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77 Cards in this Set

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What is DIC?
Uncontrolled release of plasmin and thrombin --> uncontrolled intravascular coagulation --> depletion of platelets, coag factors, fibrinogen --> hemorrhage
What causes DIC?
gram-negative sepsis, trauma, obstetric complications, pancreatitis, nephrotic syndrome, transfusion, malignancy
STOP Making New Trombi
Blood film in DIC
schistocytes
Blood test values in DIC
increased PT, APTT, fibrin, D-dimer
decreased fibrinogen, platelets
Management of DIC
FFP
platelets
What is TTP?
Deficiency of enzyme (metalloprotease) which cleaves multimers of vWF
genetic: ADAMST13
acquired: Ab directed enzyme
Blood film in TTP
schistocytes, low platelets
Blood test values in TTP
PT, APTT, fibrinogen normal
increased fibrin, D-dimer
Triad clinical features of hemolytic uremic syndrome
1. low platelets
2. hemolytic anemia
3. acute renal failure
Management of TTP/HUS
plasmaphoresis (gets rid of large vWF)
don't give platelets!
immunotherapy
What is ITP?
Ab against platelets
increased splenic destruction and clearance of platelets
Blood test values in ITP
all normal, except low platelets
big platelets on blood film
Management of non active bleeding ITP
steroids
Management of active bleeding in ITP
steroids
IVIG
tranexamic acid (anti-fibrinolytic) - stabilizes clots
life-threatening: give platelets, splenectomy
Which gene is implicated in hemachromatosis?
C282Y
affects iron absorption in the gut
Best prognosis type of Hodgkin's lymphoma,
leukocyte predominant
what is the most common type of Hodgkin's lymphoma?
nodular sclerosing
Which Hodgkin's lymphoma has the worst prognosis?
lymphocyte depleted
What is an aplastic crisis in sickle cell anemia?
due to Parvovirus B19
sudden reaction of bone marrow production
usually self-limiting, <2 weeks
increase in reticulocytes
What is the sequestration crisis in sickle cell anemia?
affects children as spleen has not undergone splenic atrophy
pooling of blood in the spleen
acute decrease in Hb and shock
urgent transfusion needed
Bugs in asplenism
encapsulated organsms
Hib
Strep pneumoniae
Neisseria meningitides
Salmonella
What crises with sickle cell anemia present with?
vaso-occlusive - painful --> can occur in the bones
aplastic
sequestration
chest
What is in the blood film in hyposplenism
* target cells
* Howell-Jolly bodies
* Cabot's rings
* Pappenheimer bodies
* siderotic granules
* acanthocytes
* schizocytes
What do tear drop poikilocytes indicate on a blood film?
myelofibrosis
What do hypersegmented neutrophils indicate on a blood film?
Megaloblastic anemia
What is seen on the blood film in iron deficiency anemia?
target cells
pencil cells
Complications of tumor lysis syndrome
* hyperkalaemia
* hyperphosphataemia
* hypocalcaemia
* hyperuricaemia
* acute renal failure
What is gene translocation associated with Burkitt's lymphoma?
c-myc, t (8:14)
Which cancer is associated with tumor lysis syndrome?
Burkitt's lymphoma
Earliest sign of spinal cord compression in cancer patients
back pain - worse on lying down or coughing
oncological emergency!
What is factor V Leiden also known as?
activated protein C resistance
What vaccines do you want to give someone with no spleen/functional asplenia?
Hib
Pneumococcus (pneumovax)
Neiserria Meningitis (Meningovax?)

Significance: asplenia = vulnerable to encapsulated bacteria

Salmonella is encapsulated but cannot be vaccinated against
Probenicid
uricosuric
Classic pentad of TTP?
1) microangiopathic haemolytic anaemia
2) thrombocytopenia
3) neurologic symptoms
4) renal function abnormalities
5) fever
Treatment for TTP
Acute:
Stop causative agents
Daily plasma exchange
Contraindicated: platelet transfusion

Chronic
Plasma exchange
Consider cyclophosphamide and reituximab
Splenectomy if refractory to treatment
What investigations differentiate Thrombotic Thrombocytopenic purpura from Haemolytic uraemic syndrome?
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
Triad of HUS
Thrombocytopenia
Acute renal failure
Microangiopathic haemolytic anaemia
Treatment for HUS
Supportive
Dialysis to support renal function
Non-pharm:
Blood transfusion for severy anaemia
What % of blood transfusions may be inappropriate
30%
Under what conditions is blood transfusion appropriate
Hb 70-100 + a need to relieve clinical signs and symptoms

Hb <70

This does NOT apply to blood component use.
Clinical features that indicate a patient is NOT tolerating anaemia?
Chest pain, dyspnoea, fatigue, dizziness, hypotension.
Physical signs a patient is not tolerating anaemia
Clinical decompensation
Conjunctival pallor
Tachypnoea
Tachycardia
Raised JVP
Heart murmurs
Ankle oedema
Postural hypotension
Altered mental state
What are the risks of blood transfusion
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)
What is the most common inherited bleeding disorder?
Von willebrands disease
What is an aplastic crisis in hemolytic anemias?
BM suppression due to overwhelming infection
What is tranexamic acid and what is it used for?
anti-fibrinolytic aka suppresses clot breakdown and reduces excessive bleeding

used in hemophilia and VWF disease
What is DDAVP and what is used for in hemotology?
vasopressin AKA ADH analogue

used to promote release of vWF --> factor VIII

used in vWF disease, mild hemophilia A, thrombocytopenia
Pathophysiology of CLL
accumulation of neoplastic lymphocytes in bone, BM, spleen, lymph nodes

characterized by clonal malignancy of mature B cells
Blood tests in CLL
lymphocytosis >5 x 10^9/L
blood film: smudge cells
BM aspirate: lymphocytes >30% of nucleated cells
flow cytometry
FISH
Pathophysiology of multiple myeloma
proliferation of plasma cells producing a monoclonal Ig (M protein)
Clinical features of multiple myeloma
CRAB
hypercalcemia
renal failure
anemia
bony lesions
T/F Urinalysis is a good test in multiple myeloma
False!

routine urinalysis will not detect light chains as dipstick detects albumin

need sulfosalicylic acid or 24 urine protein for immunofixation or electrophoresis
Which hematological malignancy is amyloidosis associated with?
multiple myeloma
What heme malignancy are Bence-Jones proteins a part of?
multiple myeloma
What investigations shows monoclonal proteins in multiple myeloma?
serum electrophoresis: monoclonal protein spike

urine protein electrophoresis: light chains in urine
What is the mechanism of hypercalcemia in multiple myeloma
increased osteclastic resorption caused by local cytokines released by myeloma cells
What GI problem is associated with hereditary spherocytosis?
gall stones
How to tell beta thalassemia trait on blood results
MCV is much lower in proportion to anemia

HbA2 >3.5%
T/F Beta thalassemia trait is usually asymptomatic
true
What are the blood results in Hemophilia?
prolonged APTT
normal PT
Rx of hemophilia A
DDAVP in mild
recombinant factor VIII
tranexamic acid (anti-fibrinolytic)
What type of dominance is hemophilia?
X-linked recessive
Complications of blood transfusion
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
Complications of tumor lysis syndrome
* hyperkalaemia
* hyperphosphataemia
* hypocalcaemia
* hyperuricaemia
* acute renal failure
T/F Hemachromatosis is associated with a raised Hb level
False, typically only ferritin and iron is increased, transferrin saturation >60% and low TIBC
What implies a poor prognosis in Hodgkin's lymphoma
B symptoms: wt loss >10% in 6 months, fever >38, night sweats
What do tear drop poikilocytes indicate?
myelofibrosis
What is important to start first, B12 or folate, when deficient in both?
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.
When is TIBC high? When is it low?
in inflammatory disease, TIBC is low as opposed to iron-deficiency when it is high
If already found to have anemia, what is the best test to determine whether a patient has hemolytic anemia?
reticulocyte count and blood smear

LDH and bilirubin would be elevated too but are not specific
Definitive diagnostic test in multiple myeloma
bone marrow biopsy showing >10% plasma cells in bone marrow
What is seen in the blood film of CLL
almost entirely mature small lymphocytes
When do you treat CLL
early treatment does not improve survival

treat only if symptomatic - lymphadenopathy, anemia, thrombocytopenia
if a patient has pancytopenia, what is the next investigation to do?
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
Rx of Hodgkin's lymphoma
Stage 1 & 2 (same side of diaphragm): radiotherapy

Stage 3 & 4 (different sides of diaphragm): chemotherapy
Commoest cause of drug-induced thrombocytopenia
Heparin

10-15% of pts on heparin get it! whoa!
Rx Heparin induced thrombocytopenia
1. stop heparin
2. use alternative anti-coag: danaparoid or lepirudin