Heme and Lymph CIS Flashcards

1
Q

Define Purpura

A

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])

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2
Q

DDx for patient with Purpura and Thrombocytopenia and abnormal coag studies?

A
  • septicemia
  • ITP
  • HUS
  • Leukemia
  • Coagulopathies
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3
Q

Are purpura palpable in thrombocytopenic purpuric disorders?

A
  • no, they are not!

- but they are in HSP vasculitis

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4
Q

Ddx for purpura and normal platelet 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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5
Q

What is the Characteristic tetrad for HSP?

A
  • Palpable purpura without thrombocytopenia and coagulopathy
  • Acute arthralgia or arthritis
  • acute abdominal pain
  • renal disease
  • IgA deposition on biopsy
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6
Q

What is IgA vasculitis?

A
-immune-mediated
vasculitis that may be
triggered by a variety of
antigens, including
various infections and
immunizations
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7
Q

Is the timeline significant for HSP?

A

-yes

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8
Q

What can HSP follow?

A

-a streptococcal infection, and present with generalized arthralgias/myalgia, rash, palpable purpura (LE and butt), abdominal pain, and renal insuficiency

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9
Q

What is AHEI?

A

-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.

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10
Q

What is hypersensitivity Vasculitits

A

-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.

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11
Q

What are the 3 primary vasculitides that we need to know about?

A
  • Granulomatosis with polyangiitis (Wegner’s)
  • Microscopic polyangiitis
  • Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)
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12
Q

What are the 3 secondary vascular inflammation small vesse vasculitides that we need to know?

A
  • SLE
  • RA
  • Infectious disease (Hep B or C)
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13
Q

What ddx would we think of if someone was doing insanity workout and got an infection. Arthralgia, ab pain, renal insufficiency, CPK elevation?

A
  • Rhabdomyolysis

- the point is that they are in excellent health prior to the onset of these symptoms

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14
Q

DDx arthritis and arthralgia until a pt develops the classical purpura of HSP (IgAV)?

A
  • SLE or some kind of Autoimmune disease
  • Rheumatic fever vs. HSP
  • Septic and toxic synovitis
  • Reactive Arthritis
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15
Q

What would favor Rheumatic fever vs. HSP?

A
  • Group A beta- hemolytic strep

- erythema marginatum, nodules and carditis= rheumatic fever

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16
Q

What would lead us to think that it is HSP instead of Rheumatic fever?

A

-hx of strep infection and clinical course of HSP tetrad

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17
Q

What do we have to know about septic and toxic synovitis?

A

-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).

18
Q

Reactive arthritis, what do we have to know about it?

A

-may be triggered bya variety of genitourinary and GI pathogens including beta-hemolytic strep infections

19
Q

Does the rash in HSP come before or after the GI manifestations?

A

-usually preces GI manifestions and seldom lags by more than a few days

20
Q

What usually happens with HSP patients?

A
  • they recover spontaneously

- thus, care is primarily supportive

21
Q

What do we do to manage HSP patients?

A
  • 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
22
Q

What are we looking for if we do an acute abdominal series or U/S on an HSP patient?

A

-Intussusception

23
Q

What are some Lab Tests we have to consider in HSP patient?

A
  • 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
24
Q

What will we see on light microscopy in skin biopsy of HSP patient?

A
  • leukocytoclastic vasculitis in postcapillary venules with IgA deposition
  • pathognomonic of HSP
25
Q

How is HSP characterized on kidney biopsy?

A

-IgA deposition in the mesangium on immunofuorescence microscopy that is identical to that in IgA nephropathy

26
Q

In general, is there a lab test that is diagnostic for HSP?

A

-no, but we can use them to rule other things out

27
Q

Are ANA, Anti-ds DNS, aANCA, and RF there in HSP?

A

-no, they’re all negative

28
Q

What is Proteinuria in HSP an indicator of?

A

-Long term kidney disease, CKD

29
Q

What imaging should we consider in an HSP patient?

A
  • Plain abdominal radiography: look for dilated loops of bowel consistent with decreased intstinal motility
  • Ab U/S: look for intussusception
30
Q

If we have a child, what imaging modality is best?

A

-Ultrasound rather than contrast enema should be the initial screening test

31
Q

What kind of intussusception is seen in more than 1/2 of the cases of intussusception in pt’s with HSP?

A

-Ileoileal intussusception

32
Q

What will doppler flow studies or radionuclie scans help us distinguish?

A
  • in a boy who presents with scrotal symptoms

- tells us if it’s from HSP or from testicular torsion

33
Q

Differences between Adult and Child HSP?

A
  • Intussusception is more common in children

- Adults get more significant renal involvement including end-stage-renal disease

34
Q

What are the Adult clinical findings for HSP?

A

-palpable purpura, arthritis, GI sypmtoms, renal insufficiency (decreasing frequency)

35
Q

Palpable vs. Non palpable purpura

A
  • Palpable is HSP vasculitis

- Non palpable is thrombocytopenia

36
Q

Is OMM indicated in the acute setting of HSP?

A

-no, we don’t want to encourage more purpura

37
Q

What are the viscerosomatic reflexes for the kidney?

A
  • (T10-L1)

- check them upon recovery

38
Q

Why would we do lymphatic tx on these patients?

A

-help normalize fluid balance and decrease risk of kidney failur

39
Q

What does the Right lymphatic duct drain?

A

-Right head and neck, right UE, all lung lobes except upper left

40
Q

What does the thoracic duct drain?

A

-everything not drained by the right lymphatic duct

41
Q

What is the sequence of the Lymphatic drainage?

A

-Thoracic inlet, Thoracic Area, Abdominal Area, UE or LE depending on which is more dysfunctional, UE or LE, Head and neck, Thoracic inlet