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77 Cards in this Set
- Front
- Back
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Causes of iron deficiency anaemia?
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1) BLOOD LOSS
GIT malignancy, peptic ulcer, IBD, diverticulitis, polyps, hookworm, schistosomiasis Chronic haemoptysis, haematuria, mentruation 2) MALABSORPTION Gastric acid (Fe into ferrous form) - hypochlorhydia (elderly), PPI, gastric surgery SI - absorption - coeliac disease 3) PHYSIOLOGICAL DEMANDS Pregnancy, infancy, puberty, menstruation |
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What are the biochemical changes associated with iron deficiency anaemia?
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INCREASED
TIBC, transferrin DECREASED Serum iron, ferritin, transferrin saturation Microcytic anaemia |
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Changes associated with iron deficiency?
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Anaemia
Growth and intellectual impairment in children Angular stomatitis, glossitis, koilonychia, plummber-vinson syndrome |
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Treatment of iron deficiency anaemia?
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Oral iron supplements 2-3mg/kg/day - split into 2-4 doses
Iv iron supplement if problems with absorption - caution of anaphylaxis |
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Where do you get B12 from?
Where is it absorbed? How long is the storage? |
Animal foods - meat, fish, eggs, milk
Terminal ileum Stores last 3 yrs |
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Factors affecting malabsorption of B12?
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Gastric acid (release from food) - hypochlorhydia, gastric surgery
Pernicious anemia - IF Drugs - metformin, phenytoin, AB, PPI, H2 antagonists SI - pancreastic insufficiency, motility disorders, hypogammaglobulinaemia (bacterial overgrowth - competition for B12) , IBD, ileal resection |
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Lab results associated with B12 deficeincy?
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Macrocytic anaemia
Hypersegmented neurophils ->5 Low B12 (<400pg/ml) Low methylmalonic acid levels Pancytopaenia |
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Folate in the diet?
Stores? Investigation? |
Green leafy vegies - but now all bread is supplemented
Stores - 3-4weeks Investigation - red cell folate |
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Causes of folate deficiency ?
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DIET
MALABSORPTION - coeliac INCREASED DEMAND - pregnancy, increased cell turn over - haemolysis DRUGS Anticonvulsants, OCP, cytotoxic drugs (methotrexate) |
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Mechanism of anaemia in chronic renal failure?
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EPO deficeincy
Uraemia toxicity - toxic effect on BM Decreased RBc survival - haemolysis Capillary fragility + poor platelet function - increased bleeding TREATMENT EPO |
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What is the role of hepcidin in anaemia of chronic disease?
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Increased levels of hepcidin results in internalisation of ferroportin (fe transport on basal side of duodenal enterocytes) - this causes accumulation of iron within the cell and prevents passage to the blood
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What is the role of hepcidin in anaemia of chronic disease?
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Increased levels of hepcidin results in internalisation of ferroportin (fe transport on basal side of duodenal enterocytes) - this causes accumulation of iron within the cell and prevents passage to the blood
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Inherited and Haematological Factors associated with Venous Thrombosis?
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HAEMATOLOGICAL DISORDERS
Primary proliferative polycythaemia Essential thrombocytopaenia Paroxysmal nocturnal haemoglobinuria Myelofibrosis ANTI-COAGULANT DEFICIENCES Antithrombin (inactivates IIa, Xa, IXa) Protein C/S Prothrombin mutation Factor V leiden (cannot be degraded by protein C) |
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Environmental factors predisposing to thrombosis?
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Dehydration
OCP Immobility Surgery - long, ortho obesity smoking HRT age |
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T/F
HCC can cause ectopic secretion of EPO? |
TRUE
leads to erythrocytosis |
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What clotting factors are Vit K dependent ?
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2,7,9,10, fibrinogen
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Cause of Vit K deficiency?
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alcoholism
hepatic insufficiency chronic illness malnutrition abdo surgery cholestatic disease IBD |
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What factors reduced oxygen binding capacity of Hb?
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Acidosis (low pH)
High CO2 High Temp High DGP - product of glycolysis |
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chromosome associated with alpha Hb ?
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16
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Chromosome associated with BETA Hb?
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11
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Tissue factor is associated with which coagulation pathway?
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Extrinsic
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Extrinsic coagulation pathway?
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VII - IX - X (+V) - II - Fibrin / XIII
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Intrinsic coagulation pathway?
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XII - XI - (+VII) - X - (+V) - II - fibrin / XIII
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PT/APTT - goes with intrinsic/extrinsic?
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PT - Extrinsic - Play tennis outside
APTT - Intrinsic - Play table tennis inside |
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What is Thalassaemia A?
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Autosomal
Missing Alpha genes - 1-4 genes deleted Path - Accumulation of beta chains damage red cell precursors - anaemia. Causes expansion of ineffective BM with severe effects on development, bone formation and growth. - also causes iron deposition (due to increased absorption and transfusions) - major cause of morbidity and mortality |
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Treatment of Thalassaemia?
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Regular transfusion
iron chelators folic acid supplementation splenectomy - if hypersplenism is causing increased need for transfusion Depends on the type and severity - Beta thalassaemia major and perhaps HbH disease (loss of 3 alpha) |
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Consequences associated with Sickle cell disease?
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1) VASO_OCCLUSIVE CRISIS (most common) - bone pain
2) SICKLE CHEST SYNDROME (after 1. - bone infarction - fat emboli to lungs - most common cause of death in SCD) 3) SEQUESTRATION CRISIS (thrombosis of venous outflow of organs - loss of function, painful enlargement 4) APLASTIC CRISIS (parvovirus infection - self limiting, anaemia may lead to heart failure) 5) OTHER - renal, iron overload, growth/developmental delay, CVD, stroke, CNS, .... |
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Consequences of Sickle cell Trait?
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resistance to malaria
rhabdomyolysis and sudden death with sustained exhausting exercise |
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Treatment of Sickle Cell Disease?
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Hydroxyurea, blood transfusions, BM transplantation
Vacciantion + prophylactic AB Vaso-occlusive crisis - REHYDRATION, oxygen, AB, analgesia, blood transfusion |
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Why must warfarin only be started while other anticoagulation is continued?
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Warfarin inhibits clotting factors but also Protein C and S and these have a shorter half life - leading to a procoaguable state in the first couple of days before the clotting factors are inhibited .
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Antithrombin (III) inactivates ?
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Thrombin
IX, X, XI Activity greatly enhanced by Heparin Lack of Antithrombin - increased DVT + PE |
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Causes of Acquired antithrombin III deficiency ?
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liver dysfunction (coagulopathy), sepsis, premature birth, kidney disease with protein loss in the urine in patients with nephrotic syndrome, or as a result of interventions such as major surgery or cardiopulmonary bypass
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Function of thrombomodulin?
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Activates protein C (which inhibits clotting by cleaving Va and VIIIa)
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MCV Values for microcytic/macrocytic/normocytic anaemia?
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Micro <80
Normo 80-95 Macro >95 |
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Causes of Microcytic anaemia?
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iron deficiency
thalassaemia ACD lead sideroblastic |
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Causes of normocytic anaemia?
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MCV 80-95
Haemolytic ACD acute blood loss mineral deficiency BM failure |
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Causes of macrocytic anaemia?
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B12 (hypersegmented neutrophils >5)
folate ALCOHOL liver dysfunction myelodysplasia aplastic |
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Haemophillia A
Factor Affected? Inheritance? Problems? |
VIII (80% Type A, 20% type B haemophillia)
Sex linked - X chromosome Complications - joint bleeding, internal bleeding, transfusion infection, inhibitor formation, |
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Treatment of Haemophilia ?
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Factor concentrate (high dose if low inhibitor present, if high inhibitor don't give) or desmopressin (oral/nasal bleed)
by pass agent Acute - life threatening - anti-fibrinolytic Joint - ortho consult Analgesia + fluids |
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Bleeding associated with platelet dysfunction?
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superficial bleeding, menorrhagic, GIT, prolonged bleeding post trauma
(VIII - joint and muscle bleeds) |
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von Willebrands Disease is associated with decrease in what clotting factor? why?
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VIII
vWF acts as a carrier protein for VIII vWD - 1/2 types - AD; 3 - recessive |
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What is the genetic mutation associated with CML(90-95%)/ALL?
Impact of mutation? |
t(9,22)
BCR/ABL constitutively active tyrosine kinase - speeds up cell cycle, inhibits DNA repair Rx - Imatinib |
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What is the genetic mutation associated with Myeloproliferative disorders?
Impact of mutation? |
JAK2 V617F mutation
Activated tyrosine kinase ---> independence/ hypersensitivity to cytokines (ie. EPO), Increased Cellular and fibrotic phases myelofibrosis (50%) thrombocytopaenia (50%) polycythaemia (100%) |
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What is the genetic mutation associated with Burkitts lymphoma?
Impact of mutation? |
t(8,14) cMYC-Ig-H
Increased c-MYC which does everything! (cell growth and proliferation) |
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What is the genetic mutation associated with Follicular lymphoma?
Impact of mutation? |
t(14,18)
Indolent form of NHL Overexpression of BCL2 - Antiapoptotic protein |
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What is the genetic mutation associated with Mantle cell lymphoma?
Impact of mutation? |
T(11,16) - Rare type of NHL
Ig heavy chain - CyclinD1 leads to deregulation of G1-S checkpoint |
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Staging system for Hodgkin's Lymphoma?
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Ann Arbor
Also for non-hodgkins I) 1 LN group II) 2 or more LN groups on 1 side of the diaphragm III) LN above and below diaphragm (or + splenic involvement IIIs) IV) LN + other organs - BM, Liver.... A absence B presence of - fever>38, night sweats, loss of wt >10% in 6 months E indicates local extra nodal extension at any stage |
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Four major subgroups of causes of bleeding disorders?
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Vascular
Thrombocytopaenia Platelet dysfunction Defective coagulation factors |
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Types of bleeding disorders caused by vascular problems?
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INHERITED
Hereditary haemorrhagic telangictasia - skin and mucosal purpura + epitaxis, GI bleed - fe deficiency CT diseases - ehlers danlos, marfans - purpura AQUIRED Normal bruising, senile purpura, purpura with infection (measles, dengue, meningococcal), henoch schonlen, scurvy, steroids |
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Causes of thrombocytopaenia ?
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1) FAILURE TO PRODUCE
- selective megakaryocyte depression - rare - congenital, drugs BM failure - all normal causes 2) INCREASED CONSUMPTION Immune - ITP.... Infections DRUGS - quinine, heparin Post transfusional purpura TTP, DIC 3) ABNORMAL DISTRIBUTION Splenomegaly 4) DILUTIONAL LOSS Massive transfusion |
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Treatment of ITP
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Steroids and IVIG
Splenectomy and immunosuppression |
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Causes of HUS
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SLE
Pregnancy Infection - ECOLI |
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What happens in TTP/ HUS
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Deficiency of ADAMST13 - which breaks down large vWF -- leads to
1) thrombocytopaenia 2) Microangiopathic haemolytic anaemia TTP - neurological and fever HUS - renal failure - may also have Diarrhoea, HT and fits Mortality - approaches 90% without treatment |
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Treatment of TTP?
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1) Plasma exchange with FFP
plus corticosteroids, antiplatelets, transfusion, folic acid 2nd - immunosuppression 3rd - splenectomy Also |
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What are the investigation results with TTP/HUS?
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Thrompocytopaenia
Schistocytes Increased LDH Coags - NORMAL Uraemia in HUS |
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What is fanconi's anaemia
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Defect in DNA repair mechanisms - leading to anaemia (macrocytic megaloblastic)
90% develop pancytopaenia by age of 40 75% also have congenital defects - short stature, skins/arms/eyes/kidneys/head... Many also develop cancer - AML TREATMENT 1) androgens and haemopoetic growth factors 2) Bone marro transplant |
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What kind of chemotherpay is chlorambucil?
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Alkylating agent
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Name an alkylating chemotherapy
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Chlorambucil
Cyclophosphamide Cisplatin |
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Name antimetabolite chemotherapy agents
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Methoexate
6mercaptopurine Cytosine arabinoside |
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Name cytotoxic antibiotic drugs
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Doxirubicin
Danorubicin (adramycin) Bleomycin |
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Name plant derived chemotherapies
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Vincristine (oncovin) , vinblastine, etoposide
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Viruses that cause cancer?
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HTLV - ATLL
EBV - Burkitts HHV8 - karposis sarcoma HVP - cervical (Also H pylori - MALT - gastric lymphoma ) |
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Benzene causes what leukaemia?
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AML
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Inherited factors predisposing to leukaemia
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Downsydnrome (20-30x)
Fanconis anaemia Ataxia telangectasia Kleinfelters |
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causes of splenomegaly?
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HAEM
Cancers, thalassaemia, PCV, sickle cell, haemolytic anaemia PORTAL HT Cirrhosis, RHF, STORAGE Gauchers - mitochondrial INFECTION Acute - speticaemia, IE, typhoid, mono chronic - malaria, TB .... SYSTEMIC Sarcoidosis, amyloidosis, collagen (SLE, RA) |
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Indications for splenectomy
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Splenic rupture
Chronic ITP Haemolytic anaemia Spherocytosis, autoimmune, thalassaemia CLL/lymphoma Myelofibrosis |
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With splenectomy - what vaccinations afe indicated?
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Encapsulaed organisms
Strep pneumonia - neisseria meningitidis Haemophilus influenzae And influenza |
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Times for PT or APTT
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PT - 10-14s
Liver, warfarin, DIC, Heparin APTT - 30-40 Liver disease heparin, haemophillia A/B, XII, vWD (50%) |
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At what level of platelets do you become symptomatic
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10x10^9
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Investigation results with DIC
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Prolonged PT
Prolonged APTT Raised D dimer Low platelets Low fibrinogen Raised fibrin split products Platients usually present when they are bleeding after consumption of coag factors and platelets |
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causes of DIC
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Seve trauma
Aminotic fluid embolis Premature separation of the placenta Cancers AML Liver disease Falciparum malaria Hemolytic transfusion reaction Snake bites |
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What is desmopression used to treat?
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HUS
VWD Mild haemophillia A |
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What causes an increased PT and APTT
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DIC
Vit K deficiency Liver disease Heparin |
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What causes an increased APTT but is associated with thrombosis
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Lupus anticoagulant
DVt and spontaneous abortion |
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Does Protein C or S deficiency alter PT or APTT?
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No
Just increased bleeding time |
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What is the most common inheritied cause for thrombosis ? F
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Factor v leiden
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What deficiency is associated with a low APTT which does not increase with heparin therapy ?
Why? |
Antithrombin III
Because this is the factor that heparin acts on - it activates AT which then inactivates clotting factors such as Xa and IIa |
