Diagnostic immunology Flashcards
_________________ 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
Monoclonal gammopathies plasma cell dyscrasias
when there are clones of plasma cells what does that mean about the antibodies produced?
all antibodies will be the same!
what are the different types of Monoclonal gammopathies plasma cell dyscrasias?
multiple myeloma
primary amyloidosis
waldenstroms macrglobulinemia
Heavy chain disease
MGUS
_________ is a type of Monoclonal gammopathies plasma cell dyscrasias that has to do with IgG, IgA, IgE, IgD, or light chains only
Multiple Myeloma
_________ is a type of Monoclonal gammopathies plasma cell dyscrasias that has to do with free light chain deposition (Excess free light chain being produced)
primary amyloidosis
what is another name for primary amyloidosis?
bence jones lymphoproliferative disorders
_________ is a type of Monoclonal gammopathies plasma cell dyscrasias that has to do with only monoclonal IgM being produced
Waldenstrom’s Macroglobulinemia
________________ is a disorder in which an abnormal protein (monoclonal protein or M protein) is in the blood.
Monoclonal gammopathy of undetermined significance (MGUS)
what causes you to have an increased chance of having MGUS?
increasing age
Symtoms: bone pain in mandibular area, history of recurrent infections, x-rays show punched out bone lesions in mandible.
What is it?
multiple myeloma
What are the holes in this xray?
sheets of malignant plasma cells
___% of patients with multiple myeloma have mandibular involvement, and ___% of these patients have this mandibular involvement as their primary presentation
30%, 15%
what is used to detect the presence of a M protein
Serum protein electrophoresis (SPE)
what can a SPE test detect? how can you tell the difference between these two?
- Multiple myeloma (if Ig is IgG, IgA, IgD, IgE)
- Waldenstrom’s macroglobulinemia (if Ig is only IgM)
what does it mean if gamma on SPE is broad? what about if the gamma is a spike?
broad: consists of many antibodies with different charges (normal)
spike: only one type of antibody being made
how would these two patients SPE be different?
1. Patient with X-linked agammaglobulinemia
2. patient with an infection
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
Number of malignant cells you have to have before you pick up a positive SPE for multiple myeloma is _________
very high
-Symptoms: suspicious lesions in the mouth
You send to oral pathology and see that there are many plasma cells, what is your next step?
stain for Kappa and Lambda light chain
how could staining for Kappa and lambda light chains help give you a differential diagnosis?
-If PC cells stain ONLY for kappa or ONLY for lambda → myeloma
-If PC cells stain for BOTH kappa and lambda → granuloma
______________ may consist of free lambda or kappa light chains that have deposited in the tissue
Amyloid deposits
how can you see amyloid deposits?
Stain tissue with congo red and look at it under polarized light → you will see apple green birefringence (amyloid deposits)
-Symptoms: history of bleeding gums
-What do you think it is?
Waldenstrom’s macroglobulinemia
In __________ the M protein is IgM and is associated with hyperviscosity syndrome
Waldenstrom’s macroglobulinemia
why does Waldenstrom’s macroglobulinemia usually have hyperviscocity with it?
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
what are oral-facial manifestations of Waldenstrom’s macroglobulinemia?
-Oozing of blood from mucosa
-Nose bleeds
-Prolonged bleeding from minor procedures
-Disturbance of vision
what is the treatment for Waldenstrom’s macroglobulinemia?
plasmapheresis to dilute the plasma, IgM level decreases, but as it rises again the symptoms will return
what are some factors to consider in obtaining gingival biopsies for IF studies in evaluating patients with bullous-like diseases?
-Area of selection
-Prevent specimen from drying out
-Do NOT place in formalin for IF studies
-Transporting specimen
-Symptoms: blister like lesion in mouth, lesions on arms/legs
What type of disease is this? what is your next step?
-Bullous disease
-Differential diagnosis, it could be either Pemphigus Vulgaris or Mucous membrane pemphigoid
how can you tell the difference between Pemphigus vulgaris vs Mucous membrane pemphigoid in a scan?
-If epidermis is messed up/not intact, it is pemphigus
-If basement membrane is blown up/not intact, it is pemphigoid
Pemphigus vulgaris (benign) causes an Antibody against ______ while
mucous membrane pemphigoid causes an Antibody against ____________
epidermis, basement membrane
-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??
Mucous membrane pemphigoid
-Symptoms: painful ulcerative lesion in mouth, rash on face/arms
What test do you run now? what do you expect to find?
-Biopsy and anti-nuclear antibody (ANA) testing
-ANA will be positive in patients with Lupus
