Proteins

Question 1

Most important signal for Acute Phase Reactants

Answer 1

Interleukin 6

Question 2

Positive Acute Phase reactants

Answer 2

C3, CRP
A1AT, A2M
Ceruloplasmin
Fibrinogen, VII, vWF
Haptoglobin

Question 3

Negative Acute Phase reactants

Answer 3

Albumin
Transthyretin (thyroxine-binding prealbumin)
Retinol binding protein
Trasferrin
Antithrombin

Question 4

Serum Electropheris Pattern

Answer 4

Albumin, Alpha-1, Alpha-2, Beta, (Beta 2), Gamma

specimen deposited close to beta, gamma interface

proteins negatively charged so move to positively charged anode

gamma globulins move the other direction because of little charge they move with buffer moving towards cathode

Question 5

SPE Pattern: Prealbumin

Answer 5

• Retinol-binding protein - Vit. A transport
• Transthyretin - T4 transport (measure of short-term nutrition)
• decrease:
  
  • malnutrition
  • nephoris
• normally only in CSF

Question 6

SPE Pattern: Albumin

Answer 6

• Function
  
  • Oncotic pressure
  • Transport
  • Amino acid reserve
• Bisalbuminemia (rare albumin variant - no significance)
• Decreased:
  
  • malnutrition, liver dz, inflammation
  • increase catabolism
    
    • t4, Pg, steroids
  • loss
    
    • nephrosis, PLE, burn
  • fluid retention (SIADH)
• Increased
  
  • dehydation
  • diabetes insipidus
  • prolonged tourniquet time

Question 7

SPE Pattern: Alpha 1

Answer 7

• A1AT - protease inhibitor
  
  • SERPIN1
    
    • MM:normal
    • MZ: +/i asymptomatic
    • ZZ: clinical dz.
  • liver dz: caused by failure to export A1AT causes internal damage
  • pulmonary dz: Faulure to elatase breakdown
  • • HDL - reverse chol. transport
• TBG - T4, T3 transport
• CBG (transcortin). - Cortisol transport
• Orosomucoprotein - Immune resp. modifier
• Prothrombin (plasma only)
• Decreased:
  
  • HDL def.
  • A1At def
• Increased
  
  • Acute inflammation (+ A1AT)

Question 8

SPE Pattern: Alpha 2

Answer 8

• Alpha-2 macroglobulin - protease inhibitor
  
  • acute phase reactant
  • increase in nephrosis (too big to get out)
• haptoglobin - Hb binding
  
  • acute phase reactant
  • some congenital def in some AA
  • protects against iron loss
• Ceruloplasmin - oxidation reduction
  
  • acute phase reactant
  • decreased in Wilson dz., nutrition, malabsorption, nephorosis, liver dz.
    *

Question 9

SPE Pattern: Beta

Answer 9

• Beta 1
  
  • Transferrin - Iron transport
    
    • up in iron deficient anemia
    • down in anemia of chronic dz.
    • Hemopexin - Heme binding
• Beta 2
  
  • LDL - Chol Transport
  • C3 - complement cascade
• Band increased:
  
  • Liver disease
  • hyperlipidemia

Question 10

SPE Pattern: Beta-Gamma Region

Answer 10

• IgA - Mucosal Immunity
• Fibrinogen
  
  • only see in plasma

Question 11

SPE Pattern: Gamma

Answer 11

• IgM - Early primary adaptive humoral response
• IgG - Later primary and secondary response
  
  • goes in interstitial, and can cross placental
• CRP - innate immunity, initiates classical complement pathway

Question 12

How to prove “band of restricted mobility” is M-spike?

Answer 12

Immunofixation (IFE)

Newer: Immunotyping via capillary electrophoresis (CE)

Older: Immunoelectrophoresis (IEP)

Question 13

MGUS

Answer 13

• Asymptomatic
• <3 g/dl
• % BM plasma cells < 10%
• No punched out lesions
• 1% per year progress to Multiple Myeloma
• No Tx at this time.

Question 14

Waldemstrom macro-globulinemia

Answer 14

IgM monoclonal gammopathy (pentamer)

Lymphoma-like

No bone lesions

hyperviscosity more so than myeloma

Question 15

Neoplastic monoclonal gammopathies

Answer 15

• Multiple myeloma (BM)
• Plasmacytoma (mass)
• Monoclonal immunoglobulin deposition dz.
• Osteosclerotic myeloma
• AL amyloidosis

Question 16

Differentiating MGUS, Smoldering myeloma, and MM

Answer 16

• MGUS
  
  • < 3g/dl (and)
  • < 10% BM plasma cells
  • no symptoms
• Smoldering myeloma
  
  • ≥ 3 g/dl (or)
  • > 10% BM plasma cells
  • no symptoms
• MM
  
  • usually present
  • usually ≥ 10% BM plasma cells
  • yes symptoms OR ≥1 ( MRI lesion, abnormal LC ratio, > 60% BM plasma cells)

Question 17

Differential Diagnosis of Hypogammaglobulinemia

Answer 17

• Primary
  
  • XLA (Brutons agammaglobulinemia)
  • CVID (Common variable immunodeficiency)
  • IgA def. (w/IgG subclass)
  • Hyper-IgM syndrome (CD40L, CD40, NEMO, AID, UNG) no class switching
• Secondary
  
  • Loss (nephrosis, burn)
  • immunosuppression
  • chemotherapy
  • irradiation
  • immunosuppression
  • light chain disease