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77 Cards in this Set
- Front
- Back
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What is DIC?
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Uncontrolled release of plasmin and thrombin --> uncontrolled intravascular coagulation --> depletion of platelets, coag factors, fibrinogen --> hemorrhage
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What causes DIC?
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gram-negative sepsis, trauma, obstetric complications, pancreatitis, nephrotic syndrome, transfusion, malignancy
STOP Making New Trombi |
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Blood film in DIC
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schistocytes
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Blood test values in DIC
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increased PT, APTT, fibrin, D-dimer
decreased fibrinogen, platelets |
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Management of DIC
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FFP
platelets |
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What is TTP?
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Deficiency of enzyme (metalloprotease) which cleaves multimers of vWF
genetic: ADAMST13 acquired: Ab directed enzyme |
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Blood film in TTP
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schistocytes, low platelets
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Blood test values in TTP
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PT, APTT, fibrinogen normal
increased fibrin, D-dimer |
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Triad clinical features of hemolytic uremic syndrome
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1. low platelets
2. hemolytic anemia 3. acute renal failure |
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Management of TTP/HUS
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plasmaphoresis (gets rid of large vWF)
don't give platelets! immunotherapy |
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What is ITP?
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Ab against platelets
increased splenic destruction and clearance of platelets |
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Blood test values in ITP
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all normal, except low platelets
big platelets on blood film |
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Management of non active bleeding ITP
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steroids
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Management of active bleeding in ITP
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steroids
IVIG tranexamic acid (anti-fibrinolytic) - stabilizes clots life-threatening: give platelets, splenectomy |
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Which gene is implicated in hemachromatosis?
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C282Y
affects iron absorption in the gut |
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Best prognosis type of Hodgkin's lymphoma,
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leukocyte predominant
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what is the most common type of Hodgkin's lymphoma?
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nodular sclerosing
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Which Hodgkin's lymphoma has the worst prognosis?
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lymphocyte depleted
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What is an aplastic crisis in sickle cell anemia?
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due to Parvovirus B19
sudden reaction of bone marrow production usually self-limiting, <2 weeks increase in reticulocytes |
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What is the sequestration crisis in sickle cell anemia?
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affects children as spleen has not undergone splenic atrophy
pooling of blood in the spleen acute decrease in Hb and shock urgent transfusion needed |
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Bugs in asplenism
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encapsulated organsms
Hib Strep pneumoniae Neisseria meningitides Salmonella |
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What crises with sickle cell anemia present with?
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vaso-occlusive - painful --> can occur in the bones
aplastic sequestration chest |
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What is in the blood film in hyposplenism
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* target cells
* Howell-Jolly bodies * Cabot's rings * Pappenheimer bodies * siderotic granules * acanthocytes * schizocytes |
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What do tear drop poikilocytes indicate on a blood film?
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myelofibrosis
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What do hypersegmented neutrophils indicate on a blood film?
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Megaloblastic anemia
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What is seen on the blood film in iron deficiency anemia?
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target cells
pencil cells |
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Complications of tumor lysis syndrome
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* hyperkalaemia
* hyperphosphataemia * hypocalcaemia * hyperuricaemia * acute renal failure |
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What is gene translocation associated with Burkitt's lymphoma?
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c-myc, t (8:14)
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Which cancer is associated with tumor lysis syndrome?
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Burkitt's lymphoma
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Earliest sign of spinal cord compression in cancer patients
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back pain - worse on lying down or coughing
oncological emergency! |
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What is factor V Leiden also known as?
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activated protein C resistance
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What vaccines do you want to give someone with no spleen/functional asplenia?
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Hib
Pneumococcus (pneumovax) Neiserria Meningitis (Meningovax?) Significance: asplenia = vulnerable to encapsulated bacteria Salmonella is encapsulated but cannot be vaccinated against |
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Probenicid
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uricosuric
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Classic pentad of TTP?
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1) microangiopathic haemolytic anaemia
2) thrombocytopenia 3) neurologic symptoms 4) renal function abnormalities 5) fever |
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Treatment for TTP
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Acute:
Stop causative agents Daily plasma exchange Contraindicated: platelet transfusion Chronic Plasma exchange Consider cyclophosphamide and reituximab Splenectomy if refractory to treatment |
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What investigations differentiate Thrombotic Thrombocytopenic purpura from Haemolytic uraemic syndrome?
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1) Acute Renal Failure is dominant feature of HUS
2) Electrophoresis for Ultra high molecular weight vWF: raised in TTP 3) ADAMST13 assay: low in familial and idiopathic TT, normal in HUS |
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Triad of HUS
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Thrombocytopenia
Acute renal failure Microangiopathic haemolytic anaemia |
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Treatment for HUS
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Supportive
Dialysis to support renal function Non-pharm: Blood transfusion for severy anaemia |
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What % of blood transfusions may be inappropriate
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30%
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Under what conditions is blood transfusion appropriate
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Hb 70-100 + a need to relieve clinical signs and symptoms
Hb <70 This does NOT apply to blood component use. |
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Clinical features that indicate a patient is NOT tolerating anaemia?
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Chest pain, dyspnoea, fatigue, dizziness, hypotension.
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Physical signs a patient is not tolerating anaemia
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Clinical decompensation
Conjunctival pallor Tachypnoea Tachycardia Raised JVP Heart murmurs Ankle oedema Postural hypotension Altered mental state |
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What are the risks of blood transfusion
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High risk (>1:100): Mild fever, skin rash, urticaria
Low risk (1:1000-10,000) Transfusion related lung injury ABO incompatibility (1:12000) Very low risk (1:10,000-100,000) Bacterial contamination Hep B Negligible risk (1:1,000,000) HIV (1 in 4 million) Hep C (1 in 3 million) |
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What is the most common inherited bleeding disorder?
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Von willebrands disease
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What is an aplastic crisis in hemolytic anemias?
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BM suppression due to overwhelming infection
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What is tranexamic acid and what is it used for?
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anti-fibrinolytic aka suppresses clot breakdown and reduces excessive bleeding
used in hemophilia and VWF disease |
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What is DDAVP and what is used for in hemotology?
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vasopressin AKA ADH analogue
used to promote release of vWF --> factor VIII used in vWF disease, mild hemophilia A, thrombocytopenia |
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Pathophysiology of CLL
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accumulation of neoplastic lymphocytes in bone, BM, spleen, lymph nodes
characterized by clonal malignancy of mature B cells |
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Blood tests in CLL
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lymphocytosis >5 x 10^9/L
blood film: smudge cells BM aspirate: lymphocytes >30% of nucleated cells flow cytometry FISH |
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Pathophysiology of multiple myeloma
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proliferation of plasma cells producing a monoclonal Ig (M protein)
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Clinical features of multiple myeloma
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CRAB
hypercalcemia renal failure anemia bony lesions |
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T/F Urinalysis is a good test in multiple myeloma
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False!
routine urinalysis will not detect light chains as dipstick detects albumin need sulfosalicylic acid or 24 urine protein for immunofixation or electrophoresis |
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Which hematological malignancy is amyloidosis associated with?
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multiple myeloma
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What heme malignancy are Bence-Jones proteins a part of?
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multiple myeloma
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What investigations shows monoclonal proteins in multiple myeloma?
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serum electrophoresis: monoclonal protein spike
urine protein electrophoresis: light chains in urine |
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What is the mechanism of hypercalcemia in multiple myeloma
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increased osteclastic resorption caused by local cytokines released by myeloma cells
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What GI problem is associated with hereditary spherocytosis?
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gall stones
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How to tell beta thalassemia trait on blood results
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MCV is much lower in proportion to anemia
HbA2 >3.5% |
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T/F Beta thalassemia trait is usually asymptomatic
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true
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What are the blood results in Hemophilia?
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prolonged APTT
normal PT |
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Rx of hemophilia A
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DDAVP in mild
recombinant factor VIII tranexamic acid (anti-fibrinolytic) |
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What type of dominance is hemophilia?
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X-linked recessive
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Complications of blood transfusion
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Complications
* haemolytic: immediate or delayed * febrile reactions * transmission of viruses, bacteria, parasites * hyperkalaemia * iron overload * ARDS * clotting abnormalities Immediate haemolytic reaction * e.g. ABO mismatch * massive intravascular haemolysis |
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Complications of tumor lysis syndrome
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* hyperkalaemia
* hyperphosphataemia * hypocalcaemia * hyperuricaemia * acute renal failure |
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T/F Hemachromatosis is associated with a raised Hb level
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False, typically only ferritin and iron is increased, transferrin saturation >60% and low TIBC
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What implies a poor prognosis in Hodgkin's lymphoma
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B symptoms: wt loss >10% in 6 months, fever >38, night sweats
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What do tear drop poikilocytes indicate?
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myelofibrosis
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What is important to start first, B12 or folate, when deficient in both?
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It is important in a patient who is also deficient in both vitamin B12 and folic acid to treat the B12 deficiency first to avoid precipitating subacute combined degeneration of the cord.
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When is TIBC high? When is it low?
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in inflammatory disease, TIBC is low as opposed to iron-deficiency when it is high
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If already found to have anemia, what is the best test to determine whether a patient has hemolytic anemia?
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reticulocyte count and blood smear
LDH and bilirubin would be elevated too but are not specific |
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Definitive diagnostic test in multiple myeloma
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bone marrow biopsy showing >10% plasma cells in bone marrow
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What is seen in the blood film of CLL
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almost entirely mature small lymphocytes
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When do you treat CLL
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early treatment does not improve survival
treat only if symptomatic - lymphadenopathy, anemia, thrombocytopenia |
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if a patient has pancytopenia, what is the next investigation to do?
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bone marrow biopsy - if all three elements (RBC, WBC, platelets) are down, the cause is usually in the bone marrow
in young pt, most likely cause is aplastic anemia, in elderly, most likely cause is myelodysplastic syndrome |
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Rx of Hodgkin's lymphoma
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Stage 1 & 2 (same side of diaphragm): radiotherapy
Stage 3 & 4 (different sides of diaphragm): chemotherapy |
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Commoest cause of drug-induced thrombocytopenia
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Heparin
10-15% of pts on heparin get it! whoa! |
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Rx Heparin induced thrombocytopenia
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1. stop heparin
2. use alternative anti-coag: danaparoid or lepirudin |
