Internal Med 9 Flashcards
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
What are the different nephritic syndrome
- PSGN
- IgA nephropathy
- Alport syndrome
- Rapidly progressive (crescentic) glomerulonephritis
What will you see in biopsy in PSGN
♣ Hypercellular glomeruli
♣ Neutrophils
♣ EM: Immune deposits – subepithelial humps
♣ IF: “starry sky” granular appearance (“lumpy bumpy”)
What lab value might indicate PSGN
♣ Decreased C3 levels (complement is activated and used up)
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
What will you see on biopsy in IgA nephropathy
♣ IgA immune complex deposited in mesangium
♣ Proliferation of mesangial cells
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”
What are the 4 causes of rapidly progressive (crescentic) glomerulonephritis
- Goodpastures
- Granulomatosis with polyangiitis (Wegener’s)
- Microscopic polyangiitis
- Diffuse proliferative glomerulonephritis
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
Describe Granulomatosis with polyangiitis
Wegener’s
• Affects kidney, lungs, and upper airway
• c-ANCA
• Negative IF
What is the most common kidney disease in lupus
Diffuse proliferative glomerulonephritis
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
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
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
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
Describe FSGS
♣ H&E – focal (affects some glomeruli), segmental (affects part of glomerulus)
♣ EM – foot process effacement
♣ IF – negative
o Poor response to steroids
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
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
Common causes of membranoproliferative glomerulonephritis
♣ Hepatitis B
♣ Hepatitis C
♣ Lupus
♣ Subacute bacterial endocarditis
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)
Describe kidney biopsy of amyloidosis
o Deposits within the mesangium
o Characterized by apple-green birefringence on Congo red staining
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)
What will you see in platelets and RBCs in acute leukemia
o Often associated with pancytopenia
♣ Blasts “crowd out” normal hematopoiesis
Presentation of acute leukemias
- Anemia = low RBC
- Infection/fever = no good WBC
- Bleeding = low platelets
How do you officially diagnose leukemias
Bone marrow biopsy
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
Risk factors associated with AML
o Chemotherapy, radiation, myelodysplastic syndrome, Down syndrome
What positive marker will you see in ALL
TdT+
What positive marker will you see in AML
MPO+
What leukemia occurs mostly in kids
ALL
Tx of ALL
Chemo with prophylaxis of CNS
Tx of CML
Responds to Imatinib (bcr-able tyrosine kinase inhibitor)
Which leukemia do you have to worry about progression
CML may progress to AML (80%) or ALL (20%)
Tx of CLL
o Young = stem cell transplant o Old (>65) and symptomatic = chemo o Old (>65) and asymptomatic = nothing
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
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
Which lymphoma has a better prognosis?
Hodgkin
Which lymphoma presents with B-symptoms
Hodgkin
What is a unique signature feature of Hodgkin lymphoma
Alcohol lymph nodes - lymph nodes become painful when pt drinks alcohol
Burkitt lymphoma is a subtype of what?
Non-Hodgkin
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
What are the ears in chemo man
C = Cisplatin and Carboplatin = ototoxicity
What is the heart in chemo man
D = doxorubicin = cardiotoxicity
What are the lungs in chemo man
B = bleomycin = pulmonary fibrosis
What are the limbs in chemo man
V = Vincristine = peripheral neuropathy
What is the bladder in chemo man
C = Cyclophosphamide = hemorrhagic cystitis
What are the bones in chemo man
M = methotrexate 5 = 5-FU 6 = 6-MP
All cause myelosuppression
What is multiple myeloma
♣ Malignant proliferation of monoclonal plasma cell within the marrow
• Recall: Plasma cells = mature B-cells that produce immunoglobulin
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
Tx of multiple myeloma
- <70 y/o with donor = stem cell transplant
* >70 or without donor = chemo
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
Describe cause of Waldenstrom macroglobulinemia
♣ B-cell lymphoma with monoclonal IgM production
• THINK: ‘W’ upside down is ‘M’
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)
