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Final Year - ICSM > Haematology_Medicine > Flashcards

Haematology_Medicine Flashcards

1
Q

Anaemia - cause categorization? Ix? Deficiency-related anaemia - causes & relevant deficiencies?

A

Hb normal range: 130-175

MCV normal range: 82-98

Categorization:

  • MICROcytic - IDA, thalassaemia, anaemia of chr disease (can be normocytic)
  • NORMOcytic - acute bleed, aplastic anaemia, mixed anaemia (micro & macro)
  • MACROcytic - B12/folate def, alcohol excess, haemolytic anaemia

Ix: FBC

  • Microcytic - haematinics (Fe profile), Hb electrophoresis (thalassemia/SCD Dx)
  • Macrocytic - B12, folate, DAT test (AI haemolytic anaemia Dx)

Deficiency-related anaemia:

  • Poor dietary intake - Fe, B12, folate
  • Malabsorption (IBD) - Fe, B12
  • Pernicious anaemia (AI parietal cell destruction -> don’t prod intrinsic factor -> escorts B12 to terminal ileum for absorption) - B12
  • Crohn’s disease (most common in terminal ileum where B12 is absorbed) - B12
  • Bleeding (GI, menstrual) - Fe
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2
Q

Haem malignancies - Types?

A

Types:

  • Leukemia
    • Lymphoid
      • Acute (ALL) - child, TdT+ve
        • Can have BCR-ABL1, t(9;22)
      • Chronic (CLL) - smear/smudge cells, IgH UNmutated = worse prognosis, 17p gene deletion
    • Myeloid
      • Acute (AML) - Auer rods, MPO expression pattern
      • Chronic (CML) - Basophils, Philadelphia chromosome (BCR-ABL1, t(9;22)), left shift
  • Lymphoma
    • Hodgkin
    • Non-Hodgkin
  • Other
    • Myeloma
    • Myelofibrosis
    • Myelodysplasia
    • Polycythaemia Vera
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3
Q

Leukaemia vs Lymphoma?

Blood cell dev? How does this relate to Leukemia?

Lymphoma types? Staging?

A

Leukemia vs Lymphoma:

  • Same disease - characterised by abn prolif of lymphocytes
  • Different location:
    • Leukemia - blood & BM
    • Lymphoma - LNs

​Blood cell development & leukemia:

  • Physiology:
    • Differentiation (gene expression, morphology & cell function change –> mature cells right @end)
    • Proliferation (number of cells increase)
  • Acute - abn differentiation (not mature) + excessive proliferation –> acute onset, excessive blast cells, likely Sx (BM failure - anaemia, recurrent inf)
  • Chronic - normal differentiation (mature) + excessive proliferation –> mature cells, can be asymptomatic

Lymphoma

  • Hodgkin - B-cell only, single group of LNs, Reed-Sternberg cells (multinucleated lymphocytes)
  • Non-Hodgkin - 90% lymphomas, B/T-cell, multiple groups of LNs
    • Common features: painless lymphadenopathy, B-Sx, pruritis
  • NOTE: type ultimately determined by LN biopsy
  • Staging: Ann-Arbor staging
    • I: 1 site, 1-side of diaphragm
    • II: 2+ sites, 1-side of diaphragm
    • III: Both sides of diaphragm
    • IV: BM/splenic/solid organ involvement
    • NOTE: stage classified as A/B – based on presence of B symptoms (fever>38, night sweats, unintentional >10% weight loss in 6 months – FLAWS)
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4
Q

Multiple myeloma - def? pathophysiology? Spectrum of disease? Ix? Dx? Mx?

A

Def: cancer of plasma cells –> excessive monoclonal Ig prod

  • Plasma cell dyscrasia (humoral immune dysfunction) – clonal plasma cell population –> proliferate –> monoclonal Ig light chains (in blood = paraprotein, in urine = Bence Jones protein)
  • Pathophysiology:
    • Normally e.g. 5 different types of plasma cells produce 5 different types of Ig
    • In MM - one type of plasma cell outcompetes the others so lots of 1 type of Ig produced

Spectrum of disease:

  • Multiple Myeloma:
    • >1 focal lesion on MRI
    • BM plasma cells >60%
    • End organ damage (1+ of CRAB(S)):
      • Calcium (>2.75) - high: lytic bone lesions –> release Ca into circulation
        • NOTE: stones, bones, abdo groans, thrones, psychiatric overtones
      • Renal (from excess Ig) – creatinine clearance <40ml/min OR creatinine >177
      • Anaemia (Hb <100g/l) - BM supression
      • Bone lesions (lytic)
      • Signs of amyloidosis – damage from misfolded protein prod
  • Smouldering/asymptomatic myeloma
    • Serum monoclonal protein >3g/dL
    • BM plasma cells 10-60% in marrow
    • NO end-organ damage (CRABS) BUT most progress to MM untreated
  • Monoclonal gammopathy of unknown significance (MGUS)
    • Serum monoclonal protein <3g/dL
    • Plasma cells <10% in BM
    • No end-organ damage (CRABS)
    • NOTE: 1-2% progress to MM, very common in elderly (if low risk – yearly bloods)

Dx: plasma cells on BF + Rouleaux cells

Ix: ESR, Ca, U&E, serum & urine electrophoresis (to identify an excess of one type of Ig = 1 large band)

  • Electrophoresis (spike in gamma region, isolated IgG Kappa):
    • Normally polyclonal bands, in myeloma = monoclonal band
  • CD138= diagnostic

​Mx:

  • MM:
    • Young –> chemo followed by autologous SCT
    • Old –> chemo followed by maintenance therapy
  • Smouldering myeloma – treat
  • MGUS – annual blood test
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5
Q

Microcytic anaemia + GI features - Ix? Mx?

A

Ix:

  • IDA + lower GI features –> 2WW for colonscopy
  • IDA + dyspepsia –> 2WW OGD (oesopho-gastro-duodenoscopy)
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7
Q

Microcytic anaemia, disproportionately low MCV - Dx?

A

Thalassaemia

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8
Q

Normocytic anaemia, reduced renal function - Dx?

A

CKD-related renal function (EPO)

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9
Q

Macrocytic anaemia + mixed upper/lower motor signs - Dx?

A

B12 def –> subacute combined degeneration of spinal cord

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10
Q

CML Mx? CML chromosome? How to identify if in accelerated (blast) phase? Dx?

A

Tyrosine kinase inhibitor e.g. Imatinib

BCR-ABL1, t(9;22)

≥20% blasts, Basophils ≥20%, progressive splenomegaly

Dx: blood film/BM aspiration, definitive = cytogenetic analysis

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11
Q

Auer rods - Dx?

A

AML

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12
Q

DIC & t(15;17) - Dx?

A

Acute promyelocytic leukaemia

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13
Q

Haemophilia A Mx? Haemophilia A vs B? vWD difference?

A

A = factor 8

B = factor 9

vWD - bleeding time prolonged because platelet dysfunction

Haemophilia - bleeding time not prolonged because no platelet dysfunction

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14
Q

Menorrhagia + prolonged bleed time - Dx?

A

vWD

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15
Q

Low platelets + low fibrinogen?

A

DIC

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16
Q

Raised INR, low Pl, deranged LFTs - Dx?

A

Liver cirrhosis

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17
Q

Haem malignancy buzzwords

A

ALL - Testicular swelling, 3-5yrs

AML - Auer rods

CML - Philadelphia chr, t(9;22), BCR-ABL1, left shift

  • Tx: Imatinib (BCR-ABL tyrosine kinase inhibitor)

CLL - Smear/smudge cells

Polycythaemia vera - JAK2 mut, high haematocrit, flushed appearance, strokes/budd chiari

Essential thrombocythemia - High platelets, strokes, ± JAK2 mut

Myelofibrosis - Dry tap, teardrop cells (poikilocytes), massive splenomegaly

Hodgkin’s lymphoma - Painful LNs w/ alcohol, Reed-Sternberg cells, EBV

Follicular lymphoma - t(14;18), centroblasts

Mantle cell lymphoma - t(11;14), mantle cells

Burkitt’s lymphoma - t(8;14), starry sky appearance, EBV, HIV

Myeloma - CRAB, bence jones protein, IgG/A >30

MGUS - NO CRAB, paraprotein <30

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18
Q

Causes of increased vs decreased reticulocyte counts?

A

Increased reticulocytes

Decreased reticulocytes

Haemolytic anaemia

Recent haemorrhage

Thalassemia

Pregnancy

Treatment response

Hypoxia

Leukaemia

Aplastic anaemia

Megaloblastic anaemia (B12, folate)

Anaemia of chronic disease

Cirrhosis

Radiation

Decreased ACTH/pituitary hormones

19
Q

ALL - associated with what gene? Presentation? Ix? Mx?

A

ALL

  • BCR-ABL1 t(9;22) assoc w/ 20-30% ALL in adults –> this genetic mutation also causes CML
  • Child Hx: 2-5yrs
    • Hepatosplenomegaly
    • Bone pain/limp
    • Fevers, CNS Sx
    • Testicular swelling (rare but specific)
  • Adult Hx: like AML, lymphadenopathy
  • Investigations:
    • Bloods - thrombocytopenia, anaemia, high WCC (blasts/lymphocytes)
      • NOTE: circulating blasts = normal
    • Blood film – high nucleus: cytoplasm ratio, can’t differentiate betw/ ALL/AML on BF
    • Dx - BM + flow cytometry:
      • TdT+
      • CD19/22 = B cells (common)
      • CD2/3/4/8 = T cells
  • Management (adults and children similar aim):
    • Induction –> consolidation –> maintenance –> remission
    • Covering all these stages = transplant ±novel targeted therapies (+ CAR T-cell therapy)
20
Q

AML - Hx? Ix? Mx? AML vs CML difference on Ix?

A
  • Hx:
    • Incidence increases w/ age
    • Pre-existing myelodysplastic syndrome (MDS)
    • Cytopenia Sx
    • NOTE: AMML causes gingival hypertrophy (MM looks like gums)
  • Investigations:
    • Bloods – anaemia (BM suppression), high WCC (neutropenia, excess circulating blasts), thrombocytopenia (BM suppression), only abnormal INR if DIC from acute promyelocytic leukaemia
    • Blood film – single Auer rod = Dx, if none –> flow cytometry – MPO expression pattern
  • Management:
    • T(15;17) acute promyelocytic leukaemia – presents w/DIC, good prognosis, ALL-trans retinoic acid (ATRA) – causes cells to differentiate/stop prolif
    • Others: manage like ALL, poor prognosis esp in elderly (can’t tolerate stem cell transplant
  • AML VS CML- Basophils in CML
21
Q

CML - gene associated? Hx? Ix? Disease phases? Mx?

A
  • Most assoc w/ Philadelphia chromosome – BCR-ABL1 fusion gene from translocation of t(9;22) –> detected w/ FISH
  • Hx/exam:
    • Age 35-55yrs
    • LUQ pain (from splenomegaly)
    • Asymptomatic if Dx in chr phase ± lethargy, fever, night sweats
    • Sx of acute leukaemia if in accelerate/blast phase (10%)
  • Investigations:
    • Bloods: raised basophils (specific), high WCC (neutrophilia), 50% thrombocytosis, low monocytes (high = CMML), unlikely sign anaemia (can be), precursor cells on blood differential (promyelocytes/myelocytes)
      • High WCC causes:
        • Acute bact inf (high neutrophil: lymphocyte ratio)
        • Acute viral inf (low neutrophil: lymphocyte ratio BUT COVID-19 –> lymphopenia)
        • Fungal/parasitic (high eosinophils)
        • Monocytosis (in TB, endocarditis, inflame conditions)
  • Features:
    • Left shift – precursor cells present
    • High WCC, eosinophilia, basophilia
    • Hypo-lobated megakaryocytes – in BM
  • Disease phases:
    • Chr (90%)
    • Accelerated (increased blasts in BM, poor Tx-response, additional chromosomal abn)
    • Blast phase (>20% blasts in BM, behaves like acute leukaemia)
  • Management:
    • Chronic phase –> Tyrosine kinase inhibitors – 1st gen = Imatinib (2nd gen – Dasatinib/Nilotinib/Bosutinib, 3rd gen – Ponatinib)
    • >90% 10yr survival –> small % need transplants
    • Blast phase – Tx similar to AML (allogenic SCT for young)
22
Q

CLL - presentation? Ix? Mx?

A
  • Presentation:
    • Asymptomatic – routine bloods
    • >50yrs (incidence increases w/ age), X2 M>F
    • Possible LNs/splenomegaly
    • ITP (immune-mediated thrombocytopenic purpura)/haemolytic anaemia
  • Investigations:
    • Bloods: only anaemia if aggressive/haemolytic anaemia, high WCC (>100, mature lymphocytes)
    • BF: smear cells/smudge cells, lymphocytosis
    • Dx: flow cytometry – Kappa/Lambda light chains
      • Mostly B cell CLL (but can be T cell)
      • Same pathology as small lymphocytic lymphoma BUT different distribution (blood/BM Vs LNs)
      • B-cells CD5 +ve (normal mature B-cells CD5 -ve), CD38 +ve = poor prognosis
      • Immunoglobin gene mutations: IgH unmutated = worse prognosis
      • FISH – 17p gene deletion (TP53 – contains p53 tumour suppressor gene) gene deletion –> worse prognosis
  • Management:
    • Staging:
      • A – no cytopenia, <3 areas lymphoid involvement –> W&W
      • B – no cytopenia, ≥3 areas lymphoid involvement –> consider Tx
      • C – cytopenia –> TREAT
        • BCL-2 inhibitors (Venetoclax) – allows the normal apoptosis of B-cells
        • BCR-tyrosine kinase inhibitors (ibrutinib, idelalisib)
        • CAR T-cell therapy (for B cell cancers e.g., B-cell lymphoma)
        • NOTE: all very expensive
      • Richters syndrome – transformation of CLL –> aggressive disease (ALL/high grade lymphoma)
23
Q

Myeloproliferative disorders - characteristics? causes? Mx?

A

ALL = tyrosine kinase disorder (JAK2)

Essential thrombocythemia

  • High Pls: >450 (other causes of raise: acute inf, chr infl, malig (5-10%), polycythaemia rubra vera)
  • JAK2 mutation in 55%
  • Mx: aspirin to reduce stroke risk, hydroxycarbamide to lower pl count

Polycythemia vera

  • _High RBC_s:
    • Haematocrit >0.52 (M) /0.48 (F)
    • Often thrombocytosis – high risk of thrombotic event (MI, stroke, Budd-Chiari)
    • JAK2 mutation in 90%
  • Causes:
    • Primary: polycythaemia rubra vera
    • Secondary: altitude, chr hypoxia (severe COPD, cyanotic HD), erythropoietin-secreting renal cancers (RCC)
      • NOTE: secondary polycythaemia = no JAK2 mutation
  • Presentation: itchy (pruritus) after shower, peptic ulcers (increased histamine)
    • If very high RBC count –> hyperviscosity Sx, splenomegaly, thrombosis, gout
  • Mx:
    • Aspirin to reduce stroke risk, hydroxycarbamide to lower pl count
    • Venesection (removing blood –> lowers haematocrit)

Myelofibrosis

  • decrease all myeloid cell lines: MASSIVE SPLENOMEGALY
    • Clonal prolif of stem cells in BM –> cytokine release + fibrosis of BM –> pancytopenia
    • Features:
      • JAK2 mutation in 50%
      • Pancytopenia
      • Massive splenomegaly (extramedullary hematopoiesis)
      • Dry tap – on BM aspiration
      • Tear drop poikilocytes – on BF (leucoerythroblastic film)
  • Mx: stem cell transplant = only cure, ruloxitinib (JAK inhibitor)
  • NOTE: CML increases all myeloid cell lines (opposite)
24
Q

Myelodysplastic syndromes

Dx? Characteristics? Ix findings? Prognosis?

A

Myelodysplastic syndromes (MDS)

  • Pre-malignant BM failure/’early AML’ (<20% blasts; NOTE: >20% blasts = AML)
    • All 3 myeloid cell lines can be affected (erythroid, megakaryocyte, granulocyte)
    • Asymptomatic –risk progression–> AML
    • Can be secondary to chemo
  • Ix:
    • Hyposegmented + hypogranular neutrophils
    • Present w/ incidental pancytopenia, can have macrocytic anaemia (normal ferritin/B12/folate/erythropoietin –> suspicious of MDS)
  • Prognosis: 30% progress to AML, risk assessed w/ IPSS score
25
Q

Classical Hodgkin’s lymphoma - peaks when? presentation? histology? most common type? assoc inf? Mx?

A
  • Peaks: young, older adults
  • Presentation: localised LNs (freq mediastinal), B-symptoms (fever, WL, NS)
    • NHL = multiple nodal sites
    • Pain in LNs after alcohol
    • Neck node “rubbery”
    • Possibly assoc w/ EBV inf
  • Histology: Reed-Sternberg cells (“Owl’s eye” inclusions) = Dx (only 1 needed)
    • Other findings – eosinophils/macrophages, reactive fibrosis
    • Dx markers: CD30/15
  • Nodular sclerosing = most common type
  • Mx: ABVD chemo + radiotherapy –> good prognosis –> sometimes SCT
26
Q

MICROangiopathic haemolytic anaemia (MAHA)

  • Haemolytic uraemic syndrome (HUS)
  • Thrombotic thrombocytopenic purpura (TTP)
  • Disseminated intravascular coagulation (DIC)
A

MICROangiopathic haemolytic anaemia (MAHA)

  • Non-immune-mediated, small vessel disease, RBC breakdown
  • Damage to endothelial BV lining –> fibrin deposition + platelet aggregation –> fragmentation of RBCs (Schistocytes)
  • It is a mechanism NOT a disease

Haemolytic Uraemic Syndrome (HUS)

  • Post-_diarrhoeal_ illness – do NOT give abx
    • E.coli O157:H7 –> Shiga-like toxin can cause glomerular endothelial injury –> platelet plug forms (platelet consumption) –> shearing of blood vessels (MAHA) + reduced renal perfusion –> renal failure
    • Can get type with complement factor H deficiency
  • Diarrhoea in child –> triad:
    • MAHA (features on peripheral blood smear e.g. schistocytes)
      • Haemolysis signs - high LDH, low haptoglobins
    • Thrombocytopenia
    • acute renal failure (self-limiting in children)
  • Supportive Mx, anti-C5 Ab (ecluzimab)

TTP

  • Pathophysiology:
    • vWF multimers are normally broken down by ADAMTS13 but in TTP Abs against this –> reduced ADAMTS13
      • Causes: unknown, cancer, pregnancy
    • Increased vWF multimers = very sticky –> attach to endothelium & platelet plug forms (platelet consumption) –> shearing of blood vessels (MAHA) + reduced end-organ perfusion (can happen anywhere) –> confusion (brain), renal failure (kidneys)
  • Pentad: MAHA, thrombocytopenia, acute renal failure, NEURO Sx, fever
    • Case: 40yrs, fever, headache, jaundice for 1wk, temp 39, confused
      • Purpura, bleeding gums, haemoglobinuria
      • Bilirubin & LDH high = MAHA
  • Ab to metalloproteinase
  • Supportive Mx - plasma exchange + FFP

DIC

  • Trigger (sepsis, tumour, pancreatitis) –> increased exposure to Tissue factor –> factor 7 converted to 7a = coagulation cascade –> lots of miniclots formed throughout circulation - platelet & coagulation factor consumption​​
  • Very bad bleeding, Low platelets, PT & aPTT low (all coagulation factors low), low fibrinogen
27
Q

Case: 44yrs, admitted for Tx of staph cellulitis –> gangrene –> BP 86, bleeding from multiple sites

Ix: blood film shows schistocytes

Dx? Def? Findings on coag screen? Tx?

A

DIC - consumptive coagulopathy where you get massive activation of clotting cascade intravascularly (use up clotting factors and platelets) –> bleeding from multiple sites

  • Mortality 70-80%

Coagulation screen: high APTT, high PT, low platelets, low fibrinogen

Tx: replace platelets, FFP & cryoprecipitate, activated protein C

28
Q

Heparin vs Warfarin - how do they work? Warfarin reversal? INR target range?

A

Heparin: potentiates antithrombin III –> inactivates thrombin + factors 9, 10, 11 (effects the intrinsic (aPTT) and common pathways)

  • LMWH: given SC OD, does not require monitoring (except late pregnancy/renal failure – anti-Xa lvls monitored)
  • Unfractionated heparin (used if renal impairment): given IV, loading dose then infusion, monitor APTT (or anti-Xa/heparin lvls in some trusts)
  • Antidote: protamine sulphate
  • SEs: bleeding/heparin-induced thrombocytopenia (HIT), osteoporosis w/ LT use
    • SEs more common with UFH

Warfarin: inhibits reductase enzyme that regenerates the active form of Vit K –> inhibits synthesis of factors 2, 7, 9, 10 + proteins C, S and Z (effects the intrinsic and extrinsic (PT/INR) pathways)

  • Risk of teratogenicity
  • How to start Warfarin:
    • Check LFTs
    • Concurrent LMWH until INR >1.8 (protein C reduced w/ warfarin = temp procoagulant state + thrombosis in venules –> skin necrosis)
  • Warfarin reversal:
    • Any bleeding: stop Warfarin AND IV vit K slowly (takes 6hrs)
      • If major bleed = ADD dried PCC/FFP (takes 30 mins)
      • INR @24hrs –> continue Tx if INR high, continue Warfarin when INR <5
    • INR >8: stop Warfarin AND oral Vit K
      • INR @24hrs –> continue Tx if INR high, continue Warfarin when INR <5
    • INR 5-8: miss dose of Warfarin –> reduce maintenance dose
  • Therapeutic INR range:
    • 2.5 (2-3) – 1st episode of DVT/PE/AF (+ cardiomyopathy, symptomatic inherited thrombophilia, mural thrombus, cardioversion
    • 3.5 (2.5-3.5) – recurrent DVT/PE/mechanical prosthetic valve (+ coronary artery graft thrombosis, antiphospholipid syndrome)
29
Q

Haematological malignancies - stem cell differentiation & associated malignancies?

A

Multipotent stem cell differentiation & associated malignancies:

  • Common myeloid progenitor:
    • Megakaryocytes (create platelets)
    • Erythrocytes
    • Myeloblasts –> Neutrophils, Basophils, Eosinophils, Monocytes
    • NOTE: immature myeloid progenitor cells increase in AML
    • NOTE: mature myeloid cells (N,B,E,M) increase in CML
  • Common lymphoid progenitor:
    • T-cells
    • B-cells:
      • Plasma cells
      • NOTE: increased replication of abn plasma cell in MM –> produce huge amounts of one type of monoclonal Ig
    • NOTE: immature lymphoid progenitor cells increase in ALL
    • NOTE: mature lymphocytes increase in CLL
    • NOTE: if you get abnormal replication of lymphocytes in lymphatic system = Lymphoma (most are B-cell lymphomas)
30
Q

Leukaemia types - buzz words

A
  • Acute myeloid leukaemia (AML)
    • Acute, adults
    • BM failure (anaemia, inf risk, bleeds)
      • Extra: Acute myelomonocytic leukaemia (AMML) causes gingival hypertrophy (MM looks like gums)
    • Auer rods
  • Acute lymphoblastic leukaemia (ALL)
    • Children
    • BM failure (anaemia, inf risk, bleeds)
    • Failure to thrive (don’t grow along the normal curve)
  • Chronic myeloid leukaemia (CML)
    • Adults, often detected incidentally (overproduction of mature cells so doesn’t always cause rampant bone marrow failure)
    • t(9;22) = Philadelphia chromosome (BCR-ABL gene)
    • Tx: Imatinib (BCR-ABL tyrosine kinase inhibitor)
  • Chronic lymphocytic leukaemia (CLL)
    • Older adults, often detected incidentally (overproduction of mature cells so doesn’t always cause rampant bone marrow failure)
    • Smudge cells (fragile lymphocytes)
31
Q

Myelodysplasia vs Myelofibrosis

A

In normal BM - stem cells –> differentiate & proliferate

Myelodysplasia - abn differentiation of myeloid progenitor cells

  • Def: BM disorder resulting in pancytopenia AND production of functionally immature blood cells (essentially it is ‘early AML’, <20% blasts)
  • Chemo is a RF
  • Key facts:
    • Pancytopenia (all 3 myeloid lines can be affected)
    • 1/3 cases –> AML

Myelofibrosis

  • Def: clonal BM disorder characterised by deposition of fibrous scar tissue (over time, less and less tissue in BM that can produce blood cells)
  • Key facts:
    • Pancytopenia
    • Tear drop cells
    • Dry tap (due to level of fibrosis)
    • Massive splenomegaly (a site where body tries to compensate for low blood cell production in BM)
32
Q

Hereditary haemorrhagic telangiectasia (HHT) - Def? Dx criteria?

A

Aka Osler-Weber-Rendu syndrome - AD condition characterised by multiple telangiectasias over skin & mucous membranes

  • 20% cases spontaneous wo/ FHx

Dx criteria (2 = possible; 3 = definitive Dx):

  • Epistaxis: spontaneous, recurrent nosebleeds
  • Telangiectases: multiple @lips/oral cavity/fingers/nose
  • Visceral lesions: GI telangiectasia, pulmonary (increased stroke risk)/hepatic/cerebral/spinal AV malformations (AVM)
  • FHx: first-degree relative w/ HHT
33
Q

SCD exposed to child with coryzal Sx & rash - what are we worried about?

A

Parvovirus B19 infection can cause pancytopenia (aplastic anaemia) in predisposing haem conditions

34
Q

Haematinics - constituents? interpretation?

Iron studies - constituents? interpretation?

A

Haematinics - serum B12, folate, Intrinsic factor, ferritin

  • Low IF –> consider pernicious anaemia (cause of B12 def)

Iron studies - MCV, Fe, ferritin, TIBC, transferrin, transferrin saturation

  • Low MCV, low Fe, low ferritin & high TIBC/transferrin –> IDA (iron def anaemia)
  • Normal MCV, low Fe, high ferritin & low TIBC/transferrin –> consider Anaemic of chronic disease/haemoglobinopathy (SCD)
35
Q

Coagulation screen - constituents? interpretation?

A

PT, aPTT, Fibrinogen - light blue test tube

  • PT /INR measures extrinsic pathway (factor 7) and common pathway - measures overall clotting factor consumption as factor 7 rarely def in isolation
    • Raised in liver disease, DIC, vit K def, Warfarin
  • aPTT measures intrinsic pathway (factor 8/9/11) & common pathways
    • Raised by same as above + intrinsic pathway issues:
      • Haemophilia A (factor 8 def - X-linked recessive)
      • Haemophilia B (factor 9 def - X-linked recessive)
      • von Willebrand disease (as vWF pairs with factor 8)
      • NOTE: antiphospholipid syndrome can cause high aPTT despite causing clots as inactivates phospholipid used in intrinsic pathway
  • DIC - PT & aPTT raised, fibrinogen & platelets low
36
Q

Types of blood products? Indications? Duration over which given?

A

Packed red blood cells (PRBCs)

  • Indications: acute blood loss, Sx/chronic anaemia (Hb ≤70/80 with CVD)
  • 1 unit blood increases Hb by 10g/L
  • Given over 2-4hrs (must be completed within 4hrs of coming out of store)

Platelets

  • Indications: haemorrhagic shock in a trauma patient, profound thrombocytopenia (<20), bleeding with thrombocytopenia, pre-op platelets <50
  • 1 adult therapeutic dose (ATD) increases Pl by 20-40
  • Given over 30mins

Fresh frozen plasma (FFP) = clotting factors

  • Indications: DIC, haemorrhage secondary to liver disease, massive haemorrhage (after PRBCs)
  • Given over 30mins

Cryoprecipitate = fibrinogen, vWF, factor 8, fibronectin

  • Indications: DIC with low fibrinogen (<1g/L), vWD, massive haemorrhage
  • Duration = STAT
37
Q

Amyloidosis - def? types? Presentation? Ix? Mx?

A

Def: aggregates of proteins with fibrillar morphology & beta-pleated sheet structure depositing in body tissues

Types: occurs as a complication of other conditions

  • AA - serum amyloid A - chronic inflammation
    • RFs:
      • Inflammatory conditions (e.g. RA, psoriatic arthritis, ankylosing spondylitis, IBD esp. Crohn’s)
      • Chr infections (bronchiectasis, TB, chr UTIs, osteomyelitis)
  • AL - Ig light chain - multiple myeloma
    • RF: monoclonal gammopathy of undetermined significance (MGUS)
  • ATTR - TransThyRetin - familial, wild-type (elderly)

Presentation:

  • Purpura around the eyes, eyelid petechiae, enlarged tongue
  • Carpal tunnel syndrome (bilateral)
  • Peripheral neuropathy (not in AA) - symmetrical sensory loss of feet initially (temp, pain –> proprioceptive)
  • Autonomic neuropathy (not in AA) - erectile dysfunction/orthostatic HTN, GI/urinary dysfunction
  • Fatigue (amyloid cardiomyopathy/nephrotic syndrome), weight loss (cardiac/hepatic amyloidosis), dyspnoea on exertion (amyloid cardiomyopathy)
  • Exam: proteinuria, high JVP + pitting oedema (from restrictive cardiomyopathy)

Ix:

  • Serum & urine immunofixation (monoclonal protein in AL)
  • Ig free light chain assay (abn kappa to lambda ratio in AL)
  • FBC (anaemia), metabolic profile (hypoalbuminaemia, high ALP, low Ca)
  • 24hr-urine collection (>3g/day = nephrotic syndrome)

Mx: treat underlying condition

38
Q

Case:

  • 45yrs, tingling in arms & legs, loss of balance
  • Exam: loss of vibration sense in both feet
  • Ix: macrocytic anaemia, gastric antrum biopsy - achlorhydria & atrophic gastritis

Dx? Presentation? Ix? Mx?

A

Dx: pernicious anaemia aka atrophic gastritis/AI gastritis

Presentation: >60yrs, female

  • Subacute combined degeneration of spinal cord from B12 def:
    • Weakness, lethargy
    • Paraesthesia, difficulty ambulating
    • Ataxia, shuffling gait, decreased proprioception, decreased vibration sense
    • Memory loss, irritability, depression, dementia
    • Exam: koilonychia, macroglossia
  • Assoc w/ AI conditions e.g. Hashimoto’s thyroiditis
  • Risk of gastric adenocarcinoma

Ix:

  • Bloods:
    • FBC, haematinics (megaloblastic anaemia from B12 def), increased serum gastrin (increases PUD)
    • Abs: anti-IF (60% but more specific) & parietal cell abs (90% but can be normal variant)
  • Imaging:
    • Biopsy of corpus/fundus stomach (absence of parietal cell-containing oxyntic glands, achlorhydria, atrophic gastritis) + intra-gastric pH (ph>6 @rest rules out Dx)

Mx:

  • If PUD with H. pylori –> triple therapy (PPI + 2abx)
  • Replace deficiency (Fe, B12, Ca/Vit D)
39
Q

What is Ham’s test for? What is there increased risk of? Tx? What can this condition transform into?

A

Paroxysmal nocturnal haemoglobinuria

  • Acquired clonal abn of RBCs –> chr complement-mediated haemolysis + increased VTE risk (e.g. Budd-Chairi)
  • RBCs lack PIG
  • Tx: Eculizumab (anti-complement C5)
  • Complication: transforms to aplastic anaemia/AML
40
Q

What is osmotic fragility test for?

A

Spherocytosis

41
Q

Case:

  • Drowsy, visual disturbances, started on chemo & steroids by haem team
  • Anxious, thirsty, palpitations
  • ECG below

Dx? What happens? When is it high risk? Tx?

A

Tumour lysis syndrome - apoptosis of tumour cells (classical cause = steroids in leukaemia)

  • Drowsy, visual disturbances –> SOL –> chemo/steroids bursts these cells
  • Tumour cells contain - uric acid, PO4, K+ intracellularly
    • Urate nephropathy –> AKI
    • PO4 –> hypocalcemia; high K –> long QT; COMBO –> torsades de pointes
  • High risk in leukostasis - massive WCC counts - drowsy, retinal haemorrhages, pul oedema
  • Tx: hydrate + allopurinol before chemo
    • Emergency - give Rasburicase (reduce uric acid)
42
Q

Sickle cell crisis - Mx?

A

ACUTE (PAINFUL CRISES)

  • Oxygen
  • IV Fluids
  • Strong analgesia (IV opiates)
  • Antibiotics
  • Cross match blood
  • Give transfusion if Hb or reticulocytes fall sharply

Final Year - ICSM (20 decks)

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