Heme and Lymph CIS Flashcards
Define Purpura
a. Non-blanchable, hemorrhagic skin lesions that result from the leakage of red blood cells into the skin
b. Can be palpable or non-palpable
c. Macular
i. (non-palpable/non-inflammatory are further divided into petechiae [<3mm] and
ecchymosis[>5mm])
DDx for patient with Purpura and Thrombocytopenia and abnormal coag studies?
- septicemia
- ITP
- HUS
- Leukemia
- Coagulopathies
Are purpura palpable in thrombocytopenic purpuric disorders?
- no, they are not!
- but they are in HSP vasculitis
Ddx for purpura and normal platelet count and coag studies
- Henoch-Schoenlein purpura (IgA Vasculitis)
- Acute hemorrhagic edema of infancy (AHEI)
- Hypersensitivity vasculitis
- Other small vessel vasculitides
What is the Characteristic tetrad for HSP?
- Palpable purpura without thrombocytopenia and coagulopathy
- Acute arthralgia or arthritis
- acute abdominal pain
- renal disease
- IgA deposition on biopsy
What is IgA vasculitis?
-immune-mediated vasculitis that may be triggered by a variety of antigens, including various infections and immunizations
Is the timeline significant for HSP?
-yes
What can HSP follow?
-a streptococcal infection, and present with generalized arthralgias/myalgia, rash, palpable purpura (LE and butt), abdominal pain, and renal insuficiency
What is AHEI?
-leukocytoclastic vasculitis
-children between the ages of four months to two years
-self-limited disease, resolves in one to three weeks
-presents with fever, purpura, ecchymosis, and inflammatory edema of the limbs
-Involvement of the kidney and the gastrointestinal tract is uncommon
-Biopsy of the skin demonstrates a leukocytoclastic vasculitis with occasional
immunoglobulin A (IgA) deposition.
What is hypersensitivity Vasculitits
-inflammation of the small vessels that occurs after exposure to:
-drugs, infection, or without an identifiable trigger
-present with fever, urticaria, lymphadenopathy, and arthralgias,
• not usually glomerulonephritis
-Histopathology shows a leukocytoclastic vasculitis primarily of the postcapillary venules, but
IgA deposition is absent.
What are the 3 primary vasculitides that we need to know about?
- Granulomatosis with polyangiitis (Wegner’s)
- Microscopic polyangiitis
- Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)
What are the 3 secondary vascular inflammation small vesse vasculitides that we need to know?
- SLE
- RA
- Infectious disease (Hep B or C)
What ddx would we think of if someone was doing insanity workout and got an infection. Arthralgia, ab pain, renal insufficiency, CPK elevation?
- Rhabdomyolysis
- the point is that they are in excellent health prior to the onset of these symptoms
DDx arthritis and arthralgia until a pt develops the classical purpura of HSP (IgAV)?
- SLE or some kind of Autoimmune disease
- Rheumatic fever vs. HSP
- Septic and toxic synovitis
- Reactive Arthritis
What would favor Rheumatic fever vs. HSP?
- Group A beta- hemolytic strep
- erythema marginatum, nodules and carditis= rheumatic fever
What would lead us to think that it is HSP instead of Rheumatic fever?
-hx of strep infection and clinical course of HSP tetrad
What do we have to know about septic and toxic synovitis?
-May present with joint symptoms similar to those seen in patients with HSP (IgAV).
-These typically involve only one or two joints, unlike the polyarthritis seen in HSP (IgAV).
-Additionally, affected joints are warm and erythematous in septic arthritis, unlike those in
HSP (IgAV).
Reactive arthritis, what do we have to know about it?
-may be triggered bya variety of genitourinary and GI pathogens including beta-hemolytic strep infections
Does the rash in HSP come before or after the GI manifestations?
-usually preces GI manifestions and seldom lags by more than a few days
What usually happens with HSP patients?
- they recover spontaneously
- thus, care is primarily supportive
What do we do to manage HSP patients?
- assess renal status!
- Corticosteroids only if we absolutely need them
- IV hydration, rehydrate and monitor
- Pain management (NSAIDS)
- Anti-emetics
- Maybe PPI becuase NSAIDS give us acute gastritis
What are we looking for if we do an acute abdominal series or U/S on an HSP patient?
-Intussusception
What are some Lab Tests we have to consider in HSP patient?
- Serum IgA levels
- Routine blood tests: check CBC, CMP, Urinalysis
- Hypocomoplementemia… check complement levels (normal in HSP), can detect recent step infections
- Skin biopsy
- Kidney biopsy: only if dx is uncertain
What will we see on light microscopy in skin biopsy of HSP patient?
- leukocytoclastic vasculitis in postcapillary venules with IgA deposition
- pathognomonic of HSP
How is HSP characterized on kidney biopsy?
-IgA deposition in the mesangium on immunofuorescence microscopy that is identical to that in IgA nephropathy
In general, is there a lab test that is diagnostic for HSP?
-no, but we can use them to rule other things out
Are ANA, Anti-ds DNS, aANCA, and RF there in HSP?
-no, they’re all negative
What is Proteinuria in HSP an indicator of?
-Long term kidney disease, CKD
What imaging should we consider in an HSP patient?
- Plain abdominal radiography: look for dilated loops of bowel consistent with decreased intstinal motility
- Ab U/S: look for intussusception
If we have a child, what imaging modality is best?
-Ultrasound rather than contrast enema should be the initial screening test
What kind of intussusception is seen in more than 1/2 of the cases of intussusception in pt’s with HSP?
-Ileoileal intussusception
What will doppler flow studies or radionuclie scans help us distinguish?
- in a boy who presents with scrotal symptoms
- tells us if it’s from HSP or from testicular torsion
Differences between Adult and Child HSP?
- Intussusception is more common in children
- Adults get more significant renal involvement including end-stage-renal disease
What are the Adult clinical findings for HSP?
-palpable purpura, arthritis, GI sypmtoms, renal insufficiency (decreasing frequency)
Palpable vs. Non palpable purpura
- Palpable is HSP vasculitis
- Non palpable is thrombocytopenia
Is OMM indicated in the acute setting of HSP?
-no, we don’t want to encourage more purpura
What are the viscerosomatic reflexes for the kidney?
- (T10-L1)
- check them upon recovery
Why would we do lymphatic tx on these patients?
-help normalize fluid balance and decrease risk of kidney failur
What does the Right lymphatic duct drain?
-Right head and neck, right UE, all lung lobes except upper left
What does the thoracic duct drain?
-everything not drained by the right lymphatic duct
What is the sequence of the Lymphatic drainage?
-Thoracic inlet, Thoracic Area, Abdominal Area, UE or LE depending on which is more dysfunctional, UE or LE, Head and neck, Thoracic inlet
