Hematology (5-10%) Complete Flashcards

1
Q

Non-cancer-associated Proximal DVT and PE: ( ______ first line, if no contraindications)

– Provoked
• Tx for _______ months (up to ___ months)

– Unprovoked or chronic risk factor
• Tx for _____ months then ______

A

Non-cancer-associated Proximal DVT and PE: (DOAC first line, if no contraindications)

– Provoked
• Tx for 3 months (up to 6 months)

– Unprovoked or chronic risk factor
• Tx for 3 - 6 months then assess bleeding risk; continue indefinitely with regular reassessment of risk: benefit

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

Cancer-associated Proximal DVT and PE: ( _______ if no contraindications)
Low/mod bleeding risk: ________ therapy (1B) while active cancer (which means metastasis and also during +6 months in remission)

_______ preferred where bleeding risk is high

A

Cancer-associated Proximal DVT and PE: (LMWH or DOAC if no contraindications)
Low/mod bleeding risk: indefinite therapy (1B) while active cancer (which means metastasis and also during +6 months in remission)

LMWH is preferred where bleeding risk is high:
- high-risk GI lesions (e.g. angiodysplasia, varices)
- unresected intraluminal GI/GU cancer
- high-risk intracranial lesion (glioma, RCC, melanoma)
- Child-Pugh class B/C
- platelet < 50
- recent bleed
- DOAC Drug Interactions

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

DOAC:
CONTRAINDICATION IN:
1. _________________
2. _________________
3. _________________
4. _________________
5. _________________

A

CONTRAINDICATION IN:
1. Liver failure: Child-Pugh B and C
2. Anti-phospholipid antibody syndrome: arterial thrombosis, triple positive, small vessel thrombosis (livedo reticularis, nephropathy)
3. Pregnancy AND Breastfeeding
4. Drug-drug interactions (e.g. doxorubicin, cyclosporine, carbamazepine, phenytoin, azoles, protease inhibitors – bleeding risk): apix/riva CYP3A4/P-gp; dabi/edox P-gp)
5. Platelets < 50

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

PAXLOVID (ritonavir) and DOAC?

A

PAXLOVID (ritonavir) = contraindicated to use w apix and riva

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

When to Use Warfarin?
MNEMONIC?

A

Be CALM

Breastfeeding
– Therapeutic anticoagulation while breastfeeding [INR generally 2- 3 depending on the indication for anticoagulation]. DOACs contraindicated, LMWH is OK.

CKD (stage 4/5)
– INR generally 2-3 depending on the indication for anticoagulation

Antiphospholipid Ab Syndrome
– APLA Syndrome associated VTE [INR 2-3] (triple positive)

LV Thrombus (+ “Likes warfarin” – pt on warfarin stable for years)
– LV Thrombus (NB observational and 1 small RCT suggests DOAC may be non inferior to VKA)

Mechanical Valve
– Mechanical valves [INR 2.5-3.5 – mitral, INR 2-3 - aortic]

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Role of thrombolysis in VTE:
DVT: ________________

A
  • limb-threatening DVT (phlegmasia cerulea dolens)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Role of thrombolysis in VTE:
PE: ________________

What about intermediate-risk PE” (i.e.: RV dysfunction on ECHO/CT, elevated troponin)

A

hemodynamic instability (sBP <90 for over 15 mins) with no high bleeding risk (Gr 2B)

What about intermediate-risk PE” (i.e.: RV dysfunction on ECHO/CT, elevated troponin): Not indicated

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

Cancer-Associated VTE – Outpatient Prophylaxis?

A
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

When to consider full-dose anticoagulation in Distal (calf) DVT?

A
  • Severe symptoms
  • Multiple deep veins involved
  • Active cancer
  • ≥5 cm long
  • Close to the popliteal vein
  • Irreversible risk factor
  • +Ddimer
  • Progression on repeat U/S
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Superficial femoral vein is ___________

A

Superficial femoral vein is DEEP VEIN (MISNOMER)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Superficial Vein thrombosis and anticoagulation:

  • ≤3 cm from saphenofemoral junction = ___________________
  • > 3cm from SFJ and ≥5 cm long = ___________________
  • > 3cm from SFJ + <5cm long = ___________________
A
  • ≤3 cm from saphenofemoral junction =
    full dose anticoagulation x 3 months
  • > 3cm from SFJ and ≥5 cm long =
    prophylactic anticoagulation x 45d (fondaparinux 2.5 mg sc daily or rivaroxaban 10 mg po daily)
  • > 3cm from SFJ + <5cm long =
    NSAIDs and monitor with serial U/S. ** Exceptions: prophylactic anticoagulation in pregnancy, cancer, surgery, trauma, prior hx of SVT/DVT
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Subsegmental PE and Anticoagulation:
Next step?

A

Leg dopplers
If positive: Def anticoagulation

If negative: Consider treatment if: active cancer/other
thrombotic risk, symptomatic, high D-dimer

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Reversal Agents for Anticoagulants:

Warfarin:

Non-bleeding:
• INR >9: __________________________
• INR 4.5-9: __________________________

Non-life-threatening bleed:
• __________________________

Life-threatening bleed/imminent procedure:
• __________________________

When to use FFP?

A

Non-bleeding:
• INR >9: hold warfarin + VitK 2.5-5 mg po
• INR 4.5-9: hold warfarin + decrease dose

Non-life-threatening bleed:
• Vit K + supportive

Life-threatening bleed/imminent procedure:
• IV Vit K + prothrombin complex (PCC)
– PCC dosing per INR:
INR 1.5-3: PCC 1000U; INR 3-5: PCC 2000U; INR> 5: PCC 3000U

When to use FFP? PCC is superior to FFP for warfarin reversal. CHOOSING WISELY CANADA – do not use FFP for VKA reversal if PCC is available

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Reversal Agents for Anticoagulants:

LMWH/Heparin: __________________________

A

Protamine 25-50 mg

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Reversal Agents for Anticoagulants:

DOACs:

Non-life-threatening: __________________________

Life-threatening bleed: __________________________ +

  1. Dabigatran:
    • __________________________
  2. Apixaban/Rivaroxaban/Edoxaban:
    • __________________________
    • __________________________
A

Non-life-threatening: supportive

Life-threatening bleed: supportive +

  1. Dabigatran:
    • Idarucizumab (Praxbind) 2.5g x 2 doses; consider dialysis (~65% removal in 4 h)
  2. Apixaban/Rivaroxaban/Edoxaban:
    • 4-factor PCC (e.g. Octaplex, Beriplex); 2000U (can be repeated)
    • Andexanet alfa (not available in Canada)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Primary hemostasis

Clinical Presentation:
• _________________________________
• _________________________________
• _________________________________
• _________________________________

Labs:
Platelets?
Coagulation profile?

DDx
1. _________________________________
_________________________________

  1. _________________________________
    ↓Quantity == e.g. _________________________________
    ↓ Quality == e.g. _________________________________
A

Primary hemostasis

Clinical Presentation:
• Mucocutaneous bleeding
• Easy bruising
• Heavy menstrual bleeding
• Petichiae (low plts)

Labs: +/- ↓ plts, often coags normal

DDx
1. VWF Deficiency
Von Willibrand Disease (VWD)

  1. Plt Disorder
    ↓Quantity == e.g. ITP
    ↓ Quality == e.g. ASA, uremia, congenital
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

Secondary Hemostasis:

Clinical Presentation:
• ___________________________
• ___________________________
• ___________________________

Labs:
___________________________

DDx [see slides specific to INR/PTT]
1. ___________________________
• ___________________________
• ___________________________
• ___________________________

  1. ___________________________
    • ___________________________
    • Medications: ___________________________
A

Secondary Hemostasis:

Clinical Presentation:
• Hemarthrosis
• Intramuscular bleeding
• Retroperitoneal bleeding

Labs: abnormal coag. tests

DDx [see slides specific to INR/PTT]
1. Coagulation factor deficiency
• Hemophilia A (Factor VIII)
• Hemophilia B (Factor IX)
• Warfarin (II, VII, IX, X)

  1. Coagulation factor inhibitor
    • Idiopathic/various diseases
    • Medications: DOACs
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

Approach to Isolated Elevated PT or aPTT

A
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

Approach to elevated aPTT & PT

A
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

VWF :↑ levels in _________________
VWF :↓ levels in _________________

A

VWF:↑ levels in stress, estrogens, pregnancy
VWF: ↓ levels in blood group O

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

Von Willebrand Disease Diagnosis

• Type 1:
• Type 2:
• Type 3:

A

Von Willebrand Disease Diagnosis

  1. Type 1:
    - Quantitative deficiency (↓VWF:Ag [<30%] = ↓VWF Rco [<30%] ie. concordant; ratio>0.7)
    – VWF < 30% or VWF 30-50% + abnormal bleeding = diagnostic

2 Type 2:
- Qualitative deficiency of VWD (↓/↔VWF Ag [<30-200%], ↓↓VWF:Rco [<30%] ie. discordant; ratio<0.7)

  1. Type 3:
    - No VWF produced (↓VWF:Ag [<5%] =↓VWF:Rco [<5%]; ↓↓ factor VIII)
    - Behaves like Hemophilia A
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

Von Willebrand Disease

Treatment:
1. Acute Bleeding/Peri-Op?

  1. Heavy menstrual bleeding?
A

Von Willebrand Disease:

Treatment:
1. Acute Bleeding/Peri-Op:
– DDAVP: boosts vWF level (can work for Type 1; not in type 2B/2N or 3)
– Blood products: plasma-derived concentrates of vWF and FVIII (e.g. Humate P)

2 Heavy menstrual bleeding:
- TXA +/- OCP

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

Immune Thrombocytopenia (ITP)
Treatment

1) NOT BLEEDING/MILD MUCOCUTANEOUS BLEEDING & PLT >30:
_________________________________________________________

2) NOT BLEEDING & PLT < 30
• 1st line:______________________________________________
• 2nd line:______________________________________________

3) ACTIVE BLEEDING
• ______________________________________________
• Life-threatening: ______________________________________

A

Immune Thrombocytopenia (ITP)
Treatment

1) NOT BLEEDING/MILD MUCOCUTANEOUS BLEEDING &
PLT >30 • Watch and wait

2) NOT BLEEDING & PLT < 30
• 1st line: Pred 0.5-2mg/kg or Dex 40 mg X 4d, IVIG (if C/I to pred; caution: hemolysis), anti-D (RhD+; caution: hemolysis)
– FLIGHT: MMF + steroids

• 2nd line: splenectomy, rituximab, TPO-R agonists

3) ACTIVE BLEEDING
• Steroids + Tranexamic Acid +/- IVIG

• Life-threatening: Plt transfusion +/- splenectomy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

Immune Thrombocytopenia (ITP)
Treatment
PREGNANCY?
- Indication for treatment?
- How to treat?
- Platelet cut off for delivery?
- Platelet cut off for neuraxial anesthesia?

A

Immune Thrombocytopenia (ITP)
Treatment
PREGNANCY:
• Tx if bleed, plt <30, delivery or plt<50+≥36 wk GA

• Steroids or IVIG (Pred>Dex as Dex crosses placenta)

• plt>50 for delivery,
- plt>80 for neuraxial anesthesia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

Secondary ITP treatment
If significant bleeding and HCV/HIV:

HCV? _________________

HIV? _________________

A

Secondary ITP treatment
1. If significant bleeding and HCV/HIV:

HCV: use IVIG (NO ROLE FOR STEROIDS)

HIV: Steroids or IVIG

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

DO NOT MISS:
Evan’s Syndrome?

A

DO NOT MISS:
Evan’s Syndrome = ITP + hemolytic anemia
- - 2⁰ Autoimm/Connective tissue diseases
- - Difficult to treat, frequent relapse

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

Heparin-induced Thrombocytopenia

4T score?

A
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
28
Q

Management of HIT:

  1. Anticoagulant Choice:
    - IV? ____________________________
    - SC? ____________________________
    - PO? ____________________________
    - Pregnancy? ____________________________
  2. Duration of treatment?
    - No thrombosis = ____________________________
    - Thrombosis = ____________________________
A

Management of HIT:
Anticoagulant Choice:
- IV: Argatroban, Danaparoid

  • SC: Fondaparinux
  • PO: Warfarin, DOACs
  • Warfarin: start when PLT ≥ 150 + overlap with non-heparin anticoagulation ≥5d once INR 2 – 3
  • DOACs: (rivaroxaban 15 BID x 3 wks if VTE or until plt recovery, if no VTE –> Rivaroxaban 20 mg daily)
  • Pregnancy: Danaparoid preferred

Duration:
- No thrombosis = min. until platelet recovery (>150), max 3 mon
- Thrombosis = 3-6 mn

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
29
Q

Thrombotic Microangiopathy (TMA)?

Peripheral blood film?
Platelets?
Thrombi?

A

Thrombotic Microangiopathy (TMA):

– Micro-angiopathic hemolytic anemia (MAHA) -> schistocytes

– ↓Plt

– Microvascular thrombi –> end organ damage

30
Q

TTP – a Hematologic Emergency:
Treatment?

A

–PLEX within 4-8h
• FFP to bridge: 3-4u then 1u q2h
–No plt transfusion unless life-threatening bleed
–Steroids (Pred 1 mg/kg/d or Solumedrol IV 1g/day)
–Folic acid 5 mg daily
–+/- ASA if ↑ trop/CNS symptoms
–DVT proph when plt >50
–Caplacizumab or Rituximab can be considered in refractory cases

31
Q

Hemolytic Anemia:
Spherocytes

A

Spherocytes:
• Warm autoimmune hemolytic anemia (IgG)
• Hereditary spherocytosis
• Delayed hemolytic transfusion reaction

32
Q

Hemolytic Anemia:
Agglutination

A

Agglutination:
• Cold autoimmune hemolytic anemia (IgM)
• Paroxysmal cold hemoglobinuria

33
Q

Warm Autoimmune Hemolytic Anemia:
Diagnosis?

Etiology?

Treatment?
1st Line: ____________

2nd Line: ____________

A

Diagnosis:
- Hemolysis labs
- Blood film: spherocytes
- DAT: +IgG (+/- C3d)

Etiology: 1o vs 2o
– 2o: Lymphoproliferative disease (e.g. CLL), autoimmune (e.g. SLE), drugs (e.g. methyldopa, NSAIDs)

Treatment:
1st line:
- Prednisone 1 – 1.5 mg/kg/day

2nd line:
- Splenectomy (>8-12 mon)
- Rituximab

34
Q

Cold Autoimmune Hemolytic Anemia:
Diagnosis?

Etiology?

Treatment?
1st Line: ____________

2nd Line: ____________

A

Diagnosis:
– Hemolysis labs
– Blood film: agglutination
– DAT +ve C3d (= IgM antibody)
– Thermal amplitude: significant if ≥28⁰C

Etiology: 1o vs 2o
– 2o: Infection (mycoplasma pneumonia, EBV), lymphoproliferative disorder (MGUS, Waldenstroms, lymphoma)

Treatment:
– #1: Warm patient!
– Treat underlying cause
Steroids not helpful
– Rituximab for refractory cases

35
Q

PCC (Prothrombin complex concentrate) vs Plasma in HIT?

A

PCC has heparin = do not use in warfarin reversal w HIT, give ffp instead

36
Q

Platelet Refractoriness?

A
37
Q

Myeloid Disorders

  1. Acute Myeloid Leukemia (AML)
    – too many __________ myeloid cells (i.e. myeloid blasts)
  2. Myeloproliferative Neoplasms (MPNs)
    – too many ________ myeloid cells
    • too many RBCs = ________________
    • too many platelets = ________________
    • too many granulocytes = ________________
  3. Chronic Myelomonocytic Leukemia (CMML)
    – features of ________________ and ________________
    • too many ________________
    • myeloid cells are ________________
  4. Myelodysplastic Syndrome (MDS)
    – bone marrow ________________
    • ________________ hematopoiesis
    • myeloid cells are ________________
A

Myeloid Disorders

  1. Acute Myeloid Leukemia (AML)
    – too many immature myeloid cells (i.e. myeloid blasts)
  2. Myeloproliferative Neoplasms (MPNs)
    – too many mature myeloid cells
    • too many RBCs = Polycythemia vera (PV)
    • too many platelets = Essential thrombocytosis (ET)
    • too many granulocytes = Chronic Myelogenous Leukemia (CML)
  3. Chronic Myelomonocytic Leukemia (CMML)
    – features of BOTH MPN and MDS
    • too many monocytes
    • myeloid cells are dysplastic
  4. Myelodysplastic Syndrome (MDS)
    – not enough myeloid cells (cytopenias) -> bone marrow failure
    • ineffective hematopoiesis
    • myeloid cells are dysplastic
38
Q

Lymphoid Disorders

  1. Acute lymphocytic leukemia (ALL)
    – too many __________ lymphoid cells
  2. Lymphoproliferative disorders (LPDs)
    – too many __________ lymphoid cells
    • __________: burden of disease is in the peripheral blood
    • __________: burden of disease is in lymph node
    • Waldenstrom Macroglobulinemia /Lymphoplasmacytic lymphoma
  3. Plasma cell dyscrasias
    – Too many __________ cells → overproduction of a __________
    • MGUS → smoldering myeloma → multiple myeloma
    • Primary amyloidosis
A

Lymphoid Disorders
1. Acute lymphocytic leukemia (ALL)
– too many immature lymphoid cells (i.e. lymphoid blasts)

  1. Lymphoproliferative disorders (LPDs)
    – too many mature lymphoid cells
    • CLL: burden of disease is in the peripheral blood
    • Lymphoma: burden of disease is in lymph node
    • Waldenstrom Macroglobulinemia /Lymphoplasmacytic lymphoma
  2. Plasma cell dyscrasias
    – Too many plasma cells → overproduction of a single antibody / light chain
    • MGUS → smoldering myeloma → multiple myeloma
    • Primary amyloidosis
39
Q

Acute Leukemia:
Diagnosis?

A

≥ 20% blasts in peripheral blood or bone
marrow*

40
Q

Blast cells + Auer rods + DIC
THINK: ___________

A

APL (Acute Promyelocytic Leukemia)

41
Q

APL
• T(__;__)
• ________ mortality from ____:

Urgent treatment? ______

A

• T(15;17) PML-RARA

• ↑early mortality from DIC:
- Intracranial hemorrhage
- Blood clots

Urgent treatment: ATRA (all-trans-retinoic acid)

42
Q

Acute Leukemia Associated Emergencies:
1. Tumor lysis

__ K, __ PO4, __ uric acid (UA), __ Ca

Treatment:
1. ______________
2. ______________
3. ______________
4. ______________

A

↑ K, ↑ PO4, ↑ uric acid (UA), ↓ Ca

Treatment:
1. Manage hyper-K, correct ext. lytes
2. IVF (target UO 80-100 mL/m2/h)
AND EITHER:
3. Allopurinol
OR
4. Rasburicase if: AKI, ↑↑ uric acid (>535 umol/L), no response to allopurinol (NOT in G6PD def)

43
Q

Acute Leukemia Associated Emergencies:
2. Leukostasis

Treatment:
1. ______________
2. ______________ MOST IMPORTANT
3. ______________
4. ______________

A

Treatment:
1. IVF
2. Cytoreduction (e.g hydroxyurea, leukapheresis, induction chemo)
3. TLS prophylaxis
4. Avoid transfusion

44
Q

Polycythemia vera:

Risk stratification (for thrombosis)
LOW RISK: Age _____ and ________
HIGH RISK: Age ____ or _______ history

Treatment:
EVERYONE: __________, optimize __________,
__________

HIGH RISK: __________
(__________ if young or pregnant)

A

Risk stratification (for thrombosis)
LOW RISK: Age < 60 and no thrombosis history
HIGH RISK: Age ≥ 60 yo or thrombosis history

Treatment:
EVERYONE: ASA 81 mg, optimize CV risk factors, Hct < 45% with phlebotomy (CYTO-PV NEJM 2013)

HIGH RISK: cytoreduction with Hydroxyurea
(interferon if young or pregnant)

45
Q

Essential Thrombocythemia (ET)
Treatment:

Very low risk (no thrombosis, age<60, JAK2-): __________________

Low risk (no thrombosis, age<60, JAK2+): __________________

Intermediate risk (no thrombosis, age≥60, JAK2-): __________________

HIGH RISK (age≥60 and JAK2+ or thrombosis hx): __________________

JAK2+ with CV risk factors: __________________

*Hold __________________ if plt > 1000-1500 or acquired vWD

A

Essential Thrombocythemia (ET)
Treatment:

Very low risk (no thrombosis, age<60, JAK2-):
observation

Low risk (no thrombosis, age<60, JAK2+):
ASA 81 mg

Intermediate risk (no thrombosis, age≥60, JAK2-): ASA 81 mg

HIGH RISK (age≥60 and JAK2+ or thrombosis hx): add hydroxyurea (interferon if young or pregnant)

JAK2+ with CV risk factors: ASA 81 mg BID

*Hold ASA if plt > 1000-1500 or acquired vWD

46
Q

Leukoerythroblastosis?

Definition? ___________________

Differentials?

The presence of leuko-erythroblastosis raises concern for transformation from ___/__ to ____________________

A

Leukoerythroblastosis?

Definition: (left shift in myeloid series with myelocytes/ metamyelocytes/ blasts along with nucleated RBCs)

Differentials?
- GCSF
- infection/marrow stress
- myelophisitic processes (ie. metastatic cancer to bone)
- MPNs including myelofibrosis/CMML

The presence of leukoerythroblastosis raises concern for transformation from PV/ET to prefibrotic myelofibrosis.

47
Q

CLL:

Presentation:
• Peripheral blood: “_________” cells

Diagnosis:
• ______________________

*Do not need _______ to make dx

A

CLL:

Presentation:
• Peripheral blood: “smudge” cells

Diagnosis:
• peripheral blood flow cytometry

*Do not need BMBx to make dx

48
Q

CLL Treatment options:

Which is associated with bleeding and Atrial fibrillation?

A

CLL Treatment options:

Tyrosine kinase inhibitors i.e. Ibrutinib (SE =
bleeding, A.fib)

49
Q

Lymphoma Classification + Treatment

A
50
Q

Chimeric Antigen Receptor (CAR)-Therapy
Toxicities:

  1. Cytokines release syndrome (CRS)
    Sx?
    Labs?
    Tx?
  2. Immune effector cell-associated neurotoxicity (ICANS)
    Sx?
    Tx?
A

Chimeric Antigen Receptor (CAR)-Therapy
Toxicities

  1. Cytokines release syndrome (CRS)
    Sx? Fever, tachypnea, headache, tachycardia, HTN, rash, hypoxia due to cytokines storm
    Labs: elevated CRP/ferritin
    Tx based on CRS grade: supportive care, +/-steroids, +/- tocilizumab
  2. Immune effector cell-associated neurotoxicity (ICANS) due to disruption in BBB, cytokines
    Sx: aphasia, tremor, altered LOC, impaired cognition, motor weakness, seizures, cerebral edema
    Tx: supportive care, CNS imaging, seizure prophylaxis/treatment; Tx concurrent CRS
51
Q

Plasma Cell Dyscrasias

A
52
Q

Waldenstrom’s Macroglobulinemia:
DEFINITION:
AKA ______________
AND
______________ in the peripheral blood

Other key features to distinguish from others?

A

AKA Lymphoplasmacytic lymphoma (an indolent lymphoma with features between B-cells & plasma cells)
AND
Monoclonal IgM in the peripheral blood

Other key features to distinguish? Hepatosplenomegaly, lymphadenopathy

53
Q

Bone Marrow Failure Syndromes?

A

Myelodysplastic Syndrome (MDS)
– Cytopenia(s) + dysplastic cells/genetic abnormalities/blasts

Aplastic anemia (AA)
– Hypocellular bone marrow
– Primary vs. secondary

Paroxysmal nocturnal hemoglobinuria (PNH)
– Bone marrow failure
– Hemolysis
– Thrombosis

54
Q

Myelodysplastic Syndrome (MDS):
Definition:

A
55
Q

Myelodysplastic Syndrome (MDS):
Presentation:
• _______ pt. w/ a _____ (often ___ Hb + ____ MCV)
• Blood film: ________________ neutrophils,
____________ platelets, macrocytosis, _____ retics

Diagnosis? _______________

A

Presentation:
• Older pt. w/ a cytopenia (often ↓Hb + ↑MCV)
• Blood film: hypolobated/hypogranular neutrophils,
hypogranular platelets, macrocytosis, ↓ retics

Diagnosis:
• Bone marrow biopsy/aspirate:
– Hypercellular (usually) +/- ring sideroblasts
– Dysplasia >10%
– Blasts <20%

56
Q

Paroxysmal Nocturnal Hemoglobinuria (PNH)
Characterized by?
1. _______________
2. _______________
3. _______________
4. ________________

Work-up?

Diagnosis?
• ___________________
• Can overlap with ___________________

Treatment?

A

Characterized by?
1. Bone marrow failure
2. Chronic intravascular (+ extravascular) hemolysis –> uncontrolled complement activation
3. Thrombosis (venous + arterial) –> common site = splanchnic, hepatic
4. thrombosis in atypical locations (ie. abdominal or cerebral veins), anemia Sx, hemolysis Sx (jaundice, red urine), pHTN Sx, decr NO Sx (erectile dysfxn, dysphagia)

Work-up?
• Hemoglobinuria (rare), hemolysis labs, Cr (renal failure from pigment nephropathy)

Diagnosis?
• Peripheral blood flow cytometry (loss of CD55 + CD59)
• Overlap w/ aplastic anemia

Treatment?
• Anti-coagulate if thrombotic events
– No role for primary prophylactic anticoagulation
• Eculizumab or pegcetacoplan (complement inhibitors)
- ↓ hemolysis + transfusion need (not curative)

57
Q
A

Blast with Auer rod

If you see blasts with Auer rods, think APL

Arrow = Auer rod

58
Q
A

Teardrops

Think myelofibrosis
(B symptoms, high LDH, splenomegaly, cytopenias, leukoerythroblastosis)

59
Q
A

CLL

60
Q
A

ROULEAUX

Associated with multiple myeloma as well as
inflammation, pregnancy (high ESR states)

61
Q
A

Spherocyte

Spherocytes lack central pallor and are smaller than normal RBC.

Think immune-mediate hemolysis (e.g. auto or allo immune)

62
Q
A

Hyper-segmented Neutrophil

≥6 lobes = hypersegmented. Think megaloblastic anemia (e.g. folate or B12 deficiency)

63
Q
A

Schistocytes (aka fragments)

All arrows pointing to examples of schistocytes. Think TMA (eg. TTP)

64
Q
A

Malaria

65
Q
A

Sickle Cell

66
Q

Blood Product “Modification”
Indications for CMV-negative blood:

A
  • Intrauterine transfusion
67
Q

Blood Product “Modification”
Indications for irradiated blood - prevent transfusion-associated ______:
1. _____________
2. _____________
3. _____________
4. _____________

A

Indications for irradiated blood - prevent transfusion-associated GVHD:

Ø T cell deficiency
Ø Hodgkin’s lymphoma
Ø Chemotherapy with purine antagonists or purine-like antagonists
Ø HSC transplant recipients:
• Allogeneic: for life
• Autologous: for 3 months

68
Q

Blood Product “Modification”
Indications for washed platelets:

  1. ______________
  2. ______________
    3, ______________
A

Indications for washed platelets:

Ø Anaphylaxis to transfusion NYD
Ø Recurrent/severe allergic transfusion reactions
Ø IgA deficiency with no IgA deficient donor available

69
Q

Thrombocytopenia:
1) Rule out ___________________

2) Should I be worried?
– Other lineages down? → __________________
– Abnormal coags? → _______________________
– Recent heparin → _________________________
– Recent transfusion → _____________________
– Is this sepsis?

A

Thrombocytopenia:
1) Rule out pseudo-thrombocytopenia
• Secondary to EDTA-associated agglutinins
• Repeat in citrated tube

2) Should I be worried?
– Other lineages down? → Marrow process, MAHA, Evans
– Abnormal coags? → DIC, APLA
– Recent heparin → HIT
– Recent transfusion → Post-transfusion purpura
– Is this sepsis?

70
Q

Pancytopenia ddx

Primary?

Secondary?

A

Pancytopenia ddx

Primary
1. Paroxysmal Nocturnal Hemoglobinuria (PNH)
2. Aplastic Anemia
3. Leukemia
4. MDS
5. Myelofibrosis

Secondary
1. Deficiency - B12/Folate, anorexia, êT4
2. Infection – granulomatous, HIV, sepsis
3. Iatrogenic – chemo, rads, ETOH
4. Infiltrative – mets, amyloid, Gaucher
5. Hypersplenism

71
Q

B12 deficiency or syphilis?

A

Distinguish Neuro B12 deficiency from Neuro Syphilis

– BOTH: Mental status changes/Dementia

– Pupils: B12: Normal
- Pupils: Syphilis: Argyle Robertson (small, no reaction to light but does accommodate)

– B12 = subacute combined degeneration of cord = corticospinal tract (UMN weakness, increased tone in legs, hyperreflexia/clonus) AND dorsal column (dec. vibration, proprioception)

– Syphilis = “Tabes Dorsalis” = dorsal column (vibration, proprioception impaired) and dorsal roots (absent reflexes in LE). Unlike B12 deficiency, tone/power should be normal.

72
Q

Differentials: DIC vs other hemolysis

A