Monitor and adjust to APTT ratio 1.5-2.0 vs I. Thromboprophylaxis: 40 mg sc once per day II. Treatment: 1.5 mg /kg once per day 6. Protamine iv 1mg/100 IU heparin in last hr (max 40mg)

Answer 28

1. • Direct oral anticoagulant (DOAC) • Factor Xa inhibitor • Reduces thrombin generation ``` 2. I. VTE prevention after knee/hip replacement II. Stroke prevention in non-valvular AF III. Acute treatment of DVT IV. Long term prevention of DVT and PE ``` 3. Plasma half life 8 hours 4. 75% liver metabolised, 25% renal excreted 5. Typical dose 15 mg bd or 20mg od 6. Specialist products (APCC, specific reversal agents) for severe bleeding

Answer 29

1. Inhibits vitamin K metabolism • Vitamin K is needed for synthesis of clotting factors II, VII, IX and X • Reduces factor levels to 20-30% 2. I. Anticoagulation when DOACs are unproven or unsafe • Mechanical heart valves • Antiphosphoilipid syndrome • Chronic kidney disease II. Long term management of VTE III. Stroke prevention in atrial fibrillation IV. With anti-platelet agent, to prevent arterial thrombosis 3. Plasma half life ~36 hours 4. Metabolised by liver 5. Typical dose 2-8 mg od 6. • Vitamin K1 1-3 mg po or iv if INR >8.0 for mild bleeding • Vitamin K factor concentrate (eg Octaplex) plus Vit K1 for severe bleeding

Answer 30

I. Group and save [40 mins] - perform IAT screening and store sample - if antibody negative blood can be rapidly issued if needed - request this if blood might be needed but not inevitable. II. “Crossmatch blood” [5-40 mins] - laboratory will assign units for specific patient - 5 minutes if recent group and save - 40 mins if antibodies/no previous G+S (IAT crossmatch)

Answer 31

I. 1. are defined as fever and other symptoms/ signs of haemolysis within 24 hours of transfusion 2. confirmed by one or more of the following: • a fall of Hb • rise in lactate dehydrogenase (LDH) • positive direct antiglobulin test (DAT) • positive crossmatch. II. 1. are defined as fever and other symptoms/ signs of haemolysis more than 24 hours after transfusion 2. confirmed by one or more of the following • a fall in Hb or failure of increment • rise in bilirubin • incompatible crossmatch not detectable pre transfusion III. 1. defined as acute dyspnoea with hypoxia and bilateral pulmonary infiltrates during or within 6 hours of transfusion, not due to circulatory overload or other likely causes ``` 2. Any 4 of the following within 6 hours of transfusion • Acute respiratory distress • Tachycardia • Increased blood pressure • Acute or worsening pulmonary oedema • Evidence of positive fluid balance ``` IV. transfusion-associated dyspnoea - respiratory distress within 24 hours of transfusion that does not meet the criteria for TRALI, transfusionassociated circulatory overload (TACO) or allergic reaction.

Answer 32

1. IRON • 1 unit red cells contains ~250mg iron • Maximum iron excretion ~3mg/day 2. Toxicity- heart / liver / pancreas / joints/ pituitary 3. Regular transfusion (several years) -iron overload 4. • minimise transfusion • long term iron chelation (desferrioxamine [s/c] or deferasirox / deferiprone [oral] )

Answer 33

1. raised pulse & BP at 15 mins 2. Slow down the infusion and give frusemide. 3. Prevention: Identify at risk patients. Give frusemide with transfusion 1 unit at a time.

Answer 34

1. def - 50% of total blood volume lost in less than 3 hours - Loss of more than one blood volume within 24 hours (around 70 mL/kg, >5 litres in a 70 kg adult) 2. - Give Tranexamic acid if less than 3 hours (bollus 10 min followed by iv infusion over 8 hours) If still bleeding: Correction of Hypofibrinogenaemia - Fresh frozen plasma (FFP) 4 units (about 15 mL/kg) - Cryoprecipitate 2 five-unit pools

Answer 35

1. Clonal B cell lymphocytosis of >5 x 109/l persisting for more than 3 months 2. median age at diagnosis 72 years ``` 3. Immunophenotyping: Looking at antigens on WBC - If score >3, CLL. - If <3, likely other form of B cell malignancies (eg mantle cell lymphoma) ``` 4. I. CHEMOTHERAPY: - GO-GO patient, aggressive chemotherapy, back to normal very quickly - elderly, gentle oral chemotherapy II. BTK INHIBITION: - Ibrutinib: brutons tyrosine kinase inhibitor Tyrosine kinase: switches on cellular cycle - very expensive, 5. 17p depletion on cytogenetics (mutation), poor diagnosis ``` 6. Infection • Low immunoglobulins Auto-immune disorders • Auto-immune haemolytic anaemia • ITP Richter’s transformation • rare Secondary malignancy • Especially basal cell carcinomas ```

Answer 36

1. infection 2. proliferative 3. inflammation 4. steroids

Answer 37

``` 1. Asymptomatic or Hypermetabolic state • Weight loss • Sweats Leucostasis (due to high WBC, Weisses Blut) • Shortness of breath • Priapism Symptoms from splenomegaly • Discomfort • Early satiety ``` 2. translocation t(9,22); phaladelphia chromosome: Formation of Bcr-Abl: - Inhibition of apoptosis - Up-regulates cell cycle progression and differentiation 3. Imatinib blocks formation of Bcr-Abl, improving the symptoms 4. monitor Bcr-Abl level 5. good nearly normal if treated

Answer 38

1. EPO production 2. COPD 3. congenital heart disease in adults 4. kidney cancer

Answer 39

1. splenectomy 2. inflammation 3. iron deficiency 4. malignancy

Answer 40

1. I. Polycythaemia rubra vera II. Essential Thrombocythaemia III. Primary Myelofibrosis 2. JAK2 mutation 3. wild type only cayses proliferation when EPO binds Mutation: doesnt need epo binding, causes proliferation without it 4.

Answer 41

``` I. • Raised red cell mass (Hct >0.52 males, >0.48 Females) • Exclude hypoxia, excess erythropoietin ``` II. • Raised platelet count (>450 x 109 /l) • Exclude reactive process, iron deficiency ::: 1) Increased risk of thrombosis 2) Transformation to Myelofibrosis / Acute myeloid leukaemia

Answer 42

• Venesection to maintain the Hct to <0.45 • Aspirin 75 mg/d unless contraindicated • Cytoreduction should be considered if: –Poor tolerance of venesection; –Symptomatic or progressive splenomegaly; –Other evidence of disease progression, e.g. weight loss, night sweats; –Thrombocytosis.

Answer 43

1. Symptomatic anaemia 2. Discomfort from splenomegaly • Hypermetabolic symptoms (weight loss, sweats) 3. bone marrow becomes thickened (due to fibrin) so spleen starts producing RBC, and if spleen cant liver takes over 4. depends on the score, but in high risk survival 1/2 years 5. ruxolotinib: JAK2 inhibitor

Answer 44

``` 1. I. Enlarged Lymph nodes II. B Symptoms ◦ Drenching Night sweats ◦ Unexplained Fevers >38’C ◦ Weight loss >10% in last 6 months III. Other symptoms ◦ Fatigue ◦ Itching ◦ Symptoms related to cytopenias ``` 2.  Full Blood Count (FBC)  Renal function (can it handle chemotherapy)  Liver function (can it handle chemotherapy)  LDH (raised, used as a marker)  Calcium (raised) ``` 3.  Lymph node biopsy 4. Staging scan: - (CT Neck,Chest,Abdo,Pelvis or PET scan) - Bone marrow biopsy ``` 5. - Stages 1-4 and tells you how many places you have lymphoma in: I. one place II. more than one on the same side of diaphragm III. both sides of diaphragm IV. exta lymphatic ( eg lungs) A (absence of B sx) or B (presence of B sx) 6. - Stage IA RCHOP x4 + Local Radiotherapy ``` - Stage IIA – IV RCHOP x 6-8cycles RCHOP chemotherapy: Rituximab – monoclonal antibody Cyclophosphamide Hydroxydaunorubicin chemotherapy Oncovin Prednisolone Steroids ``` Given every 3 weeks Risk of neutropenic sepsis day 7-10

Answer 45

``` Rituximab – monoclonal antibody Cyclophosphamide Hydroxydaunorubicin chemotherapy Oncovin Prednisolone Steroids ``` - Given every 3 weeks for non-hodgkin lymphoma - Risk of neutropenic sepsis day 7-10

Answer 46

``` Types ◦ Follicular Lymphoma ◦ Marginal Zone Lymphoma ◦ (Mantle Cell Lymphoma) ◦ Small lymphocytic Lymphoma ``` Management  Active monitoring  Radiotherapy  Chemotherapy/ Monoclonal Antibodies/ Targetted therapies

Answer 47

1. Treat with - ABVD +/- Radiotherapy 2. >85% 5 year survival* 3. Late effects of treatment - Second malignancy eg Lung/Breast cancer - Heart or lung problems

Answer 48

1. Cancer of plasma cells increased clonal plasma cells Monoclonal protein 2.  Immunofixation of serum and urine  Bone marrow biopsy: monoclonal paraprotein ``` 3.  Serum monoclonal protein <30g/L  Clonal bone marrow plasma cells <10%  Absence of end-organ damage (have paraprotein of no significance)  Active monitoring ``` 4. Serum monoclonal protein (IgG or IgA) ≥30g/L  or urinary monoclonal protein ≥500mg per 24h  and/or clonal bone marrow plasma cells 10-60%  Absence of myeloma defining events or amyloidosis 5. I. Vigorously rehydrate with at least 3 litres of normal saline daily II. Stop nephrotoxic drugs e.g. NSAIDs III. Administer high dose dexamethasone (sterioid, anti-myeloma effect) 6.

Answer 49

``` I. hypercalcaemia, sx: - CNS dysfunction - confusion, coma - Muscle weakness - Constipation - Thirst, polyuria - Shortening of the Q-T interval on ECG Mx: Hydration with IV fluids + Bisphosphonates – zoledronic acid/ pamidronate ``` ``` II. hyper viscosity syndrome: ----->Presentation ◦ blurred vision ◦ headaches ◦ mucosal bleeding ◦ dyspnoea due to heart failure ``` Mx: - plasma exchange - isovolaemic venesection (if cant plasma exchange) III. Spinal cord compression Mx:  Dexamethasone 40 mg daily for 4 days  Urgent MRI whole spine  Neurosurgical/spinal surgical review if bony lesion to decompress or stabilise spine  Local radiotherapy - if non-bony lesions ASAP (within 24 hours)

Answer 50

I. Traditional Chemotherapy ◦ E.g. Cyclophosphamide, Melphalan II. Steroids ◦ E.g. Dexamethasone III. Immune modulatory drugs ◦ anti-angiogenic, anti-inflammatory, anti-proliferative effects ◦ E.g. thalidomide, lenalidomide IV. Proteosome inhibitors ◦ involved in the removal, breakdown and recycling of damaged proteins or those that are no longer needed by the cell. Proteosome inhibitors cause cell death ◦ E.g. bortezomib V. Bisphosphonates ◦ Block the action of osteoclasts ◦ zoledronic acid, pamidronate VI. thromboprophylaxis

Answer 51

``` 1. I. Causes: - Iron deficiency - Thalassaemia - Anaemia of chronic disorder II. - Acute blood loss - Haemolysis - Anaemia of chronic disorder - Bone marrow infiltration - Combined haematinic deficiency III. - B12/folate deficiency - Haemolysis - Hypothyroidism - Liver disease - Alcohol excess - Myelodysplasia ```

Answer 52

1. ageing --> suppressed bone marrow 2. male post puberty higher than female 3. Hb rises with altitude 4. increased Hb 5. increased RBC, but more plasma so looks diluted,

Answer 53

1. Clinical Features (if severe) - Angular stomatitis - Glossitis - Koilonychia - Pharyngeal and oesophogeal webs 2. blood test - Microcytic hypochromic (pale) anaemia - Low serum ferritin (beware: acute phase protein) - Absent iron stores in bone marrow 3. • Dietary (80% from meat, 20% from vegetables) • Physiological (infancy, adolescence, pregnancy) • Blood loss • Malabsorption e.g. coeliac disease 4. duodenum

Answer 54

1. Depression of erythropoiesis 2. Main defect is failure of transport of iron from RE system to developing red cells ``` 3. • Chronic infection/inflammation • Malignancy • Uraemia • Endocrine disorders ``` 4. • Correction of underlying cause • Erythropoietin (+ iron)

Answer 55

``` Macrocytic – red cells in peripheral blood are large. _ Erythropoiesis may or may not be megaloblastic. ``` ``` Megaloblastic – red cell precursors are abnormally large because of impaired DNA synthesis. _ The cell continues to grow but does not divide as it is incapable of replicating DNA. _ RNA synthesis and translation not affected. ```

Answer 56

1. Megaloblastic anaemia 2. mainly in the liver. 3. Synthesised by micro-organisms: only present naturally in animal produce. 4. Absorption: combines with intrinsic factor secreted by gastric parietal cells and absorbed in terminal ileum. ``` 5. I. Dietary: veganism, rare II. Intrinsic factor deficiency: - Pernicious anaemia - Gastrectomy - Congenital III. Intestinal malabsorption: - Disease of terminal ileum e.g. Crohns - Blind loops & small bowel diverticulae ``` ``` 6. Clinical features I. Anaemia II. Jaundice III. Glossitis IV. Neurological deficit ``` 7. Laboratory features I. Anaemia, neutropaenia, thrombocytopaenia II. Low serum vitamin B12 III. Antibodies against parietal cells or intrinsic factor IV. Megaloblastic change in bone marrow V. Features of haemolysis 8. Mx I. Vitamin B12 replacement as IM injection most common – 3x per week for 2 weeks then maintenance phase. II. Oral folic acid replacement. Care: risk subacute combined degeneration of the cord (with permanent neurological sequelae) if folic acid replaced in absence of vitamin B12. Replace B before F!

Answer 57

1. Megaloblastic anaemia 2. Dietary sources: green vegetables, liver, nuts, cereals. 3. Absorbed in the jejenum 4. • Dietary – common in elderly, poor diet related to alcohol misuse. • Increased utilisation – e.g. in pregnancy, malignancy, haematological disorders with rapid cell turnover. • Malabsorption e.g. coeliac disease. • Drugs e.g. anticonvulsants. • Excessive loss e.g. renal dialysis 5. Similar to vitamin B12 deficiency but neurological problems do NOT occur 6. Laboratory features - Peripheral blood and bone marrow features identical to vitamin B12 deficiency. - Diagnosis made by measuring low serum (and sometimes red cell) folate levels. 7. I. Vitamin B12 replacement as IM injection most common – 3x per week for 2 weeks then maintenance phase. II. Oral folic acid replacement. Care: risk subacute combined degeneration of the cord (with permanent neurological sequelae) if folic acid replaced in absence of vitamin B12. Replace B before F!

Answer 58

1. Reduced red cell survival (normal life span=120d) 2. RBC breakdown can be: • intravascular (within the vessels, releasing free Hb) • extravascular (by reticulo-endothelial system) 3. Erythroid expansion and increased production (up to 7 fold) may partly compensate

Answer 59

1. I. Membrane: HS, Hereditary spherocytosis (auto-dominant) II. Enzymes: - Glucose 6 phosphate dehydrogenase deficiency (X linked) - Pyruvate kinase deficiency (autosomal recessive) III. Haemoglobin: Sickle cell anaemia; Thalassaemia (autosomal recessive) 2. I. Immune: a) Autoimmune (primary or secondary to lymphoproliferative, autoimmune disorders, infections, drugs) b) Alloimmune: - antibodies do not belong to the host - e.g. haemolytic disease of the newborn, or incompatible blood transfusion II. Infections –many mechanisms III. Drugs and chemicals –many mechanisms IV. Mechanical: - MAHA (microangiopathic haemolytic anaemia): small vessels due to overactive endothelium, mechanical break down of red cells - prosthetic heart valves V. Other rare types: e.g. Paroxysmal Nocturnal Haemoglobinuria

Answer 60

1. Anaemia 2. Jaundice 3. Splenomegaly 4. Skeletal abnormalities (congenital forms) 5. Gallstones (pigment stones suggests chronic haemolysis) 6. Haemoglobinuria (denotes intravascular haemolysis), very dark urine

Answer 61

I. Blood film is the most useful investigation II. Coombs test is used to detect immune coating on RBC by Ig and complement. If positive it suggests immune cause Interperation: Increased RBC production • Reticulocytosis, polychromasia • May become folate deficient Increased RBC destruction • Increased indirect bilirubin (unconjugated) • Increased LDH • Absent haptoglobins • Intravascular: haemoglobinaemia, haemoglobinuria, haemosiderinuria

Answer 62

1. I. Reduced or absent synthesis of one or more of the globin chains of adult hemoglobin. II. Imbalance in globin synthesis. 2. - Alpha Thalassaemia - Beta Thalassaemia

Answer 63

1. Beta Thalassemia trait (carrier) - Asymptomatic, normal life span 2. Beta Thalassemia intermedia - Variable phenotype and life span 3. Beta Thalassemia major - No Beta globin and no HbA - Early death if untreated

Answer 64

1. Complete absence of HbA 2. - Excess α chains accumulate and damage red cells - Ineffective erythropoiesis - Excessive RBCs Destruction 3.  Iron Overload  Extra-medullary hematopoiesis

Answer 65

1. Symptomatic anaemia in first few months of life. 2. Jaundice 3. Growth retardation failure to thrive. 4. Medullary hyperplasiaBony abnormalities especially of the facial bones. 5. Extra medullary haematopoesisEnlarged spleen and liver . 6. Increased risk of thromboses 7. Pulmonary hypertension & congestive heart failure

Answer 66

1. Regular blood transfusions (Life long): Transfusions 2 aims: prevent symptomatic anaemia, Also, suppress marrow hyperplasia with skeletal consequences. (However, with transfusions come the inevitable problem of iron overload (currently the life limiting factor) esp heart, liver, endocrine.) 2. Iron chelation e.g. Desferrioxamine, Deferiprone, Deferasirox 3. Folic acid 4. Bone marrow transplantation in early life

Answer 67

1. - deficient/absent alpha subunits - Excess beta subunits - Excess gamma subunits newborns 2. (Silent Carrier: 1 gene not working) I. Trait: 2 genes not working II. Hemoglobin H Disease: 3 genes not working III. Major (Hemoglobin Bart’s): ALL 4 not working

Answer 68

1. reduced alpha chains (3 out of 4) 2. jaundice, hepatosplenomegaly, leg ulcers, gallstones 3. folic acid, transfusions, splenectomy

Answer 69

1. no alpha chains produced, Mainly gammma chains | 2. Intrauterine death and stillborn

Answer 70

``` Normal: • Disc-Shaped • Deformable • Life span of 120 days Sickle: • Sickle-Shaped • Rigid • Lives for 20 days or less ```

Answer 71

1. Haemolysis • increased reticulocytes, sickle cells , target cells and hyposplenic changes • usually well-adjusted to anaemia: HbS has reduced oxygen affinity • red cells contain  80% HbS (remainder HbF) 2. Vaso-occlusion • acute episodes of bone pain, worsening anaemia, pulmonary and neurological complications • Precipitated by cold, dehydration, infection 3. Hyposplenism and risk of infection with encapsulated bacteria

Answer 72

1. Acute chest syndrome, PE, infection, opiod excess 2. Ischaemic: more common in children and older adults, and lowest in adults aged 20 to 29 years 3. Haemorrhagic: most frequent in the 20- to 29-year age group 4. Urgent neuroimaging, exchange transfusion to lower HbS %.

Answer 73

Sx: Fever, respiratory symptoms, new infiltrate on CXR mx: Patients need O2, IV fluid, antibiotics, incentive spirometry, analgesia and reduction of HbS% by transfusion.

Answer 74

- Usually asymptomatic | - Inability to concentrate urine (Occasionally renal papilliary necrosis)

Answer 75

The sickle cell solubility test relies on the relative insolubility of HbS in concentrated phosphate buffers compared to Hb A and other Hb variants. - Hb S precipitates causing a cloudy solution

Haemotology Flashcards

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