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Flashcards in Internal Med 9 Deck (53):
1

What are the general principles of nephritic syndrome

• Proteinuria < 3.5 g/day
• Hematuria and RBC casts
• Azotemia – increased serum BUN and Creatine
• Hypertension – salt retention with periorbital edema
• Oliguria

2

What are the different nephritic syndrome

- PSGN
- IgA nephropathy
- Alport syndrome
- Rapidly progressive (crescentic) glomerulonephritis

3

What will you see in biopsy in PSGN

♣ Hypercellular glomeruli
♣ Neutrophils
♣ EM: Immune deposits – subepithelial humps
♣ IF: “starry sky” granular appearance (“lumpy bumpy”)

4

What lab value might indicate PSGN

♣ Decreased C3 levels (complement is activated and used up)

5

Describe Henoch Schonlein purpura and what renal disease it is associated with

♣ Palpable purpura on butt and legs
♣ Arthralgias
♣ Abdominal pain
♣ Renal disease - IgA nephropathy

6

What will you see on biopsy in IgA nephropathy

♣ IgA immune complex deposited in mesangium
♣ Proliferation of mesangial cells

7

Describe Alport syndrome

o Due to defect in Type IV collagen (basement membrane)
- Leads to splitting of the basement membrane longitudinally

o Presents as isolated hematuria, sensory hearing loss, and ocular disturbances
♣ “Can’t see, can’t pee, can’t hear high C”

8

What are the 4 causes of rapidly progressive (crescentic) glomerulonephritis

- Goodpastures
- Granulomatosis with polyangiitis (Wegener's)
- Microscopic polyangiitis
- Diffuse proliferative glomerulonephritis

9

Describe Goodpasture's syndrome

• Type II HSR – antibodies against glomerular basement membrane
o Deposits comprised of IgG and C3
• Linear IF pattern
• Affects kidneys and the lungs (alveolar basement membrane)
• Presents as hematuria + hemoptysis

10

Describe Granulomatosis with polyangiitis

Wegener's
• Affects kidney, lungs, and upper airway
• c-ANCA
• Negative IF

11

What is the most common kidney disease in lupus

Diffuse proliferative glomerulonephritis

12

Describe DPGN

• Anti-dsDNA seen subendothelially and within mesangium
• Granular IF
• Light microscopy – deposits make basement membrane look thick – “wire looping” of capillaries
o THINK: wire lupus
• Often presents as nephrotic + nephritic syndrome

13

Describe general principles of nephrotic syndrome

• Proteinuria > 3.5 g/day
• Hypoalbuminemia – protein lost in urine
• Edema – decreased oncotic pressure due to hypoalbuminemia
• Increased risk of infection – loss of immunoglobulin in urine
• Increased risk of thrombosis – loss of antithrombin III
• Hyperlipidemia – liver tries to make more lipoproteins in order to make up for decreased oncotic pressure

14

What are the subtypes of nephrotic syndrome

Foot process effacement
- Minimal change disease
- focal segmental glomerulosclerosis

Thickening of basement membrane:
- membranous nephropathy
- membranoproliferative glomerulonephritis

Other
- Diabetic nephropathy
- Amyloidosis

15

Describe minimal change disease

♣ H&E – normal
♣ EM – foot process effacement
♣ IF – negative

o Most common cause of nephrotic syndrome in children
o Triggered by infections or immunizations
o Treatment – steroids

16

Describe FSGS

♣ H&E – focal (affects some glomeruli), segmental (affects part of glomerulus)
♣ EM – foot process effacement
♣ IF – negative
o Poor response to steroids

17

Describe membranous nephropathy

o This is the nephrotic presentation of SLE
o Biopsy:
♣ H&E – thickening of basement membrane
♣ EM – “spike and dome” appearance with subepithelial deposits (IgG and C3)
♣ IF – granular

18

Describe membranoproliferative glomerulonephritis

• H&E – thick basement membrane with “tram track” appearance
o Due to mesangium proliferating between the basement membrane
o THINK: membranoproliferative = membrane proliferating = 2 membranes
• EM – subendothelial deposits
• IF – granular

19

Common causes of membranoproliferative glomerulonephritis

♣ Hepatitis B
♣ Hepatitis C
♣ Lupus
♣ Subacute bacterial endocarditis

20

Biopsy of diabetic nephropathy

o Non-enzymatic glycosylation of basement membrane
o Will show sclerosing of the mesangium with Kimmelstien-Wilson nodules (round acellular nodules)

21

Describe kidney biopsy of amyloidosis

o Deposits within the mesangium
o Characterized by apple-green birefringence on Congo red staining

22

What chromosome is involved in CML

o t(9;22) – Philedalphia chromosome
♣ THINK: Carry Mai, Lord, because she is able
• Mai’s birthday is 9/22
• BCR-ABL (because she is able)

23

What will you see in platelets and RBCs in acute leukemia

o Often associated with pancytopenia
♣ Blasts “crowd out” normal hematopoiesis

24

Presentation of acute leukemias

- Anemia = low RBC
- Infection/fever = no good WBC
- Bleeding = low platelets

25

How do you officially diagnose leukemias

Bone marrow biopsy

26

Describe characteristics of AML

When you think AML, think "All my life" = musicals
o Sound of music = Auer sounds like Fuer (Hitler) – for auer rods
o I am 16 going on 17 (15;17)
o Poke Hitler in the eye with a carrot – treat with retinoic acid

27

Risk factors associated with AML

o Chemotherapy, radiation, myelodysplastic syndrome, Down syndrome

28

What positive marker will you see in ALL

TdT+

29

What positive marker will you see in AML

MPO+

30

What leukemia occurs mostly in kids

ALL

31

Tx of ALL

Chemo with prophylaxis of CNS

32

Tx of CML

Responds to Imatinib (bcr-able tyrosine kinase inhibitor)

33

Which leukemia do you have to worry about progression

CML may progress to AML (80%) or ALL (20%)

34

Tx of CLL

o Young = stem cell transplant
o Old (>65) and symptomatic = chemo
o Old (>65) and asymptomatic = nothing

35

Next step in management of non-tender lymphadenopathy

- This most likely means cancer! Either lymphoma or met
- Inflammatory lymph node would be usually be painful

So you you do an excisional biopsy!!

Do not choose FNA

36

What will you see on biopsy of Hodgkin lymphoma

Reed-Sternberg cells
♣ Large cells with multiple/bi-lobed nuclei with clearing around the nuclei (“owl-eye”)
♣ RS cells are CD15+ and CD30+
• THINK: 2 owl eyes x 15 = 30

37

Which lymphoma has a better prognosis?

Hodgkin

38

Which lymphoma presents with B-symptoms

Hodgkin

39

What is a unique signature feature of Hodgkin lymphoma

Alcohol lymph nodes - lymph nodes become painful when pt drinks alcohol

40

Burkitt lymphoma is a subtype of what?

Non-Hodgkin

41

What chromosomes are involved in Burkitt

o t(8;14) c-myc (8) and heavy chain Ig (14)
♣ THINK: “B” in Burkitt = 8 and “tt” = 14

42

What are the ears in chemo man

C = Cisplatin and Carboplatin = ototoxicity

43

What is the heart in chemo man

D = doxorubicin = cardiotoxicity

44

What are the lungs in chemo man

B = bleomycin = pulmonary fibrosis

45

What are the limbs in chemo man

V = Vincristine = peripheral neuropathy

46

What is the bladder in chemo man

C = Cyclophosphamide = hemorrhagic cystitis

47

What are the bones in chemo man

M = methotrexate
5 = 5-FU
6 = 6-MP

All cause myelosuppression

48

What is multiple myeloma

♣ Malignant proliferation of monoclonal plasma cell within the marrow
• Recall: Plasma cells = mature B-cells that produce immunoglobulin

49

Describe clinical presentation of multiple myeloma

• THINK: CRAB
o C = hyperCalcemia
o R = renal failure
o A = anemia
o B = bone pain

• Anemia – plasma cells packed in bone marrow inhibit production of other cells
• Renal insufficiency – excessive antibodies plug up kidney and form casts
• Back pain – plasma cells stimulate osteoclasts
• Hypercalcemia – plasma cells stimulate osteoclasts

50

Tx of multiple myeloma

• <70 y/o with donor = stem cell transplant
• >70 or without donor = chemo

51

What is monoclonal gammopathy of undetermined significance (MGUS)

♣ Monocloncal proliferation of plasma cells
♣ Production of monoclonal immunoglobulins = M spike
♣ No symptoms
♣ 1-2% progress to multiple myeloma

52

Describe cause of Waldenstrom macroglobulinemia

♣ B-cell lymphoma with monoclonal IgM production
• THINK: ‘W’ upside down is ‘M’

53

Describe presentation of Waldenstrom

• M spike due to increased IgM
• Hyperviscosity:
o Blurred vision
o Raynoud phenomenon
• Amyloidosis
♣ No lytic bone lesions (vs. multiple myeloma)