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{| class="wikitable"
{| class="wikitable"
! style="background: #4479BA; width: 100px;" | {{fontcolor|#FFF|Amyloisosis subtype}}
! rowspan="2" style="background: #4479BA; width: 100px;" | {{fontcolor|#FFF|Amyloisosis subtype}}
! style="background: #4479BA; width: 100px;" | {{fontcolor|#FFF|Protein}}
! rowspan="2" style="background: #4479BA; width: 100px;" | {{fontcolor|#FFF|Protein}}
! style="background: #4479BA; width: 100px;" | {{fontcolor|#FFF|Involved organs severity}}
! colspan="3" style="background: #4479BA; width: 100px;" | Organ involvement (%)
! style="background: #4479BA; width: 100px;" | {{fontcolor|#FFF|Involved organs severity}}
! style="background: #4479BA; width: 100px;" | {{fontcolor|#FFF|Amyloisosis subtype}}
|-
|-
! style="background: #4479BA; width: 100px;" | {{fontcolor|#FFF|Amyloisosis subtype}}
! style="background: #4479BA; width: 100px;" | Kidney
! style="background: #4479BA; width: 100px;" | {{fontcolor|#FFF|Amyloisosis subtype}}
! style="background: #4479BA; width: 100px;" | Liver
! style="background: #4479BA; width: 100px;" | {{fontcolor|#FFF|Amyloisosis subtype}}
! style="background: #4479BA; width: 100px;" | Heart
! style="background: #4479BA; width: 100px;" | {{fontcolor|#FFF|Amyloisosis subtype}}
! style="background: #4479BA; width: 100px;" | {{fontcolor|#FFF|Amyloisosis subtype}}
|-
|-
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" |Primary
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" |Primary
|_
|Monoclonal immunoglobulin light chains
|[[Anemia]]
|50-100
|[[MRI]] is the best radiologic tool to differentiate between retroperitoneal masses.
|10-25
|
|50-100
|-
|-
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" |Secondary
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" |Secondary
|
|Serum amyloid A
|[[Leukocytosis]], positive inflammatory markers
|50-100
|
|<10
|
|_
|-
|-
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" |DRA (dialysis related amyloidosis)
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" |DRA (dialysis related amyloidosis)
|
|Beta‐2 microglobulin
|positive [[tumor marker]]
|_
|
|_
|
|_
|-
|-
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" |Senile systemic (cardiac) amyloidosis
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" |Senile systemic (cardiac) amyloidosis
|Transthyretin (wild type)
|_
|_
|[[Diabetes mellitus|DM type II]], [[amylase]] and [[lipase]] levels may be slightly elevated
|_
|
|50-100
|
|-
|-
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" |Meretoja syndrome
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" |Meretoja syndrome
|
|Gelsolin
|
|_
|
|_
|
|_
|}
|}



Revision as of 05:46, 10 November 2019

Amyloisosis subtype Protein Organ involvement (%)
Kidney Liver Heart
Primary Monoclonal immunoglobulin light chains 50-100 10-25 50-100
Secondary Serum amyloid A 50-100 <10 _
DRA (dialysis related amyloidosis) Beta‐2 microglobulin _ _ _
Senile systemic (cardiac) amyloidosis Transthyretin (wild type) _ _ 50-100
Meretoja syndrome Gelsolin _ _ _
 
 
 
 
Infection/Inflammation
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Increased production of IL-1/IL-6/TNF-α
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Upregulation of hepatic serum amyloid A production
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
SAA production uptake by macrophages
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
C-terminal cleavage of SAA
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
β-sheet configuration of SAA
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Fibril deposition in extracellular space
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Binding of glycosaminoglycan, serum amyloid P, and lipid components
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Resistant to proteolysis
 
 
 
 
The above algorithm is adopted from International Journal of Nephrology and Renovascular Disease












Associated Conditions
Conditions Examples
Chronic infections
Monogenic periodic fever syndromes
Conditions predisposing to recurrent infections
Neoplasia
Inflammatory Arthritis
Systemic Vasculitis
Others
Organ System Involvement Differential Diagnosis Causes Clinical Features Laboratory Findings Gold Standard Test Therapy
Nephrotic Syndrome and Renal Failure Secondary (AA) Amyloidosis
  • Biopsy and congo-red staining of the sample
Primary (AL) Amyloidosis


Diabetic Nephropathy
Minimal Change Disease
Focal Segmental Glomerulosclerosis
  • Biopsy:
    • Podocyte foot process effacement
    • Capillary lumen abolished by the segmental increase in matrix
Fabry's Disease
  • Deficient alpha galactosidase A
Light Chain Deposition Disease
  • Biopsy:
    • Non-amyloid granules
Membranous Glomerulonephritis
Fibrillary-Immunotactoid Glomerulopathy
  • Biopsy:
    • Polycloncal IgG deposits
    • Infiltration of glomerular structures by amorphous acellular material (nonbranching fibrils 12-24nm in diameter)
    • Ig heavy-chain and one light-chain subclass
Organ System Involvement Differential Diagnosis Causes Clinical Features Laboratory Findings Gold Standard Test Therapy
Polyneuropathy POEMS syndrome (Demyelinating)
Metabolic Syndrome (Axonal pathology)
Vitamin Deficiencies (Axonal Pathology)



Guillain-Barre Syndrome (Demyelinating)
  • Delayed F waves
  • Clinical diagnostic criteria (progressive weakness of more than two limbs, areflexia, and progression for no more than four weeks)
Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) (Mixed axonal and demyelinatiing)


  • EFNS/PNS criteria
  • Koski criteria
Multifocal Motor Neuropathy
  • Progressive, asymmetric, distal and upper limb predominant weakness
  • No significant sensory abnormalities
  • Areflexia
  • Clinical criteria (EFNS/PNS):
    • Slowly progressive or step-wise progressive, focal, asymmetric limb weakness; i.e., motor involvement in the motor nerve distribution of at least two nerves for > 1 month.
    • No objective sensory abnormalities except for minor vibration sense abnormalities in the lower limbs


Organ System Involvement Differential Diagnosis Causes Features Laboratory Findings Gold Standard Test Therapy
Organomegaly (Hepatosplenomegaly and Lymphadenopathy) Malaria
Kala-azar


Infective Hepatitis
Chronic Myelogenous Leukemia (CML)
Lymphoma
Primary (AL) Amyloidosis
  • Typical green birefringence under polarized light after Congo red staining (appears in red under normal light)
  • Congo red staining
  • Melphalan-prednisone/dexamethasone
  • Dexamethasone plus Cyclophosphamide-thalidomide
  • Stem cell transplantation
Gaucher's Disease



Example #1

The patient presented with S.O.B. one year after hysterectomy for a leiomyomatous uterus.