Heme Exam 2 Flashcards

1
Q

Direct Factor Xa inhibitors

A

Rivoraxaban

Apixaban

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2
Q

Indirect Factor Xa inhibitors

A

Heparin
LWMH
Fondaparinux

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3
Q

Direct Thrombin Inhibitors

A

Dabigatran

Argatroban

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4
Q

ITP: labs

A
  • decreased platelet count

- order HCV, HIV, bone marrow bx to rule out secondary causes

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5
Q

ITP: tx

A
-corticosteroid +/- IVIG or Rh IG
recurrent/persistently
below 30,000 platelets:
-refer
-Rituximab
-spelenectomy

consider/tx H. pylori infx

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6
Q

Classic HUS causes

A

E. coli 0157:H7 or 0145

Shiga toxin

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7
Q

TMA pentad

A
  1. microangiopathic hemolytic anemia
  2. thrombocytopenia
  3. fever
  4. kidney dz
  5. neuro sx
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8
Q

TMA: tx

A

plasma exchange, tx until platelets and LD normalize

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9
Q

HIT: labs

A
  • ELISA: screens for PF4 antibody

- confirm w/ serotonin assay

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10
Q

HIT: tx

A
  • US of LEs to r/o DVT

- switch to direct thrombin inhibitor: Argatroban (once platelets recovered, switch to Warfarin)

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11
Q

Causes of congenital qualitative platelet disorders

A
  • Bernard-Soulier syndrome: abnormal platelet membrane expression
  • Glanzmann Thromboasthenia: abnormality of Gp IIb/IIIa receptors on platelet
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12
Q

Drugs that cause qualitative platelet disorders

A
  • salicylates
  • NSAIDS
  • SSRIs
  • Abx
  • Alcohol
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13
Q

vWF dz: tx

A
  • Type 1: DDAVP (vasopressin) causes transient rise in Factor VIII levels and vWF
  • Other types: may require vWF products and Factor VIII concentrates
  • Type 2B: NO DDAVP (causes severe thrombocytopenia)
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14
Q

Hemophilia A: long-term tx

A
  • plasma-derived or recombinant Factor VIII
  • severe: prophylactic factor infusions 2-3x per week
  • mild and moderate: tx as needed for bleeding and for high risk events (sports, surgery, dental procedures)
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15
Q

Hemophilia A: acute bleeding episodes tx

A
  • mild: DDAVP (vasopressin) IV/intranasal
  • adjunct therapy: tranexamic acid (dental visits)
  • moderate and severe pts: give Factor VIII
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16
Q

DDAVP (vasopressin): MOA

A

causes transient rise in Factor VIII levels and vWF

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17
Q

tranexamic acid: MOA

A

decreases plasmin formation and fibrinolysis by inhibiting plasminogen binding sites

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18
Q

Hemophilia B: long-term tx

A
  • plasma-derived or recombinant Factor IX
  • severe: prophylactic factor infusions 2-3x per week
  • mild and moderate: tx as needed for bleeding and for high risk events (sports, surgery, dental procedures)
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19
Q

Hemophilia B: acute bleeding episodes tx

A
  • Factor IX
  • adjunct therapy: tranexamic acid for dental procedures
  • DDAVP DOES NOT WORK for Hemophilia B
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20
Q

Hemophilia C: tx

A
  • Fresh Frozen Plasma (FFP)

- adjunct therapy: tranexamic acid (dental procedures)

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21
Q

DIC: causative gram - bacteria

A
  • Pseudomonas aeruginosa
  • E. coli
  • Proteus vulgaris
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22
Q

DIC: causative gram + bacteria

A
  • group A strep toxic shock syndrome

- S. aureus

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23
Q

Vitamin K deficiency: tx

A
  • underlying cause
  • Vitamin K IV/oral
  • Severe hemorrhage: fresh frozen plasma (FFP)
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24
Q

SVT: tx

A

-rest
-local heat
-elevate extremity
-NSAIDS (usually aspirin)
-removal of foreign body (IV/PICC)
-abx (if cellulitis present)
»S. aureus = most likely pathogen
»inpatient: vancomycin, possibly cefazolin
»outpatient: Bactrim or Cephalexin
-compression stockings

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25
DVT: tx
-rest -local heat -elevate extremity -heparin w/ warfarin bridge >>when INR reaches 2-3: stop heparin >>continue warfarin for at least 3 months (>6 months - indefinitely, if recurrent DVT) -compression stockings -IVC filter for pts who have contraindication of anticoag therapy or high risk for recurrence -recheck Doppler at 3 months
26
Westermark sign
prominent/dilated central pulmonary artery seen on chest Xray, suggests PE
27
Hampton’s Hump
pleural areas of hemorrhage seen on chest xray, suggests PE
28
PE: tx
-UFH: IV, inpatient or -LMWH: subq, out or inpatient, no monitoring/labs needed - start Warfarin at same time as Heparin - goal INR: 2-3 - continue UFH/LMWH x 5 days and until INR 2 x 2 days
29
PE: tx in cancer pts
LMWH - enoxaparin (lovenox) - dalteparin - tinzparin - nadroparin - reviparin
30
Warfarin: contraindications
pregnancy
31
PE: tx (newer anticoagulants)
Fondaparinux Rivaroxaban (approved for immediate monotherapy) Apixaban (approved for immediate monotherapy) Dabigatran (must bridge w/ heparin first)
32
VTE/DVT/PE: absolute contraindications to tx
intracranial bleeding recent brain, eye, or spinal cord surgery malignant hypertension
33
Polycythemia vera: sx
``` Neuro sx Erythromelalgia* Aquagenic pruritus Hepato/spleno-megaly Thrombotic events (often initial sx) (more) ```
34
Polycythemia vera: labs
- polycythemia labs - JAK2 mutation screening + - Vitamin B12 very high - Hyperuricemia - Bone marrow bx: hypercellularity
35
Polycythemia vera: WHO dx criteria
``` *Requires either all 3 major or first 2 + minor* Major: 1) Hgb >16.5 men, >16 women or Hematocrit >49% men, >48% women 2) Bone marrow bx: hypercellularity w/ trilineage growth 3) JAK2 mutation Minor: subnormal serum EPO level ```
36
Polycythemia vera: tx
- refer to heme - PHLEBOTOMY: weekly until hct <45%, then periodically to maintain this level - Aspirin: reduces thrombotic events - Hydroxyurea: decreases RBCs, only for special indications - New tx: ruxolitimib (JAK 2 inhibitor) - Low iron diet may help reduce frequency of phelbotomy
37
Hydroxyurea: indications for tx of polycythemia veria
- if phleb not feasible - high phleb requirements - thrombocytosis/thrombotic events
38
HH: sx
``` weakness, lethargy, impotence, arthralgia Class Triad (late stage): -cirrhosis -DM -bronzing of skin ```
39
HH: labs
-Transferrin sat: >45% -Serum ferritin: >200 ng/mL in men >150 ng/mL in women -mildly elevated transaminases if suspected by above labs: -genetic testings for HFE mutation -consider liver biopsy
40
HH: criteria for proceeding directly to tx w/ liver biopsy
<40 y/o - Homozygeous C282Y mutation - Labs indicate iron overload - Normal transaminases
41
HH: first-line tx
- PHELBOTOMY: reduce total iron levels and normalize ferritin levels (goal 50-150 ng/mL) - frequency: 1-2 per week until iron stores normalized, then less frequently for life - each 500 mL unit removed 200-250 mg iron and reduces serum ferritin by approx 30 ng/mL - CONTRAINDICATION: Hgb <12.5 g/dL
42
HH: additional mgmt (besides phleb)
- PPIs can reduce iron absorption - AVOID: iron and Vitamin C supplements, raw shellfish - if intolerant of phleb, iron chelation needed: desferoxamine - liver transplant if advanced cirrhosis
43
siderophilic bacterium which necessitates that HH pts avoid shellfish
vibrio vulnificans
44
HH with cirrhosis: mgmt in terms of screening
US every 6-12 months: - if lesion < 1 cm, screen every 3-6 months - if lesions > 1 cm, refer for 4 phase multidetector CT and biopsy (doing this to screen for hepatocellular carcinoma)
45
Whole blood: transfusion indications
trauma >25% blood loss | military and emergencies
46
pRBC: transfusion indications
- anemia: surgery, trauma, symptomatic - active bleeding - 1 unit of pRBCs should increase Hgb by ~1 gram
47
Platelets: transfusion indications
therapeutic: -tx of active bleeding w/ thrombocytopenia -prep for invasive procedure when thrombocytopenic prophylactic: -prevent bleeding *beware of HLA matching*
48
Fresh Frozen Plasma: transfusion indications
-corrects coagulopathies (liver dz, DIC, supratherapeutic warfarin levels)
49
Cryoprecipitate: transfusion indications
- contains factor 8, 13, fibrinogen, and vWF - lack of/low fibrinogen (<100 mL/dL) - Factor VIII deficiency - vWF disease -cryo more concentrated than FFP- useful if pt fluid overloaded
50
Transfusion rxn: mgmt
- stop transfusion - hemodynamic support - telemetry/frequent VS - labs: retest ABO compatibility, Rh antibody testing, direct antiglobulin (Coombs), LDH, bilirubin, DIC, serial Hgbs to monitor for hemolysis
51
AML: bone marrow findings
Auer rods* Blasts >20% FISH
52
AML: favorable risk cytogenetics
t(8;21), inv(16) | p13;q22
53
AML: tx
Induction therapy (7+3): - cytarabine (7 days) - anthracycline (idarubicin or daunorubicin) (3 days) additional target therapies if not candidate for induction: -azacitidine, decitabine, or clofarabine after induction>>> consolidation: -high dose cytarabine Stem Cell Transplant?
54
ALL: bone marrow bx findings
``` NO Auer rods Blasts >20% FISH B lineage: CD19 and CD10 T lineage: Express CD2, CD5, CD7, TdT do NOT express CD3, CD4, CD8 (mature T cell markers) ```
55
ALL: favorable risk crytogenics
hyperdiploidy: >50 chromosomes T(12:21)
56
ALL: poor risk crytogenetics
hypodiploidy: <44 chromosomes t(9:22) “Philadelphia chromosome” t(4;11)
57
ALL: tx
Combo therapy: -daunorubicin, vincristine, prednisone, asparaginase + TKI if Philly chromosome (+) dasatinib CNS prophylaxis Relapsed dz: -blinatumomab and ionatuzamab SCT CART-19 for relapsed dz
58
CLL: bone marrow bx findings
``` hypercellular lymphocytosis *Smudge cells Flow and FISH -B lymph lineage: CD 19 -T lymph lineage: CD5 +/- bx of lymph node/organs ```
59
CLL: tx
early stages: observation combo therapy: -purine analogs (fludarabine, pentostatin) -alkylating agents (chlorambuicl, cyclophosphamide, bendamustine) -monoclonal antibodies (rituximab, ofatumumab, obinutuzumab) -Brutons TKI (ibrutinib)
60
CML: bone marrow bx findings
- hypercellular w/ left myeloid shift - BCR/ABL gene detected by PCR* - basophilia and eosinophilia - myeloblasts - t (9:22) Philly chromosome
61
CML: tx
- not usually emergent - normalize heme abnormalties - suppress malignant BCL/ABL expressing clone - TKIs: imatinib, nilotinib, dasatinib
62
Hodgkin’s Lymphoma: tx
1st: ABVD +/- radiation to affected sites interim PET scans to evaluate dz status relapse after ABVD: high dose chemo and SCT
63
Non-Hodgkin Lymphoma: lab findings
- increased LDH | - hypercalcemia and hyperuricemia
64
NHL: tx
Indolent: -R-CHOP ``` Aggressive: -R-CHOP +/- INRT -R-EPOCH +/- INRT Burkitt’s: CODOX-M/IVAC Intrathecal chemo ```
65
MM: tx
``` MGUS & Smoldering myeloma: observe MM: -immunomodulatory agent (lenalidomide) -proteasome inhibitor (bortezomib or carfilzomib) -moderate-high dose dexamethasone -Auto SCT ```
66
Spinal cord compression: tx
Steroids* radiation/chemo surgery
67
Superior vena cava syndrome: tx
- malignany: chemo vs. radiation - steroids and sx mgmt - elevate HOB, lasix, O2
68
increased ICP: tx
chemo/radiation steroids lumbar puncture
69
Tumor lysis syndrome: lab abnormalities
- hyperuricemia, hypoclcemia, hyperkalemia, hyperphosphatemia - azotemia: increased Cr and BUN - increased LDH
70
Tumor lysis syndrome: tx
- correct electrolytes - IV fluids (flush kidneys) (but prevent fluid overload) - Xanthine oxidase inhibitors: allopurinol and rasburicase