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| Year : 2020 | Volume
: 63
| Issue : 3 | Page : 506-508 |
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| Multiple myeloma associated with GI tract amyloidosis – Some queries |
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Sanjay A Pai1, Reinhold Paul Linke2
1 Consultant Pathologist and Head of Pathology, Columbia Asia Referral Hospital, Malleswaram, Bangalore, India
2 Reference Center of Amyloid Diseases, Vinzenz-Schuepfer-Str. 20a, Munich, Germany
Click here for correspondence address and email
| Date of Submission | 07-Dec-2019 |
| Date of Decision | 27-Feb-2020 |
| Date of Acceptance | 18-Mar-2020 |
| Date of Web Publication | 7-Aug-2020 |
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How to cite this article:
Pai SA, Linke RP. Multiple myeloma associated with GI tract amyloidosis – Some queries. Indian J Pathol Microbiol 2020;63:506-8 |
Dear Editor,
We read with interest, the report by Ahuja et al. of multiple myeloma associated with extensive gastrointestinal tract amyloidosis and have some concerns.[1] The case is remarkably similar to the report that we had published some time ago.[2]
Our patient was a 50-year-old man who presented with protein-losing enteropathy and was clinically and endoscopically diagnosed as having Crohn's disease. However, biopsies showed amyloid deposits in the stomach, duodenum, ileum, and colon. Subsequent immunohistochemical classification showed that it was s an amyloid light-chain (AL) amyloidosis in a patient with multiple myeloma.[3]
Likewise, the patient described by Ahuja et al. had hypoproteinemia and it is likely that he too had a protein-losing enteropathy. However, there are considerable lacunae in this report:
- We are not informed about the exact classification of the amyloid and the isotype of the immunoglobulin heavy and light chain
- Was a kidney biopsy performed? The kidney can be the most sensitive organ for the detection of AL amyloidosis. Was there evidence of other organs being involved?
- We are informed that the bone marrow was negative for amyloid by using Congo Red stain. However, given that amyloid is difficult to detect in the marrow, it would have been ideal to evaluate it by a more sensitive method as Congo Red Fluorescence [3]
- The young age of the patient makes us wonder whether amyloidosis is a familial trait. What was the family history?
- What treatment was offered to the patient? We presume that the patient is undergoing treatment currently.
Finally, the authors refer to “early diagnosis and management of the disease”. It must be remembered that, by definition, amyloidosis is a chronic progressive condition and gets deposited usually only in advanced stages of multiple myeloma and usually not in the early stages or at young age. Among patients with systemic amyloidosis, the involvement of the gastrointestinal tract is common. In one series, 164 of 741 (22.1%) patients diagnosed with systemic AL amyloidosis were found to have involvement of the gastrointestinal tract.[4]
There are 36 different types of human amyloid proteins and some of these have specific therapies.[5] Hence, the exact classification of the amyloid protein is essential and should be the first step in the management of the patient.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
 Reply by the Principal Authors | |  |
Dear Editor,
I thank the authors for showing interest in our case and raising queries to better understand the disease.
I will like to point wise answer all the questions-
- Patient was referred to higher centre for proper management and advised immunofixation. Later, it was reported as λ light chain restriction. Therefore, it was AL λ amyloidosis. We didn't performed AA immunohistochemistry (IHC), however kappa and lamda IHC cocktail was used on paraffin sections from the bone biopsy to established clonality of plasma cells.
- Kidney biopsy was not performed, however his biochemical profile revealed low albumin (2.0 g/dl) and heavy protienuria (13 g/day) suggesting that kidney was involved.
- We agree, congo red fluorescence is more sensitive method for detection of amyloid, however it was not done due to lack of Texas red filter in our lab. By staining with congo red and examination under polarized light we could offer diagnosis easily in this case. Moreover, the management was not going to change based on its positivity.
- There was no family history of amyloidosis or plasma cell dyscrasia.
- Patient was referred to higher centre with medical oncology and bone marrow transplant facility for further management.
Based on our experience, we recommended early diagnosis in context to gastrointestinal amyloidosis, wherein we suggest early biopsy to reach a definitive diagnosis rather than treating patient empirically based on the symptoms.
| Comments by the Corresponding Author | | |
Dear Editor,
We do not agree with the author that light chain restriction proves AL amyloidosis, since light chain restriction can also occur with any other amyloid type, most often with old-age wild-type ATTR. Therefore, the light chain maker of soluble native light chains is not reliable as compared to the amyloidotic light chain which represents an insoluble conformationally changed light chain. Therefore, the only proof for classifying amyloid is the examination of the immunohistochemical identity of the amyloidotic light chain which is provided with ALl-specific ((homologous) antibodies which we use or by mass spectroscopy. Since antibodies against the soluble light chain may be unreliable and may miss the earliest amyloid deposits, they could lead to the delay of the earliest stages of amyloidosis which are the central goal of early diagnosis as the authors point out. Unfortunately, these early stages are missed in too many patients. The classification can only be provided immunohistochemically since the earliest amyloid deposits may not be classified by mass spectroscopy which has been reported to be insensitive as compared to immunohistochemistry.
| References | | |
| 1. | Ahuja A, Sharma V, Bhardwaj M, Marwah S, Lamoria S. Extensive gastrointestinal amyloidosis due to multiple myeloma mimicking inflammatory bowel disease in a young male. Indian J Pathol Microbiol 2019;62:638-40.  [ PUBMED] [Full text] |
| 2. | Pai SA, Chandra S, Pai S, Kini D, Linke RP. Gastrointestinal ALκ amyloidosis presenting as protein-losing enteropathy correctly diagnosed by immunohistochemistry using amyloid-type-specific antibodies. Natl Med J India 2015;28:129-31. |
| 3. | Linke RP. On typing amyloidosis using immunohistochemistry. Detailed illustrations, review and a note on mass spectrometry. Prog Histochem Cyto 2012;47:61-132. |
| 4. | Shimzaki S, Hata H, Iida S, Ueda M, Katoh N, Sekijima Y, et al. Nationwide survey of 741 patients with systemic amyloid light- chain amyloidosis in Japan. Intern Med 2018;57:181-7. |
| 5. | Benson MD, Buxbaum JN, Eisenberg DS, Merlini G, Saraiva MJ, Sekijima Y, et al. Amyloid nomenclature 2018: Recommendations by the International society of amyloidosis (ISA) nomenclature committee. Amyloid 2018;25:215-8. |
Correspondence Address:
Sanjay A Pai
Consultant Pathologist and Head of Pathology, Columbia Asia Referral Hospital, Malleswaram, Bangalore - 560 055
India
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/IJPM.IJPM_962_19
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