CIS-Heme/Lymph/Oncology

Question 1

non-blanchable, hemorrhagic skin lesions that result from leakage of RBCs into skin, can be palpable or non-palpable

Answer 1

purpura

Question 2

characterize purpura in thrombocytopenic purpuric disorders

Answer 2

non-palpable

Question 3

which demographic is more likely to experience intussusception in HSP? what about renal involvement, including end-stage renal disease?

Answer 3

Intussusception-children, much less common in adults

renal disease-Adults&raquo_space;children

Question 4

Characteristic tetrad for HSP?

Answer 4

MANDATORY: Palpable purpura w/o thrombocytopenia and coagulopathy
PLUS, one or more of the following:
-acute arthralgia and/or arthritis
-acute abdominal pain
-renal disease
-IgA deposition on biopsy

Question 5

considerations for dx of HSP?

Answer 5

• may follow strep infx (sore throat)
• present with generalized arthralgias/myalgias, rash, palpable purport (esp on LE and buttocks), abdominal pain, and renal insufficiency

Question 6

HSP is what type of Ig-mediated vascultis?

Answer 6

IgA

Question 7

what else to consider in DDx for arthritis and arthalgia in this case?

Answer 7

• Autoimmune: SLE, Juvenile idiopathic arthritis
• Rheumatic fever –> evidence of recent group A beta-hemolytic strep infx, consider the JONES criteria
• Septic and toxic synovitis (transient synovitis) –> only involves 1 or 2 joints, HSP is a polyarthritis
• Reactive arthritis

Question 8

a significant proportion of cases of HSP are triggered by what infx?

Answer 8

streptococcal

Question 9

how do you manage pts presenting with HSP?

Answer 9

• vast majority recover spontaneously and care is primarily supportive
• Assess renal status, overall fluid/electrolytes. Steroids can be used but only when absolutely needed
• pain mgm –> NSAIDS, dep on renal status
• anti-emetics
• PPI to minimize gastritis/PUD from NSAID or steroids
• Acute abdominal series or US to screen for intussuception

Question 10

in management of HSP, what should you do empirically until labs return?

Answer 10

-**IV hydration –> IV fluid rate and saline/D5/LR is empiric until labs return. goal is to initiate rehydration and monitor

Question 11

this lab is elevated in 50-70% of HSP pts and higher levels associated with renal involvement

Answer 11

Serum IgA levels

Question 12

When doing routine blood tests such as CBC, CMP, and UA in HSP pts, what lab components are necessary to confirm when clinical features do not allow conclusive distinction of HSP (IgAV) from other diseases that present with purport d/t thrombocytopenia or coagulopathy?

Answer 12

normal platelet count and coag studies (PT time)

Question 13

levels of complement in HSP?

Answer 13

typically normal

Question 14

what does light microscopy from a skin biopsy sample show of the BVs of superficial dermis in an HSP pt?

Answer 14

leukocytoclastic vasculitis in postcapillary venues with IgA deposition that is PATHOGNOMONIC OF HSP

Question 15

what does IF microscopy show from a kidney biopsy of a HSP pt?

Answer 15

IgA deposition in mesangium that is identical to that in IgA nephropathy

Question 16

__ is an indicator of long-term kidney disease (CKD) in HSP

Answer 16

Proteinuria

Question 17

for a child w/ HSP who you suspect intussusception, what imaging should you consider?

Answer 17

US rather than contrast enemas should be initial screening test

Question 18

location/type of intussusception in more than 1/2 cases in pts with HSP?

Answer 18

ileoileal intussusception

Question 19

imaging modality to distinguish scrotal pain caused by HSP from testicular torsion?

Answer 19

doppler flow studies and/or radionuclide scans

Question 20

OMM in acute setting?

Answer 20

contraindicated d/t vasculitis

Question 21

OMM once pt recovers?

Answer 21

• viscerosomatics of kidney (T10-L1)
• viscerosomatics for upper and lower GI (upper GI=T5-9, lower=T10-L2
• Lymphatics