Myeloma and Lymphoma Flashcards
What is B cell differentiation?
B cells converting from immature to mature B cells
Where does the first stage of B cell differentiation occur and what happens?
Bone marrow
pro B cells to naive B cells
gain immoglobulins
Where does the second stage of B cell differentiation occur and what happens?
secondary lymphoid organs
Antigen dependent stage
B cells get antigens presented to them to develop surface immunoglobulins
plasma cells are made
What marker is unique to plasma cells?
CD38 - useful for diagnosing myeloma
What is the Plasma Cell Differentiation Pathway?
final stage in producing plasma cells
details not important - there just to show that if there are errors in any stage = increased proliferation = myeloma
What are the light chains in immunoglobulins?
kappa
lambda
What is protein electrophoresis?
laboratory technique whereby serum is placed in a gel and exposed to an electric current
used to diagnose myeloma
What in protein electrophoresis is important to diagnosing myeloma?
gamma globulin
m spike seen - neoplastic cells only producing one type of immunoglobulin
What is immunofixation?
next step after electrophoresis
(Anti-immunoglobulin antisera) gel added with antibodies to those globulins to identify what is there
positive result = complex precipitates
What is myeloma a malignancy of?
plasma cells
What is the common age and ethnic origin that suffer with myeloma?
70 years
Afro-Caribbean
What can present in patients before they myeloma or can help to predict the rate of the disease?
asymptomatic monoclonal gammopathy of undetermined significance (MGUS) - can be normal or high risk
amyloidosis
POEMS syndrome - combination of a plasma-cell proliferative disorder
Solitary plasmacytoma (with minimal bone marrow involvement) - discrete, solitary mass of neoplastic monoclonal plasma cells in either bone or soft tissue
myeloma with adverse cellular features
Apart from seeing more than 10% of neoplastics plasma cells and/or extramedullary plasmacytomas, what else should the patient present with in order to diagnose a patient with myeloma?
CRAB features
MDEs - Myeloma defining events
What are the CRAB features?
calcium elevation
renal insufficiency
anaemia
bone lesions
What are myeloma defining events?
> 60% neoplatic plasma cells on biopsy
Serum Free Light Chains ratio >100mg/L- kappa to lambda chain ratio abnormal
bone focal lesion on MRI measuring >5mm
If a patient has myeloma defining events but no symptoms, what is this known as?
“asymptomatic / smouldering” myeloma cases
How do kidney problems arise in myeloma?
light chains clog up the glomerulus
What is the management of acute kidney injury with suspected myeloma?
MEDICAL EMERGANCY
STEROIDS
What other diseases patients with MGUS progress to have?
Waldenstrom’s macroglobulinaemia
Primary Amyloaid light chain amyloidosis
Lymphoproliferative disorders
What is used to determine the risk of a patient with MGUS progressing to myeloma?
low para-protein
IgG rather than IgM/IgA
abnormal SFLC ratio
all mean low chance of progression to myeloma
What is amyloid light chain amyloidosis?
Light chain fragments misfold and self-aggregate to form beta-pleated fibrils
Nephrotic-range proteinuria containing mainly albumin and small monoclonal light chain component
Cardiac and liver involvement
End stage renal failure
peripheral neuropathy
What is follicular lymphoma?
Neoplastic disorder of lymphoid tissue
Type of non-Hodgkin lymphoma - characterised by slowly enlarging lymph nodes
Who is follicular lymphoma more common in?
Incidence rises with age
M=F
What is used to determine the prognosticate of follicular lymphoma?
Follicular International Prognostic Index (FLIPI)
What is night sweats a classic symptom of?
lymphadenopathy
What is seen histologically on a biopsy for a Hodgkin’s lymphoma?
Reed–Sternberg cell = speckled looking cell
usually surrounded by inflammatory cells i.e. eosinophils
lots of T cells
