Haematology Flashcards

Question

Investigations for Acute myeloid leukaemia (AML)

Answer 1

FBC - ↓ Hb - ↓ platelets - ↓ neutrophils clotting - DIC common - ↑PT, ↑aPTT, ↓fibrinogen LDH (↑) Bone marrow biopsy -≥20% blast cells LFTs, U&Es, blood film/smear

Answer 2

induction -cytarabine + daunorubicin consolidation -cytarabine / daunorubicin / mitoxantrone Stem cell transplant

Answer 3

presents younger than other AMLs (~25y/o) often has DIC/thrombocytopenia at presentation has Auer-rods on histology treated with arsenic trioxide (ATO) or all-trans relinoic acid (ATRA)

Answer 4

ALL = most common leukaemia in children CLL = most common leukaemia in adults -often has lymphadenopathy AML = most common acute leukaemia in adults CML= only ~15% of adult leukaemias - rarely has lymphadenopathy - associated with Philadelphia chromosome

Answer 5

malignant monoclonal expansion of B lymphocytes with accumulation of abnormal lymphocytes in blood / bone marrow / spleen / lymphnodes / liver most common leukaemia in adults, usually diagnosed ~70y/o

Answer 6

variable presentation with insidious onset, may be symptomatic & incidentally found on blood test ``` symmetrically enlarged painless lymph nodes (NB CML rarely has lymphadenopathy) susceptibility to infection anorexia, weight loss hepatosplenomegaly, abdo discomfort brusing, petichae ```

Answer 7

FBC - ↓ Hb - ↑↑ WCC - ↓ platelets Blood film -smear cells & smudge cells Immunophenotyping -key investigations Bone marrow aspirate / lymph node biopsy T53 gene test -done before treatment

Answer 8

chemotherapy stem cell transplant if CD20 +ve -rituximab TP53 targeted therapy -ibrutinib

Answer 9

a myeloproliferative disorder of pluripotent haemopoietic stem cells affecting one or all cell lines usually seen in adults age 60-65 yrs associated with Philadelphia chromosome in ~95% of pts

Answer 10

often asymptomatic & incidentally found on blood test, otherwise symptoms have insidious onset ``` fatigue, night sweats, weight loss abdo fullness, abdo distension LUQ pain splenomegaly & hepatomegaly easy bruising ``` NB CML only rarely has enlarged lymph nodes, but they are common in CLL = key differentiating factor

Answer 11

FBC - ↓ Hb - ↑ WCC (>100x10^9/L) - ↓/↑ platelets Blood film - all stages of maturation seen - looks like bone marrow aspire LDH (↑) leukocyte alkaline phosphate (↓) bone marrow aspirate & biopsy cytogenetics Fluorescent in situ hybridisation (FISH) quantitive reverse transcriptase PCR

Answer 12

a plasma cell dyscrasia characterised by terminally differentiated plasma cells, infiltration of the bone marrow by plasma cells and presence of a monoclonal immunoglobulin in the serum and/or urine classified by immunoglobulin type (IgG most common) 2nd most common haematological malignancy median age of presentation is 70yrs

Answer 13

classified by immunoglobulin type -IgG most common type 2nd most common haematological malignancy median age of presentation is 70yrs

Answer 14

classified by immunoglobulin type -IgG most common type 2nd most common haematological malignancy median age of presentation is 70yrs more common in men & afro-carribbeans

Answer 15

bone pain -particularly back pain pathological fractures lethargy, anorexia, night sweats, weight loss ↑ Ca2+ (constipation, nausea, confusion) dehydration, thirst, pallor easy bruising / bleeding ↑susceptibility to infection & repeated infections

Answer 16

bone pain -particularly back pain pathological fractures lethargy, anorexia, night sweats, weight loss ↑ Ca2+ (constipation, nausea, confusion) Renal damage (dehydration, thirst, pallor) easy bruising / bleeding ↑susceptibility to infection & repeated infections

Answer 17

FBC - ↓ Hb - ↓ platelets U&Es - ↑ urea - ↑ creatinine serum / urine electrophoresis - ↑ monoclonal IgA / IgG - Bence-Joyce proteins in urine Bone marrow aspiration & biopsy -monoclonal plasma cell infiltration X-ray - rain drop skill - randomly placed dark spots due to bone lysis Ca2+ (↑) Whole body MRI

Answer 18

Symptomatic multiple myeloma is defined at diagnosis by the presence of all 3 of the following: - Monoclonal plasma cells in the bone marrow >10% - Monoclonal protein within serum / urine (as determined by electrophoresis) - Evidence of end-organ damage e.g. hypercalcaemia, elevated creatinine, anaemia or lytic bone lesions/fractures

Answer 19

currently deemed incurable elderly pts/not suitable for transplant -Thalidomide + an Alkylating agent + Dexamethasone suitable for transplant - Bortezomib + dexamthasone - followed by stem cell transplant NB pts should be mounted every 3 months with blood tests & electrophoresis

Answer 20

refers to an increased number of Red blood cells in the body

Answer 21

Relative causes: -dehydration, stress (gaissbock syndrome) Primary: -polycythaemia vera Secondary - COPD - high altitude - OSA - uterine fibroids (cause ↑ EPO)

Answer 22

myeloproliferative disorder characterised by erythropoietic independent rise in erythrocyte & platelets count associated with a mutation in the JAK2 gene

Answer 23

maybe be asymptomatic or incidentally found on blood tests hyper viscosity syndrome -mucosal bleeding, visual changes, neurological symptoms (dizziness, headache, tinnitus) Pruritus -typically after contact with warm water plethoric appearance - flushed face with purple hue - cyanotic lips erythromyalgia -warmth, pain, erythema, infarction of distal extremities thrombosis -e.g. stroke, MI, PE, DVT, thrombophlebitis haemorrhage -from gums, easy bruising, GI bleeds splenomegaly, hypertension

Answer 24

FBC - ↑ Hb/RBC - ↑ haematocrit - ↑ platelets - ↑ WCC - ↓ MCV ``` Jak2 gene mutation (+ve) LDH (↑) erythropoietin (↓) ferritin (often ↓) LFTs ``` NB in secondary causes of polycythaemia there is only an isolated ↑ Hb/RBC and no ↑ platelets

Answer 25

Venesection (1st line) Myelosuppression (2nd line) - e.g. with hydroxyurea or phosphorus-32 - ↑ risk of secondary leukaemia JAK2 inhibitors -e.g. ruxolitinib Aspirin - low dose - to decrease risk of thromboembolic events NB ~5% of pts progress to myelofibrosis or acute leukaemia (↑ risk of this with myelosuppression therapy)

Answer 26

refers ti a low neutrophil count i.e. <1.5x10^9 (normal range (2.0-7.5 x10^9) important to recognise as it predisposes to infection

Answer 27

Viral -HIV, EBV, hepatitis Medications: -cytotoxics/chemotherapy, carbimazole, clozapine Benign ethnic neutropenia - seen in black africans, afro-carribbean individuals - no treatment required Haemtological malignacies - aplastic anaemia - myelodysplastic malignancy SLE, RA, haemodialysis

Answer 28

oral temp ≥38.5°C / ≥2 consecutive readings ≥38.0°C with a neutrophil count <0.5 x10^9 often associated with cancer therapy

Answer 29

Only investigate after starting Abx ``` FBC (↓ neutrophils) blood cultures CXR U&Es LFTs ```

Answer 30

fever tachycardia hypotension recent chemo therapy

Answer 31

a platelets count below the normal range i.e. <150 x10^9/L

Answer 32

Most severe - immune thrombocytopenia (ITP) - Disseminated intravascular coagulation (DIC) - thrombotic thrombocytopenic purpura (TTP) - haematological malignancies Others - heparin induced thrombocytopenia (HIT) - HELLP syndrome - Antiphospholipid syndrome - Medication (quinine, aspirin, thiazides, sulphonamides)

Answer 33

can be caused the use of EDTA as an anticoagulant | has low platelet count but without suggestive symptoms

Answer 34

``` FBC Blood film coagulation screen U&Es LFTs consider bone marrow biopsy ``` NB rapidly falling platelet count even if within normal range is worrying

Answer 35

treat any significant bleeds consult haematology consider replacing platelets -e.g. if anticipated need for urgent surgery, significant bleeding or neurological symptoms NB rapidly falling platelet count even if within normal range is worrying

Answer 36

epistaxis -usually excessive, prolonged, frequent ``` bleeding gums ↑ bleeding from tooth extractions spontaneous bruising menorrhagia PPH excessive bleeding after surgery ```

Answer 37

An autoimmune disorder with autoantibodies to platelets causing thrombocytopenia, which may be triggered by viral infections NB in children it is usually acute, following a viral infection or vaccination but is generally self limiting (lasts 1-2 weeks)

Answer 38

often asymptomatic & incidentally picked up on FBC petechiae, bruising, epistaxis absent splenomegaly major haemorrhages e.g. intracranial bleeds are uncommon NB presence of splenomegaly should make you consider other diagnosis

Answer 39

FBC shows platelet count <100 x10^9/L & blood film shows normal platelets 1st line -oral prednisolone if active bleed or urgent need to ↑ platelets use IVIG (human immunoglobulin

Answer 40

FBC shows platelet count <100 x10^9/L & blood film shows normal platelets 1st line -oral prednisolone if active bleed or urgent need to ↑ platelets use IVIG (human immunoglobulin)

Answer 41

thrombotic microangiopathic where micro thrombi consisting primarily of platelets occlude microvasculature due to deficiency of ADAMTS13 should be treated as an emergency most common in adults usually aged ~40 yrs

Answer 42

FBC - ↓ platelets - ↓ Hb - ↑ reticulocytes - ↓ haptoglobin U&Es - ↑ urea - ↑ creatinine urinalysis -proteinuria ± haematuria Blood film -schisticytes (erythrocyte fragements) LDH (↑↑) direct coombs test (-ve) pretreatment ADAMTS13 activity levels & ADAMTS13

Answer 43

Fever Fluctuating neurological signs - delirium, altered mental status - stroke, seizures - headache, dizziness Microangiopathic haemolytic anemia - fatigue, dyspnoea, pallor - jaundice, myalgia, arthralgia Impaired renal function: -haematuria, oliguria, proteinuria Purpura -non palpable small purpuric spots or petechiae

Answer 44

Direct -checks if antibodies are stuck to RBC surface In-driect -checks for free antibodies in serum help to rule out haemolytic anaemia

Answer 45

Plasmaphoresis -IV plasma exchange Steroids -high dose prednisolone Rituximab -if severe NB is treated as an emergency

Answer 46

Pentad of - fever - fluctuating neurological signs - microangiopathic haemolytic anemia - thrombocytopenia - renal failure

Answer 47

The most common hereditary coagulopathy due to the deficiency or abnormal function of von Willebrand factor (vWF) characteristically behaves like a platelet disorder as vWF promotes platelet adhesion & platelet plug formation + vWF binds factor VIII preventing its clearance inherited in an autosomal dominant fashion

Answer 48

The most common hereditary coagulopathy due to the deficiency or abnormal function of von Willebrand factor (vWF) characteristically behaves like a platelet disorder as vWF promotes platelet adhesion & platelet plug formation + vWF binds factor VIII preventing its clearance inherited mostly in an autosomal dominant fashion

Answer 49

often asymptomatic bleeding tendency from mucosa - epistaxis - menorrhagia (common in women) prolonged bleeding from minor injuries / post surgery NB haemoarthrosis are rare

Answer 50

Coagulation profile - PT (normal) - aPTT (may be ↑) - bleeding time (↑) ``` FBC (normal) Factor VIII (may be ↓) vWF antigen (↓) ristocetin cofactor assay (defective platelet aggregation) ```

Answer 51

TXA for mild bleeding Desmopressin (DDAVP) -stimulates vWF release vWF containing factor VIII concentrate -for severe bleeding or surgical prophylaxis

Answer 52

a platelets count >450 x10^9/L Aetiology - reactive (platelets = acute phase reactant_ - e.g. stress, surgery, infection - malignancy - e.g. CML - hyposplenism / post splenectomy - essential thrombocytosis

Answer 53

most common myeloproliferative neoplasm due to sustained dysregulates megakaryocytic proliferation, usually seen in pts age 50-60yrs

Answer 54

commonly asymptomatic erythromelalgia -burning pain & erythema of hands and feet thrombotic events (stroke/TIA/retinal artery occlusion) Vasomotor symptoms -headache, visual disturbance, ocular migraines splenomegaly/hepatomegaly bleeding -primarily GI, simulates duodenal ulcer following duodenal arcade thrombosis

Answer 55

FBC -platelets >600 x10^9 Blood smear -immature precursor cells e.g. myelocytes Bone marrow aspirate - hypercellularity - megakaryocyte hyperplasia LDH (↑) CRP/ESR (normal)

Answer 56

Hydroxyurea (hydroxycarbamide) -to ↓ platelet count low dose aspirin -↓ thrombotic risk NB Interferon-alpha is preferred in young pts & pregnant women over hydroxyurea

Answer 57

an X-linked recessive inherited bleeding disorder resulting in the deficiency of coagulation factors

Answer 58

X-linked recessive inheritance hence only seen in males

Answer 59

Haemophilia A - deficiency in clotting factor VIII - accounts for ~80% of cases Haemophilia B - deficiency in clotting factor IX - accounts for ~20% of cases Haemophilia C - deficiency of factor XI - very rare - seen in Ashkenazi jews

Answer 60

repeated haemarthrosis - especially of knees - can lead to haemophilic arthropathy frequent brusing & haematomas oral mucosal bleeding, epistaxis, excessive bleeding after minor procedures musculoskeletal bleeding/haematomas NB female carriers may present with mild symptoms

Answer 61

Coagulation screen - aPTT ↑ - PT normal - bleeding time normal Factor VIII/IX assay - ↓ / absent joint x-rays -may show degenerative joint disease

Answer 62

prophylactic factor VIII if acute bleeding -FFP/recombinant factor VIII

Answer 63

if acute bleeding - 1st line = recombinant factor IX - 2nd line = PCC or plasma derived factor IX

Answer 64

a syndrome characterised by systemic activation of the coagulation cascade resulting in the formation of intravascular thrombi & the depletion of platelets + coagulation factors

Answer 65

-Sepsis -trauma (trauma induced coagulopathy) -malignancy -obstetric complications (e.g. HELLP syndrome, amniotic fluid embolus) -organ failure (e.g. pancreatitis, fulminant hepatitis) transfusion reaction

Answer 66

``` oliguria, hypotension, tachycardia large bruises spontaneous bleeding at venipuncture sites / soft palate / site of trauma massive haemorrhage organ failure -ARDS -PE -purpura fulminans (DIC + extensive skin necrosis) -Waterhouse-Friedirchsen syndrome ```

Answer 67

Coagulation screen - PT ↑ - aPTT ↑ - bleeding time ↑ - Fibrinogen ↓ FBC -platelets ↓ D-Dimer ↑

Answer 68

treat underlying cause blood products as needed anticoagulation - if hypercoaguability is the main problem - therapeutic dose LMWH/UFH do not use antifibrinolytics e.g. TXA in pts with DIC

Answer 69

a group of hereditary haemoglobin disorders characterised by mutations in the alpha or beta global chains inherited in autosomal recessive pattern

Answer 70

deficient production of α-globin | 2 α genes present on each chromosome 16

Answer 71

Normal (α,α / α,α) a+ heterozygous i.e. silent carrier (α,- / α,α) - i.e. one allele affected - borderline Hb & MCV - pt asymptomatic a+ homozygous (α,- / α,- or α,α / -,-) - i.e. two alleles affected - slightly low Hb, slightly low MCV & MCH - pt asymptomatic HbH disease (α,- / -,-) - i.e. three alleles affected - microcytic hypo chromic anaemia - splenomegaly & skeletal deformities α Thalassaemia major / Hb Barts (-,- / -,-) - i.e. all four alleles affected - hydrops fettles & intrauterine death - incompatible with life - Hb Barts = γ chain tetramere

Answer 72

FBC - Hb ↓ - MCV ↓ - MCH ↓ - reticulocytes ↑ - RBC count ↑ Iron studies - iron ↑ - ferritin ↑ Blood film + supra vital stain -Heinz bodies (HbH inclusion bodies of β-chain tetrameres)

Answer 73

FBC - Hb ↓ - MCV ↓ - MCH ↓ - reticulocytes ↑ - RBC count ↑ Iron studies - iron ↑ - ferritin ↑ Blood film + supra vital stain -Heinz bodies (HbH inclusion bodies of β-chain tetrameres)

Answer 74

no management for silent carriers or asymptomatic pts folic acid supplements for most pts Blood transfusions -especially in HbH disease Splenectomy if hyposplenism Iron chelation -e.g. desferrioxamine to prevent iron overload

Answer 75

deficient production of β globes one β global gene on each chromosome 11

Answer 76

Normal (β / β) β Thalassaemia trait (β / -) - one functional allele, one non functional - HbA2 >4% - slight anaemia, ↓MCV, ↓MCH - asymptomatic β Thalassaemia major (- / -) - absence of β chains - HbF >90% - severe haemolytic anemia ↓↓MCV, ↓↓MCH - hepatosplenomegaly & skeletal deformities - chronic diffusion dependency - presents in 1st year of life with failure to thrive

Answer 77

FBC - Hb ↓ - MCV ↓ - MCH ↓ - reticulocytes ↑ Blood film + supra vital stain - Target cells - Teardrop cells Haemoglobin analysis - minimal/no HbA - HbF ↑ - HbA2 ↑ LDH (↑)

Answer 78

Repeated transfusions -risk of iron overload iron chelation -e.g. with IV desferrioxamine or PO deferrasirox Folic acid supplements as required consider splenectomy

Answer 79

Autosomal recessive single gene defect in the β chain of haemoglobin leading to production of sickle haemoglobin (HbS) more common in people of african descent most common form of intrinsic haemolytic anaemia

Answer 80

i.e. heterozygous individuals carrying HBSA people with sickle cell trait are generally asymptomatic but may present with gross haematuria due to renal papillary necrosis protective against malaria

Answer 81

symptoms begin age 3-6 months as HbF levels drop pallor, jaundice, lethargy, growth restriction, weakness failure to thrive splenomegaly further presentations with crisis

Answer 82

Most common type of sickle cell crisis due to obstruction of microcirculation by sickle RBCs leading to ischaemia precipitated by cold, infection, dehydration, hypoxia etc. causes severe pain, swollen joints, dactylitis, priapism (in men) may affect major organs e.g. stroke if brain, bowel ischaemia if mesentery, renal papillary necrosis (loin pain &haematuria)

Answer 83

a type of sickle cell crisis essentially a vast-occlusive crisis of the lung vasculature presents with dyspnoea, hypoxia, chest pain, tachypnoea & new infiltrates on CXR symptoms + new infiltrates = diagnostic

Answer 84

temporary cessation of erythropoiesis leading to severe anaemia in sickle cell pts usually due to parvovirus B19 infections leads to rapid ↓Hb (~1 week), due to bone marrow suppression the reticulocyte count is ↓ FBC (↓Hb, ↓reticulocytes) requires transfusions

Answer 85

mainly seen in babies/young children, sickling in organs e.g. spleen/lungs cause blood pooling and worsening of anaemia presents with splenomegaly, LUQ pain, shock (tachycardia, hypotension) FBC (↓Hb, ↑reticulocytes) NB recurrent spleen sequestration is an indication for splenectomy

Answer 86

rare sickle cell crisis ↓Hb due to ↑ rate of haemolysis

Answer 87

FBC - Hb ↓ - reticulocytes ↑ (if ↓ indicates aplastic crisis) Blood smear - presence of sickle cells - Howell-Jolly bodies - nucleated RBCs - target cells Haemoglobin electrophoresis - gold standard - no HbA - ~90% HbSS - ~10% HbF U&Es, Lung function tests, LFTs

Answer 88

heel prick blood spot test on day 3-10 after birth OR pre-conceptual screening in high risk groups

Answer 89

Crisis - high dose analgesia e.g. opiates - O2, IV fluids - blood transfusions / exchange transfusion - Abx if signs of infection folic acid supplements oral penicillin prophylaxis from diagnosis unconjugated pneumococcal vaccine from age 2yrs -repeat every 5 years exchange transfusions - as required - should be done if a stroke occurs hydroxycarbamide (hydroxyurea) - to ↑ HbF levels - helps ↓ frequency of crisis NB most pts have crisis plan which includes analgesic regiments, this should be followed, these pts are often deemed to be opiate seeking and left in pain

Answer 90

most common RBC enzyme defect leading to ↑ susceptibility to oxidative stress X-linked recessive inheritance i.e. male only usually seen in mediterranean & african people generally managed by avoiding triggers for crisis & blood transfusion if necessary

Answer 91

often asymptomatic recurring haemolytic crisis -sudden onset back/abdo pain, jaundice, dark urine, transient splenomegaly, pallor precipitants include fava beans. chlorquine, isoniazid, NSAIDs, sylph-group drugs

Answer 92

FBC - Hb ↓ - reticulocytes ↑ Urinalysis -haemoglobinuria Blood smear -Heinz bodies & bite cells G6PD enzyme assay -3 month after acute episodes ``` Indirect bilirubin (↑) LDH (↑) ```

Answer 93

hereditary abnormality of RBCs caused by defects in structural membrane proteins leading to ↓ lifespan of RBCs inherited in autosomal dominant fashion most common cause of hereditary haemolytic anaemia in people of northern european descent

Answer 94

``` failure to thrive jaundice splenomegaly LUQ pain black pigmented gallstones pallor anaemia ```

Answer 95

FBC - Hb ↓ - reticulocytes ↑ Blood smear -spherocytes EMA binding test -↓ binding of dye to RBC membranes Unconjugated bilirubin (↑) LDH (↑) direct antiglobulin test (-ve) NB direct antiglobulin test is +ve in other haemolytic anaemias

Answer 96

folate replacement splenectomy transfusions if necessary

Answer 97

a group of disorders that result in premature destruction of RBCs leading to a normocytic anaemia Types: - Intrinsic: due to abnormalities in the RBCs - Extrinsic: due to external causes e.g. mechanical damage or immune mediated Mechanism - Intravascular: due to complement fixation, trauma or other extrinsic factors e.g. G6PD/TTP/DIC/ABO mismatch - Extravascular: most common type, RBCs removed by phagocytic system due to intrinsic defects e.g. spherocytosis

Answer 98

Hereditary -G6PD, spherocytosis, sickle cell anaemia Acquired immune: -autoimmune haemolytic anaemia, transfusion reactions, haemolytic disease of the new born, drugs e.g. penicillin Acquired non immune: -DIC, TTP, HUS, malignancy, pre-eclampsia, prosthetic heart valves, paroxysmal nocturnal haemoglobinuria

Answer 99

may be sudden with tachycardia, dyspnoea, angina, weakness and mild jaundice gallstones (due to ↑ bilirubin) haematuria (if intravascular)

Answer 100

FBC - Hb ↓ - Reticulocytes ↑ - MCV/MCH normal direct antiglobulin test (direct coombs test) - if +ve suggests immune aetiology - if -ve non immune aetiology blood smear - abnormal forms - fragments LDH (↑) haptoglobin (↓) bilirubin (↑) urinalysis (haematuria)

Answer 101

Triad of AKI, microangiopathic haemolytic anaemia (combs -ve) and thrombocytopenia generally seen in young children, ~90% of cases are due to shiga-toxin producing E.coli (0157:H7) presents with diarrhoea illness preceding onset of thrombocytopenia, microangiopathic haemolytic anaemia and AKI Investigations include Hb ↓, haptoglobin ↓, bilirubin ↑, LDH ↑, reticulocytes ↑, platelets ↓, urea ↑, creatinine ↑ & ↑ schistocytes management is supportive (IV fluids, blood transfusions), with consideration for plasma exchange or eculizumab

Answer 102

a condition defined by pancytopenia with hypo cellular bone marrow and absence of abnormal cells diagnosis requires ≥2 of - Hb <100g/L - platelets <50x10^9 /L - neutrophil count <1.5x10^9 /L

Answer 103

~75% of cases are idiopathic congenital e.g. Flaconi syndrome, drugs e.g. cytotoxics/phenytoin/sulphonamides, radiation, parovirus

Answer 104

fatigue, malaise, pallor, dyspnoea ankle oedema purpura, petechiae mucosal bleeding, retinal haemorrhage no lymphadenopathy no hepatosplenomegaly

Answer 105

FBC - Hb <100g/L - platelets <50x10^9 /L - neutrophil count <1.5x10^9 /L - reticulocytes ↓ - MCV normal Bone marrow biopsy - hypocellular bone marrow - no abnormal cells

Answer 106

removal of underlying causes e.g. if drug induced haemopoietic stem cell transplant (HSCT) immunsuppression e.g. ATG + ciclosporin supportive care including blood/platelet transfusion

Answer 107

a low level of haemoglobin with a normal MCV Mechanism includes ↓blood volume and/or ↓erythropoiesis

Answer 108

Haemolytic anaemias -sickle cell, HUS, G6PD, hereditary spherocytosis, pyruvate kinase deficiency, TTP, DIC, malaria, mechanical destruction Aplastic anaemia -idiopathic, Falconi syndomre Acute blood loss anaemia of chronic disease anaemia of CKD (falls under anaemia of chronic disease)

Answer 109

Haemolytic anaemias -sickle cell, HUS, G6PD, hereditary spherocytosis, pyruvate kinase deficiency, TTP, DIC, malaria, mechanical destruction Aplastic anaemia -idiopathic, Falconi syndomre Acute blood loss anaemia of chronic disease (most common normocytic anaemia) anaemia of CKD (falls under anaemia of chronic disease)

Answer 110

anaemia secondary to chronic inflammation, 2nd most common anaemia overall aetiology include chronic infections e.g. TB, malignancy, inflammatory conditions e.g. RA/SLE Investigations include FBC (normocytic, normochromic anaemia), serum iron (↓), total iron binding capacity (↓), ferritin (↑), reticulocyte count (↓), ESR/CRP (↑) management include treatment of underlying cause, blood transfusion or use of recombinant human erythropoietin/NESP e.g. darbepoetin e.g. in CKD

Answer 111

a condition with ↓Hb & ↓MCV due to insufficient Hb production Aetiology - iron deficiency anaemia (most common) - lead poisoning - late phase anaemia of chronic disease - Thalassaemia - sideroblastic anaemia

Answer 112

most common type of anaemia due to a deficiency in iron

Answer 113

excessive blood loss e.g. menorrhagia, GI bleeding dietary inadequacy (e.g. vegans/vegetarians) malabsorption (e.g. coeliac disease, IBD) increased demand (e.g., pregnancy) NB GI bleeding is the most common cause in men & post menopausal women and should prompt consideration of colon cancer

Answer 114

often found incidentally ``` fatigue, SOB on exertion, pallor, palpitations koilonychia (spoon nails) atrophic glossitis angular stomatitis changes in hair / hair loss brittle nails angular chelitis ``` dysphagia due to oesophageal webs (Plummer-Vinson syndrome)

Answer 115

FBC - microcytic hypo chromic anaemia - reticulocytes ↓ iron studies - serum iron ↓ - total iron binding capacity ↑ - transferrin ↑ - transferrin saturation ↓ - ferritin ↓ Blood film - anisopoikilocytosis (red blood cells of different sizes and shapes) - target cells (hypo chromic RBCs) - 'pencil' poikilocytes

Answer 116

Treat underlying cause PO ferrous sulfate/ferrous salts -side effects include constipation, dark stools, nausea, diarrhoea, abdo pain Parenteral iron supplements are reserved for severe cases Iron rich diet -e.g. dark green leafy veg, meat, iron fortified bread NB consider urgent referral if pt with new IDA age >60yrs or <50yrs presenting with rectal bleeding (?colon cancer)

Answer 117

a condition with ↓Hb & ↑MCV due to insufficent nucleus maturation reactive to cytoplasmic expansion due ti defective DNA synthesis or defective DNA repair Aetiology - Megaloblastic anaemia (characterised by hyperhsegmented neutrophils) - Vit B12 deficiency - Folate deficiency - normoblastic anaemia - alcohol abuse, liver disease, pregnancy, myelodysplasia, hypothyroidism

Answer 118

autoimmune disorder causing Vit B12 deficiency due to autoantibodies against intrinsic factor* ± gastric parietal cells accounts for ~80% of megaloblastic anaemias NB ↑ risk of gastric cancer

Answer 119

fatigue, lethargy, palpitations, faintness, dyspnoea pallor + jaundice (gives lemon twinge) peripheral neuropathy -typical symmetrical affecting arms & legs subacute combined degeneration of the spinal cord - progressive weakness, ataxia, parasthesia - can progress to spasticity, paraplegia neuropsychiatric features -memory loss, poor concentration, confusion, depression, irritability

Answer 120

FBC - Hb↓ - MCV↑ Blood smear - hypersegmented neutrophils - megakaryocytes Serum Vit B12 (↓) Folate (normal or ↓) anti intrinsic factor antibodies (+ve) anti parietal cell antibodies (may be +ve)

Answer 121

Hb levels <70g/L Hb levels <80g/L in ACS ongoing haemorrhage & chronic anaemia requiring transfusions do not have to meet these thresholds

Answer 122

thought to be due to leaked cytokines that accumulate during storage presents with fever, chills and flushing managed with temporarily stopping infusion, paracetamol, restarting transfusion at slower rate with increased monitoring

Answer 123

due to foreign plasma proteins presents with urticaria, pruritus (i.e. only cutaneous symptoms) managed by temporarily stopping infusion, antihistamines, restarting transfusion at slower rate with increased monitoring

Answer 124

pt presents with SOB, dyspnoea, wheezing, angioedema, hypotension and respiratory distress managed by stopping transfusion, 500mcg adrenaline IM (0.5ml 1:1000) & supportive measures

Answer 125

due to ABO incompatibility (this is a never event) presents within minutes of starting transfusion with fever, abdo pain, hypotension, tachycardia, dyspnoea, chills & flushing management -STOP transfusion, supportive care, fluid resuscitation NB blood should be returned to lab for confirmation i.e. direct coombs test & repeat crossmatch

Answer 126

due to ABO incompatibility (this is a never event) presents within minutes of starting transfusion with fever, abdo pain, hypotension, tachycardia, dyspnoea, chills & flushing can lead to DIC & renal failure management -STOP transfusion, supportive care, fluid resuscitation NB blood should be returned to lab for confirmation i.e. direct coombs test & repeat crossmatch

Answer 127

fluid overload secondary to excessive rate of transfusion or pre-existing HF presents with hypertension, extended JVP S4 heart sound, peripheral oedema, pulmonary oedema (on CXR) and dyspnoea managed by stopping/slowing transfusion & IV diuretics e.g. furosemide

Answer 128

non cardiogenic pulmonary oedema due ↑ vascular permeability secondary to neutrophil activation presents with hypoxia, dyspnoea, hypotension, fever, no signs of fluid overload, SOB & pulmonary infiltrates on CXR managed by stopping transfusion, giving O2 -consider mechanical ventilation if required

Answer 129

24h - 28 days post infusion generally mild symptoms including mild fever, jaundice, anaemia, chest/abdo/back pain generally self limiting and do not need treatment

Answer 130

500ml bolus of crystalloids (usually NaCl 0.9%) over 15 min -consider 250ml bolus if elderly or known HF if no response then can repeat boluses to a maximum of 2000ml - juniors should get senior help at 1000ml - ITU support is needed at 2000ml

Answer 131

Daily requirements over 24h - 25-30ml/kg of fluid - 1mmol/kg of K+/Na+/Cl- - 50-100g of glucose Nb weight based K+ should be rounded to the nearest common fluid available so in 67kg person give 60mmol (one 40mmol & one 20mmol) K+ replacement rate should not exceed 10mmol/h

Answer 132

K+ replacement rate should not exceed 10mmol/h

Answer 133

flow rate = drip factor X (volume (ml)/minutes) drip factor is found on the giving set packaging flow rate is in drops/min`

Answer 134

an oncological emergency resulting from the rapid destruction of tumour cells leading to a massive release of intracellular components generally associated with the introduction of combination chemotherapy

Answer 135

an oncological emergency resulting from the rapid destruction of tumour cells leading to a massive release of intracellular components generally associated with the introduction of combination chemotherapy associated with high grade lymphomas & leukemias especially Burkitts lymphoma

Answer 136

Hyperkalaemia (K+ ↑) hyperuricaemia (uric acid ↑) hyperphosphataemia (Phosphate ↑) hypocalcaemia (Ca2+ ↓)

Answer 137

typically within 1-5 days of staring chemo Renal failure -oedema, lethargy, oliguria Hyperkalaemia -arrhythmias (syncope, palpitations, chest pain) nausea, vomiting Hypocalcaemia -seizures, tetany, muscle cramps, perioral paraesthesia

Answer 138

``` uric acid > 475umol/l or 25% ↑ potassium > 6 mmol/l or 25% ↑ phosphate > 1.125mmol/l or 25% ↑ calcium < 1.75mmol/l or 25% ↓ serum creatinine ≥1.5x upper limit of normal LDH ↑ FBC U&Es ```

Answer 139

Prevention is key - High risk pt = IV allopurinol or IV rasburicase + ↑ hydration - low risk pts = PO allopurinol - meds to be given when chemo commences Treatment: - IV fluids & hydration - IV Rasburicase (do not combine with allopurinol as ↓ activity of Rasburicase) - cardiac monitoring - IV calcium gluconate (if symptomatic ↓ Ca2+)

Answer 140

an immune mediated prothrombotic disorder characterised by a sudden drop in platelet count in a pt receiving heparin containing products due to antibody formation against heparin platelet factor 4 (PF4) usually occurs within 5-10days of initiating heparin NB although platelet levels are low its a prothrombotic condition (platelet levels are low as they are activated & used up)

Answer 141

Thrombotic manifestations - mainly DVT, PE - less commonly acute limb ischaemia, MI, stroke localised skin necrosis at heparin injection sites NB venous thrombosis is more common than retail thrombosis NB bleeding is very uncommon

Answer 142

Investigations -FBC (↓platelets >50%) Mangement - stop heparin - address need for ongoing coagulation with argabotran/fondaparinux/danaparoid NB there should be lifetime avoidance of heparin

Answer 143

i.e. activated protein C resistance =i most common inherited thrombophilia, affects ~5% of UK population leads to ↑ risk of VTEs, but usually not screened for

Haematology Flashcards

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