Myeloma Flashcards
Dx POEMS
Polyradiculopathy
Paraprotein
Plts 1 of 3 major - criteria (Castleman disease, sclerotic bone lesions, or elevated VEGF)
Plus
1 of 3 minor- oedema, organomegaly, papilloedma skin changes thrombocytosis, endocrinopathy
Which cytokines correlates best w poems
Veg f
Tests at dx
The diagnosis is made based on a composite of clinical and laboratory features. Most notably, the constellation of peripheral neuropathy (especially demyelinating) and any of the following should elicit an in-depth search for POEMS syndrome: monoclonal protein (especially when λ restricted), thrombocytosis, anasarca, or papilledema. The requirements set forth in Table 1 are designed to retain both sensitivity and specificity, potentially erring on the side of specificity. Making the diagnosis can be a challenge, but a good history and physical examination followed by appropriate testing (most notably radiographic assessment of bones,34 measurement of VEGF,14,18,35,36 and careful analysis of a bone marrow biopsy37) can differentiate this syndrome from other conditions, such as chronic inflammatory polyradiculoneuropathy (CIDP), monoclonal gammopathy of undetermined significance, neuropathy, and immunoglobulin light chain amyloid neuropathy. Other important baseline tests include: complete blood count, creatinine, creatinine clearance, serum and urine protein electrophoresis with immunofixation, serum immunoglobulin free light chains, thyroid-stimulating hormone, prolactin, parathyroid hormone, testosterone (or estradiol), luteinizing hormone, follicle-stimulating hormone, plasma VEGF, bone marrow aspirate, and biopsy with immunohistochemical stains to document λ-restricted plasma cells, pulmonary function tests, electromyelogram with nerve conduction studies, and PET/CT, with special attention to the bone windows of the CT.
High risk dx
del(17p), del(1p), t(4;14), and t(14;16)) or the presence of circulating plasma cells (≥20%).
T14:20
Extramedullary plasma cell means what
High risk dx- circulating or plasmacytoma
New dx criteria for myeloma
2
Clonal bone marrow plasma cells >10% or biopsy-proven bony or extramedullary plasmacytoma
Any 1 or more of the below listed myeloma defining events (which has to be attributed to the underlying plasma cell proliferative disorder):
• Hypercalcemia:
• Renal failure
• Anemia:
• Bone lesions: 1 or more osteolytic lesions on skeletal
radiography (ie, X-ray), low-dose CT, or PET-CT
• Clonal bone marrow plasma cell percentage >60%
• Involved/uninvolved serum free light chain ratio >100,and the involved serum free light chain concentration 10 mg/dL or higher
• Two or more focal lesions based on MRI studies of the skeleton
Both criteria have to be fulfilled.
Aberrant plasma cell on flow
Decreased cd 38
Cd 56 exp
Loss cd 19/45
Dx mgus
Serum M protein
< 30g/L
• BM clonal plasma
cells < 10%
• NO mde
Smouldering
Serum M protein
(IgG or A) ≥ 30g/L
and/or
• BM clonal plasma
cells ≥ 10%
• NO mde
Standard baseline investigation
FBC and film
• Biochemistry, incl. calcium and creatinine
• Serum EPG, immunofixation
• Quantification of serum Igs (nephelometry)
• Urinalysis, 24‐hr urine EPG & immunofixation
• BM aspirate and/or biopsy
• Cytogenetics (metaphase karyotype & FISH)
• Serum 2microglobulin and LDH
• Serum free light chains
Myeloma cr
No serum or urine M-protein by immunofixation,
BM≤5% pcs, disappearance of plasmacytomas
Plasmablastc mrorphology
large numbers said to be
the most important distinguishing
feature from reactive
– Numbers of plasmablasts correlates with
prognosis
Standard risk
11:14
6:14
Hyper diploid
Intermediate risk
11:14
Competitive genomic hybridisation
Global assessment
Not requiring metaphase
But only detects copy number changes not balance translocations nor inversions
