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