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pulmonary > heme!! > Flashcards

heme!! Flashcards

1
Q

thrombotic microangiopathies

A
  • include TTP (thrombotic thrombocytopenic purpura) and HUS (hemolytic uremic syndrome)
  • thrombocytopenia secondary to insult causing platelet activation, produces thrombi in microcirculation causing hemolytic anemia (fibrin shears RBC – shistocytes!!)
  • causes poor perfusion and organ dysf
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2
Q

pentad a/w thrombotic microangiopathies

A
  1. microangiopathic hemolytic anemia
  2. thrombocytopenia
  3. fever
  4. kidney dz (esp hus!!)
  5. transient neuro dysf (esp TTP!!)
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3
Q

TTP - patho

A

deficiency in ADAMTS13 which cleaves multimers of vWF normally
w/ deficiency, get large clumps of vWF that deposit and activate platelets
can be acquired or immune (antibodies against ADAMTS13 - GET POSITIVE COOMBS)

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4
Q

TTP - pres

A
  1. anemia - pale, fatigue, SOB
  2. bleeding - purpura, petechiae
  3. transient neuro def - HA, confusion, lethargy, coma, seizures, paresis, delirium
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5
Q

HUS - patho

A
  • normal ADAMTS13…a/w ECOLI 0157:H7 AND O145 (THINK ABOUT KIDS EATING HAMBURGERS!!!!)
  • e.coli relsease shiga toxin causing endo cell dysf and platelet activation
    • TOXIN HAS INCREASED AFFINITY FOR RENAL VESSELS!!
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6
Q

HUS - pres

A
  1. anemia
  2. bleding
  3. KIDNEY FAILURE (esp in kids!)
    * rarely have neuro sx!
    * **h/o diarrhea!!
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7
Q

TTP and HUS labs / dx

A

-evidence of hemolytic anemia: normochromic w/ elevated INDIRECT BILIRUBIN, LDH and decreased HAPTOGLOBIN
**check K and EKG!!
-blood smear: reticulocytosis and shistocytosis
-normal coag tests!
TTP: positive coombs and more severe thrombocytopenia!!
HUS: negative coombs! positive stool culture!

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8
Q

treatment TTP

A
  1. DAILY IMMEDIATE PLASMA EXCHANGE!!! ASAP and continue until platelets and LDH normal >2 days! then taper slowly and monitor!
    • if refractory, do twice daily!
  2. prednisone for immune mediated TTP
  3. FFP if plasma exchange not available
  4. RBC transfusion w/ sig anemi
    - w/ refractory: rituximab, corticosteroids, IVIG, vincristine, cyclophosphadmide, splenectomy
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9
Q

treatment HUS

A

kids: most self-limiting - FLUIDS TO PROTECT KIDNEYS! DONT TREAT W/ ANTIBIOTICS - WILL INCREASE TOXIN RELEASE!
adult: a/w malignancies, SLE, chem, AI
- same as ttp: daily plasma exchange, hemodialysis w/ renal failure

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10
Q

contraindication w/ TTP and HUS

A

PLATELET TRANSFUSIONS!!!! WORSENS THROMBOSIS!!!!

only give if severe life-threatening bleeding!

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11
Q

pruritis after hot shower / bath w/ hx DVT…

A

think about POLYCYTHEMIA VERA!

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12
Q

polycythemia vera dx

A 
1st rule out secondary w/ erythropoietin level - should be LOW w/ PV! (will be high w/ secondary!)
3 major or 2 major + 2 minor
major: 
1. increased red cell mass >32 ml/kg female or >36 ml/kg male
2. normal ox sat >92%
3. splenomegaly
minor:
1. throbocytosis >400,000
2. leukocytosis >12,000
3. leukocyte alk phos >4000
4. increased B12
**definitive w/ biopsy!
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13
Q

PV pres

A

sx d/t:

  1. increased viscosity: HA, malaise, fatigue, dizzy, PRURITIS!, vision changes, syncope, FACIAL FLUSHING
  2. increased thrombotic events: MI, CVA, DVT/PE
  3. bleeding / increased bruising
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14
Q

PV other lab findings….

A
  1. hyperuricemia
  2. high cholesterol
  3. high histamine..PRURITIS!
  4. low erythropoietin! w/ high it is secondary i.e. hypoxia
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15
Q

PV treatment

A

1st line / mainstay: phlebotomy q 2-3 d to reduce hematocrit to about 45%

  1. ASA 81 mg and adequate hydration
  2. Hydroxurea - 2nd line if phlebotomy not posible or high risk i.e. >60 / h/x of clot
  3. allopurinol for increased uric acid
  4. antihistamines for pruritis
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16
Q

main cause of death in PV

A

thrombotic events

17
Q

key sx multiple myeloma

A

HIGH CALCIUM + HIGH PROTEIN + LOW HGB + RENAL FAILURE!

18
Q

MM patho…

A

malignant proliferation of single plasma line - abundant proliferation of abnormal immunoglobulines = M protein!

  • proliferates in bone marrow causing PUNCHED OUT LYTIC BONE LESIONS seen on xray and pancytopenia!
  • can be entire protein or components (KAPPA / LAMBDA light chains!)
19
Q

CRAB w/ MM…

A

key sx:
high calcium, renal failure, anemia (normocytic normochromic) and bone pain / lesions
-more in african americans!

20
Q

MM sx (most to least common):

A
  1. Anemia (n,n): d/t bone marrow infiltration and renal failure
  2. bone pain - worse w/ movement, NOT worse at night - most in face, back and chest (axial skeleton)
  3. renal failure / inc crt d/t hypercalcemia and BENCE JONES proteins (myeloma kidney)
  4. hypercalcemia
  5. weight loss
  6. recurrent infections d/t decreased effective immunoglobulins - #1 cause of death! most lung or UTI
  7. cord compression - MEDICAL EMERGENCY! GET mri AND START STEROIDS!
    * Extrameduallary plasmacytomas - purplish mass dx w/ PET
21
Q

testing if suspect MM…

A
  1. SPEP/UPEP (serum / urine protein electrophoresis) - blood or u/a w/ 24 hr urine
    +
  2. immunofixation of serum / urine - done w/ abnormal spep/upep
    *KAPPA is predominant light chain seen
  3. serum free light chains (FLC) assay - measures amout of kappa and lambda and gives K:L ratio
  4. U/A: w/ myeloma cast nephropathy see no / minimal proteinuria b/c U/A detects ALBUMIN! not bence-jones!
    -w/ amyloidosis / light chain deposition dz see more significant proteinuria!
  5. peripheral blood smear: pancytopenia w/ ROULEAUX!!!! = coin stacks - hyperglobulinemia makes RBC sticky!
  6. BM bx: for dx, must have at least 10% abnormal plasma cells!!
  7. bone survey w/ xray -include humerus and femur for PUNCHED OUT LYTIC BONE LESIONS!! then f/u CT/MRI w/ negative or w/ neuro sx / compression
22
Q

diagnostic criteria for MM:

A
  1. at least 10% abnormal plasma cells plus one of:
    A. organ / tissue impairment (CRAB)
    B. presence of biomarker near inevitable progression to end organ damage - 60% clonal cells, FLC ratio 100+, MRI w/ 1+ lesion
23
Q

other similar diseases…

A

Waldenstrom macroglobulinemia: proliferation of lymphocytes producing Igm paraprotein - leads to viscous blood

  • dx: >5g/dl of IgM, bence-jones proteinuria BUT NO LYTIC BONE LESIONS!
    2. amyloidosis / light chain deposition disease: see amyloid on biopsy - different b/c deposits cause nephrotic syndrome, heart failure, liver failure
  • *no bone lesions! w/ modest proteinuria and 20% or less plasma cells on bm bx
24
Q

tx MM

A

if young and asx: hematopoietic cell transplant!! DO NOT DO CHEMO PRIOR! CANNOT DO HCT AFTER CHEMO!
if not transplant candidate, do systemic chemo
*prognosis: 2-4 yrs, 5 yr survival = 10%

pulmonary (16 decks)

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