Haemotology Flashcards

Question

What is anaemia of chronic disease

Answer 1

microcytic/ normocytic Reduced Hb related to chronic inflammatory disorders, chronic infections and malignancy

Answer 2

Inflammatory cytokines lead to reduced sensitivity of the marrow to erythropoietin and failure to incorporate iron into developing red cells.

Answer 3

Poor use of iron in erythropoiesis Cytokine-induced shortening of RBC survival Decreased production of and response to erythropoietin

Answer 4

Chronic infection, chronic inflammatory disease or malignancy Vasculitis Autoimmune disorders - Rheumatoid Renal failure

Answer 5

Ferritin normal (or increased in normocytic/microcytic anaemia) Check blood film – B12, folate, TSH and tests for haemolysis

Answer 6

Treat underlying disease Erythropoietin – helps raise Hb levels IV iron can overcome functional iron deficiency

Answer 7

flu-like symptoms, hypertension, mild rise in platelet count

Answer 8

A form of anaemia in which the bone marrow produces ringed sideroblasts rather than healthy red blood cells.

Answer 9

ineffective erythropoiesis, leading to increased iron absorption and iron loading in marrow – the body has enough iron, but cannot incorporate it into Hb.

Answer 10

Congenital – rare X-linked Chemotherapy Anti-TB drugs Alcohol excess

Answer 11

Increased ferritin Hypochromic blood film Disease-defining sideroblasts in the marrow

Answer 12

Remove the cause Pyridoxine Repeated transfusions for severe anaemia

Answer 13

Unusually large blood cells – low haemoglobin.

Answer 14

Megaloblastic Non-megaloblastic Other haematological

Answer 15

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

Answer 16

alcohol excess, reticulocytosis, liver disease, hypothyroidism.

Answer 17

myelodysplasia, myeloma, myeloproliferative disorders, aplastic anaemia

Answer 18

Test serum folate and B12 Blood film shows hypersegmental neutrophils for B12 deficiency Bone Marrow results in similar appearance for both.

Answer 19

1. Megaloblastic marrow 2. Normoblastic marrow 3. Abnormal erythropoiesis 4. Increased erythropoiesis

Answer 20

Maternal folate deficiency causes foetal neural tube defects.

Answer 21

green vegetables, nuts, yeast, and liver; it is synthesised by gut bacteria

Answer 22

Poor diet e.g. poverty, alcoholics, elderly Increased demand e.g. pregnancy or increased cell turnover Malabsorption e.g. coeliac disease Alcohol Drugs: anti-epileptics, methotrexate

Answer 23

Blood film – anisocytosis and poikilocytosis with large oval macrocytes Serum and red cell folate assay

Answer 24

Assess for underlying cause e.g. poor diet, malabsorption Treat with folic acid 5mg/day PO for 4 months Pregnancy: prophylactic doses of 400mcg/day are given until 12 weeks Helps prevent spina bifida

Answer 25

An autoimmune condition in which atrophic gastritis leads to a lack of IF secretion from the parietal cells of the stomach. Dietary B12 therefore remains unbound and consequently cannot be absorbed by the terminal ileum.

Answer 26

Dietary Malabsorption during digestion Congenital metabolic errors

Answer 27

Autoimmune gastritis affecting the fundus. Parietal and chief cells are replaced with mucin-secreting cells, leading to no intrinsic factor secretion. IF is required for B12 absorption = B12 deficiency. B12 and folate are needed for DNA synthesis so red cell development is arrested and they remain as large immature cells (megaloblastic). These may develop into abnormally large red cells – macrocytes.

Answer 28

Thyroid disease Vitiligo Addison’s disease Hypoparathyroidism

Answer 29

Symptoms of anaemia Mild jaundice due to haemolysis B12 deficiency causes

Answer 30

Neurological – paraesthesia, peripheral neuropathy, subacute combined degeneration of spinal cord Irritability, depression, psychosis and dementia

Answer 31

FBC: Decreased Hb Increased MCV WCC and platelets low if severe Reticulocytes may be low as production impaired Megaloblasts in the marrow Parietal cell antibodies IF antibodies

Answer 32

Treat cause Malabsorption – hydroxocobalamin to replenish B12 stores Dietary – oral B12

Answer 33

Anaemia due to haemolysis, the abnormal breakdown of red blood cells.

Answer 34

Drug-induced: causing formation of RBC autoantibodies from binding to RBC membranes or production of immune complexes. Autoimmune haemolytic anaemia: mediated by autoantibodies causing mainly extravascular haemolysis and spherocytosis. Paroxysmal cold haemoglobinuria: seen with viruses/syphilis Isoimmune: acute transfusion reaction

Answer 35

Autoimmune hepatitis; hepatitis B and C; post flu and other vaccinations, drugs

Answer 36

Mechanical disruption of RBCs in circulation, causing intravascular haemolysis and schistocytes

Answer 37

haemolytic-uraemic syndrome, eclampsia.

Answer 38

RBC lysis and blackwater fever (haemoglobinuria)

Answer 39

G6PD deficiency – chief RBC enzyme defect. Pyruvate kinase deficiency – decreased ATP production causes decreased RBC survival

Answer 40

Hereditary spherocytosis Hereditary elliptocytosis

Answer 41

Sickle-cell disease and thalassaemia

Answer 42

Anaemia due to bone marrow failure – defined as pancytopenia with hypoplastic marrow (bone marrow stops making cells).

Answer 43

reduced numbers of all major cell lines (red, white and platelets)

Answer 44

Autoimmune Drugs Viruses – parvovirus, hepatitis Irradiation Inherited – Fanconi anaemia

Answer 45

A reduction in pluripotent stem cells together with a fault in those remaining, or an immune reaction against them so that they are unable to repopulate the bone marrow.

Answer 46

Symptoms and signs of anaemia Bruising with minimal trauma Blood blisters in mouth

Answer 47

Pancytopenia Virtual absence of reticulocytes Hypocellular aplastic bone marrow with increased fatty spaces

Answer 48

Support the blood count Bone marrow transplant Immunosuppression can be helpful but no curative

Answer 49

A condition marked by an abnormal increase in the number of circulating red blood cells.

Answer 50

Caused by clonal proliferation of haematopoietic myeloid stem cells in the bone marrow. These cells retain the ability to differentiate into RBCs, causing an excess of RBCs. Somatic mutation in a single haematopoietic stem cell.

Answer 51

Vague symptoms due to hyper viscosity; Headaches Dizziness Tinnitus Visual disturbance Characteristic; Itching after a hot bath Erythromelalgia Burning sensation in fingers and toes

Answer 52

Facial plethora Splenomegaly Gout may occur due to increased urate from RBC turnover Features of thrombosis

Answer 53

Chronic myeloid leukaemia (CML) Essential thrombocytosis Primary myelofibrosis

Answer 54

increased RCC, increased Hb, increased PCV, increased WBC and platelets, increased HCT

Answer 55

Aim to keep HCT (haematocrit) <0.45 to decrease risk of thrombosis. Younger patients with low risk – venesection >60 years high risk – hydroxycarbamide is used. Low dose aspirin

Answer 56

can progress to acute myeloid leukaemia.

Answer 57

Genetic diseases of haemoglobin. Categorised by thalassaemia syndromes and structural haemoglobin variants.

Answer 58

Sickle cell anaemia is an inherited autosomal recessive disorder in which the production of abnormal haemoglobin results in vaso-occlusive crises. It arises from an amino acid substitution in the gene coding for the beta haemoglobin chain which leads to the production of HbS rather than HbA. HbS is 50 times less soluble than HbA Under conditions of low oxygen tension, HbS polymerises into rod-like aggregates which cause the red cell to adopt a sickle shape

Answer 59

HbS is caused by a point mutation in the β-globin gene on chromosome 11, which causes a replacement of valine for glutamic acid in the sixth position of the β-chain. Homozygotes (SS) – sickle cell anaemia Heterozygotes (AS) – sickle cell trait (protection against falciparum malaria)

Answer 60

Asymptomatic Asthenia (abnormal weakness/ lack of energy) Jaundice Ulcers around the ankles Bone deformities Infections – more vulnerable

Answer 61

Due to microvascular occlusion Can affect the ribs, spine, pelvis, tummy, legs and arms and hands/feet (particularly in children). Affects the marrow, causing severe pain, triggered by cold, dehydration, infection or hypoxia.

Answer 62

Painful crisis Sickle chest syndrome Stroke

Answer 63

Renal impairment Pulmonary hypertension Joint damage

Answer 64

Neonatal screening Hb electrophoresis – one major single HbS band and no normal HbA. Blood test - Increased reticulocytes, increased bilirubin. Film – sickle cells and target cells. Sickle solubility test - +ve (could be HbAS/HbSS)

Answer 65

Disease modifying treatment Transfusion Hydroxycarbamide Stem cell transplant Prophylaxis in cases of hyposplenism and immunocompromise.

Answer 66

A group of inherited red cell disorders caused by the underproduction of α- or β-globin chains.

Answer 67

Underproduction of either α- or β-globin chains causes accumulation or excess unpaired chains. This leads to the destruction of developing red cells and premature removal of circulating red cells in the spleen The anaemia in thalassaemia is therefore a combination of ineffective erythropoiesis and haemolysis in the spleen

Answer 68

alpha (gene deletions) and beta (point mutations)

Answer 69

Caused by point mutations in β-globin genes on chromosome 11, leading to decreased β chain production, or its absence.

Answer 70

β thalassaemia minor A carrier state that is usually asymptomatic. β thalassaemia intermedia Intermediate state with moderate anaemia, not requiring regular transfusions. May have splenomegaly. β thalassaemia major

Answer 71

Significant abnormalities in both β-globin genes, presenting with severe anaemia and failure to thrive (<1yr). Inadequate production of haemoglobin causes the severe anaemia, and children who are affected will require lifelong transfusions if they develop to adulthood

Answer 72

Failure to feed Listless (lacking energy) Crying Pale Extra-medullary haematopoiesis causes the characteristic head shape (skull bossing) and hepatosplenomegaly.

Answer 73

FBC – 40-70g/L Hb, MCV and MCH very low Film – large and small very pale red cells, nucleated RBCs Hb electrophoresis

Answer 74

Regular transfusions Iron chelation (removal of excess iron) Endocrine supplementation A histocompatible marrow transplant can offer chance of cure.

Answer 75

Ferritin Cardiac and liver MRI Endocrine testing – gonadal function, diabetes screening, growth and puberty

Answer 76

Thalassaemia major requires lifelong blood transfusions to grow and develop. This consequently increases the body iron load – there is no means for the body to eliminate the excessive iron. Excess iron is deposited mainly in the liver and spleen – leading to liver fibrosis and cirrhosis Deposited in endocrine glands and heart – diabetes, heart failure and premature death.

Answer 77

There are two separate α-globin genes on each chromosome 16 – four genes termed αα/αα. If all 4 genes are deleted, death is in utero – Bart’s hydrops. If 3 genes are deleted (--/-α), HbH disease occurs – moderate anaemia and features of haemolysis – hepatosplenomegaly, leg ulcers, jaundice. If 2 genes are deleted (--/αα) – asymptomatic carrier state – decreased MCV If one gene is deleted – normal clinical state.

Answer 78

Autosomal dominant conditions which result in an abnormally shaped red cell. Deficiency of red cell membrane proteins caused by a variety of genetic lesions

Answer 79

Autosomal dominant Shortage of red blood cells Less deformable spherical RBCs, so trapped in spleen -> extravascular haemolysis.

Answer 80

Splenomegaly Jaundice

Answer 81

fbc- Mild if Hb >110g/L and reticulocytes <6%. Increased bilirubin

Answer 82

Autosomal dominant Abnormally large number of the erythrocytes are elliptical. Mostly asymptomatic (small protection from malaria) 10% display a most severe phenotype

Answer 83

X-linked recessive Most are asymptomatic Lack of enzyme that maintains protective protein against oxidant injury.

Answer 84

May get oxidative crises due to decreased glutathione production. In attacks – rapid anaemia and jaundice. Precipitants – drugs, exposure to broad beans, illness, henna Drugs = primaquine, sulphonamides, quinolones, dapsone, nitrofurantoin

Answer 85

Enzyme assay Film – bite and blister cells

Answer 86

Transfuse if severe

Answer 87

Autosomal recessive Decreased ATP production causes decreased RBC survival

Answer 88

Variable chronic haemolysis Homozygotes have neonatal jaundice, later in life haemolysis and splenomegaly Prone to aplastic crisis in Parvovirus B19 infection.

Answer 89

enzyme assay

Answer 90

Often not needed – splenectomy may help. Transfuse during severe crisis

Answer 91

Produced in bone marrow Megakaryocyte fragments Regulated by thrombopoietin – produced in the liver

Answer 92

Low platelets = reduced bound TPO = increased free TPO = increased megakaryocyte stimulation = increased platelets.

Answer 93

Following damage to endothelium: Platelets adhere to vascular endothelium via collagen & vWF (von Willebrand factor) Binding of platelets to collagen stimulates cytoskeleton shape change within the platelets, and they spread out This increases their surface area and results in their activation, leading to the release of platelet granule contents including ADP, fibrinogen, thrombin and calcium. These components facilitate the clotting cascade ending with the production of fibrin Aggregation of platelets then occurs, which involves the cross-linking of activated platelets by fibrin Activated platelets also provide a negatively charged phospholipid surface, which allows coagulation factors to bind and enhance the clotting cascade

Answer 94

Mucosal bleeding Easy bruising Petechiae, purpura (red/purple discoloured skin) Traumatic haematomas

Answer 95

Aplastic anaemia Megaloblastic anaemia Marrow infiltration (leukaemia, metastatic malignancy, lymphoma, myeloma) Marrow suppression Marrow replacement

Answer 96

Immune thrombocytopenia Thrombotic thrombocytopenia

Answer 97

IgG antibodies form a platelet and megakaryocyte surface glycoproteins Opsonised platelets are removed by reticuloendothelial system Primary – may follow viral infection/ immunisation especially in children Secondary – occurs in association with malignancies and infections

Answer 98

Widespread adhesion and aggregation of platelets -> microvascular thrombosis -> profound thrombocytopenia

Answer 99

Congenital – Von Willebrand disease Acquired – uraemia, drugs

Answer 100

Dysfunctional production of platelets in bone marrow

Answer 101

abnormal size of platelets; absence of platelet alpha granules

Answer 102

FBC – isolated thrombocytopenia Raised lactate dehydrogenase levels (from ischaemic or necrotic cells) Physical examination – signs of bleeding or splenomegaly

Answer 103

Smoking Hypertension Diabetes Hyperlipidaemia Obesity/ sedentary lifestyle Stress/ type A personality

Answer 104

Glycosaminoglycan Binds to antithrombin and increases its activity (indirect thrombin inhibitor) Given by continuous infusion

Answer 105

Once daily, weight-adjusted dose given subcutaneously Used for treatment and prophylaxis of thrombosis

Answer 106

Inhibits cyclo-oxygenase irreversibly Inhibits thromboxane formation and hence platelet aggregation

Answer 107

Prevents synthesis of active factors II, VII, IX and X Antagonist of vitamin K Prolongs prothrombin time

Answer 108

Direct oral anticoagulant drugs Directly acting on factor II and X No blood test or monitoring Used for extending thromboprophylaxis Not used in pregnancy

Answer 109

Deep vein thrombosis a blood clot that develops within a deep vein in the body, usually in the leg.

Answer 110

is the obstruction of a blood vessel by a blood clot that has become dislodged from another site in the circulation.

Answer 111

Surgery Immobilisation Long haul flights

Answer 112

Factor V Leiden Antithrombin deficiency

Answer 113

Anti-phospholipid syndrome Lupus anticoagulant

Answer 114

Sluggish blood flow and/or increased blood coagulability overcomes natural anticoagulant activity and causes thrombus to form in the deep leg veins The thrombus may enlarge in size as it propagates along the lumen of the vein

Answer 115

Surgery, immobility, leg fracture, POP OC pill, HRT, pregnancy Long haul flights Inherited thrombophilia – genetic predisposition towards Caucasian

Answer 116

Surgery, immobility, leg fracture, POP OC pill, HRT, pregnancy Long haul flights Inherited thrombophilia – genetic predisposition towards Caucasian

Answer 117

Hydration and early mobilisation Compression stockings Foot pumps LMW heparin

Answer 118

Symptoms and signs are non-specific, clinical diagnosis unreliable Symptoms; Pain Swelling Signs; Tenderness Swelling Warmth Decolourisation

Answer 119

D-dimer: normal excludes diagnosis Positive does not confirm diagnosis – surgery/ pregnancy/infection can provide positive D-dimer (not useful for inpatients) Ultrasound compression test proximal veins Venogram for calf, recurrence uncertain

Answer 120

Oral warfarin LMWH (low molecular weight heparin) DOAC (direct oral anticoagulant) Compression stockings Treat underlying cause – malignancy, thrombophilia

Answer 121

Pulmonary embolism A blockage in one of the pulmonary arteries in your lungs, often caused by blood clots that travel to the lungs from the legs (DVTs).

Answer 122

A large PE is haemodynamically significant as it will block inflow of blood to lungs, so they can’t oxygenate the blood. It can lead to hypotension, cyanosis, severe dyspnoea and right heart strain/failure.

Answer 123

Breathlessness Pleuritic chest pain Cyanosis

Answer 124

Tachycardia Tachypnoea Pleural No signs of alternative diagnosis

Answer 125

CXR usually normal ECG – sinus tachycardia Blood gases: type 1 respiratory failure, decreased O2 and CO2 D-dimer: normal excludes diagnosis Ventilation/perfusion scan: mismatch defects

Answer 126

LMW heparin DOAC Treat the cause If cannot anti-coagulate, consider IVC filter (inferior vena cava)

Answer 127

Osler-Weber-Rendu syndrome, connective tissue disease

Answer 128

senile purpura, infection, steroids, scurvy

Answer 129

haemophilia, von Willebrand’s disease

Answer 130

anticoagulants, liver disease, vitamin K deficiency

Answer 131

An inherited bleeding tendency caused by a quantitative or qualitative deficiency of vWF.

Answer 132

vWF acts as an adhesion molecule which allows platelets to bind to subendothelial tissues and it also acts as a carrier for factor VIII Lack of vWF activity leads to a bleeding tendency due to a combination of failure of platelet adhesion and factor VIII deficiency

Answer 133

Mucosal bleeding, particularly nosebleeds, and bleeding after injury or surgery are the main manifestations

Answer 134

An inherited disorder of haemostasis characterised by bleeding tendency due to a deficiency of either factor VIII (haemophilia A) or factor IX (haemophilia B).

Answer 135

The factor VIII and factor IX genes are both located on the X chromosome, so haemophilia demonstrates sex-linked inheritance, with males being predominantly affected. Both disorders are X-linked recessive.

Answer 136

Factors VIII and IX together form the factor VIII-factor IX complex which activates factor X in the clotting cascade Lack of these factors impairs clotting

Answer 137

Factor VIII deficiency; inherited an x-linked recessive pattern

Answer 138

Depends on severity, often early in life or after surgery/trauma Bleeds into joints leading to crippling arthropathy Bleeding into joints causing haematomas

Answer 139

Increased APTT and decreased factor VIII assay

Answer 140

activated partial thromboplastin time, measures the length of time that it takes for clotting to occur when specific reagents are added to plasma

Answer 141

Avoid NSAIDs and IM injections Minor bleeding – pressure and elevation of the part Desmopressin raises factor VIII levels

Answer 142

Factor IX deficiency, behaves clinically like haemophilia A. Treat with recombinant factor IX.

Answer 143

a bleeding diathesis causing big mucosal bleeds caused by suddenly appearing autoantibodies that interfere with factor VIII.

Answer 144

produces a complicated bleeding disorder with decreased synthesis of clotting factors, decreased absorption of vitamin K, abnormalities of platelet function.

Answer 145

Malignant proliferation of haemopoietic cells.

Answer 146

A malignancy of lymphoid cells, affecting B/T lymphocyte cell linages, arresting maturation and promoting uncontrolled proliferation of immature blast cells, with marrow failure and tissue infiltration.

Answer 147

Marrow failure: anaemia Fatigue Shortness of breath Infection Tonsillitis Fevers/ rigors Bleeding – thrombocytopenia Hepato/splenomegaly, lymphadenopathy

Answer 148

Characteristic blast cells on blood film and bone marrow. WCC usually high CXR and CT scan to look for mediastinal and abdominal lymphadenopathy Lumbar puncture should be performed to look for CNS involvement

Answer 149

Educate and motivate patient to promote engagement with therapy. Support Blood/platelet transfusion IV fluids Hickman line for IV access Infections – immediate IV antibiotics and give prophylactic antivirals, antifungals and antibiotics Chemotherapy

Answer 150

Neoplastic proliferation of blast cells derived from marrow myeloid elements.

Answer 151

Marrow failure Anaemia Infection Bleeding Infiltration Hepatosplenomegaly Splenomegaly Gum hypertrophy Skin involvement

Answer 152

WCC is often increased Bone marrow biopsy – AML differentiated from ALL by Auer rods. Immunophenotyping

Answer 153

Predisposition to infection by both the disease and the treatment; may be bacterial, fungal or viral – prophylaxis is given for each during therapy. Chemotherapy causes increased plasma urate levels

Answer 154

Chemotherapy Daunorubicin and cytarabine Supportive measures Blood/platelet transfusion IV fluids Hickman line for IV access Walking exercises can relieve fatigue Bone marrow transplant Pluripotent haematopoietic stem cells

Answer 155

Characterised by an uncontrolled clonal proliferation of myeloid (blood-forming tissue in the bone marrow) cells.

Answer 156

A hybrid chromosome comprising reciprocal translocation between the long arms of chromosome 9 and the long arm of chromosome 22 – t(9;22).

Answer 157

Weight loss Tiredness Fever Sweats May be features of gout Bleeding Abdominal discomfort

Answer 158

Splenomegaly Hepatomegaly Anaemia Bruising

Answer 159

WBC – increased neutrophils, monocytes, basophils, eosinophils Film: left shift + basophilia Philadelphia chromosome Cytogenetic analysis of blood or bone marrow for Ph Increased urate

Answer 160

Target molecular therapy – tyrosine kinase inhibitors: Imatinib Hydroxycarbamide

Answer 161

nausea, cramps, oedema, rash, headache

Answer 162

The most common leukaemia. The hallmark is a progressive accumulation of a malignant clone of functionally incompetent beta cells. Mutations, trisomies and deletions influence risk.

Answer 163

Often none – surprise finding on routine FBC Anaemic Infection-prone Weight loss Sweats Anorexia (if severe)

Answer 164

Enlarged, rubbery, non-tender nodes Splenomegaly Hepatomegaly

Answer 165

Increased lymphocytes Autoimmune haemolysis Marrow infiltration; decreased Hb, neutrophils, platelets

Answer 166

Autoimmune haemolysis Increased infection due to hypogammaglobulinemia Marrow failure

Answer 167

Supportive care; Transfusions IV human immunoglobulin Stem cell transplantation Radiotherapy helps lymphadenopathy and splenomegaly Chemotherapy – rituximab and fludarabine (first line)

Answer 168

A malignant growth of white blood cells – predominantly the lymph nodes but also found in the blood, bone marrow, liver and spleen.

Answer 169

Lymphomas are caused by malignant proliferations of lymphocytes. These accumulate in the lymph nodes causing lymphadenopathy.

Answer 170

Characteristic cells with mirror-image nuclei are found, called Reed-Sternberg.

Answer 171

Enlarged, non-tender, rubbery superficial lymph nodes. Node size will fluctuate and become matted Constitutional upset – fever, weight loss, night sweats, pruritus, lethargy. Alcohol-induced lymph pain

Answer 172

Lymphadenopathy (abnormal lymph nodes) Cachexia (weakness and wasting of the body due to severe chronic illness) Anaemia Spleno/hepatomegaly

Answer 173

Tissue diagnosis; Lymph node excision biopsy if possible. Image guided needle biopsy Imaging; CXR CT/PET of thorax, abdo and pelvis

Answer 174

1 Confined to single lymph node region 2 Involvement of two or more nodal areas on the same side of the diaphragm 3 Involvement of nodes on both sides of the diaphragm 4 Spread beyond the lymph nodes on both sides of the diaphragm

Answer 175

Each Ann-Arbor stage is either ‘A’ (no systemic symptoms) or ‘B’ (presence of B symptoms) – weight loss, unexplained fever, night sweats. B indicates worse disease.

Answer 176

chemotherapy Stages IA-IIA: radiotherapy + short courses of chemotherapy Stages IIA-IVB (with >3 areas involved): longer courses of chemotherapy Relapsed disease: high-dose chemotherapy followed by autologous stem cell transplant. Good long term survival – must minimise long term effects of treatment

Answer 177

All lymphomas without Reed-Sternberg cells.

Answer 178

Immunodeficiency Drugs HIV Infection Infection from EBV transform cells Helicobacter pylori

Answer 179

Superficial lymphadenopathy Extra-nodal disease Fever Night sweats Weight loss Pancytopenia from marrow involvement (anaemia, bleeding) then local symptoms caused by lymphoma locations

Answer 180

Bloods: FBC (anaemia), U&E, LFT Marrow and node biopsy: classify high/low grade Staging: CT/PET of chest, abdomen, pelvis

Answer 181

Low-grade often incurable and widely disseminated (e.g. follicular lymphoma) Symptomless – no treatment needed

Answer 182

normally none needed and incurable Radiotherapy curative in localised disease Chemotherapy Remission maintained by rituximab

Answer 183

High-grade More aggressive, often curable (e.g. Burkitt’s lymphoma) Often rapidly enlarging lymphadenopathy with systemic symptoms

Answer 184

Granulocyte colony-stimulating factors (G-CSFs) help neutropenia ‘R-CHOP’ chemotherapy regimen: rituximab, cyclophosphamide, hydroxydaunorubicin, oncovin, prednisolone.

Answer 185

A malignant tumour of the bone marrow involving plasma cells (a type of WBC).

Answer 186

neoplastic proliferation of bone marrow plasma cells.

Answer 187

Monoclonal protein in serum/urine Lytic bone lesions/ CRAB end organ damage Excess plasma cells in bone marrow

Answer 188

an abnormal state of a body part

Answer 189

Plasma cell dyscrasias– an abnormal proliferation of a single clone of plasma/lymphoplasmacytic cells leading to secretion of immunoglobulin, causing the dysfunction of many organs. Clonal expansion -> monoclonal gammopathy of undetermined significance (MGUS) -> early myeloma -> late myeloma -> plasma cell leukaemia

Answer 190

Clonal proliferation of bone marrow cells, usually capable of monoclonal antibodies (IgA/IgG). Can be associated with excretion of light chains in the urine. Bone destruction – increased osteoclastic activity -> bone pain and osteolytic lesions. Infiltration of bone marrow – reduced function. AKI – due to light chain and amyloid deposition, hypercalcaemia and hyperuricaemia.

Answer 191

Tiredness and malaise Bone destruction; Bone/back pain +/- fractures Bone marrow infiltration; Recurrent bacterial infections (+anaemia) Non-specific

Answer 192

Anaemia Abnormal FBC Osteolytic lesions Renal failure Hypercalcaemia Raised globulins Raised ESR

Answer 193

Primary lymphoma of bone Metastatic bone disease

Answer 194

Protein in the blood (monoclonal protein band in serum or urine electrophoresis). Bone marrow plasma cells in excess of 10% present in a biopsy CRAB (calcium renal anaemia bone) Chromosome abnormalities; T(11;14) most common 13q- associated with treatment resistance and poorer prognosis

Answer 195

Analgesia for bone pain Bisphosphonate to reduce fracture rates and bone pain Local radiotherapy can help rapidly in focal disease Orthopaedic procedures (vertebroplasty) in vertebral collapse Transfusions to correct anaemia Infection – treat rapidly with broad-spectrum antibiotics Acute kidney infection - hydration Chemotherapy with stem cell transplant if possible

Answer 196

An infectious disease caused by protozoan parasites from the Plasmodium family – transmitted by the bite of the Anopheles mosquito or by a contaminated needle or transfusion.

Answer 197

The bite of an infected female Anopheles mosquito. Plasmodium sporozoites are passed on via bite of mosquito to human host. Travel to liver, where maturation occurs forming schizonts. May be a dormant stage before rupture – merozoites released which enter RBCs Undergo asexual reproduction larger trophozoites and erythrocytic schizonts The rupture of erythrocytic schizonts produces clinical manifestations of malaria

Answer 198

Fever – consider everyone to have malaria with fever who has visited a malarial area Headache Malaise Myalgia Diarrhoea Cough Delayed diagnosis – jaundice, confusion, seizures

Answer 199

Dengue Typhoid Hepatitis Meningitis/ encephalitis Viral haemorrhagic fever

Answer 200

Immediate blood testing is mandatory in the UK Detailed travel history – country, date of return, stopovers in other countries. Microscopy of thick and thin blood smear with giemsa stain If first blood film comes back negative, serial blood films should be repeated at 12-24h and at 24h after. Rapid diagnostic test detection of parasite antigen

Answer 201

Non-falciparum = chloroquine Falciparum: non-complicated = chloroquine/hydroxychloroquine. Complicated = artesunate, quinine sulfate