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P4T1 > Heme and Lymph CIS > Flashcards

Heme and Lymph CIS Flashcards

1
Q

purpura

A
  • non-blanchable, hemorrhagic skin lesions that result from the leakage of RBCs into skin
  • can be palpable or non-palpable
  • macular- non-palpable/non-infl- further divided into petechiae (<3 mm) and ecchymosis (>5 mm)
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2
Q

Purpura + Thrombocytopenia and abnormal coag studies-diff dx

A
  • septicemia (meningococcal)
  • ITP (immune thrombocytopenia)
  • HUS (E coli 0157:H7)
  • leukemia
  • coagulopathies
  • **purpura are non-palpable in thrombocytopenia purpuric disorders!!!
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3
Q

Purpura + normal plt count and coag studies

A
  • Henoch-Schoenlein Purpura (IgA vasculitis)
  • Acute Hemorrhagic edema of infancy (AHEI)
  • hypersensitivity vasculitis
  • other small vessel vasculitides
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4
Q

Henoch-Schoenlein Purpura

A
  • more straightfwd in adults than in children

- intussusception of bowel is much less common in adults!!!

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5
Q

Henoch-Schoenlein Purpura- tetrad

A
  • Mandatory- palpatory purpura w/o thrombocytopenia and coagulopathy!!!
  • plus 1 or more of:
  • acute arthralgia and/or arthritis
  • acute abd pain
  • renal dz
  • IgA deposition on bx
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6
Q

IgA vasculitis (IgA nephropathy)

A
  • immune-mediated vasculitis- may be triggered by a variety of ag’s (infections, immunizations)
  • timeline is significant and typical for HSP!!!
  • dx- may follow a streptococcal infection (sore throat), w generalized arthralgias/myalgias, rash, palpable purpura (LE and buttocks), abd pain, renal insuff
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7
Q

Acute hemorrhagic edema of infancy

A
  • leukocytoclastic vasculitis
  • children 4 months- 2 yrs
  • self-limited- resolves in 1-3 wks
  • fever, purpura, ecchymosis, infl edema of limbs
  • kidney and GI tract involvement is UNCOMMON
  • bx of skin- leukocytoclastic vasculitis with occasional IgA deposition
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8
Q

Hypersensitivity vasculitis

A
  • infl of small vessels that occurs after exposure to- drugs, infections, or w/o an identifiable trigger
  • fever, urticaria, lymphadenopathy, arthralgias (not usually glomerulonephritis)
  • histo- leukocytoclastic vasculitis primarily of postcapillary venules, but IgA deposition is absent
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9
Q

Other small vessel vasculitides- primary vasculitides

A
  • granulomatosis with polyangiitis (Wegener’s)
  • microscopic polyangiitis
  • eosinophilic granulomatosis with polyangiitis (Churg-Strauss Syndrome)
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10
Q

Other small vessel vasculitides- secondary vascular infl

A
  • SLE
  • RA
  • infectious dz (hep B or C)
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11
Q

Rhabdomyolysis

A
  • infection + doing insanity workout
  • sx- arthralgias, abd pain, renal insuff, CPK elevation
  • *our pt was in excellent health prior to onset of these sx’s
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12
Q

DDX arthritis and arthalgia

A

until a pt develops the classical purpura of HSP (igAV)

  • autoimmune diseases- SLE, juvenile idiopathic arthritis
  • rheumatic fever vs HSP
  • septic and toxic synovitis (also called transient synovitis)
  • reactive arthritis
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13
Q

Rheumatic fever vs HSP

A
  • recent group A beta-hemolytic streptococci infection
    • throat culture, + rapid streptococcal ag test, or elevated anti-streptolysin O titers
  • clinical course of Jones Criteria- rash is diff, erythema marginatum, nodules, carditis
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14
Q

Septic and Toxic synovitis (transient synovitis)

A
  • may have joint sx’s
  • 1 or 2 joints, unlike the polyarthritis seen in HSP
  • affected joints are warm and erythematous (unlike in HSP)
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15
Q

reactive arthritis

A

-may be triggered by genitourinary and GI pathogens (including beta-hemolytic streptococcal infections)

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16
Q

DDX abd pain

A
  • rash of HSP usually precedes GI manifestations
  • evaluation for potential acute abdomen
  • radiologic studies used to screen for surgical causes of abd pain also used in pts with HSP who develop GI complications (intussusception, bowel infarction, or perforation)
17
Q

HSP- plan to manage pts

A
  • majority of pts recover spontaneously
  • assess renal status, overall fluid/electrolyte status- in severe cases corticosteroids have been used!!
  • steroids- “one-shot” thing- reserve them for when you really need them
  • IV hydration= IV fluid rate and type of solution (saline/D5/LR) is empiric until labs return- goal is to initiate rehydration and monitor!!!
  • pain management
  • anti-emetics
  • PPI to minimize acute gastritis/PUD from NSAID or steroids
  • acute abd series (plan film xray) or US is done- screen for intussusception!!!
18
Q

HSP- lab tests to consider

A
  • serum IgA levels- 50-70% and higher levels are assoc w renal involvement
  • check CBC, CMP, urinalysis
  • normal plt count and coag studies (prothrombin time) are necessary!!!
  • hypocomplementemia- check complement levels
  • skin bx- small BVs of superficial dermis- usually adequate do dx HSP
  • light microscopy studies- leukocytoclastic vasculitis in postcapillary venules with IgA deposition- pathognomonic of HSP
  • direct immunofluorescence- IgA
19
Q

HSP- lab tests to consider- 2

A
  • kidney bx- reserved for if dx is uncertain or if clinical evidence of renal involvement
  • IgA deposition in mesangium on immunofluorescence microscopy- identical to that in IgA nephropathy
  • monitor urine analysis- if protein, worry about if kidney dz will become chronic- proteinuria in HSP is an indicator of long term kidney disease, CKD
  • ANA, anti-ds DNA, ANCA, RF- neg in HSP
20
Q

HSP- imaging

A
  • in pts with significant abd sx’s
  • plain abd radiography- dilated loops of bowel (deci ntestinal motility), may see intussusception
  • abd US- detect intussusception
  • doppler flow studies and/or radionuclide scans- in boys w scrotal sx’s- distinguish from testicular torsion
21
Q

for a child- if intussusception is considered

A
  • US rather than contrast enema should be initial screening test!!!!
  • ileoileal intussusception- seen in > 1/2 cases in pts with HSP
  • contrast enema, indicated in children w signs of an intussusception, neither detect nor help reduce ileoileal intussusception
22
Q

adult vs child HSP

A
  • intussusception- children&raquo_space; Adults
  • significant renal involvement including end-stage renal dz- adults&raquo_space; children
  • adult clinical findings- palpable purpura, arthritis, GI sx’s, renal insuff (1/3) (creatinine clearance < 50), sometimes persistent renal impairment including end-stage renal dz
23
Q

palpable vs non-palpable purpura

A
  • palpable- HSP vasculitis

- non-palpable- thromboctyopenia

24
Q

OMM?

A
  • contraindicated due to vasculitis!!!
  • Upon recovery:
  • viscerosomatic reflexes for kidney (T10-L1)
  • viscerosomatic reflxes for upper and lower GI tract (T5-9, T10-L2)
  • lymphatic tx

P4T1 (7 decks)

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