Haematology Flashcards

Question

WHAT IS NHL?

Answer 1

This includes all lymphomas without Reed–Sternberg cells —a diverse group.

Answer 2

Low grade e.g. Follicular Lymphoma High grade e.g. Diffuse Large B Cell Lymphoma Very high grade e.g. Burkitt’s Lymphoma

Answer 3

B-cells Diffuse large B-cell lymphoma (DLBCL) is commonest.

Answer 4

1. Elderly 2. Caucasians 3. History of viral infection (specifically Epstein-Barr virus) 4. Family history 5. Certain chemical agents (pesticides, solvents) 6. History of chemotherapy or radiotherapy 7. Immunodeficiency (transplant, HIV, diabetes mellitus) 8. Autoimmune disease (SLE, Sjogren's, coeliac disease)

Answer 5

1. Painless lymphadenopathy 2. Weight loss \>10% 3. Night sweats 4. Pyrexia \>38C 5. + GI symptoms if small bowel lymphomas 6. Skin involvement in T-cell lymphomas

Answer 6

**_Marrow and node biopsy_** Diagnostic **_Blood_** FBC, U&E, LFT **_Cytology_** LP for CSF cytology if CNS signs.

Answer 7

**_Low grade (follicular)_** W&W **_High grade (diffuse large B cell)_** Chemo BMT for relapse R-CHOP = Rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone.

Answer 8

Malignant proliferation of haemopoietic cells.

Answer 9

Acute Myeloid leukaemia (AML) Chronic Myeloid Leukaemia (CML) Acute Lymphoblastic Leukaemia (ALL) Chronic Lymphocytic Leukaemia (CLL).

Answer 10

Myeloblasts.

Answer 11

Basophils, Neutrophils and Eosinophils.

Answer 12

Lymphoblast.

Answer 13

B lymphocytes.

Answer 14

**Children** ALL AML **Adults** AML CML CLL

Answer 15

Sudan black stain

Answer 16

A lymphoma

Answer 17

Clonal expansion of myeloblasts in BM, blood and other tissues

Answer 18

More than 20% blasts cells in bone marrow

Answer 19

The commonest acute leukaemia of adults

Answer 20

Aetiology usually not obvious but risk increased in: 1. Preceeding Haematological disorders 2. Prior chemotherapy 3. Exposure to ionising radiation

Answer 21

**_Marrow failure_** Symptoms of anaemia, infection or bleeding. **_Fever_** **_Infiltration_** Hepatomegaly and splenomegaly GUM HYPERTROPHY + BLEEDING

Answer 22

1. **FBC** * ↓RBC * ↓PLT * WCC variable * Usually w/ neutropenia 2. **Bone marrow biopsy** * **Blast cells** may be few in the peripheral blood * 20 out of 100 white blood cells 3. **Clotting screen** * DIC may occur 4. **Blood film** * ****Aurer rods

Answer 23

1. Auer rods are diagnostic of AML 2. Myeloperoxidase makes this

Answer 24

1. **Supportive** * (blood/platelets/fluids) 2. **Treat Infection** 3. **Chemotherapy** * (remission induction (regenerate) ➔ consolidation(intensification) ➔ maintenance: steroids 4. **Bone Marrow Transplantation** * During 1st remission

Answer 25

Allopurinol Gout

Answer 26

Build up of premyelocytes from a problem with the retinoic acid receptor

Answer 27

1. Build up of premyelocytes 2. Lots of auer rods 3. Increase coagualation risk 4. Medical emergency

Answer 28

All trans retionic acid ATRA

Answer 29

CML is characterized by an uncontrolled clonal proliferation of myeloid cells Only partialy differentiate

Answer 30

Divide too quickly Spill out into blood and crowd it

Answer 31

1. Liver and spleen 2. Cause hepatosplenomegaly

Answer 32

Usually 40-60yrs age Rare in childhood Slight male dominance

Answer 33

Philadelphia Chromosome t(9;22) 80% BCR/ABL on 22 Causes an activated tyrosine kinase Worse prognosis if absent

Answer 34

**Symptoms** 1. Weight loss 2. Tiredness 3. Fever 4. Sweats 5. Bleeding (platelet dysfunction) 6. Abdominal discomfort (splenic enlargement). **Signs** 1. Splenomegaly (\>75%)— OFTEN MASSIVE 2. Hepatomegaly, anaemia, bruising

Answer 35

1. **FBC** * Raised WCC \>30x10^9 – all myeloid cells raised – neutrophils, macrophages, basophils, eosinophils). * Hb low or normal * Platlets variable * **can cause thrombocytosis** 2. **Cytogenetics (karyotype, FISH or PCR)** * Philadelphia Chromosome

Answer 36

1. **Matinib** is now considered first-line treatment * Inhibitor of the tyrosine kinase associated with the BCR-ABL defect * Very high response rate in chronic phase CML 2. **Hydroxyurea** 3. **Interferon-alpha** 4. **Allogenic bone marrow transplant**

Answer 37

Malignancy of B/T lymphocyte cell lines

Answer 38

Most common paediatric malignancy Rarer in adults

Answer 39

**_Genetic susceptibility_** Translocations **_+ an environmental trigger_**

Answer 40

1. CNS involvement (cranial nerve palsies and meningism) 2. Anaemia, bleeding, vulnerability to infection 3. Lymphadenopathy 4. Orchidomegaly

Answer 41

1. **FBC** * ↓RBC, ↓PLT, WCC variable, usually w/ neutropenia 2. **Blood film** * Blasts cells unless confined to bone marrow (+ bone marrow aspiration) 30 in 100 cells * Terminal deoxynucleotide transferase 3. **Clotting screen** * DIC may occur 4. **Lumbar puncture** * CNS involvement 5. **Cytogenetics**

Answer 42

1. **_Chemotherapy_** Remission induction (regenerate) ➔ consolidation (intensification) ➔ maintenance: steroids 2. **_Bone Marrow Transplantation_** During 1st remission 3. **_Supportive_** Blood/platelets/fluids

Answer 43

Accumulation of mature B cells that have escaped programmed cell death

Answer 44

* Don't divide quickly enough

Answer 45

1. Most common leukaemia 2. Generally elderly but 20% \<55yrs

Answer 46

**_Symptoms_** 1. Often none, surprise finding on a routine FBC. 2. May be anaemic or infection-prone. 3. If severe: 'B' symptoms - weight loss, sweats, anorexia. **_Signs_** 1. Enlarged, rubbery, non-tender nodes. 2. Splenomegaly, hepatomegaly.

Answer 47

1. Rai Staging 2. 1-5 based on examination and FBC

Answer 48

1. Increased WBCs greater than 15000 x 10^9 per litre 2. **_Film_** Smudge cells (also know as smear cells) B cell that have been broken

Answer 49

1. Autoimmune haemolysis 2. Increased Infection due to hypogammaglobulinaemia 3. Marrow failure

Answer 50

1. Rituximab 2. Chemotherapy 3. Bone marrow transplant + Radio

Answer 51

**_Disorders of quality (abnormal molecule or variant haemoglobins)_** Sickle cell disease **_Disorders of quantity (reduced production)_** a or b thalassaemia

Answer 52

Normal Hb 2xa, 2xb Foetal Hb 2xa, 2xg

Answer 53

Haemoglobin F to haemoglobin A.

Answer 54

1. Sickle-cell anaemia is an autosomal recessive disorder causing production of abnormal beta globin chains. 2. Microcytic anaemia

Answer 55

Variant haemoglobin arising because of a point mutation in the b globin gene.

Answer 56

Carriers of HbS are symptom free Carriage offers protection against falciparum malaria Sickle cell diseases arise in the homozygous state (SS) or in combined heterozygotes (SC or Sb thalassaemia).

Answer 57

Thiamine for adenine in 6th codon Beta globin gene Glutamic acid leaves Valine comes in

Answer 58

1. HbS polymerizes when deoxygenated 2. RBCs deform, producing sickle cells 3. Which are fragile and haemolyse, and also block small vessels 4. Sickling cells appear at acidosis at at time when they deposit lots of oxygen

Answer 59

1. Fatigue and Anaemia 2. Pain Crises 3. Dactylitis and Arthritis 4. Bacterial Infections 5. Leg Ulcers 6. Aseptic Necrosis and Bone Infarcts

Answer 60

1. Haemolytic 2. Vaso-occlusive ‘painful’ crisis 3. Aplastic crisis (sudden reduction in marrow production) - reduced reticulocytes 4. Sequestration crisis (There is pooling of blood in the spleen ± liver) - increased reticulocytes

Answer 61

1. **Hb ELECTROPHARESIS** * For dx * HbSS present and absent HbA 2. **Screen neonate** * blood/heel prick test 3. **FBC** * Low Hb, high reticulocyte count 4. **Blood film** * SICKLED erythrocytes * Howell-Jolly bodies * Taget cells

Answer 62

**_Acute complications_** Painful crisis Sickle chest syndrome Stroke **_Chronic complications_** Renal impairment Pulmonary hypertension Joint damage

Answer 63

1. **Prophylactic ABX** * Daily penicillin. * Pneumococcal & influenza vaccine * Folic acid 2. **Pain relief for crisis** * NSAIDs/paracetamol 3. **Hydroxyurea** * Hydroxycarbamide - REDUCE COMPLICATIONS * Increase conc. of HbF 4. **Transfusions** * Bone marrow transplant

Answer 64

Salmonella enteridis

Answer 65

The thalassaemias are genetic diseases of unbalanced Hb synthesis, with under-production (or no production) of one globin chain

Answer 66

Unmatched globins precipitate Damaging RBC membranes Causing their haemolysis while still in the marrow.

Answer 67

**_Thalassaemia Major_** Transfusion dependent **_Thalassaemia Intermedia_** Less severe anaemia and can survive without regular blood transfusions **_Thalassaemia Carrier/heterozygote_** Asymptomatic.

Answer 68

Microcytic

Answer 69

In homozygous beta-thalassaemia, little/no normal beta chain production EXCESS alpha chains. Alpha chains combine w. whatever beta, delta or gamma chains available increased production of HbA2& HbF.

Answer 70

Point mutations in beta-globin genes Chromosome 11 Decreased beta-chain production (beta+) or absence (beta0)

Answer 71

Beta thalassaemia major (**Cooley’s anaemia**) denotes abnormalities in both beta-globin genes.

Answer 72

6 -12 months

Answer 73

1. Failure to feed, listless, crying, pale. 2. **Bones widen due to increased activity** * Skull bossing 3. **Anaemia** 4. **Hepatosplenomegaly** * Lots of RBCs need to be destroyed

Answer 74

This is a carrier state

Answer 75

1. Usually asymptomatic 2. **Mild, well-tolerated anaemia (Hb \>90g/L)** * Mild hypochromic, microcytic anaemia - microcytosis is characteristically disproportionate to the anaemia

Answer 76

Moderate anaemia but not requiring transfusions There may be splenomegaly

Answer 77

1. **FBC & film** * Hypochromic & microcytic anaemia * Target cells are seen * Reduced reticulocytes and nucleated RBCs 2. **Dx by Hb electrophoresis** * Absent/low HbA. * HbA2 & HbF raised

Answer 78

**_BLOOD TRANSFUSIONS_** **_Give iron chelation_** Desferrioxamine Decrease iron loading **_Ascorbic acid_** Increased urinary excretion of iron. Long term folic acid supplements.

Answer 79

Children require lifelong blood transfusions. Consequence is an progressive increase in body iron load. Can't eliminate the excessive iron Patients inexorably develop a clinically worsening hemosiderosis Liver and spleen, leading to liver fibrosis and cirrhosis. Endocrine glands and the heart, resulting in diabetes, heart failure and premature death. Death ultimately occurs, mainly due to cardiac hemosiderosis.

Answer 80

**_4 deletions_** HbBarts, infants are STILLBORN(hydrops fetalis). **_3 deletions_** SEVERE anaemia. **_2 deletions_** Often ASYMP. carrier, may have mild anaemia. **_1 deletion_** Close to normal.

Answer 81

Autosomal dominant conditions. Deficiency of red cell membrane proteins caused by a variety of genetic lesions.

Answer 82

Spherocytosis & elliptocytosis most common. Neonatal jaundice.

Answer 83

1. Anemia due to hemolysis 2. Either in the blood vessels (intravascular hemolysis) or 3. Elsewhere in the human body (extravascular)

Answer 84

**_Genetic_** **Red cell membrane abnormalities:** Hereditary spherocytosis, elliptocytosis **Haemoglobin abnormalities** Sickle cell anaemia, thalassaemia **Enzyme defects** G6PD deficiency, pyruvate kinase deficien **_Acquired_** Systemic lupus erythematosus (SLE) Lymphoma Chronic lymphatic leukaemia (CLL)

Answer 85

Where the red blood cells are shaped elliptically. Horizontal interactions.

Answer 86

Where the red blood cells are shaped spherically. Vertical interactions.

Answer 87

RBC haemolyse because of different causes Haemolgobin released. Haptoglobin binds to haemoglobin to recycle it

Answer 88

Pallor Jaunidce Splenomegaly

Answer 89

1. ↑Unconjugated bilirubin 2. ↑LDH 3. Coombs test +ve * Detect antibodies stuck on surface of RBC 4. Reduced reticulocytes

Answer 90

1. Transfusion 2. Folic acid 3. Discontinue medications 4. Iron 5. Autoimmune haemolytic anaemia therapy

Answer 91

1. failure to thrive 2. jaundice, gallstones 3. splenomegaly 4. aplastic crisis precipitated by parvovirus infection 5. degree of haemolysis variable 6. MCHC elevated

Answer 92

1. Patients with a family history of HS, typical clinical features and laboratory investigations (spherocytes, raised mean corpuscular haemoglobin concentration [MCHC], increase in reticulocytes) do not require any additional tests 2. If the diagnosis is equivocal the BJH recommend the **EMA binding test** and the **cryohaemolysis test** 3. For atypical presentations electrophoresis analysis of erythrocyte membranes is the method of choice

Answer 93

Inherited enzyme deficiencies leading to shortened red cell lifespan from oxidative damage.

Answer 94

1. **Provides the fuel for the red cell** * NAD+ to NAPH 2. **Generates redox capacity to protect red cell** * Gluthathione

Answer 95

1. Caused by a wide variety of mutations within G6PD gene 2. X linked but women may also be affected **Drugs** 1. Sulphonamides 2. Nitrofurans 3. Antimalarials - chloroquine, primaquine 4. Antipyretics 5. Aspirin 6. Chloramphenicol

Answer 96

1. Most asymptomatic 2. But may get oxidative crises due to decreased glutathione production, precipitated by drugs 3. In attacks, there is rapid anaemia and jaundice

Answer 97

G6PD enzyme assay **_Film_** Heinz boides Bite- and blister-cells

Answer 98

1. Avoid precipitants (eg, henna) 2. Transfuse if severe

Answer 99

Anucleate cells formed by fragmentation of megakaryocyte (MK) cytoplasm in bone marrow.

Answer 100

1) Platelets adhere to vascular endothelium via collagen & vWF (von Willebrand factor) 2) Binding of platelets to collagen stimulates shape change 3) Activation, leading to the release of platelet granule contents including ADP, fibrinogen, thrombin and calcium. Ends with production of fibrin 4) Aggregation of platelets then occurs, cross-linking by fibrin 5) Activated platelets also provide a negatively charged phospholipid surface, which allows coagulation factors to bind

Answer 101

Contents that help the formation of fibrin, and provide a surface for clotting factors Va and Xa to function better.

Answer 102

Platelets P2Y12.

Answer 103

1. **Mucosal bleeding** * Epistaxis, gum bleeding, menorrhagia 2. **Easy bruising** 3. **Petechiae** * Red or purple spot on the skin, caused by a minor bleed from broken capillary blood vessels 4. **Purpura** * Red or purple discolored spots on the skin that do not blanch on applying pressure 5. **Traumatic haematomas** * (inc subdural)

Answer 104

**_Reduced platelet number (thrombocytopenia)._** Decrease in production. Increase in destruction. **_Normal numbers but reduced function._** Congenital abnormality in platelet function Medication e.g aspirin Von Willebrand disease (reduced VWF activity) Uraemia

Answer 105

Decreased platelet production.

Answer 106

**_Congenital thrombocytopenia_** Absent / reduced / malfunctioning megakaryocytes in BM **_Infiltration of bone marrow_** Leukaemia, metastatic malignancy, lymphoma, myeloma, myelofibrosis.

Answer 107

**_Reduced platelet production by bone marrow_** Low B12 / folate Reduced TPO (e.g. liver disease) Medication: Methotrexate, chemotherapy Toxins: e.g. Alcohol Infections: e.g. viral (e.g. HIV) TB Aplastic anaemia (auto immune) **_Dysfunctional production of platelets in BM_** Myelodysplasia.

Answer 108

**_Autoimmune_** Immune thrombocytopenia (ITP) Primary, or secondary **_Hypersplenism_** Portal hypertension, splenomegaly **_Drug related immune destruction_** E.g. Heparin induced thrombocytopenia.

Answer 109

1. IgG antibodies form to platelet and megakaryocyte surface glycoproteins 2. Antibodies are directed against the glycoprotein IIb/IIIa or Ib-V-IX complex. 3. Opsonized platelets are removed by reticuloendothelial system

Answer 110

May follow viral infection / immunisation esp in children Varicella

Answer 111

Occurs in association with some malignancies, such as: 1. Chronic Lymphocytic Leukaemia(CLL) 2. Infections e.g. HIV / HepC

Answer 112

Chronic ITP runs a fluctuating course of bleeding, purpura, epistaxis and menorrhagia. There is no splenomegaly.

Answer 113

1. Easy bruising 2. Purpura 3. Epistaxis (nose bleed) 4. Menorrhagia (heavy bleed at period)

Answer 114

1. FBC * Thrombocytopenia 2. Blood film 3. Increased megakaryocytes in marrow 4. Antiplatelet autoantibodies often present

Answer 115

1. First-line treatment for ITP is **oral prednisolone** 2. Pooled normal human immunoglobulin (IVIG) may also be used * It raises the platelet count quicker than steroids, therefore may be used if active bleeding or an urgent invasive procedure is required 3. Splenectomy is now less commonly used

Answer 116

1. Small blood clots develop throughout the bloodstream 2. Blocking small blood vessels maybe causing organ ischemia 3. Also depleting platelet supply leading to bleeding

Answer 117

Sepsis Malginancy Obstetric complications Intravascular haemolysis

Answer 118

Consumption coagulopathy, consumes platelets and clotting factors

Answer 119

**_Primary haemostasis_** 1. Vasocontriction 2. Platelets then adhere to the damaged endothelial wall 3. These then activate other platelets and form a plug **_Coagulation cascade_** 1. One coagulation factor gets proteolytically cleaved which cleaves other coagulation factors 2. This then cleaves fibrinogen into fibin which forms a mesh **_Fibrinolysis_** 1. This stops the clot from becoming too big.

Answer 120

1. Sepsis 2. Malignancy 3. Trauma 4. Obstetrics complications.

Answer 121

1. Underlying malignancy favours clotting 2. Widespread clot formation leading to 3. Ischaemia, necrosis and organ damage 4. Kidneys, Liver, Lungs and Brain. 5. Depletes clotting factors and platelets 6. Fibrin degradation products in circulation 7. Interferes with clot formation

Answer 122

1. Bleeding 2. Internal bleeding 3. Bruising 4. Ischaemia

Answer 123

**_FBC_** 1. ↓ platelets 2. ↓ fibrinogen 3. ↑ PT & APTT 4. ↑ fibrinogen degradation products 5. schistocytes due to microangiopathic haemolytic anaemia **_Chronic DIC_** Could look normal due to compensation

Answer 124

LOW circulating coagulation factors

Answer 125

1. FFP: contains clotting factors - FIRST LINE 2. Platelets 3. Cryoprecipitate: contains fibrinogen and some clotting factors 4. Underlying cause

Answer 126

In TTP, blood clots form in small blood vessels throughout the body

Answer 127

1. Abnormally large and sticky multimers of **von Willebrand's factor** cause platelets to clump within vessels 1. **ADAMTS13** (a metalloprotease enzyme) which **breakdowns** ('cleaves') large multimers of v**on Willebrand's factor** **** 1. Overlaps with haemolytic uraemic syndrome (HUS)

Answer 128

1. **Idiopathic (40%)** 2. Post-infection e.g. urinary, gastrointestinal 3. Pregnancy 4. Drugs: ciclosporin, oral contraceptive pill, penicillin, clopidogrel, aciclovir 5. Tumours 6. SLE 7. HIV

Answer 129

1. Rare, typically adult females 2. Fever 3. Fluctuating neuro signs (microemboli) 4. Microangiopathic haemolytic anaemia 5. Thrombocytopenia 6. Renal failure

Answer 130

**_Blood and protein in urine_** Haematuria/proteinuria **_Blood film_** Fragmented RBC - **Schistiocytes** Decrease platelets Decrease Hb **_Clotting tests are normal_**

Answer 131

Plasma exchange using FFP Steroids - prednisolone

Answer 132

Warfarin and Heparin

Answer 133

IgG antibody against platelet-heparin complex IgG/plt/heparin complex causes pltactivation and THROMBOSIS

Answer 134

After Cardiac bypass surgery

Answer 135

Bruising Bleeding Melena Epistaxis Hematemesis Haemoptysis

Answer 136

1. Stop drug straight away 2. **Heparin** - Protamine 3. **Warfarin** - Vitamin K/ Phytomenadione

Answer 137

**_Microcytic_** \<80 **_Normocytic_** 80-100 **_Macrocytic_** \>100

Answer 138

Iron deficiency Haemoglobinopathies e.g. Thalassaemia, sickle cell Anaemia of chronic disease e.g. CKD and so lack of EPO

Answer 139

1. Acute blood loss 2. Combined haematinic deficiency 3. Anaemia of chronic disease e.g. CKD and so lack of EPO

Answer 140

B12/Folate deficiency Alcohol excess/liver disease Metabolic disease e.g. hypothyroidism | (Megaloblastic)

Answer 141

Male Hb = 131 – 166 g/L Female Hb = 110 – 147 g/L

Answer 142

Microcytic

Answer 143

**_Blood loss_** eg menorrhagia or GI bleeding **_Poor diet_** May cause IDA in babies or children (but rarely in adults), those on special diets, or wherever there is poverty. **_Malabsorption_** (eg coeliac disease) is a cause of refractory IDA. **_Increased demand_** Pregnant women and child/adolesence

Answer 144

**_Koilonychia_** Spoon shaped nails **_Atrophic glossitis_** Painful tongue **_Angular stomatitis_** Corners of mouth inflammed **_Brittle hair and nails_** **_RARELY_** Post-cricoid webs (Plummer-Vinson syndrome).

Answer 145

1. **FBC & film** * MCV\<80 * Variation in size (anisocytosis) & shape (poikilocytosis) of cells. * Low serum iron & ferritin * Low reticulocytes (due to reduced Hb production) 2. **High TIBC** 3. **Ix of GI tract** 4. **Coeliac serology** ## Footnote **IF HAEMOGLOBIN \<10 WOMEN AND \<11 MEN THEY NEED URGENT UPPER AND LOWER ENDOSCOPE**

Answer 146

1. Oral iron, eg ferrous sulfate 2. **IV ferrous sulfate** for surgery * If deficient and interval for surgery short

Answer 147

Inflammation

Answer 148

A megaloblast is a cell in which nuclear maturation is delayed compared with the cytoplasm. This occurs with B12 and folate deficiency, as they are both required for DNA synthesis.

Answer 149

Green veg, liver, nuts and yeast.

Answer 150

Dietary Malabsorption Increased req (e.g. pregnancy); Folate antagonists

Answer 151

**_Low folate_** **_Low Hb_** **_Film_** Macrocytic anaemia - hypersegmented polymorphs and Megaloblasts

Answer 152

Folic acid supplements With B12

Answer 153

Deficiency in B12

Answer 154

Found in meat, fish & dairy products. NOT in plants!! Body stores last 4yrs! (in liver)

Answer 155

Binds to intrinsic factor & absorbed in terminal ileum Prod of DNA impaired w deficiency Decreased RBC prod.

Answer 156

1. **Pernicious anaemia**: most common cause 2. Post **gastrectomy** 3. **Vegan diet or a poor diet** 4. Disorders/surgery of terminal ileum (site of absorption) 5. Crohn's: either diease activity or following ileocaecal resection

Answer 157

1. **Blood film:** * Megoblasts and hypersegmented polymorphs 2. **Bone marrow biopsy:** * IF antibodies * Schilling test (medical procedure used to determine whether you're absorbing vitamin B-12 properly)

Answer 158

Vitmain B12 injections or tablets

Answer 159

This is caused by an autoimmune atrophic gastritis, leading to achlorhydria and lack of gastric intrinsic factor secretion.

Answer 160

**Other autoimmune diseases** Thyroid disease Vtiligo Addison’s disease Hypoparathyroidism.

Answer 161

1. Intrinsic factor (IF) antibodies 2. MCV increase 3. Serum B12 lower 4. Reticulocytes lower or normal as production impaired, 5. Hypersegmented polymorphs 6. Megaloblasts in the marrow

Answer 162

Treat the cause if possible. If a low B12 is due to malabsorption, injections are required. Replenish stores with hydroxocobalamin (B12) If the cause is dietary, then oral B12 can be given after the initial acute course.

Answer 163

The marrow is responsible for haemopoiesis. In adults, this normally takes place in the central skeleton (vertebrae, sternum, ribs, skull) and proximal long bones. In some anaemias (eg thalassaemia), increased demand induces haematopoiesis beyond the marrow (extramedullary haematopoiesis), in liver and spleen, causing organomegaly.

Answer 164

Pancytopenia is reduction in all the major cell lines: Red cells White cells Platelets

Answer 165

PANCYTOPENIA w. HYPOCELLULARITY (aplasia) of the bone marrow.

Answer 166

Decreased number of pluripotent stem cells. BM replaced by fat.

Answer 167

1. Mostly AI 2. Triggered by drugs (cytotoxics, chloramphenicol, sulphonamides, phenytoin, gold) 3. Irradiation 4. Fanconi anaemia (=inherited form) 5. Infections (hepatitis)

Answer 168

1. **Bone marrow exam for dx** * HYPOCELLULAR 2. **FBC** * Pancytopenia

Answer 169

Withdrawal of offending agent, supportive care & some definitive tx: 1. Blood & platelet transfusions 2. Prophylactic ABX/prompttx of infections **_\<40yo (curative)_** 1. Bone marrow transplant **_\>40yo_** 1. Immunosuppression w. anti-thymocyteglobulin & ciclosporin.

Answer 170

These are caused by proliferation of a clone of haematopoietic myeloid stem cells in the marrow. While the cells proliferate, they also retain the ability to differentiate into RBCS, WBCS or platelets.

Answer 171

RBC Polycythaemia rubra vera (PRV) WBC Chronic myeloid leukaemia (CML, p352) Platelets Essential thrombocythaemia Fibroblasts Myelofibrosis

Answer 172

Deficiency of the bone marrow, produces too many red blood cells. Genetic disorder.

Answer 173

**_Primary_** Polycythaemia Rubra Vera (PRV) JAK2 gene - increased sensitivty to EPO **_Reactive/secondary_** EPO excess, altitude, lung disease

Answer 174

May present with no symptoms May have Easy bleeding/bruising Fatigue Dizziness Headaches

Answer 175

1. FBC 2. Bone marrow biopsy 3. Genetic testing for JAK2 gene

Answer 176

1. **Aspirin** Reduces the risk of thrombotic events 2. **Venesection** First-line treatment to keep the haemoglobin in the normal range 3. **Chemotherapy** Hydroxyurea - slight increased risk of secondary leukaemia Phosphorus-32 therapy

Answer 177

1. 5-15% of patients progress to myelofibrosis 2. 5-15% of patients progress to **acute myeloid leukaemia** (risk increased with chemotherapy treatment)

Answer 178

Malaria is a disease caused by Plasmodium protozoa which is spread by the female Anopheles mosquito. There are four different species which cause disease in man: Plasmodium falciparum Plasmodium vivax Plasmodium ovale Plasmodium malariae

Answer 179

Fever paroxysms reflect synchronous release of flocks merozoites from mature schizonts **3 phases:** 1. Shivering (1h): “I feel so cold.” 2. Hot stage (2–6h): T≈41°C, flushed, dry skin; nausea/vomiting; headache. 3. Sweats (~3h) as T° falls.95

Answer 180

Anaemia, jaundice, and hepatosplenomegaly. No rash or lymphadenopathy. Anaemia is common, eg from haemolysis of parasitized RBCS (often serious in children). Thrombocytopenia.

Answer 181

Microscopy of films Rapid diagnostic test

Answer 182

Chloroquine

Answer 183

Deep vein thrombosis, a subset of venous thromboembolism (VTE), occurs in normal veins, usually the deep calf veins of the leg and propagates more proximally

Answer 184

1. Pain and tenderness 2. Pain may vary from an ache to cramping, from dull to sharp and from mild to severe, be intermittent or constant 3. Aggravated by movement and standing up and relived by elevating the leg 4. Local swelling and warmth 5. Mild pyrexia 6. Persistent tachycardia

Answer 185

Well's score D-dimer

Answer 186

Stop anticoagulation and repeat scan in 6-8 days

Answer 187

**NOAC** 1. 3 months - provoked 2. 6 months - unprovoked **UNLESS** 1. Severe renal impairment \<15/min 2. Antiphospholipid syndrome then LMWH

Answer 188

**_INR\>8 or active bleeding_** STOP warfarin Give vit K Restart warfarin when \<5 **_INR 5-8_** 1. Minor bleeding - STOP warfarin, give vit K 2. No bleeding - withhold 1 or 2 does of warfarin

Answer 189

**Apixaban or rivaroxiban** - Andexanet alfa **Dabigatran** - Idarucizumab Haemodialysis if treatment methods don't work

Answer 190

Protamine sulphate

Answer 191

1. Accelerated hypertension potentially leading to encephalopathy and seizures (blood pressure increases in 25% of patients) 2. Bone aches 3. Flu-like symptoms 4. Skin rashes, urticaria 5. Pure red cell aplasia\* (due to antibodies against erythropoietin) 6. Raised PCV increases risk of thrombosis (e.g. Fistula) 7. Iron deficiency 2nd to increased erythropoiesis

Answer 192

Neutropenic sepsis is a relatively common complication of cancer therapy, usually as a consequence of chemotherapy?

Answer 193

1. It most commonly occurs 7-14 days after chemotherapy. 2. It may be defined as a neutrophil count of \< 0.5 \* 109 in a patient who is having anticancer treatment and has one of the following: * A temperature higher than 38ºC or * Other signs or symptoms consistent with clinically significant sepsis

Answer 194

1. Piperacillin with tazobactam (Tazocin) 2. Started asap - do not wait for blood results

Answer 195

Haemophilia is an X-linked recessive disorder of coagulation

Answer 196

1. **Haemoarthroses (blood in joints)** 2. Haematomas 3. Prolonged bleeding after surgery or trauma

Answer 197

1. Prolonged APTT 2. Bleeding time, thrombin time, prothrombin time normal

Answer 198

1. Haemolytic uraemic syndrome is generally seen in young children and produces a triad of: 2. acute kidney injury 3. microangiopathic haemolytic anaemia 4. thrombocytopenia

Answer 199

1. full blood count: anaemia, thrombocytopaenia, **fragmented blood film** 2. Decreased haptoglobins 3. U&E: acute kidney injury 4. stool culture * looking for evidence of STEC infection * PCR for Shiga toxins

Answer 200

1. Treatment is supportive e.g. Fluids, blood transfusion and dialysis if required 2. There is no role for antibiotics, despite the preceding diarrhoeal illness in many patients 3. The indications for plasma exchange in HUS are complicated. As a general rule plasma exchange is reserved for severe cases of HUS not associated with diarrhoea 4. Eculizumab (a C5 inhibitor monoclonal antibody) has evidence of greater efficiency than plasma exchange alone in the treatment of adult atypical HUS

Answer 201

1. Fever 2. Abdominal pain 3. Hypotension

Answer 202

1. Von Willebrand's disease is the most common inherited bleeding disorder. 2. The majority of cases are inherited in an autosomal dominant fashion\* and characteristically behaves like a platelet disorder i.e. epistaxis and menorrhagia are common whilst haemoarthroses and muscle haematomas are rare

Answer 203

1. large glycoprotein which forms massive multimers up to 1,000,000 Da in size 2. promotes platelet adhesion to damaged endothelium 3. carrier molecule for factor VIII

Answer 204

1. prolonged bleeding time 2. APTT may be prolonged 3. factor VIII levels may be moderately reduced 4. defective platelet aggregation with ristocetin

Answer 205

1. tranexamic acid for mild bleeding 2. desmopressin (DDAVP): raises levels of vWF by inducing release of vWF from Weibel-Palade bodies in endothelial cells 3. factor VIII concentrate

Answer 206

1. May be normal 2. Fat emboli tend to lodge distally and therefore CTPA may not show any vascular occlusion, a ground glass appearance may be seen at the periphery

Answer 207

1. Prompt fixation of long bone fractures 2. Some debate regarding benefit Vs. risk of medullary reaming in femoral shaft/ tibial fractures in terms of increasing risk (probably does not). 3. DVT prophylaxis 4. General supportive care

Answer 208

1. The 2011 NICE guidelines suggest a target haemoglobin of 10 - 12 g/dl 2. Determination and optimisation of iron status should be carried out prior to the administration of erythropoiesis-stimulating agents (ESA). Many patients, especially those on haemodialysis, will require IV iron 3. ESAs such as erythropoietin and darbepoetin should be used in those 'who are likely to benefit in terms of quality of life and physical function'

Answer 209

To prevent graft versus host disease

Answer 210

Macrocytic anaemia and thrombocytopenia

Answer 211

1. Haemophilia 2. Von Wilebrand's disease 3. Vitmain K deficiency

Answer 212

1. Vitamin K deficiency

Answer 213

Factor V Leiden (activated protein C resistance) is the most common inherited thrombophilia, being present in around 5% of the UK population.

Answer 214

Protein C resistance

Answer 215

1. Tumour lysis syndrome (TLS) is a potentially deadly condition related to the treatment of high-grade lymphomas and leukaemias. 2. It can occur in the absence of chemotherapy but is usually triggered by the introduction of combination chemotherapy. 3. On occasion, it can occur with steroid treatment alone

Answer 216

1. A high potassium 2. High phosphate 3. Low calcium

Answer 217

1. Patients at high risk of TLS should be given * IV allopurinol or * IV rasburicase Immediately prior to and during the first days of chemotherapy.

Answer 218

Sideroblastic anaemia is a condition where red cells fail to completely form haem, whose biosynthesis takes place partly in the mitochondrion. This leads to deposits of iron in the mitochondria that form a ring around the nucleus called a ring sideroblast. It may be congenital or acquired.

Answer 219

1. full blood count * hypochromic microcytic anaemia (more so in congenital) 2. iron studies * high ferritin * high iron * high transferrin saturation 3. blood film * basophilic stippling of red blood cells 4. bone marrow * Prussian blue staining will show ringed sideroblasts

Answer 220

1. supportive 2. treat any underlying cause 3. pyridoxine may help

Answer 221

Mean corpuscular haemoglobin

Haematology Flashcards

(251 cards)