Haemotology Flashcards

Question

Investigations carried out for microcytic anaemia

Answer 1

Iron studies +/- Mentzer Index

Answer 2

Reticulocyte Count

Answer 3

1. Decreased production of erythropoesis 2. Blood Loss 3. Increased destruction

Answer 4

Disseminated Intravascular Coagulation Thrombotic Thrombocytopenic Purpura Hemolytic uremic syndrome

Answer 5

Lactate Dehydrogenase Haemoglobin: -Globin -Unconjugated bilirubin -iron

Answer 6

Fe deficiency Alpha & Beta Thalassemia Sideroblastic

Answer 7

Anaemia due to a decrease in iron

Answer 8

Anaemia Fatigue Headache Palpitations Pallor Nausea Iron Deficiency Pica (Pica is the abnormal craving or appetite for non-food substances, such as dirt, ice, paint, or clay.) Nail changes (koilonychia-spoon shaped nails) Hair Loss Glossitis and Angular stomatitis Restless Leg Syndrome Patients with iron deficiency anemia are often asymptomatic and have limited findings on examination. Remember Elder patient presenting with iron deficiency must be investigated for colon cancer malignancy even in the absence of Foecal blood

Answer 9

Decrease in the iron intake or impaired Absorption Vegan diet Low socioeconomic status Gastrectomy Coeliac Disease Increase in iron loss Menorrhagia Hookworm NSAID use Gastrointestinal Bleeding Increase in iron demand Pregnancy Infancy Side note Our body needs iron 1mg/day. Pregnant women need 5-6mg/day

Answer 10

Anaemia Vitamin B12 deficiency Folate deficiency Thalessemia - also hypochromic and microcytic Anaemia of chronic disease Sideroblastic Iron Disorder Atransferrinaemia Lead toxicity

Answer 11

The lower the haemoglobin the more likely there is to be serious underlying pathology and the more urgent is the need for investigation. Patients without a clear physiological explanation for iron deficiency (especially men and postmenopausal women) should be evaluated by gastroscopy/colonoscopy to exclude a source of gastrointestinal bleeding, particularly a malignant lesion. Full blood count Haemoglobin/Haematocrit: decreased Mean cell volume (MCV): decreased Red cell distribution of width: increased because as iron decreases cells become smaller, increasing distribution *Think In anaemia of chronic disease, the RDW decreases the red blood cells of similar size.* Peripheral blood smear: microcytic, hypochromic red blood cells Iron studies Serum iron: decreased Total iron-binding capacity: increased Transferrin Saturation: decreased (transferrin is being overproduced to compensate for low iron) Serum Ferritin: reduced. Ferritin reflects iron stores and is the most accurate test to diagnose iron deficiency anaemia. Endoscopy: check for peptic ulcer disease, coeliac disease, and other gastrointestinal bleeding conditions Colonoscopy Coeliac disease screening at any age? Remember, IDA may be effectively diagnosed by full blood examination and serum ferritin level in most cases. Serum iron levels should not be used to diagnose iron deficiency. Side note A complete blood count can help determine the mean corpuscular volume or red blood cell size. Although iron deficiency is the most common cause of microcytic anaemia, up to 40 per cent of patients with iron deficiency anaemia will have normocytic erythrocytes.

Answer 12

Diagnosis of iron deficiency anaemia requires laboratory-confirmed evidence of anaemia and evidence of low iron stores. Anaemia is defined as a haemoglobin level two standard deviations below normal for age and sex. Think: Iron deficiency anaemia and thalassaemia trait are the commonest causes of microcytic anaemia, but they may coexist. Serum ferritin and haemoglobin A2 quantitation are the two most important investigations to distinguish between iron deficiency anaemia and thalassaemia trait

Answer 13

Once iron deficiency anaemia is identified, the goal is to determine the underlying aetiology. Decreased iron intake Poverty Starving Patient not taking oral iron therapy Patient taking an iron supplement or multivitamin tablet with insufficient iron content Inadequate diet or impaired absorption Concomitant consumption of inhibitors of iron absorption (eg, tea, calcium, antacids, tetracycline, within 2 hours of iron ingestion) Coexisting inflammation with functional iron deficiency Intestinal mucosal disorders (eg, coeliac disease, inflammatory bowel disease) Impaired gastric acid secretion (including use of proton pump inhibitors) Gastric/intestinal bypass procedures Helicobacter pylori colonisation Controlled-release iron formulations may contribute (ie, potential for limited iron absorption in some patients Increased iron loss Occult, undiagnosed or recurrent gastrointestinal blood loss (eg, peptic ulcer, malignancy, angiodysplasia, small bowel lesion, parasites) Other source of recurrent blood loss (eg, menorrhagia due to uterine pathology or an inherited bleeding disorder such as von Willebrand disorder) Multiple sources of recurrent blood loss (eg, hereditary haemorrhagic telangiectasia) Ongoing urinary iron losses (eg, significant valve haemolysis) Renal failure responding to erythropoietin-stimulating agents Increased iron requirements Coexisting condition interfering with bone marrow response Superimposed infection, inflammation, malignancy or renal failure Concomitant B12 or folate deficiency Coexisting primary bone marrow disease or suppression Unknown cause (congenital iron deficiency) Patients with an underlying condition that causes iron deficiency anemia should be treated or referred to a subspecialist (e.g., gynecologist, gastroenterologist) for definitive treatment. Remember Gastrointestinal, Genitourinary sources of bleeding should always be excluded

Answer 14

1. Iron is required to form the haem moiety in haemoglobin, myoglobin, and haem enzymes, also known as cytochromes. 2. Iron can also be stored in the form of ferritin 3. Iron Deficiency can be a result of: Decreased iron intake because of inadequate diet or impaired absorption Increased iron loss Increased iron requirements Depletion of Iron stores 4. Anaemia then results in decreased oxygen-carrying capacity and the resultant symptoms of fatigue, low energy level, and dyspnoea on exertion.

Answer 15

Oral iron replacement OR Parenteral iron replacement Blood transfusion - serious cases with low haemoglobin Ascorbic acid (helps in the absorption of iron) Improve diet Remember: Oral iron therapy, in appropriate doses and for a sufficient duration, is an effective first-line strategy for most patients.

Answer 16

Diastolic heart failure Impaired muscular performance Cognitive impairment Developmental delays

Answer 17

Iron reacts with protoporphyrin (pigment in RBC) converts it to haem. Haem is essential for haemoglobin. Therefore less iron-less haem-less haemoglobin

Answer 18

Iron deficiency (condition) It takes up most volume in blood and therefore becomes Microcytic- decrease in MCV less than 80fl.

Answer 19

Low birth weight History of prematurity Exposure to lead Exclusive breastfeeding beyond four months of life Weaning to whole milk and complementary foods without iron-fortified foods. Although iron deficiency anemia is associated with cognitive delays in children, it is unclear if iron supplementation improves cognitive outcomes.

Answer 20

Early symptoms: poor attention span, irritability Later symptoms & signs: cognitive deficits, lethargy, pallor, poor growth, weakness, listlessness, dyspnoea/ tachypnoea, conjunctival pallor, tachycardia pica, poor feeding, cardiomegaly & signs of cardiac failure

Answer 21

Genetic condition Two types (alpha and beta) Missing a globin chain: alpha: 1aplha + 2beta beta: 2alpha + 1beta

Answer 22

Mediterranean ancestry

Answer 23

microcytic

Answer 24

Bone stem cell transplant Iron Transfusion

Answer 25

Autosomal recessive Deletion of alpha globin genes on chromosme 16

Answer 26

1. Defective alpha gene: Silent carrier 2. Defective alpha gene: Alpha thalassemia Minor = mild symptoms 3. Defective alpha genes: Haemoglobin H (HbH) disease (excess Beta chains). Enlargens liver, spleen and bone containing bone marrow 4. Alpha genes deleted: Hb Bart's Hydrops foetal

Answer 27

+Haemoglobin electrophoresis Confirmed by: genetic testing

Answer 28

Autosomal recessive Mutation in Beta globin gene on chromsome 11. This results in a reduced or absent Beta globin chain synthesis

Answer 29

Confirmed : Haemoglobin electrophoresis. Low HbA, High HbF, HbA2

Answer 30

type of blood disorder where there’s a buildup of iron in the RBC’s in the body causing them to be immature and dysfunctional

Answer 31

Congenital abnormality Acquired cause: -Vitamin B6 deficiency -Excessive alcohol use -Lead poisoning =Decrease in functional haem -> damage to other organs -> anaemia and fatigue

Answer 32

Removal of Toxins Pyridoxine, thiamine & folic acid

Answer 33

Haemolytic – Sickle cell, Hereditary spherocytosis, G6PDH deficiency, Malaria, Autoimmune Haemolytic Non-Haemolytic – CKD, Aplastic

Answer 34

Anaemia due to premature destruction of red blood cells

Answer 35

Intravascularly: haemolysis occuring within vasculature Extravascularly: haemolysis occuring outside vasculature, typically in organs such as spleen or liver

Answer 36

Erythropoietin

Answer 37

Clearence of old/damaged RBC

Answer 38

1.Mechanical Valve (sheer stress) -> haemolysis 2.Microangiopathic haemolysis 3.Cold autoimmune haemolytic anaemia 4.Osmotic lysis following infusion of hypotonic solution 5.Acute transfusion reactions

Answer 39

Further subdivided into extracorpuscular and intracorpuscular

Answer 40

Sickle cell anaemia is an autosomal recessive disorder causing production of abnormal ß-globin chains. A single amino acid is substituted in the ß-globin chain (Glu to Val at position 6). This results in the production of HbS (haemoglobin Sickle) rather than HbA. The common variants of sickle cell disease are: Sickle cell anaemia (SS disease) is the most common Sickle cell trait - causes no disability and protects from malaria except in hypoxia. Sickle ß Thalassemia (HbS/ßthal) Sickle haemoglobin C disease (HbSC)

Answer 41

A missense mutation in the Beta Globin chain

Answer 42

When its not bound to oxygen

Answer 43

Splenic infarction (<2yo) Increase risk of infection Failure to thrive Chronic renal failure Gallstone Iron overload Lung damage - Hypoxia → fibrosis → pulmonary hypertension Aplastic crisis - Paravirus B19 infection causing drop haemotocrit Remember Paravirus B19 infection causing drop haemotocrit in sickle cell and thalassaemia. Treatment is immunoglobulins

Answer 44

Sickle cell disease can be diagnosed in newborns, as well as older persons, by hemoglobin electrophoresis, isoelectric focusing, high-performance liquid chromatography or DNA analysis FBC Blood smear - sickle cells and target cells Remember Sickle cell trait have normal blood smear, sickle cell anaemia does not! Side note Target cells are found in Thalassaemia too. Sickle solubility test The parents of the affected child with sickle cell aneamia will show features of sickle cell trait. Sickle solubility test is where a mixture of Hb S in a reducing solution such as sodium dithionite gives a turbid appearance because of precipitation of Hb S, whereas normal Hb gives a clear solution.

Answer 45

Autoimmune haemolysis Hereditary spherocytosis G6PD deficiency

Answer 46

Overview Newborns are usually asymptomatic because babies still have fetal haemoglobin Vaso-occlusive crisis Dactylitis (children) Mesenteric Ischaemia CNS infarction - seziures, stroke, cognitive defects Avascular necrosis (neck of femur) Leg ulcers Priapism Fever - infection Acute chest syndrome a new infiltrate on chest x-ray associated with one or more NEW symptoms: fever, cough, sputum production, dyspnea, or hypoxia. Acute splenic sequestration Splenomegaly Hepatomegaly Aplastic crisis - due to parovirus infection, with a sudden reduction in bone marrow production Remember children typically present with acute dactylitis. Males can present with priapism

Answer 47

Substituting one amino acid in the haemoglobin molecule results in sickle haemoglobin. Amino acid changed from Glu to Val. As a result, RBCs sickle in low oxygen states, causing occlusion of blood vessels, increased viscosity, and inflammation. The average life span of these sickle RBCs is 20 days (120days is normal)

Answer 48

Genetic Mutation (ankyrin and spectrin) results in a spherical haemoglobin-loses its biconcave shape. Not good at delivering oxygen effectively as it gets stuck

Answer 49

Family History of anaemia of haemolysis Northern European

Answer 50

Splenomegaly Haemolysis

Answer 51

Lab: Coombs test Clinically: Anaemia, jaundice, gall stones, splenomegaly

Answer 52

There is no cure for HS, but it can be treated. The severity of your symptoms will determine which course of treatment you receive. Options include: Surgery: In moderate or severe diseases, removing the spleen can prevent common complications that result from hereditary spherocytosis. Your red blood cells will still have their spherical shape but live longer. Removing the spleen can also prevent gallstones. Not everyone with this condition needs to have their spleen removed. Some mild cases can be treated without surgery. Your doctor might think less invasive measures are better suited for you. For example, surgery is not recommended for children younger than 5 years. Vitamins: Folic acid, a B vitamin, is usually recommended for everyone with HS. It helps you make new red blood cells. A daily dose of oral folic acid is the main treatment option for young children and people with mild cases of HS. Transfusion: You may need red blood cell transfusions for severe anaemia. Light therapy: The doctor might use light therapy, also called phototherapy, for severe infant jaundice. Vaccination: Getting routine and recommended vaccinations are also important to prevent infectious complications. Infections can trigger the destruction of red blood cells in people with HS.

Answer 53

Infections Food Medications e.g Sulfa-drugs & antimarials

Answer 54

Jaundice Dark Urine Back Pain Anaemia

Answer 55

Blood Smear: Heinz Bodies Bite Cells

Answer 56

Confirmed with Enzyme Assay

Answer 57

Avoid Oxidant Factors Transfusion (if haemolysis is SEVERE)

Answer 58

A condition where the immune system makes antibodies against specific antigens on RBC.

Answer 59

Pathway by which RBC are destroyed

Answer 60

Warm: corticosteroids Cold: Plasmapheresis

Answer 61

Form of pancytopenia resulting form the autoimmune destruction of haematopoirtic stem cells

Answer 62

Medications Viruses Toxins Genetic Disorders

Answer 63

Stem cell transplant Immunosuppressive therapy

Answer 64

Anaemia Thrombocytopenia Neutropoenia Deficient thrombocytes, leukocytes

Answer 65

Diagnosis: Bone marrow biopsy.

Answer 66

Impaired renal function > 3 months based on abnormal structure or function, or GFR <60 for > 3 months with evidence of kidney dysfunction

Answer 67

Patients with CKD are often asymptomatic until the advanced stages. Fatigue (Anaemia) Breathlessness (fluid overload/acidosis) Pruritis (itching) Restless legs Bone pain Leg swelling Severe Chronic Kidney Disease (GFR <20mL/min) Pericarditis Serositis Encephalopathy Gastrointestinal bleeding Uraemic neuropathy Examination Pallor Lemon tinge skin (Uremia) Scratch marks from pruritis Pericardial rub (Uraemic complication) Pleural effusions Palpable kidneys (Polycystic Kidney Disease or hydronephrosis) Peripheral Oedema

Answer 68

Remember Proteinuria, which is a clinical marker for CKD, is also indicative of an increased risk of cardiovascular disease

Answer 69

Diabetic Nephropathy Overview Type II diabetes Mellitus is the leading cause of Chronic Kidney Disease. It is classified as a secondary nephrotic syndrome. 20% of people with Type II diabetes will develop end stage kidney disease. Everyone with Diabetes should be screened yearly for microalbuminuria. Clinical features - Nephrotic Syndrome with signs and symptoms of diabetes (hyperglycemia) Pathological features Diabetic kidney disease is defined by characteristic structural and functional changes. The predominant structural changes include Mesengial expansion Glomerular basement membrane thickening Glomerular sclerosis

Answer 70

The majority of patients with CKD stages 1–3 do not progress to kidney failure. The risk of death from CV disease is far higher than the risk of progression. Mild to moderate CKD is usually managed in general practice or by other physicians caring for the patient. Referral to nephrologist should be considered if: Proteinuria with haematuria Stage 4-5 CKD Suspected rare cause of CKD Poorly controlled BP Rapidly falling eGFR Remember Normal GFR is >90mL/min/1.73m² (130L/day) Think Low eGFR and raised urine albumin are markers for death, CVD, End-Stage Kidney disease, Acute Kidney Injury

Answer 71

Hyperkalaemia Sodium and volume overload Metabolic acidosis Hyperphosphataemia Hypocalcaemia Anaemia *Hyperkalaemia on ECG is characterised by Peak T wave and later widened QRS complex Hypocalcaemia on ECG is charactersied by QT complex prolongation primarily by prolonging the ST segment. No change is T wave*

Answer 72

Determining Renal Function GFR - creatinine clearance and plasma creatinine/urea level Tubular function - glucosuria, EUC, CMP, plasma albumin Urine analysis CT scan - for renal artery stenosis or Urinary tract obstruction Determine renal structure Ultrasound - small kidneys suggest chronic disease CT scan Cystoscopy Assess effects of Chronic Kidney Disease on body FBC Serum ferritin and iron CMP LFT parathyroid hormone level Nerve conduction studies Arterial Doppler studies

Answer 73

General and limited progression/complication of CKD Exclude Acute Kidney injury Education Stop Smoking Weight reduction Encourage exercise Avoid alcohol Vaccination Fluid intake and diet Fluid and salt restriction are often important to prevent volume overload. A low-protein diet has been shown to slow the progression of renal failure in patients with CKD Phosphate restriction Potassium restriction Cardiovascular risk reduction Hypertension - ACE inhibitors or ARBs Hypercholesterolaemia - Statins Aspirin prophylaxis if not contraindicated Think CKD with hyperkalaemia be careful with using ACE inhibitors or K+ sparing diuretics. Symptomatic treatment (usually associated with uraemia) Anorexia Taste disturbance Dyspepsia Constipation Dyspnoea Dry skin and pruritus Anxiety and Depression Confusion Restless legs Treat complications Anaemia Erythopoesis stimulating agents Oral/intravenous iron Acidosis Phosphate/calcium/bones Hyperphosphataemia - Dietary restriction, phosphate binders (aluminium hydroxide) Vitamin D Hypocalcaemia - calcimimetics Secondart/Tertiary hyperparathyroidism - Parathyroidectomy Preparation for renal replacement therapy Consider when to start dialysis Consider suitability for transplant Dialysis Peritoneal dialysis Haemodialysis Indications for Dialysis (AEIOU) Acidosis Electrolytes - refractory hyperkalaemia Ingestions/intoxication - Barbiturates, lithium, alcohol, salicylates, theophyline Overload - Pulmonary oedema Uraemia Complications - pericarditis, refractory pulmonary oedema and encephalopathy

Answer 74

Megaloblastic – B12 deficiency, Folate deficiency Non-megaloblastic – Hypothyroidism, Alcohol excess, Liver disease

Answer 75

Vitamin B12 (cobalamin) deficiency is a common cause of megaloblastic anaemia, a variety of neuropsychiatric symptoms, and elevated serum homocysteine levels, especially in older persons. The recognition and treatment of vitamin B12 deficiency is critical since it is a reversible cause of bone marrow failure and demyelinating nervous system disease

Answer 76

Vitamin B12 is found in meat, fish, and dairy products. NOT in plants. The liver can store Vitamin B12 and stores are sufficient for up to 5 years. The daily requirement of vitamin B12 is about 2.4 μg. Side note Vitamin B12 is found almost exclusively in animal-based foods and is therefore a nutrient of potential concern for those following a vegetarian or vegan diet. Vegans, and anyone who significantly limits intake of animal-based foods, require vitamin B12-fortified foods or supplements. *Remember Vitamin B12 (cobalamin) is a water-soluble vitamin that is crucial to normal neurologic function, red blood cell production, and DNA synthesis.*

Answer 77

Risk Factors and Aetiology Decreased ileal absorption Crohn's disease Ileal resection Tapeworm infestation Decreased intrinsic factor Atrophic gastritis Pernicious anaemia Postgastrectomy syndrome Genetic Transcobalamin II deficiency Inadequate intake Alcohol abuse Older persons Vegetarians Alcohol abuse Prolonged medication use Metformin Proton pump inhibitors Histamine h2 blockers

Answer 78

Clinical manifestations of megaloblastic anemia include pallor, tachycardia, weakness, fatigue, and palpitations. The specific mechanism by which vitamin B12deficiency affects the neurologic system is unknown Remember There are extensive hepatic stores of vitamin B12. There may be a five- to 10-year delay between the onset of deficiency and the appearance of clinical symptoms Fatigue Short of breath Headache Pallor, hyperpigmentation Muscle Weakness Dizziness and Confusion Paraesthesia (numbness) Ataxia (loss of normal control of body movements) Atropic Glosstis (smooth erythematous surface of tongue) Remember Peripheral neuropathy is the most common symptom of vitamin B12 deficiency. Folate deficiency alone typically has no neurological symptoms.

Answer 79

Pernicious anaemia (autoimmune-mediated chronic atrophic gastritis) - a most common cause of severe vitamin b12 deficiency Postsurgical malabsorption Dietary deficiencies Vitamin B12malabsorption from food

Answer 80

High-dose oral Vitamin B12 supplements Parenteral cyanocobalamin or hydroxocobalamin Multivitamins - for vegans

Answer 81

Neurolgical deficits Haemotological deficits Gastric cancer (pernicious anaemia)

Answer 82

Pernicious Anaemia is a condition where there is lack of intrisinc factor, a glycoprotein responsible for the absorption of Vitamin B12. Vitamin B12 (Cobalamin) is an essential vitamin responsible for many physiological process in our body. Vitamin B12 deficiency causes megaloblastic anaemia and maybe accompanied by neurological abnormalities.

Answer 83

Infertility Gastric Polyps Gastric Cancer and Gastric Carcinoid Tumour Neurological deficits

Answer 84

Decrease in Folate : Impairs cell division Increase homocysteine

Answer 85

Lack of thyroid hormones

Answer 86

Primary Hypothyroidism: Clinical state resulting from underproduction of T4 and T3. Low free T4 with an elevated TSH is diagnostic of primary hypothyroidism. Autoimmune thyroiditis (Hashimoto's disease) is the most common cause of primary hypothyroidism. Secondary (Central) Hypothyroidism: The result of anterior pituitary or hypothalamic dysfunction.

Answer 87

Clonal proliferation of hematopoietic stem cells in the bone marrow

Answer 88

Clonal proliferation of hematopoietic stem cells in the bone marrow that develops rapidly requires immediate treatment and often presents with symptoms. In acute leukaemia, the cells in the bone marrow are immature (blasts).

Answer 89

clonal proliferation of hematopoietic stem cells in the bone marrow that develops slowly, treatment may be delated and often presents asymptomatically. In chronic leukaemia the cells in the bone marrow are still able to mature.

Answer 90

Bone marrow aspirate Bone marrow biopsy Cytogenetic testing Flow cytometry with immunophenotyping Molecular testing Peripheral smear

Answer 91

A patient with suspected leukemia should be referred to a hematologist-oncologist to confirm the diagnosis and initiate treatment. Treatment depends on type of leukaemia

Answer 92

Tumour Lysis Syndrome Disseminated Intravascular Coagulopathy - Widespread activation of coagulation, from release of procoagulants into the circulation with consumption of clotting factors and platelets, with ↑risk of bleeding Hyperviscosity Death

Answer 93

Lots of partially developed white blood cells in the blood over a long period of time

Answer 94

Chromosomal abnormalities

Answer 95

Both cells dont work effectively CML Cells: Divide too quickly CLL cells: don't die as they should

Answer 96

Chronic: Cells mature only partially Acute: Cell don't mature

Answer 97

Lymphoma is malignancy originating the lymph nodes. There are two types: Hodgkins lymphoma Non-hodgkins lymphoma

Answer 98

1.Lymphadenopathy 2.Constitutional symptoms Fever Night sweats Weight loss

Answer 99

Lymphoma Leukaemia Metastasis Infection Connective tissue disease - rheumatoid arthritis, SLE Infiltration - sarcoidosis Drugs - phenytoin

Answer 100

So, Hodgkin lymphoma is a tumor derived from lymphocytes - specifically B-cells which mainly reside in lymph nodes.

Answer 101

**Classical HL – (90-95% of cases). **The tumour cells in this group are derived from germinal centre B cells, but typically fail to express many of the genes and gene products that define normal germinal centre B cells. Classical HL is further divided into the following subtypes: Nodular sclerosis classical HL (NSHL) - (most common) Mixed cellularity classical HL (MCHL) Lymphocyte-rich classical HL (LRHL) Lymphocyte-depleted classical HL (LDHL) **Nodular lymphocyte predominant HL – The tumour cells in this subtype retain the immunophenotypic features of germinal centre B cells.

Answer 102

History of EBV infection Family history Young adults from higher socio-economic status Immunosuppression Autoimmune disorders

Answer 103

“Non-Hodgkin” refers to the absence of a key cell that’s seen in Hodgkin lymphoma, the Reed-Sternberg cell. Non-Hodgkin lymphoma is a tumor derived from lymphocytes - specifically B-cells and T-cells, which mainly live in the lymph nodes and move through the blood and lymphatic system.

Answer 104

Diffuse large B-cell lymphoma (DLBCL) Follicular lymphoma (FL) The rest have 1% or lower incidence

Answer 105

Night sweats Weight loss Fatigue Fever Lympadenopathy Splenomegaly Shortness of breath Cough Anaemia

Answer 106

Malignant disease of plasma cells in the bone marrow

Answer 107

Older people

Answer 108

No known hereditary/genetic component or definitive environmental risk factor

Answer 109

Pmnemonic: CRAB Calcium Elevated Renal Disease Anaemia Bone Lesions

Answer 110

Smoldering multiple myeloma (SMM): Asymptomatic Symptomatic multiple myeloma Non-secretory mutliple myeloma: Less common (3%)

Answer 111

Genetic mutations t(14,11) t(14,6)

Answer 112

Alcohol consumption Obesity Radiation Exposure Family History

Answer 113

1. Spinal Cord compression 2. Hypercalcaemia 3. Symptomatic Hyperviscosity 4. Light Chain Nephropathy

Answer 114

Excessive red blood cells =Can result in hyperviscous blood which is prone to clotting

Answer 115

Primary : Known as polycythaemia vera Secondary:

Answer 116

Mutations in bone marrow haematopoietic stem cells cause the excessive production of RBC 90% due to mutation in JAK2 (tyrosine kinase involved in erythropoietin receptor signalling). This means that epo which is usually produced by the kidneys in response to hypoxia is not needed for the proliferation of RBC

Answer 117

Excessive epo Appropriate: normal physiological adaptations due to hypoxia like COPD, sleep apnea Inappropriate: opposite to appropriate e.g Renal cell carcinoma

Answer 118

Incidental finding in asymptomatic patients Hyperviscosity: visual changes, muscosal bleeding, headaches Splenomegaly Pruritus Plethora Erythromelagia

Answer 119

1. Thrombotic events: DVT 2. Myelofibrosis

Answer 120

Initial FBC shows high RBC & haemoglobin 1. Exclude secondary polycythaemia 2. JAK2 mutation 3. Serum EPO -Primary: EPO Low -Secondary: EPO High

Answer 121

1.Aspirin 2.Venesection 3.Chemotherapy

Answer 122

Anaemia <45%

Answer 123

Polycythaemia >45%

Answer 124

Leukopenia <4,800ul

Answer 125

Leucocytosis >11,000 ul

Answer 126

Thrombocytopenia <150,000ul

Answer 127

Thrombocytosis >450,000 ul

Answer 128

Diseases that: Decrease platelet production Sequestration in spleen Increase in platelet destruction (non-immune/immune mechanisms)

Answer 129

Bleeding from mucocutaneous areas: platelet count <30,000 Disorders like HIT can paradoxically present with thrombotic events

Answer 130

Treating underlying cause: Transfusing platelets when platelet count <10,000

Answer 131

Quantitative or qualitative deficiency in VWF -> increases risk of bleeding

Answer 132

Most cases are inherited -People can acquire it

Answer 133

Type I: Moderate symptoms Type II: Moderate symptoms Type III: Severe Bleeding

Answer 134

Heavy Menstrual periods Mucocutaneous/Nose bleeding

Answer 135

Present with severe bleeding in joints and muscles. Rarely bleeding in the gut

Answer 136

Serum tests: -VWF antigen -Ristocetin: Test activity

Answer 137

Bleeding disorder caused by an impaired coagulation cascade X-Linked recessive

Answer 138

Mutations in genes for factor VIII

Answer 139

Mutations in genes for factor IX

Answer 140

Supplementing missing factor

Answer 141

Liver Failure Vitamin K deficiency Autoimmunity against a clotting factor Disseminated intravascular coagulation: consumes clotting factors

Answer 142

Symptoms depend on degree of mutation

Answer 143

Bleeding in the brain: -> Stroke -> Increased intracranial pressure

Answer 144

When haemostasis starts to run out of control resulting in widespread clotting leading to organ ischaemia. At the same time it depletes clotting factors and increase bleeding

Answer 145

Oxygen IV fluid

Answer 146

Caused when tumour cells release there intra-cellular content into the bloodstream either spontaneously or as a result of treatment. This leads to an increased: DNA purine and pyramidine Hyperkalemia Hyperphosphataemia Hypocalcaemia Lactic Acid

Answer 147

Acute Kidney Injury Arryhythmia Tetany Seizures

Answer 148

Large Tumour burden Rapidly dividing cells: acute leukaemia, high grade lymphoma **Chemotherapy**

Answer 149

Spontaneously (Before treatment) Within weeks after treatment

Answer 150

1. Identify those at risk and therefore provide: Prophylaxis Intravenous hydration Allopurinol Rasquricase 2. Treat tumour lysis syndrome and it's complications Dialysis

Answer 151

Cairo and Bishop diagnosis

Answer 152

The aPTT is 1 of several blood coagulation tests. It measures how long it takes your blood to form a clot. Normally, when 1 of your blood vessels is damaged, proteins in your blood called clotting factors come together in a certain order to form blood clots and quickly stop bleeding. The aPTT test can be used to look at how well those clotting factors are working.

Answer 153

A prothrombin time (PT) test measures how long it takes for a clot to form in a blood sample. An INR (international normalized ratio) is a type of calculation based on PT test results

Answer 154

Diagnosis Diagnosis of iron deficiency anemia requires laboratory-confirmed evidence of anemia, as well as evidence of low iron stores. Anemia is defined as a hemoglobin level two standard deviations below normal for age and sex Think Iron deficiency anaemia and thalassaemia trait are the commonest causes of microcytic anaemia, but they may coexist. Serum ferritin and haemoglobin A2 quantitation are the two most important investigations to distinguish between iron deficiency anaemia and thalassaemia trait

Answer 155

Autoimmune haemolysis Hereditary spherocytosis G6PD deficiency

Answer 156

Clonal proliferation of hematopoietic stem cells in the bone marrow

Answer 157

Clonal proliferation of hematopoietic stem cells in the bone marrow

Answer 158

occurs when leukaemia cells enter the lymph node and change into a high-grade, fast-growing non-Hodgkin's lymphoma. Patients often become unwell very suddenly. Ritcher's transformation is indicated by one of the following symptoms: lymph node swelling fever without infection weight loss night sweats nausea abdominal pain

Answer 159

Complications anaemia hypogammaglobulinaemia leading to recurrent infections warm autoimmune haemolytic anaemia in 10-15% of patients transformation to high-grade lymphoma (Richter's transformation)

Answer 160

Hereditary spherocytosis

Answer 161

monoclonal IgG or IgA paraprotein band, osteolytic lesions, increased infections

Answer 162

- multiple myeloma

Answer 163

Bence-Jones protein A Bence Jones protein is a monoclonal globulin protein or immunoglobulin light chain produced by neoplastic plasma cells. Presence of Bence Jones protein in the urine associated with anaemia and hypercalcemia make multiple myeloma the most likely diagnosis.

Answer 164

through increased osteoclast activity in response to cytokines released by the myeloma cells

Answer 165

Hypogammaglobulinaemia

Answer 166

anaemia: lethargy weight loss and sweating are common splenomegaly may be marked → abdo discomfort an increase in granulocytes at different stages of maturation +/- thrombocytosis decreased leukocyte alkaline phosphatase may undergo blast transformation (AML in 80%, ALL in 20%)

Answer 167

imatinib 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 hydroxyurea interferon-alpha allogenic bone marrow transplant

Answer 168

warm autoimmune haemolytic anaemia

Answer 169

Philadelphia chromosome, where a translocation between chromosome 9 and 22 causes the formation of the BCR-ABL gene.

Answer 170

Under 5 and over 45 – acute lymphoblastic leukaemia (ALL) Over 55 – chronic lymphocytic leukaemia (CeLLmates) Over 65 – chronic myeloid leukaemia (CoMmon) Over 75 – acute myeloid leukaemia (AMbitions)