Diagnostic immunology

Question 1

_________________ is a group of disorders in which there is an abnormal proliferation of a clone of Ig secreting cells (usually plasma cells) resulting in the production of a monoclonal (M) antibody

Answer 1

Monoclonal gammopathies plasma cell dyscrasias

Question 2

when there are clones of plasma cells what does that mean about the antibodies produced?

Answer 2

all antibodies will be the same!

Question 3

what are the different types of Monoclonal gammopathies plasma cell dyscrasias?

Answer 3

multiple myeloma
primary amyloidosis
waldenstroms macrglobulinemia
Heavy chain disease
MGUS

Question 4

_________ is a type of Monoclonal gammopathies plasma cell dyscrasias that has to do with IgG, IgA, IgE, IgD, or light chains only

Answer 4

Multiple Myeloma

Question 5

_________ is a type of Monoclonal gammopathies plasma cell dyscrasias that has to do with free light chain deposition (Excess free light chain being produced)

Answer 5

primary amyloidosis

Question 6

what is another name for primary amyloidosis?

Answer 6

bence jones lymphoproliferative disorders

Question 7

_________ is a type of Monoclonal gammopathies plasma cell dyscrasias that has to do with only monoclonal IgM being produced

Answer 7

Waldenstrom’s Macroglobulinemia

Question 8

________________ is a disorder in which an abnormal protein (monoclonal protein or M protein) is in the blood.

Answer 8

Monoclonal gammopathy of undetermined significance (MGUS)

Question 9

what causes you to have an increased chance of having MGUS?

Answer 9

increasing age

Question 10

Symtoms: bone pain in mandibular area, history of recurrent infections, x-rays show punched out bone lesions in mandible.
What is it?

Answer 10

multiple myeloma

Question 11

What are the holes in this xray?

Answer 11

sheets of malignant plasma cells

Question 12

___% of patients with multiple myeloma have mandibular involvement, and ___% of these patients have this mandibular involvement as their primary presentation

Answer 12

30%, 15%

Question 13

what is used to detect the presence of a M protein

Answer 13

Serum protein electrophoresis (SPE)

Question 14

what can a SPE test detect? how can you tell the difference between these two?

Answer 14

1. Multiple myeloma (if Ig is IgG, IgA, IgD, IgE)
2. Waldenstrom’s macroglobulinemia (if Ig is only IgM)

Question 15

what does it mean if gamma on SPE is broad? what about if the gamma is a spike?

Answer 15

broad: consists of many antibodies with different charges (normal)
spike: only one type of antibody being made

Question 16

how would these two patients SPE be different?
1. Patient with X-linked agammaglobulinemia
2. patient with an infection

Answer 16

1.gamma globulin region would be at baseline since they aren’t making any
2. would have a polyclonal response that is just much greater than in a healthy person

Question 17

Number of malignant cells you have to have before you pick up a positive SPE for multiple myeloma is _________

Answer 17

very high

Question 18

-Symptoms: suspicious lesions in the mouth
You send to oral pathology and see that there are many plasma cells, what is your next step?

Answer 18

stain for Kappa and Lambda light chain

Question 19

how could staining for Kappa and lambda light chains help give you a differential diagnosis?

Answer 19

-If PC cells stain ONLY for kappa or ONLY for lambda → myeloma
-If PC cells stain for BOTH kappa and lambda → granuloma

Question 20

______________ may consist of free lambda or kappa light chains that have deposited in the tissue

Answer 20

Amyloid deposits

Question 21

how can you see amyloid deposits?

Answer 21

Stain tissue with congo red and look at it under polarized light → you will see apple green birefringence (amyloid deposits)

Question 22

-Symptoms: history of bleeding gums
-What do you think it is?

Answer 22

Waldenstrom’s macroglobulinemia

Question 23

In __________ the M protein is IgM and is associated with hyperviscosity syndrome

Answer 23

Waldenstrom’s macroglobulinemia

Question 24

why does Waldenstrom’s macroglobulinemia usually have hyperviscocity with it?

Answer 24

IgM is a large antibody and it doesn’t readily leave the vascular system, an increase IgM in blood causes blood to be highly viscous

Question 25

what are oral-facial manifestations of Waldenstrom’s macroglobulinemia?

Answer 25

-Oozing of blood from mucosa
-Nose bleeds
-Prolonged bleeding from minor procedures
-Disturbance of vision

Question 26

what is the treatment for Waldenstrom’s macroglobulinemia?

Answer 26

plasmapheresis to dilute the plasma, IgM level decreases, but as it rises again the symptoms will return

Question 27

what are some factors to consider in obtaining gingival biopsies for IF studies in evaluating patients with bullous-like diseases?

Answer 27

-Area of selection
-Prevent specimen from drying out
-Do NOT place in formalin for IF studies
-Transporting specimen

Question 28

-Symptoms: blister like lesion in mouth, lesions on arms/legs
What type of disease is this? what is your next step?

Answer 28

-Bullous disease
-Differential diagnosis, it could be either Pemphigus Vulgaris or Mucous membrane pemphigoid

Question 29

how can you tell the difference between Pemphigus vulgaris vs Mucous membrane pemphigoid in a scan?

Answer 29

-If epidermis is messed up/not intact, it is pemphigus
-If basement membrane is blown up/not intact, it is pemphigoid

Question 30

Pemphigus vulgaris (benign) causes an Antibody against ______ while
mucous membrane pemphigoid causes an Antibody against ____________

Answer 30

epidermis, basement membrane

Question 31

-Symptoms: similar blister like lesions to patient 3, but lesions present in mouth and NOT skin, lesion of conjunctiva
-Biopsy shows that epidermis is intact but the dermis is separated

what do you think it is??

Answer 31

Mucous membrane pemphigoid

Question 32

-Symptoms: painful ulcerative lesion in mouth, rash on face/arms
What test do you run now? what do you expect to find?

Answer 32

-Biopsy and anti-nuclear antibody (ANA) testing
-ANA will be positive in patients with Lupus