Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

ACP Hematology > T5 Bleeding, Thrombotic disorders and Transfusion > Flashcards

T5 Bleeding, Thrombotic disorders and Transfusion Flashcards

1
Q

45/M flew back from USA 2 days ago, swollen right leg for 2 days.
No history of leg trauma.
Afebrile, painful to touch. No superficial wound. Left leg normal.
Q1. DDx? (2)

A

Q1. DDx

  1. Deep vein thrombosis
  2. Superficial thrombophlebitis (inflammation of superficial vein)

Others

  • Cellulitis (inflammation and swelling of limbs)
  • Baker’s cyst (fluid-filled cyst behind knee)
  • Fracture
  • Hematoma
  • Acute arterial ischemia

(not varicose: enlarged, swollen, twisted veins)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

45/M flew back from USA 2 days ago, swollen right leg for 2 days.
No history of leg trauma.
Afebrile, painful to touch. No superficial wound. Left leg normal.

Q2. Investigations?

A
  1. Doppler USG of lower limbs
    - detects of flow in vessels
    - can identify venous flow obstruction
  2. Venography
    - gold standard to diagnose DVT but invasive
    - contrast injected to veins
  3. D-dimer
    - from the degradation of cross-linked fibrin
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Patient’s

  • D-dimer is 2000 ug/L (rf: <500 ug/L)
  • CBC, PT, APTT normal
  • developed SOB, chest tightness while waiting for further investigations
  • Pulse oximetry is 82%,
  • BP 90/60, pulse 110 bpm
  • Sinus tachycardia
  • ABG: type I respiratory failure

Final diagnosis?

A
  • Type I respiratory failure = Hypoxemic respiratory failure
    (c. f. Hypercapnic respiratory failure in type II RF - failure to remove CO2 from lungs)

Diagnosis:
- Pulmonary embolism from DVT of the right LL

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

What is the use of D-dimer in a patient with suspected DVT?

Sensitivity and specificity? Is it a good diagnostic tool?

A

D-dimer

  • specific cross-linked derivatives of fibrin
  • produced when cross-linked fibrin is degraded by plasmin &raquo_space;> FDP (fibrin degradation products) that includes D-dimer
  • level is increased when there is venous thromboembolism/ malignancy/ pregnancy/ tissue injury.

Good sensitivity but poor specificity.

  • Normal levels - good to exclude DVT
  • Elevated levels - NOT used for diagnosing DVT
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Patient’s

  • D-dimer is 2000 ug/L (rf: <500 ug/L)
  • CBC, PT, APTT normal
  • developed SOB, chest tightness while waiting for further investigations
  • Pulse oximetry is 82%,
  • BP 90/60, pulse 110 bpm
  • Sinus tachycardia
  • ABG: type I respiratory failure

Other than D-dimer, Doppler ultrasound and venography, further investigations of suspected condition on top of DVT? (4)

A
  1. High-resolution CT (for probable PE)
  2. V/Q scan: detects areas of lung being ventilated but not perfused
  3. CT pulmonary angiogram: detects filling defects in pulmonary arteries, using contrast
  4. Pulmonary angiogram: detects filling detects in pulmonary arteries, invasive, using contrast
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Treatment for the patient with pulmonary embolism + DVT?

A

Acute anticoagulation management

  1. UFH (IV)
  2. LMWH
  3. Fondaparinux
  4. Rivaroxiban (oral Xa inhibitors, NOAC)
  5. Apixaban (oral Xa inhibitors, NOAC)

Clot removal

  1. Surgical thrombectomy
  2. (Catheter-directed) Thombolysis
  • UFH/LMWH for few days until international normalized ratio (INR) in therapeutic range (2-3) for 2 consecutive days
  • Warfarin for 3 months, with a target INR of 2.5
// 
NOAC (novel oral anticoagulant)
1. Factor Xa inhibitors
- Apixaban (oral Xa inhibitors)
- Rivaroxaban (oral Xa inhibitors)
2. Direct thrombin inhibitors
- Dabigatran (oral IIa inhibitors)
- Argatroban
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

3/M presented with Right knee swelling and pain after a fall at the playground.
The patient’s maternal uncle passed away at the age of 5 because of intracranial hemorrhage.

PT 12s (10-12s)
APTT 80s (28-40s)
Platelets 180 x 10^9/L (140-380)
R/LFT normal.

What factor deficiencies cause an isolated APTT?
What is the most likely defect according to history?

A

Factor deficiencies of 8,9,11,12

Isolated APTT
1. Intrinsic pathway defect: Factor deficiencies in factor 8,9,11,12 OR inhibitors of factors/ contact factor deficiency

  1. Lupus anticoagulant
  2. Heparin administration
    (potentiates antithrombin III to inhibit factor IIa (thrombin) and Xa)

DDx for clotting problems

  1. Hemophilia A - Factor 8 deficiency
    - platelet count normal
    - APTT increased
    - PT normal

// Hemophilia B - Factor 9 deficiency; Hemophilia C: Factor 11 deficiency

  1. Vitamin K deficiency
    - for factor 2,7,9,10
    - due to warfarin
    - PT and APTT increased
  2. Liver problem
    - all factors deficiency?

Most likely defect:
Hemophilia A/B

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

3/M presented with Right knee swelling and pain after a fall at the playground.
The patient’s maternal uncle passed away at the age of 5 because of intracranial hemorrhage.

PT 12s (10-12s)
APTT 80s (28-40s)
Platelets 180 x 10^9/L (140-380)
R/LFT normal.

What investigations will give the diagnosis?

A
  • Mixing test to differentiate factor deficiency/ inhibitors.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

The mixing test showed corrected APTT on mixing the test sample with an equal volume of normal plasma.

Factor VIII 2% (rf: 50-200%)
Factor IX 80% (rf :50-200%)
Factor XI,XII normal.

What is the diagnosis?
What is the mode of the inheritance of the disease?
What is the severity of the disease?

A

Diagnosis:

  • Hemophilia A (Factor 8 deficiency)
  • X-linked recessive

Severity is related to factor level
<1% - severe - spontaneous bleeding (first years of life)
1-5% - Moderate - bleeding with mild injury
6-30% - Mild - bleeding with surgery/trauma

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

What are the clinical features of Hemophilia A? (4)

A
  1. Hemarthrosis (MC)
  2. Soft tissue hematomas (e.g. muscles)
  3. Muscle atrophy
  4. Shortened tendons
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

What are the therapeutic agents of Hemophilia A?

A
  1. Replacement therapy
    a. Factor 8 concentrates, either plasma-derived/recombinant products
  • complications: inhibitor formation and infection
  1. Pharmacological treatment
    b. DDAVP (desmopressin) for mild-moderate Hemophilia A (not for Hemophilia B!)

c. Antifibrinolytic e.g. Tranexamic acid
> useful adjunctive therapy for mucosal bleeding e.g. dental extraction

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

The 3-year-old boy has been given factor 8 concentrate for his right knee hemarthrosis.
He has been subsequently treated with factor 8 concentrate several times, after injury and surgery.
2 years later, admitted with massive hemorrhage in right buttock.

APTT before factor 8 infusion: >120s
APTT after factor 8 infusion: 115s
rf: 28-40s

What has happened? How to investigate and manage?

A
  1. Testing for factor 8 inhibitor with quantification of inhibitor titer
  • there is the development of antibodies (inhibitors) to the infused factor 8
  • this renders the patient refractory to further replacement therapy such that a VERY LARGE dose of factor concentrate have to be used
  1. Alternative treatments
    - Porcine factor 8 concentrate
    - Recombinant factor 7a
    - Immunosuppresion
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q 
Case 3 (new) 
30/F admitted for symptomatic anemia and menorrhagia. Good past health
Hb 7.0
MCV 60
RDW 19%
Reticulocyte 2%
WBC 7x 10^9/L
Plt 460 x 10^9/L

Would you consider red cell transfusion? Justify your decision.
If you are going to transfuse her, how many units would you prescribe?

A

Yes. (but borderline decision)

  • Hb <7: transfuse according to the rate of ongoing blood loss
  • Hb 7-10: consider symptoms, co-morbidities and rate of blood loss (patients >65/with CV/respi disease may tolerate anemia poorly)
  • Single unit transfusion for red cell transfusion
  • ONE standard unit of blood for stable and normovolemic in-patients that are NOT actively bleeding
  • Reassess the patient before transfusing another unit
  • Clinical decision based on symptoms not only Hb levels
  • If the patient’s symptoms have settled, further transfusion may cause hypervolemia
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

What are the components of pre-transfusion compatibility testing?

A
  1. Type
    - ABO + Rh(D) typing on patient’s RBC
  2. Screen
    - Antibody screening on patient’s plasma/serum
    - Antibody identification in screen +ve cases
    - Serological crossmatch with donor’s antigen -ve, ABO compatible RBCs
  • if antibody -ve, computer crossmatch only
  • then issue RBC
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

30/F admitted for symptomatic anemia and menorrhagia. Good past health.
Gave red cell transfusion,
She developed a sudden drop in BP in 15 mins after transfusion of red cells.
Also showed fever, flushing, shortness of breath, pain in lumbar region.

Suspected DDx (5)? 
General Management? (5)
A

Acute transfusion reactions

  1. Acute hemolytic transfusion reaction (AHTR)
    - fever, pain, bleeding, shock
  2. Septic reaction due to bacterial contamination
    - same as above
  3. Anaphylaxis
    - Urticaria, SOB, shock
  4. Transfusion-related acute lung injury (TRALI)
    - Fever, SOB, shock
  5. Transfusion-associated circulatory overload (TACO)
    - SOB, increased JVP, ankle edema
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

30/F admitted for symptomatic anemia and menorrhagia. Good past health.
Gave red cell transfusion,
She developed a sudden drop in BP in 15 mins after transfusion of red cells.
Also showed fever, flushing, shortness of breath, pain in the lumbar region.

General Management? (5)

A

Mx:

  1. Stop the transfusion;
  2. maintain venous access (cardiovascular support with fluid resuscitation and maintenance of UO by IV fluids)
  3. Monitor vital signs (temperature, BP, pulse, RR, O2 saturation)
  4. Check identification details between patient and compatibility of blood component
  5. Perform visual inspection of the component (cloudiness may implicate contamination)
17
Q

30/F admitted for symptomatic anemia and menorrhagia. Good past health.
Gave red cell transfusion,
She developed a sudden drop in BP in 15 mins after transfusion of red cells.
Also showed fever, flushing, shortness of breath, pain in the lumbar region.

General investigations?

A
  1. CBC and blood film: red cells agglutinate, spherocytosis (hemolytic anemia)
  2. RFT: renal impairment
  3. Clotting factor: DIC (increased PT, APTT, TT, reduced platelets)
  4. Reduced haptoglobin (because the need to bind to increased free Hb), Increased bilirubin, Increased LDH (tissue damage),
    direct Coomb’s test +ve: Hemolysis
  5. Blood culture on both patient and donor unit: differentiate Acute hemolytic transfusion reaction VS septic reaction
  6. Blood group: ABO incompatibility
  7. Indirect Coomb’s test: detect autoantibodies
18
Q

Case 4:
26/F hospitalized with fever, proteinuria, deranged RFT.
- Serological studies reveal +ve antinuclear antibody (ANA) result (1:160)
- Kidney biopsy: membranoproliferative glomerulonephritis
- PT 12s (10-12)
- APTT 79s (28-40)
- APTT after 1:1 mixing with normal plasma: 61s

What coagulation disorder? How to investigate?

A

DDx:
Isolated APTT
1. Intrinsic pathway defect: Factor deficiencies in factor 8,9,11,12 OR inhibitors of factors/ contact factor deficiency

  1. Lupus anticoagulant
  2. Heparin administration
    (potentiates antithrombin III to inhibit factor IIa (thrombin) and Xa)
  • Should be Lupus anticoagulant in this case (ANA +ve)

Investigations

  • presence of antiphospholipid antibody
    e. g. assay for lupus anticoagulant, anti-cardiolipin antibody, anti-beta2 glycoprotein antibody
19
Q

What is antiphospholipid syndrome?

A

(an autoimmune disease with antiphospholipid antibodies attacking binding to phospholipid-binding proteins > easy clotting)
(can occur as primary disease/ with SLE/autoimmune disease)

  1. Clinical criteria
    - Vascular thrombosis (arterial/venous/small-vessel thrombosis) +
    - Recurrent miscarriage (pregnancy morbidity)
  2. Laboratory criteria
    a. Lupus anticoagulant
    b. Anticardiolipin antibody
    c. Anti-beta 2 glycoprotein antibody

-all have to be present on 2 or more occasions at least 12 weeks apart

20
Q

What problem can antiphospholipid syndrome patients have?

A
  1. Arterial thrombosis (stroke, limb ischemia, mitral valve vegetation)
  2. Venous thrombosis (DVT, PE)
  3. Renal insufficency (deranged RFT)
  4. Pregnancy - miscarriages, pre-eclampsia…
21
Q

Patient of antiphospholipid syndrome treated with slow correction of abnormal coagulation results and renal function.

Petechiae seen in both legs during tapering of treatment
Hb 6.6g/dL (reduced)
Reticulocyte 13% (increased) 
WBC: 5x 10^9/L
Platelet 12x10^9 (reduced) 
DAT+ve 
Serum haptoglobin <0.04 *(0.3-2.0)
LDH 704 (103-199)
Total bilirubin 63 (<19 umol/L) 
Blood film: confirmed thrombocytopenia, polychromasia, spherocytosis

Bone marrow: normal cell count, no leukemic cells or dysplastic changes

Final diagnosis?

A

A. Hemolytic Anemia

  • low Hb→ anemia
  • serum haptoglobin low →hemolytic anemia
  • increase in reticulocyte, LDH, bilirubin

peripheral blood film: polychromasia, spherocytosis

DAT (direct Coomb’s test) +ve→ Autoimmune Hemolytic Anemia (AIHA)

Spherocytosis → warm AHIA

Warm AHIA (DCT: anti-IgG+ve + spherocytosis)
Cold AHIA (ACT: anti-C3d +ve, less marked spherocytosis, red cell agglutination in cold)

B. ITP
- Thrombocytopenia + normal bone marrow
- Petechiae
Rule out platelet deficiency due to reduced production (eg hematological malignancies, MDS, aplastic anemia, etc)
- With underlying SLE/ APS →
Interpretation: immune thrombocytopenia purpura (ITP) (platelet level <30)
(autoimmune attack of platelets)

A+B
= Evan’s syndrome

22
Q

Treatment for Evan’s syndrome in this patient with SLE?

A

AHIA + ITP (immune thrombocytopenia purpura)

  1. Treat underlying SLE
    - NSAIDs
    - Steroids
    - anti-malarial drugs (hydroxychloroquine/ chloroquine)
  2. Immunosuppression for ITP and AIHA

AIHA:

  • Corticosteroids
  • Splenectomy
  • Rituximab (anti-CD20)

ITP:
Platelet 12 x10^9/L (< 20-30 x10^9/L) → treatment indicated
Initial treatment of newly diagnosed ITP:
- Anti-D, IVI g→ inhibit antibody-mediated destruction of platelet
- Corticosteroids→ immunosuppression

Subsequent treatment:
Splenectomy
Rituximab
TPO-RA

23
Q

The patient with Evan’s syndrome complained SOB. Hb further dropped to 5.0 and red cell transfusion is decided. What are the potential problems in pre-transfusion compatibility testing? (2)

A

AHIA (autoimmune hemolytic anemia) frequently have anemia of sufficient severity as to require a blood transfusion.

Problems

  1. Autoantibody in the patient’s serum reacts with normal RBC.
  2. Autoantibody may mask the presence of red cell alloantibody capable of causing a hemolytic transfusion.
24
Q

75/F with UTI, given intravenous antibiotics treatment.
Noticed hematuria one week later.
APTT 53s (24-37s)
PT >60s (10-12s)

APTT and PT on admission were normal.
What are the ddx? (3)
What lab tests would be useful and what results would you expect for the aforementioned diagnosis?

A
  1. Sepsis with DIC
    - sepsis from UTI
  2. Liver disease
  3. Vitamin K deficiency (Factor 2,7,9,10)
    - malabsorption due to prolonged use of antibiotics (gut flora)
    - Vitamin K antagonist (warfarin)
  • Platelet count (decrease in DIC, acute liver failure)
  • LFT (liver)
  • D-dimer (Fibrinolysis: increase in DIC, DVT, liver diseases )
  • Fibrinogen (increase in acute phase reaction, aging, pregnancy, OCP; decrease in DIC, liver disease, massive transfusion)
  • Mixing studies
  1. DIC
    - decreased Plt, decreased fibrinogen, increased D-dimer
  2. Liver diseases
    - deranged liver function, increased D-dimer…
  3. Vitamin K deficiency - decreased factor 2,7,9,10 in mixing studies and coagulation factor assays
25
Q

APTT and PT elevated after antibiotic use.
Patient has normal platelet counts, D-dimer and liver function.

Factor 2: 15%
Factor 5: 166%
Factor 7: 3%
Factor 8: 150%
Factor 9: 28%
Factor 10: 6%
Factor 11: 85%
Factor 12: 22%

Interpret the test results and give your diagnosis?

A

Multiple coagulation factors deficiency due to vitamin K deficiency, antibiotic-related.

Causes of vitamin K deficiency

  • Hemorrhagic disease of the newborn
  • Biliary obstruction
  • Disturbed gut flora e.g. antibiotic therapy
  • Malabsorption of vitamin K (tropical sprue (flattening of villi), gluten-induced enteropathy)
  • Vitamin K antagonist therapy e.g. warfarin
  • Liver disease: poor enterohepatic circulation for vitamin K recycling
26
Q

Management for a patient who has vitamin K deficiency due to antibiotic use?

A
  1. Blood products
    - Platelets
    - Plasma
    - Cryoprecipitate
    - Factor concentrates
  2. Drugs
    - Desmopressin (DDAVP)
    - Prothrombin complex concentrate (warfarin emergency handling): rich in 2,7,9,10
    - Recombinant human factor 7a
    - Anti-fibrinolytic agents e.g. tranexamic acid

ACP Hematology (16 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions