Home › Research › Drug development
Amyloidosis: a convoluted story
Robert A. Kyle. British Journal of Haematology, 2001-09-01
Topic: Plasma and spinal-fluid biomarkers
However, the fibrils in primary amyloidosis (AL) consist of the variable portion of a monoclonal light chain (κ or λ); secondary amyloidosis (AA) fibrils consist of protein A, a non-immunoglobulin; familial amyloidosis fibrils are usually composed of mutated transthyretin (prealbumin); senile systemic amyloid fibrils consist of normal transthyretin; and amyloid associated with long-term dialysis consists of β2-microglobulin. Carl Rokitansky (1842) stated that patients with tuberculosis or syphilis had liver enlargement as a result of infiltration by a grey albuminous, gelatinous substance. He concluded that the infiltrating substance was albuminous and not fatty. Budd noted that all four of his cases were associated with tuberculosis involving bone. Gairdner concluded that the waxy liver represented a true degeneration in which there was a ‘metamorphosis of its glandular structure into a much more dense “albuminous material” than in the normal condition.’ He believed that this resulted in a reduction in function and loss of the typical structural characteristics of the liver. Billroth, stated that lardaceous and waxy infiltration was not the result of cellulose degeneration, and that it had no resemblance to starch granules. The first case of amyloidosis associated with multiple myeloma was reported by Weber (1867). The autopsy revealed spontaneous fractures of the sternum with replacement by a greyish red substance containing many small nucleated cells. He found that frozen sections revealed marked staining of the amyloid with Congo red, but staining of paraffin sections was unsatisfactory. Cohen & Calkins (1959) first recognized that all types of amyloid demonstrated a non-branching fibrillar structure when viewed under the electron microscope. He postulated that increased and of protein was & demonstrated the of amyloid in plasma cells and believed that this was evidence of the of origin of amyloid. that amyloid be able to be as a substance, and that its to other then be also stated that amyloid in the tissues was to the of protein by the He emphasized the of amyloid in the lung and tissue in multiple myeloma in to its in liver, spleen and kidneys in secondary amyloidosis. He also thought there was a between the amyloid associated with and the associated with protein. The of primary amyloidosis with multiple myeloma or a plasma was reported by & et that protein an in primary amyloidosis. & reported that of amyloid fibrils a They this as a structure by t
Key findings
- However, the fibrils in primary amyloidosis (AL) consist of the variable portion of a monoclonal light chain (κ or λ); secondary amyloidosis (AA) fibrils consist of protein A, a non-immunoglobulin; familial amyloidosis fibrils are usually composed of mutated transthyretin (prealbumin); senile systemic amyloid fibrils consist of normal transthyretin; and amyloid associated with long-term dialysis consists of β2-microglobulin.
- Carl Rokitansky (1842) stated that patients with tuberculosis or syphilis had liver enlargement as a result of infiltration by a grey albuminous, gelatinous substance.
- He concluded that the infiltrating substance was albuminous and not fatty.
- Budd noted that all four of his cases were associated with tuberculosis involving bone.
- Gairdner concluded that the waxy liver represented a true degeneration in which there was a ‘metamorphosis of its glandular structure into a much more dense “albuminous material” than in the normal condition.’ He believed that this resulted in a reduction in function and loss of the typical structural characteristics of the liver.
New to this subject? Start with our plain-English guide: Treatments →
Study details
- Participants60
- Evidence strengthLow
How this study scored 43/100
Published in a peer-reviewed journal. Scored zero for study design, effect reported and randomised / blinded because the record does not state it. We have no data on journal's citation record, times cited, citations per year, impact for its field and year, cited by studies here, still being cited, senior author's track record, institution's track record and multi-centre collaboration, so those were left out: it earned 25 of the 58 points we could judge it on, which is 43 out of 100.
| Component | Points | Why |
|---|---|---|
| Journal tier | 7 / 10 | Tier 3 — peer-reviewed journal (British Journal of Haematology) |
| Journal's citation record | — / 4 | No journal record from OpenAlex — left out of this score |
| Study design | 0 / 12 | No study design recorded — scores zero, not guessed (not stated in the record — scored 0) |
| Strength of evidence | 1 / 5 | Rated “low” in our summary |
| Peer-review validation | 10 / 10 | Peer-reviewed. Published as an article in a named peer-reviewed journal. |
| How recent | 1 / 5 | 2001 — older than 20 years, still counted |
| Sample size | 1 / 4 | 60 participants |
| Effect reported | 0 / 3 | No effect size reported — scores zero (not stated in the record — scored 0) |
| Studied in people | 3 / 3 | Studied in people (participant count stated) |
| Randomised / blinded | 0 / 2 | No randomisation or blinding stated — scores zero (not stated in the record — scored 0) |
| Times cited | — / 7 | No citation count (not found in OpenAlex) — left out of this score |
| Citations per year | — / 5 | No citation count or no year — left out of this score |
| Impact for its field and year | — / 6 | No field-weighted citation impact (OpenAlex has none yet) — left out of this score |
| Cited by studies here | — / 6 | Not yet checked against this library's citation graph — left out of this score |
| Still being cited | — / 4 | No yearly citation counts — left out of this score |
| Senior author's track record | — / 4 | Senior author's record not available — left out of this score |
| Institution's track record | — / 3 | No institution listed in OpenAlex — left out of this score |
| Multi-centre collaboration | — / 3 | Institutions not listed — left out of this score |
| Indexed in PubMed | 2 / 2 | Indexed in PubMed (PMID 11552976) |
| Free to read | 0 / 2 | Behind a paywall — scores zero |
| Points | 25 / 58 | Scored on 11 of 20 parts. We have no data for the rest, so they are left out: 25 of the 58 points we could judge it on is 43 out of 100. |
| Total | 43 / 100 | — |
What each of these 20 components measures, and what this score is not →
Read the original
Free copy unknown: no free-copy check has an answer for this record yet. That does not mean it is paywalled.
Cite this study APA, Vancouver, BibTeX · download .bib or .ris
Built from the record alzhub holds for this paper, not looked up. Volume, issue and page numbers are not held in the record. Check the citation against the original before you use it.
APA 7th
Kyle, R. A. (2001). Amyloidosis: a convoluted story. British Journal of Haematology. https://doi.org/10.1046/j.1365-2141.2001.02999.x
Vancouver
Kyle RA. Amyloidosis: a convoluted story. British Journal of Haematology. 2001. doi:10.1046/j.1365-2141.2001.02999.x. PMID: 11552976.
BibTeX
@article{kyle2001-11552976,
author = {Kyle, Robert A.},
title = {{Amyloidosis: a convoluted story}},
journal = {British Journal of Haematology},
year = {2001},
doi = {10.1046/j.1365-2141.2001.02999.x},
pmid = {11552976},
url = {https://doi.org/10.1046/j.1365-2141.2001.02999.x}
}