Heme and Lymph CIS

Question 1

purpura

Answer 1

• 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)

Question 2

Purpura + Thrombocytopenia and abnormal coag studies-diff dx

Answer 2

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

Question 3

Purpura + normal plt count and coag studies

Answer 3

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

Question 4

Henoch-Schoenlein Purpura

Answer 4

• more straightfwd in adults than in children

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

Question 5

Henoch-Schoenlein Purpura- tetrad

Answer 5

• 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

Question 6

IgA vasculitis (IgA nephropathy)

Answer 6

• 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

Question 7

Acute hemorrhagic edema of infancy

Answer 7

• 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

Question 8

Hypersensitivity vasculitis

Answer 8

• 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

Question 9

Other small vessel vasculitides- primary vasculitides

Answer 9

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

Question 10

Other small vessel vasculitides- secondary vascular infl

Answer 10

• SLE
• RA
• infectious dz (hep B or C)

Question 11

Rhabdomyolysis

Answer 11

• 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

Question 12

DDX arthritis and arthalgia

Answer 12

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

Question 13

Rheumatic fever vs HSP

Answer 13

• 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

Question 14

Septic and Toxic synovitis (transient synovitis)

Answer 14

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

Question 15

reactive arthritis

Answer 15

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

Question 16

DDX abd pain

Answer 16

• 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)

Question 17

HSP- plan to manage pts

Answer 17

• 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!!!

Question 18

HSP- lab tests to consider

Answer 18

• 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

Question 19

HSP- lab tests to consider- 2

Answer 19

• 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

Question 20

HSP- imaging

Answer 20

• 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

Question 21

for a child- if intussusception is considered

Answer 21

• 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

Question 22

adult vs child HSP

Answer 22

• 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

Question 23

palpable vs non-palpable purpura

Answer 23

• palpable- HSP vasculitis

- non-palpable- thromboctyopenia

Question 24

OMM?

Answer 24

• 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