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Myeloma (Pathophysiology/ Symptoms (Hyperviscosity syndrome (血液高度黏稠症候群)…
Myeloma
Pathophysiology/ Symptoms
Bone pain
back or chest
常見
extremities 不常見
induced by movement
vertebral collapse
變矮
Plasmacytomas
of the ribs
expanding costal lesions
soft tissue masses
Infection
肺炎,泌尿道感染
Streptococcus pneumoniae
gram-negative organisms
原因
immune dysfunction
impaired lymphocyte function
suppression of normal plasma cell function
hypogammaglobulinemia
physical factors
hypoventilation
rib cage and spine
pathologic fractures
pain
Neurologic disease
原因
paravertebral plasmacytoma
collapsed bone (rare)
Radiculopathy
脊椎神經壓迫
造成半身麻痺或無法控制尿液、大便之排泄
thoracic or lumbosacral area
類型
Cord compression
原因
extramedullary plasmacytoma
bone fragment due to
fracture
of a vertebral body (5%)
症狀
severe back pain
weakness or paresthesias of the lower extremities
bladder or bowel dysfunction or incontinence
medical
emergency
避免 permanent paraplegia
Peripheral neuropathy
原因
amyloidosis
POEMS syndrome (osteosclerotic myeloma)
polyneuropathy
,organomegaly,endocrinopathy,M-protein,skin changes
paraneoplastic mechanism
CNS involvement
原因
Intracranial plasmacytomas
myelomatous lesions of the skull
plasmacytomas involving the clivus or base of the skull
Leptomeningeal myelomatosis
hyperviscosity
or high blood levels of
ammonia
Rare
Myeloma cell lines 產生ammonia
Hyperviscosity syndrome
(血液高度黏稠症候群)
特殊蛋白的形成
出血傾向
神經症狀
眩暈、嗜睡、末梢神經症狀、痙攣
狹心症、鬱血性
心臟
衰竭
全身症狀
易疲勞、全身無力、食慾不振、
體重減輕
視網膜病變
視網膜出血、視乳突的水腫等,造成視力障礙
Hypercalcemia
便秘、意識障礙、心律不整
low anion gap
鈣當作 unmeasured cation
cationic IgG molecule
原因
binding of the
monoclonal protein
with calcium
Bone lesion (
osteolytic
)
Anemia
normocytic, normochromic
fatigue and pallor
原因
bone marrow replacement
kidney damage
dilution in the case of a large
M-protein
Renal disease
serum creatinine concentration 增加
導致Renal failure
原因
light chain cast nephropathy
(myeloma kidney)
high involved free light chain (FLC) levels (>1500 mg/L)
Bence-Jones 蛋白
(輕鍊: ϰ 或 λ)
沈積於腎小管 (distal convoluted tubule + collecting duct)
形成
tubular (myeloma) cast
hypercalcemia
其他:
concurrent light chain (AL)
amyloidosis
,
light chain deposition disease,
drug-induced renal damage
Spectrum of disease
infiltration of
plasma cells
into the
bone
or other organs or to kidney damage
from excess
light chains
Extramedullary plasmacytomas (EP) (7%)
large, purplish, subcutaneous masses
Risk factor/ Epidemiology
60~80 歲男性
(66歲, >40歲)
男女比=2:1
t (4,14) = (FGFR , IgH)
血液惡性腫瘤之 14%
所有癌症之 3.93%
每年新發生100~200名
發生率3/100,000
症狀
●Anemia – 73 percent
●Bone pain – 58 percent
●Elevated creatinine – 48 percent
●Fatigue/generalized weakness – 32 percent
●Hypercalcemia – 28 percent
●Weight loss – 24 percent, one-half of whom had lost ≥9 kg
PATHOLOGIC FEATURES
Monoclonal proteins
Light chain myeloma
Nonsecretory myeloma
Oligo-secretory myeloma
Laboratory artifacts
Typical pattern
Urinalysis
Peripheral smear
Bone marrow examination
Percent plasma cells
Morphology
Immunophenotype
Cytogenetics
Free light chain assay
IMAGING
Choice of modality
CT, MRI, and PET
Skeletal surveys
DIAGNOSIS
Diagnostic criteria