Flashcards in Haematology 11 - Plasma cell myeloma and Monoclonal Gammopathy of Uncertain Significance Deck (42)
What immunoglobin is produced by myeloma cells?
One single type (eith IgG or IgA) which is known as paraprotein or M spike
What are Bence Jones proteins?
Urine monoclonal free light chains
What is Waldenstrom’s-Lymphoplacytic lymphoma?
A lymphoplasmocytic lymphoma with IgM paraprotein that causes visual disturbances
What is the name of the premalignant condition that always precedes myeloma?
Monoclonal gammaopathy of uncertain significance (MGUS)
What are the two most significant risk factors for myeloma?
Black > causasians/ asians
What are the diagnostic criteria for MGUS?
Serum M-protein <30g/L
BM clonal plasma cells <10%
What is smouldering myeloma?
Serum M-protein >30g/L
BM clonal plasma cells >10%
What % of plasma cells is there in symptomatic multiple myeloma?
What is the most notable interaction of myeloma cells with the bone marrow micro-environment?
Produce RANK ligand which stimulates osteoclasts to cause bone resorptions
What is the incidence of IgM myeloma?
Very rare (<1% of myelomas)
What does CRAB stand for in myeloma diagnosis?
Calcium (hypercalcaemia, >2.75)
Renal (creatinine >177/ eGFR <40)
Bone disease (see lytic lesions)
What is the most common and 2nd most common cytogenetic abnormality in myeloma?
1. Hyperdiploid karyotype
2. IgH gene rearrangement
What are the 3 2014 Myeloma Defining Events
BM plasma cells >60%
involved:uninvolved FLC ratio >100
>1 focal lesion on MRI
Which part of the skeleton is affected by myeloma?
Proximal skeleton (spine, skull, knees)
Where are myeloma patients most likely to feel pain?
Back, chest wall, pelvis
What % of myeloma patients present with bone disease?
What scan is necessary to detect bone lesions in myeloma?
Whole body CT is first line (X ray is obsolete for this use)
PET scan can also be used
Gold-standard = whole body diffusion-weighted MRI as this shows active vs treated disease
What are the 2 most likely emergency presentations of myeloma?
What tests should be done to diagnose myeloma?
Serum protein electrophoresis
Serum free light chains
Bone marrow aspirate and biopsy for immunohistochemistry
FISH (for prognostic)
What is the best way to treat cord compression in myeloma?
What risk does myeloma present to the kidney?
Cast nephropathy - FLCs and Bence Jones proteinuria cause proximal tubule cell injury
How should myeloma kidney disease be treated in an emergency?
Bortezomib-based therapy is the cornerstone of myeloma kidney disease treatment - once the patients can become independent from dialysis their outcomes improve dramatically
How does myeloma affect immunity?
Serum levels of normal Igs reduced
BM micro-environment interference also impairs myeloid, T and NK cells
Chemo also impairs immune response
What is tested for in BM biopsy in suspected myeloma?
Immunohistochemistry for CD138 - specific for myeloma cells in BM
What test can detect cytogenetic abnormalities of prognostic significance in myeloma?
What are the 3 parameters in the international staging system for myeloma?
Serum beta microglobulin
How does AL amyloidosis link to myeloma?
Misfolded FLCs aggregate into amyloid in target organs
How does amyloidosis affect the kidney?
Give 5 features of the clinical presentation of AL amyloidodis?
Unexplained heart failure
Abnormal liver function tests