Heme Exam 2 Flashcards
Direct Factor Xa inhibitors
Rivoraxaban
Apixaban
Indirect Factor Xa inhibitors
Heparin
LWMH
Fondaparinux
Direct Thrombin Inhibitors
Dabigatran
Argatroban
ITP: labs
- decreased platelet count
- order HCV, HIV, bone marrow bx to rule out secondary causes
ITP: tx
-corticosteroid +/- IVIG or Rh IG recurrent/persistently below 30,000 platelets: -refer -Rituximab -spelenectomy
consider/tx H. pylori infx
Classic HUS causes
E. coli 0157:H7 or 0145
Shiga toxin
TMA pentad
- microangiopathic hemolytic anemia
- thrombocytopenia
- fever
- kidney dz
- neuro sx
TMA: tx
plasma exchange, tx until platelets and LD normalize
HIT: labs
- ELISA: screens for PF4 antibody
- confirm w/ serotonin assay
HIT: tx
- US of LEs to r/o DVT
- switch to direct thrombin inhibitor: Argatroban (once platelets recovered, switch to Warfarin)
Causes of congenital qualitative platelet disorders
- Bernard-Soulier syndrome: abnormal platelet membrane expression
- Glanzmann Thromboasthenia: abnormality of Gp IIb/IIIa receptors on platelet
Drugs that cause qualitative platelet disorders
- salicylates
- NSAIDS
- SSRIs
- Abx
- Alcohol
vWF dz: tx
- 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)
Hemophilia A: long-term tx
- 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)
Hemophilia A: acute bleeding episodes tx
- mild: DDAVP (vasopressin) IV/intranasal
- adjunct therapy: tranexamic acid (dental visits)
- moderate and severe pts: give Factor VIII
DDAVP (vasopressin): MOA
causes transient rise in Factor VIII levels and vWF
tranexamic acid: MOA
decreases plasmin formation and fibrinolysis by inhibiting plasminogen binding sites
Hemophilia B: long-term tx
- 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)
Hemophilia B: acute bleeding episodes tx
- Factor IX
- adjunct therapy: tranexamic acid for dental procedures
- DDAVP DOES NOT WORK for Hemophilia B
Hemophilia C: tx
- Fresh Frozen Plasma (FFP)
- adjunct therapy: tranexamic acid (dental procedures)
DIC: causative gram - bacteria
- Pseudomonas aeruginosa
- E. coli
- Proteus vulgaris
DIC: causative gram + bacteria
- group A strep toxic shock syndrome
- S. aureus
Vitamin K deficiency: tx
- underlying cause
- Vitamin K IV/oral
- Severe hemorrhage: fresh frozen plasma (FFP)
SVT: tx
-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
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
Westermark sign
prominent/dilated central pulmonary artery seen on chest Xray, suggests PE
Hampton’s Hump
pleural areas of hemorrhage seen on chest xray, suggests PE
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
PE: tx in cancer pts
LMWH
- enoxaparin (lovenox)
- dalteparin
- tinzparin
- nadroparin
- reviparin
Warfarin: contraindications
pregnancy
PE: tx (newer anticoagulants)
Fondaparinux
Rivaroxaban (approved for immediate monotherapy)
Apixaban (approved for immediate monotherapy)
Dabigatran (must bridge w/ heparin first)
VTE/DVT/PE: absolute contraindications to tx
intracranial bleeding
recent brain, eye, or spinal cord surgery
malignant hypertension
Polycythemia vera: sx
Neuro sx Erythromelalgia* Aquagenic pruritus Hepato/spleno-megaly Thrombotic events (often initial sx) (more)
Polycythemia vera: labs
- polycythemia labs
- JAK2 mutation screening +
- Vitamin B12 very high
- Hyperuricemia
- Bone marrow bx: hypercellularity
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
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
Hydroxyurea: indications for tx of polycythemia veria
- if phleb not feasible
- high phleb requirements
- thrombocytosis/thrombotic events
HH: sx
weakness, lethargy, impotence, arthralgia Class Triad (late stage): -cirrhosis -DM -bronzing of skin
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
HH: criteria for proceeding directly to tx w/ liver biopsy
<40 y/o
- Homozygeous C282Y mutation
- Labs indicate iron overload
- Normal transaminases
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
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
siderophilic bacterium which necessitates that HH pts avoid shellfish
vibrio vulnificans
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)
Whole blood: transfusion indications
trauma >25% blood loss
military and emergencies
pRBC: transfusion indications
- anemia: surgery, trauma, symptomatic
- active bleeding
- 1 unit of pRBCs should increase Hgb by ~1 gram
Platelets: transfusion indications
therapeutic:
-tx of active bleeding w/ thrombocytopenia
-prep for invasive procedure when thrombocytopenic
prophylactic:
-prevent bleeding
beware of HLA matching
Fresh Frozen Plasma: transfusion indications
-corrects coagulopathies (liver dz, DIC, supratherapeutic warfarin levels)
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
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
AML: bone marrow findings
Auer rods*
Blasts >20%
FISH
AML: favorable risk cytogenetics
t(8;21), inv(16)
p13;q22
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?
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)
ALL: favorable risk crytogenics
hyperdiploidy: >50 chromosomes
T(12:21)
ALL: poor risk crytogenetics
hypodiploidy: <44 chromosomes
t(9:22) “Philadelphia chromosome”
t(4;11)
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
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
CLL: tx
early stages: observation
combo therapy:
-purine analogs (fludarabine, pentostatin)
-alkylating agents (chlorambuicl, cyclophosphamide, bendamustine)
-monoclonal antibodies (rituximab, ofatumumab, obinutuzumab)
-Brutons TKI (ibrutinib)
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
CML: tx
- not usually emergent
- normalize heme abnormalties
- suppress malignant BCL/ABL expressing clone
- TKIs: imatinib, nilotinib, dasatinib
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
Non-Hodgkin Lymphoma: lab findings
- increased LDH
- hypercalcemia and hyperuricemia
NHL: tx
Indolent:
-R-CHOP
Aggressive: -R-CHOP +/- INRT -R-EPOCH +/- INRT Burkitt’s: CODOX-M/IVAC Intrathecal chemo
MM: tx
MGUS & Smoldering myeloma: observe MM: -immunomodulatory agent (lenalidomide) -proteasome inhibitor (bortezomib or carfilzomib) -moderate-high dose dexamethasone -Auto SCT
Spinal cord compression: tx
Steroids*
radiation/chemo
surgery
Superior vena cava syndrome: tx
- malignany: chemo vs. radiation
- steroids and sx mgmt
- elevate HOB, lasix, O2
increased ICP: tx
chemo/radiation
steroids
lumbar puncture
Tumor lysis syndrome: lab abnormalities
- hyperuricemia, hypoclcemia, hyperkalemia, hyperphosphatemia
- azotemia: increased Cr and BUN
- increased LDH
Tumor lysis syndrome: tx
- correct electrolytes
- IV fluids (flush kidneys) (but prevent fluid overload)
- Xanthine oxidase inhibitors: allopurinol and rasburicase
