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Clinical Medicine Jeff > Heme Exam 2 > Flashcards

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
Q

DVT: tx

A

-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

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26
Q

Westermark sign

A

prominent/dilated central pulmonary artery seen on chest Xray, suggests PE

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27
Q

Hampton’s Hump

A

pleural areas of hemorrhage seen on chest xray, suggests PE

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28
Q

PE: tx

A

-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
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29
Q

PE: tx in cancer pts

A

LMWH

  • enoxaparin (lovenox)
  • dalteparin
  • tinzparin
  • nadroparin
  • reviparin
30
Q

Warfarin: contraindications

A

pregnancy

31
Q

PE: tx (newer anticoagulants)

A

Fondaparinux
Rivaroxaban (approved for immediate monotherapy)
Apixaban (approved for immediate monotherapy)
Dabigatran (must bridge w/ heparin first)

32
Q

VTE/DVT/PE: absolute contraindications to tx

A

intracranial bleeding
recent brain, eye, or spinal cord surgery
malignant hypertension

33
Q

Polycythemia vera: sx

A 
Neuro sx
Erythromelalgia*
Aquagenic pruritus
Hepato/spleno-megaly
Thrombotic events (often initial sx)
(more)
34
Q

Polycythemia vera: labs

A
  • polycythemia labs
  • JAK2 mutation screening +
  • Vitamin B12 very high
  • Hyperuricemia
  • Bone marrow bx: hypercellularity
35
Q

Polycythemia vera: WHO dx criteria

A 
*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
Q

Polycythemia vera: tx

A
  • 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
Q

Hydroxyurea: indications for tx of polycythemia veria

A
  • if phleb not feasible
  • high phleb requirements
  • thrombocytosis/thrombotic events
38
Q

HH: sx

A 
weakness, lethargy, impotence, arthralgia
Class Triad (late stage):
-cirrhosis
-DM
-bronzing of skin
39
Q

HH: labs

A

-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
Q

HH: criteria for proceeding directly to tx w/ liver biopsy

A

<40 y/o

  • Homozygeous C282Y mutation
  • Labs indicate iron overload
  • Normal transaminases
41
Q

HH: first-line tx

A
  • 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
Q

HH: additional mgmt (besides phleb)

A
  • 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
Q

siderophilic bacterium which necessitates that HH pts avoid shellfish

A

vibrio vulnificans

44
Q

HH with cirrhosis: mgmt in terms of screening

A

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
Q

Whole blood: transfusion indications

A

trauma >25% blood loss

military and emergencies

46
Q

pRBC: transfusion indications

A
  • anemia: surgery, trauma, symptomatic
  • active bleeding
  • 1 unit of pRBCs should increase Hgb by ~1 gram
47
Q

Platelets: transfusion indications

A

therapeutic:
-tx of active bleeding w/ thrombocytopenia
-prep for invasive procedure when thrombocytopenic
prophylactic:
-prevent bleeding
beware of HLA matching

48
Q

Fresh Frozen Plasma: transfusion indications

A

-corrects coagulopathies (liver dz, DIC, supratherapeutic warfarin levels)

49
Q

Cryoprecipitate: transfusion indications

A
  • 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
Q

Transfusion rxn: mgmt

A
  • 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
Q

AML: bone marrow findings

A

Auer rods*
Blasts >20%
FISH

52
Q

AML: favorable risk cytogenetics

A

t(8;21), inv(16)

p13;q22

53
Q

AML: tx

A

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
Q

ALL: bone marrow bx findings

A 
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
Q

ALL: favorable risk crytogenics

A

hyperdiploidy: >50 chromosomes

T(12:21)

56
Q

ALL: poor risk crytogenetics

A

hypodiploidy: <44 chromosomes

t(9:22) “Philadelphia chromosome”
t(4;11)

57
Q

ALL: tx

A

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
Q

CLL: bone marrow bx findings

A 
hypercellular
lymphocytosis
*Smudge cells
Flow and FISH
-B lymph lineage: CD 19
-T lymph lineage: CD5
\+/- bx of lymph node/organs
59
Q

CLL: tx

A

early stages: observation
combo therapy:
-purine analogs (fludarabine, pentostatin)
-alkylating agents (chlorambuicl, cyclophosphamide, bendamustine)
-monoclonal antibodies (rituximab, ofatumumab, obinutuzumab)
-Brutons TKI (ibrutinib)

60
Q

CML: bone marrow bx findings

A
  • hypercellular w/ left myeloid shift
  • BCR/ABL gene detected by PCR*
  • basophilia and eosinophilia
  • myeloblasts
  • t (9:22) Philly chromosome
61
Q

CML: tx

A
  • not usually emergent
  • normalize heme abnormalties
  • suppress malignant BCL/ABL expressing clone
  • TKIs: imatinib, nilotinib, dasatinib
62
Q

Hodgkin’s Lymphoma: tx

A

1st: ABVD
+/- radiation to affected sites

interim PET scans to evaluate dz status

relapse after ABVD: high dose chemo and SCT

63
Q

Non-Hodgkin Lymphoma: lab findings

A
  • increased LDH

- hypercalcemia and hyperuricemia

64
Q

NHL: tx

A

Indolent:
-R-CHOP

Aggressive:
-R-CHOP +/- INRT
-R-EPOCH +/- INRT
Burkitt’s: CODOX-M/IVAC
Intrathecal chemo
65
Q

MM: tx

A 
MGUS &amp; Smoldering myeloma: observe
MM:
-immunomodulatory agent (lenalidomide)
-proteasome inhibitor (bortezomib or carfilzomib)
-moderate-high dose dexamethasone
-Auto SCT
66
Q

Spinal cord compression: tx

A

Steroids*

radiation/chemo
surgery

67
Q

Superior vena cava syndrome: tx

A
  • malignany: chemo vs. radiation
  • steroids and sx mgmt
  • elevate HOB, lasix, O2
68
Q

increased ICP: tx

A

chemo/radiation
steroids
lumbar puncture

69
Q

Tumor lysis syndrome: lab abnormalities

A
  • hyperuricemia, hypoclcemia, hyperkalemia, hyperphosphatemia
  • azotemia: increased Cr and BUN
  • increased LDH
70
Q

Tumor lysis syndrome: tx

A
  • correct electrolytes
  • IV fluids (flush kidneys) (but prevent fluid overload)
  • Xanthine oxidase inhibitors: allopurinol and rasburicase

Clinical Medicine Jeff (7 decks)

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