Haematology Flashcards

Question

Investigations bleeding disorder

Answer 1

FBC (with platelet count), blood film. aPTT, PT (INR) Fibrinogen, TT (if strong bleeding history): - PFA 100 - vWF Atg (ELISA), ristocetin Cofactor, FVIII activity assay LFTs, EUCs Iron studies

Answer 2

Fucking Heavy Bleeding is a MEDICAL emergency - Family Hx (bleeding or disorder) - Hospitalisations, transfusions, previous episodes - Bruising, telangectasias, petechiae - Menorrhagia - Estrogen (OCP, HRT, pregnancy) - Drugs - Iron deficiency - Challenges (surgery, trauma, dentist) - Associated medical conditions - Lab abnormalities previously

Answer 3

pre-test probability (VT OILCAMP) - veins (collateral, non varicose) - tenderness along venous system - oedema (pitting) > opposite - immobility >3 day or surgery < 4 weeks - leg swollen - calf swollen >3cm opposite - alternative diagnosis more likely (minus points) - malignancy < 6months - paralysis ``` 0 = low = 3% 1-2= moderate = 17% 3-8 = high = 50 to 75% ```

Answer 4

``` Rouleaux formation Anaemia Leukopenia Thrombocytopenia Monoclonal plasma cell -rare on blood film ->presence indicates plasma cell leukemia -readily detected with flow cytometry ```

Answer 5

Urinalysis -sulfosalicylic acid ``` FBC -differential -blood film EUC CMP -calcium Lactate dehydrogenase Albumin Beta 2 microglobulin CRP/ESR ``` SPEP/immunofixation USEP/immunofixation sFLC Bone marrow biopsy and aspirate - HnE/immunophenotyping/FISH - percent plasma cells - morphology - immunophenotype Cross sectional imaging - whole body low dose non con CT - >most accessible - whole body MRI - >most sensitive for bone involvement - whole body FDG PET/CT - >most sensitive for extramedullary disease Skeletal survey - backup if cross sectional contraindicated - AP chest - AP C/T/L spine - AP humeri and femora - AP pelvis - AP and lateral of skull - findings - >punched out lytic lesions - >osteopenia - >fractures

Answer 6

Clonal bone marrow plasma cells >10% or Biopsy proven plasmacytoma + ``` 1. presence of organ impairment (CRAB) -hypercalcaemia -renal dysfunction (1 boney lesion -anaemia -boney lytic lesions ``` 2. evidence of inevitable organ impairment - >60% plasma cells - involved:uninvolved FLC ratio 100:1 - MRI with >1 focal lesion M protein usually >30g/L

Answer 7

Blood dyscrasia (SWAMPS) - Smouldering MM - Waldestroms macroglobulinaemia - AL amyloidosis - MGUS - POEMS - Solitary plasmacytoma

Answer 8

A Feared Mistake for Haematologists - Allergic reaction - Febrile non haemolytic reaction (anti-leukocyte) - Modulation with immunosuppression - Haemolytic anaemia (acute and delayed)

Answer 9

Haematologists Are Very Concerned about Injecting Blood Products - Hypothermia - Acute lung injury (leukocytes vs alloantibodies) - Volume overload - Citrate toxicity (alkalosis, calcium) - Immunologic (A Feared Mistake for Haematologists) - Bleeding due to dilutional coagulopathy - Purpura (exposure to platelet antigen)

Answer 10

Fatty streak and intimal thickening - earliest change - fatty streak = accumulation of lipid laden macrophages within intima - intimal thickening = proliferation of smooth muscle cells and proteoglycan matrix within intima Pathologic intimal thickening - extracellular lipid pools = lipid deposits, hyaluron and proteoglycans - no inflammation Inflammation and plague progression - deposition of LDL within intima - LDL becomes oxidised, which leads to increased expression of adhesion molecules on endothelial cells and chemotaxis - LDL activates TLRs on macrophages and drive inflammation within intima Fibroatheroma - collagen cap overlying necrotic core - fibrous cap made of collagen synthesised by smooth muscle cells - necrotic core formed by apoptosis of foam cells, which increases plaque size - haemorrhage into plaque by leaky vasa vasorum also contributes - as plaque grows there is positive remodelling of artery to increase lumen diameter and compensate for obstruction Thin fibrous cap - thought to develop from fibroatheromas though may arise de novo - thinning may be due to release of MMP that degrade collagen, or release of interferon gamma by T cells and subsequent smooth muscle cell apoptosis or collagen synthesis inhibition - more vulnerable to rupture Rupture or erosion rupture most common -large necrotic core, thin fibrous cap containing macrophages and T cell, limited smooth muscle cells -progressive degradation of cap by MMP, smooth muscle apoptosis and shear forces erosion -smaller necrotic core, intact fibrous cap, fewer macrophages and T cells and loss of endothelial lining -greater accumulation of hyaluron and proteoglycan may drive de-epithelialisation thrombus formation -in either case, exposure of blood to procoagulation subepithelial tissue, platelet activation/aggregation and coagulation

Answer 11

MM cast nephropathy - large waxy, laminated casts - >precipitated light chains and tam-horsfall - dipstick normal - sulfosalacylic acid positive AL amyloidosis - positive dipstick - minimal Bence Protein (UPEP/fixation)

Answer 12

MGUS - M protein <30g/L - clonal bone marrow <10% - no organ damage symptoms Smouldering MM - M protein >30 - clonal bone marrow 10-60% - no symptoms Waldenstrom macroglobulinaemia - lymphoplasmacytic lymphoma in bone marrow - IgM monoclonal gammopathy in blood - Absence of CD56 in bone marrow plasma cells - IgM M protein rare in MM Solitary plasmacytoma - normal bone marrow - absence of CRA Al amyloidosis - fewer bone plasma cells - no lytic bone lesions - modest Bence Jone proteinuria - positive dipstick ``` POEMS 1)peripheral neuropathy 2)monoclonal plasma cell disorder 3) -osteosclerotic lesions or -elevated VEGF or -Castlemans disease ->enlarged lymph nodes single region of body 4) with addition of -endocrinopathy -skin changes -organomegaly ```

Answer 13

Continuous doppler over dorsalis pedis or posterior tibial Ankle cuff inflated Released -> record systolic with return of pulse Repeat with other pulse on ipsilateral Repeat above on contralateral Measure brachial pulse with same method bilaterally Divide highest pulse for each lower limb by highest brachial Results - 0.9-1.3 = normal - >1.3 = calcified vessels - 0.4-0.9 = arterial obstruction, correlated with claudication - <0.4 = multilevel disease, non healing ulcer, gangrene

Answer 14

Percent plasma cells - determined by HnE staining - usually >10% Morphology - mature plasma cell - >oval - >abundant basophilic cytoplasm - >eccentrically placed nucleus - >clock face chromatin, no nucleoli - immature plasma cell - >dispersed nuclear chromatin - >prominent nucleoli - >high N:C - intracellular Ig accumulations - >Morula cells (bluish white grape like) - >Russel bodies (cherry red, round) - >Flame cells (vermilion staining glycogen rich IgA) - >Gaucher cells (overstuffed fibrils) - >crystalline rods Immunophenotype - determined by immunohistochemistry, immunoflurescence, flow cytometry - presence of kappa or lambda in plasma cells, but not both - normal ratio K:L = 2:1 - >4:1 or 1:2 = monoclonality - CD receptors - >no CD19 - >CD45 rare - >most CD56 positive Cytogenetics - FISH - no specific changes

Answer 15

Detection of M protein - SPEP = 80% sensitivity - SPEP + immunofixation = 90% - " " + light chain assay or UPEP/urine immuno fixation = 97% - remainder are non secretory MM Serum and Urine Protein Electrophoresis (SPEP/UPEP) - >gamma, beta or alpha 2 mobility - >narrow spike on densitometer tracing - >dense discrete band on agarose band Serum immunofixation - confirms presence of M protein - determines type of M protein - >IgG/IgA/IgM/IgD - >Kappa or Lambda light chains - possible results - >heavy chains + light chains - >light chains alone (Bence Jones) - reduction in uninvolved Ig in most cases - >less frequently, reduction of both Free light chain assay - anti sera to constant region of Kappa and Lambda light chains - >measures light chains not bound to heavy chains in serum - light chain MM detected by abnormality in ratio of K:L - >detected in >90% of MM - absolute FLC level also prognostic Light chain myeloma - approx 20% of cases - normal serum proteins - readily detected by sFLC, UPEP and urine immunofixation Non secretory - approx 60% will have abnormal sFLC ratios - majority of remainder will have M protein detectable in neoplastic cell cytoplasm - hypogammaglobinaemia seen in 50% of non secretory

Answer 16

Marrow damage: - infiltration - fibrosis Decreased production: - Aplastic - Iron deficiency Systemic: - Renal disease - Hypothyroidism

Answer 17

CBC: normocytic, normochromic anaemia Reticulocyte count: low response Blood film: nil Bone marrow: M:E 2-3

Answer 18

CBC: normocytic, may be macrocytic with reticulocytosis normochromic (unless iron deficient) Reticulocyte count: high Blood film: polychromatophilic macrocytes Bone marrow: nil.

Answer 19

Defect in haem synthesis. Iron is taken up by mitochondria but not incorporated into haem. Iron encrusted mitochondria surround nucleus, forming ring. Seen in myelodysplasia

Answer 20

CBC: Macrocytosis or microcytosis Normochromic or hypochromic Reticulocyte count: low Blood film: May show specific anomaly Marrow: M:E of 1, reflecting ineffective erythropoesis.

Answer 21

Dense blue circular inclusions in RBC that represent nuclear remnants and defective spleen function

Answer 22

small RBC without central pallor. Seen in hereditary spherocytosis and haemolytic anaemias

Answer 23

teardrop shaped RBC. Seen in haemolytic anaemias, iron deficiency, thalassaemia, myelofibrosis, myelodysplastic syndrome

Answer 24

RBC fragments, seen in microangiopathic haemolytic anaemia

Answer 25

Spiculated RBCs seen in liver disease, renal disease and splenectomy

Answer 26

Slit shaped central pallow in RBC, seen in inherited red cell membrane defects and alcoholism

Answer 27

RBC with central pallor that has dense center (bulls eye). Seen in thalassaemia, iron deficiency, cholestatic liver disease

Answer 28

Stacked coin agglutination. Abnormal serum protein levels (eg. multiple myeloma)

Answer 29

Denatured, agglutinated haemoglobin in G6PD

Answer 30

Bite cell. Removal of Heinz body

Answer 31

Mutliple toxic granules in neutrophils indicating severe trauma or infection (eg. sepsis)

Answer 32

Group of haemolytic disorders characterised by - anaemia - schistocytes Haemolytic uraemia syndrome - triad of - >microangiopathic anaemia - >thrombocytopaenia - >AKI - usually associated with diarrhoea - >shiga producing E coli Thrombotic thrombocytopaenic purpura - pentad of - >microangiopathic anaemia - >purpura - >AKI - >neurological signs - >fever - caused by - >deficiency in ADAMTS-13 - >vWF multimeres not cleaved causing platelet clumping Disseminated intravascular coagulation - acquired disorder - >trauma - >sepsis and infection - >malignancy - >toxic reactions - activation of coagulation pathways - >intravascular thrombi - >consumption of platelets and clotting factors - presents with - >trigger - >bleeding and purpura - >thrombocytopaenia - >prolonged aPTT/PT - >low fibrinogen - >high d-dimer/fibrin degradation production

Haematology Flashcards

(56 cards)