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158 Cards in this Set
- Front
- Back
What is the life cycle of an erythrocyte? |
Erytroblast to Reticulocyte and then erythrocyte |
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What stimulates erythropoiesis? |
Erythropoeitin |
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Where is it made and why? |
In the kidneys in response to hypoxia |
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What is reticulocyte count a measure of? |
Red cell production |
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What regulates production of platelets? |
Thrombopoietin |
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Where is it produced? |
The liver |
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What do neutrophils do? |
Ingest and destroy bacteria and fungi |
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What do increased neutrophils show? |
Infection or inflammation |
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Decreased neutrophils? |
Marrow failure Sepsis Autoimmune disease |
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What do monocytes do? |
Ingest and destroy bacteria and fungi |
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What are monocytes known as in tissues? |
Macrophages |
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When are eosinophils increased? |
In allergic reactions and parasitic infection |
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What types of lymphocytes are there? |
B, T & NK |
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Where are B and T cells produced and where do they mature? |
B - bone marrow for both T - produced in bone marrow, mature in thymus |
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What can cause splenomegaly? |
Infections - EBV, TB Malignancies - Leukaemia Portal Hypertension Haemolytic disorders - hereditary spherocytosis Connective tissue disorders - SLE |
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What is anaemia? |
Reduction in red cells or their Hb count |
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What materials are essential for red cell production? |
Iron B12 Folic acid Erythropoietin |
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What is hereditary spherocytosis? |
Defects in red cell membrane proteins causes cells to be spherical |
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How does it present? |
Anaemia Jaundice Splenomegaly Pigment gallstones |
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Management? |
Folic acid Transfusion Splenectomy |
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What does G6PD do? |
Prevents red cells from oxidative damage |
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How is G6PD deficiency passed on? |
X-linked |
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What is seen on blood film? |
Blister cells |
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How does it present? |
Neonatal jaundice and anaemia Often precipitated by infection or eating broad beans |
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How is sickle cell caused? |
Beta gene is replaced with sickle gene |
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What happens? |
Causes the production of sickle cells, which causes increased haemolysis which leads to vaso-occlusion |
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How does it present? |
Sickle cell crises - bones and chest commonly affected Increased infection risk Anaemia |
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What is the life long management of sickle cell? |
Vaccinations Penicillin prophylaxis Folic acid Blood transfusions Hydroxycarbamide Bone marrow transplant |
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What is the management of a sickle cell crisis? |
Hydration Oxygen Quick treatment Analgesia - opiates |
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What causes thalassaemias? |
Reduced or absent globin chain production due to mutation or deletion in gene |
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Which type is incompatible with life? |
Alpha thalassaemia |
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What does beta thalassaemia major present like? |
Severe anaemia at 3-6 months Expansion of bone marrow Splenomegaly Growth retardation |
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Treatment? |
Chronic transfusion support with deferasirox as iron chelation therapy BMT is curative |
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Explain the RhD blood groupings |
RhD negative people can only receive RhD negative blood, but RhD positive can receive any. |
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Who is given anti-D as an injection to prevent rhesus disease? |
Pregnant RhD negative women |
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What is donor blood screened for? |
Hep B Hep C HIV Syphilis |
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What is readily available in case of emergency? |
O- red cells and AB plasma |
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What is the massive haemorrhage policy? |
Immediate supply of: 6 units of red cells 4 units of FFP 1 units of platelets |
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What is a minor reaction to a blood transfusion? |
Fever and hives |
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Major reaction? |
Fever Hives Respiratory distress Hypotension ↑HR |
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What is the normal range of Hb in males? |
12-70 (140-180) >70 (116-156) |
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In females? |
12-70 (120-160) >70 (108-143) |
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What are the clinical features of anaemia? |
Tiredness Breathlessness Ankle oedema Dizziness Chest pain Factors relating to underlying cause |
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What tests should be done? |
MCH and MCV |
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Types of microcytic anaemia? |
Iron-deficiency anaemia Thalassaemia Sideroblastic anaemia |
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Causes of IDA? |
Blood loss Poor diet Malabsorption |
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What signs may be present in IDA? |
Koilonychia Atrophic glossitis Angular cheilosis |
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What test is used to confirm diagnosis? |
Serum ferritin - will be reduced |
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Treatment? |
Treat the cause Give iron tablets |
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Causes of normocytic anaemia? |
Acute blood loss Anaemia of chronic disease Bone marrow failure Renal failure Hypothyroidism |
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Causes of macrocytic anaemia? |
B12 or folate deficiency Alcohol excess Myelodysplastic syndromes |
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Where is iron stored in the plasma? |
Transferrin in plasma Ferritin in macrophages and hepatocytes |
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What does hepcidin do? |
Reduces intestinal iron absorption and iron mobilisation |
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When is hepcidin produced? |
In response to inflammation so can cause anaemia (anaemia of chronic disease) |
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What does B12/folate deficiency show on blood film? |
Megaloblastic anemia |
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What tests should be done? |
Blood film Bone marrow biopsy possible |
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What are causes of B12 deficiency? |
Pernicious anaemia Gastric/ileal disease |
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What is pernicious anaemia? |
Antibodies are produced against intrinsic factor and gastric parietal cells |
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Treatment? |
B12 IM injections |
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Causes of folate deficiency? |
Diet Increased requirement GI pathology |
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Treatment? |
Oral folate replacement |
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What is used to form a clot? |
Platelets Von willebrand factor Coagulation factors |
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What activates the formation of a clot? |
Abnormal surfaces and physiological activators |
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How is the clot formed? |
Platelets adhere to the collagen and form a plug. A fibrin meshwork forms over the top |
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What is the cause of haemophilia A & B? |
A - Absent factor VIII B - Absent factor IX |
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How does the clot remain confined to the site of the injury? |
Due to TFPI, Protein C & S and antithrombin which are natural anticoagulants |
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What gets rid of clot after it is needed? |
Fibrinolytic system |
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How does it work? |
t-PA and u-PA activate plasminogen to form plasmin which breaks down fibrin clot. |
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What marker is released as part of this process? |
D-dimer |
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What is a thrombus? |
A clot arising in the wrong place |
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What is a thromboembolism? |
The movement of a clot along a vessel |
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What is an arterial thrombus made up of? |
Platelets and fibrin |
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What is it secondary to? |
Atherosclerosis |
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What examples? |
Coronary - MI, angina Cerebrovascular - Stroke, TIA |
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RFs for Art thrombus? |
Age Smoking HT Diabetes |
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What is used to treat? |
Thrombolysis and antiplatelets |
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What is a venous thrombus made up of? |
Fibrin and red cells |
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What are the causes of VT? |
Stasis Hypercoaguability Vessel damage |
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What examples? |
DVT PE |
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Risk factors for VT? |
Age Pregnancy Surgery Immobility |
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What are used as probability scores for VT? |
Wells Geneva |
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What marker is elevated in VT? |
D-dimer |
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What tests can be done? |
Doppler US V/Q scan |
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What is used to treat? |
LMW Heparin |
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What are the causes of DIC? |
Septicaemia Malignancy Eclampsia |
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What is the platelet pattern of bleeding? |
Mucosal Epistaxis Purpura Menorrhagia GI bleeding |
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What are purpura? |
Red/purple spots that do not blanch under pressure |
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What is coagulation factor pattern of bleeding? |
Articular Muscle haematoma CNS retro-peritoneal bleeding Post-surgical bleeding |
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What is the inheritance of Haemophilia? |
X-linked |
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Which type is more common? |
A |
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Presentation? |
Coagulation Factor type bleeding |
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Complications? |
Synovitis Chronic arthropathy Neurovascular compression |
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Diagnosis? |
Prolonged APTT Normal prothrombin time and bleeding time Reduced factor 8/9 |
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Treatment? |
Factor 8 or 9 replacement DDAVP is given for A Tranexamic acid |
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What is the inheritance of VWD? |
Autosomal dominant |
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Presentation? |
Platelet type bleeding |
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Diagnosis? |
Increased APTT Increased bleeding time ↓VwF |
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What should not be given to patients with VwF disease? |
NSAIDs |
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Treatment? |
vWF concentrate or DDAVO Tranexamic acid |
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What is the presentation of thrombocytopenia? |
Mucosal/Platelet type bleeding with petechiae and ecchymosis |
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Tell me about acute leukaemia? |
Leukaemic cells do not differentiate Bone marrow failure Rapidly fatal if untreated Potentially curable |
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Chronic leukaemia? |
Cells retain ability to differentiate Proliferation without Bone marrow failure Survival for a few years Not curable without BMT |
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Presentation of AML? |
Bone marrow failure Hepatomegaly Splenomegaly |
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How does BMF present? |
Anaemia Platelet type bleeding Susceptible to infection |
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Investigations? |
Bone marrow biopsy Immunophenotyping done to differentiate from ALL |
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What is seen on microscopy in AML? |
Auer rods |
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Treatment? |
Supportive Care - Blood transfusions, IV fluids, Allopurinol Chemotherapy - Intensive BMT |
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Who gets CML? |
People between 40-60 |
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What genetic component is present in CML? |
Philadelphia chromosome |
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Symptoms of CML |
Chronic and insidious Systemic - weight loss, tiredness, fever, sweats and gout Platelet type bleeding |
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How does gout occur? |
Due to purine breakdown |
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Signs of CML? |
Splenomegaly Hepatomegaly Anaemia Bruising |
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Diagnosis |
↑↑WBC Blood film shows all stages of white cell differentiation Bone marrow and blood cells contain PH |
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Treatment |
Imatinib - Tyrosine kinase inhibitor Stem cell transplantation is an option |
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What is an example of a myelodysplastic syndrome? |
Polycythaemia Vera |
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What is polycythaemia vera? |
Malignant proliferation of a clone derived from one pluripotent stem cell which leads to excess porliferation of RBCs, WBCs and platelets |
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What does this lead to? |
Hyperviscosity Thrombosis |
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Presentation? |
Itch after a hot bath Erythromelalgia - burning sensation in toes and fingers Headaches, dizziness, tinnitus and visual disturbance are possible |
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Signs? |
Facial flushing Splenomegaly |
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Investigations? |
↑Hb concentration ↑Haematocrit |
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What mutation is present? |
JAK2 |
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Treatment? |
Keep haematocrit below 0.45 (Prevent thrombosis) Venesection in younger patients Hydroxycarbamide |
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Complications? |
Can progress to AML |
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What is the most common cancer of children? |
Acute Lymphoblastic Leukaemia |
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What is it? |
Neoplastic disorder of lymphoblasts which mostly affects B-cells |
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Presentation? |
2-3 history of bone marrow failure or bone/joint pain |
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Signs? |
Splenomegaly Hepatomegaly Lymphadenopathy Weight Loss |
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Diagnosis? |
>20% lymphoblasts present in bone marrow High WCC |
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What is the treatment? |
Supportive - blood transfusions, IV fluids, allopurinol Antibiotic prophylaxis Chemotherapy Stem cell transplantation |
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What is Chronic Lympocytic Leukaemia? |
Accumulation of mature B cells that have escaped programmed cell death and undergone cell-cycle arrest in the G0/G1 phase |
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Presentation? |
Often asymptomatic Anaemic or prone to infection |
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Signs |
Lymphadenopathy Splenomegaly |
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How does it often present? |
As a surprise finding on a routine FBC |
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How is diagnosed |
Increased number of lymphocytes on blood film |
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When is treatment indicated? |
Only if symptomatic, progressive BMF or massive lymphadenopathy |
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What is the treatment? |
Fludarabine Cyclophosphamide Rituximab |
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What is lymphoma? |
Malignant proliferation of lymphocytes |
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What happens? |
The lymphocytes acccumulate in lymph nodes causing lymphadenopathy. |
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What is the difference between Hodgkins and Non-Hodgkins? |
Reed-sternberg cells are present in hodgkins lymphoma |
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Presentation? |
Lymphadenopathy Hepatosplenomegaly Extranodal disease B-symptoms - fever, night sweats and weight loss Bone marrow involvement |
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How is it staged? |
Lymph node biopsy |
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What age gets hodgkins? |
Bimodal age curve 1st peak 15-35 2nd peak in elderly |
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What infection is associated with HL? |
EBV infection |
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Treatment |
Combination chemotherapy |
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How many subtypes of NHL are there? |
50 subtypes |
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What type of cell are they most derived from? |
B-cells |
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How does low-grade present? |
Often Asymptomatic Responds to chemo Incurable |
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How does high grade present? |
Often aggressive Requires combination chemo Can be cured |
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What is the most common High grade? |
Diffuse large B-cell lymphoma |
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Low grade? |
Follicular lymphoma |
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What is myeloma? |
Neoplastic disorder of plasma cells which results in excessive production of an immunoglobulin |
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What immunoglobulins are most common? |
IgG and IgA |
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Presentation? |
Bone lesions Bone marrow failure Renal Failure Prone to infections |
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What do the bone lesions cause? |
Pathological fractures Backache Vertebral collapse Hypercalcaemia |
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Investigations |
Monoclonal protein in serum or urine electrophoresis Increased plasma cells on marrow biopsy X-ray reveals bone lesions |
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Treatment |
Chemo Bisphosphonates Blood transfusion Surgery |
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What is a complication of any haematological malignancy? |
Infection |
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What are measures taken to prevent this? |
Antimicrobial prophylaxis Stem cell transplant Vaccinations IV Immunoglobulin replacement |