Clinical Features of AL Amyloidosis Presenting with ‘Raccoon Eyes’: A Case Report and Literature Review

Objective: This study presents and examines the clinical profiles of 28 patients with immunoglobulin light chain (AL) Amyloidosis who exhibit the characteristic “raccoon eye” appearance. It seeks to delineate the typical clinical manifestations, diagnostic approaches, and therapeutic strategies associated with this condition, thereby aiming to augment the clinical awareness and treatment proficiency among physicians.

Methods: We present a case of “raccoon eye” in a 63-year-old male patient diagnosed with AL amyloidosis and detail his clinical presentation. Furthermore, we conducted a comprehensive literature review of related case reports in the PubMed database and emphasized the clinical features and treatment outcomes associated with AL amyloidosis featuring this rare form of periorbital purpura.

Results: We identified 27 cases of AL amyloidosis characterized by "raccoon eye" from the PubMed database, and including our reported case, a total of 28 patients were analyzed. Among them, 13 were male (46.4%) and 15 were female (53.6%), with a male-to-female ratio of 0.87:1. The average age for males was 66.31 ± 7.239 years, and for females, it was 66.00 ± 10.282 years. In these 28 patients, the majority exhibited renal and cardiac involvement. Renal involvement manifested as proteinuria or impaired renal function, while cardiac involvement was primarily characterized by left ventricular hypertrophy and early elevation of NT-proBNP levels. Additionally, some patients experienced involvement of the liver, lungs, nervous system, and macroglossia. AL amyloidosis can also present with nonspecific symptoms, mainly fatigue and unconscious weight loss. Six patients suffered from the disease within months after the appearance of the “raccoon eye” sign.

Conclusion: When the typical symptom of “raccoon eye” appears in patients, clinicians should be alert to the possibility of AL amyloidosis and promptly establish a diagnosis and initiate treatment.

"Raccoon eyes" denotes periorbital purpura resulting from the Valsalva maneuver or minor trauma. While it is seen in a limited number of patients, it represents a highly distinctive feature of immunoglobulin light chain (AL) Amyloidosis [1]. However, once this sign appears, patients have a high mortality rate and poor prognosis. Hence, this study documents a case of AL amyloidosis manifesting with "raccoon eyes" and leverages this case to conduct a comprehensive review of the literature, with the goal of elevating clinicians' awareness and proficiency in diagnosing and managing immunoglobulin light chain amyloidosis that presents with "raccoon eyes".

Amyloidosis is a category of disorders marked by the accumulation of insoluble, misfolded proteins within tissues [2]. This process involves the misfiling of either native or mutant proteins, leading to their aggregation as insoluble fibrils in the extracellular spaces of various organs [3]. The four predominant types of amyloid include immunoglobulin light chain (AL), Amyloid A (AA), Transthyretin (ATTR), and Amyloid Beta peptide (AB) [4]. Light chain amyloidosis, namely AL amyloidosis, is the most frequently occurring type of systemic amyloidosis [2].

AL amyloidosis impairs organ function, leading to multi-organ dysfunction, organ failure, and death within 6 months of diagnosis in 24-37% of patients, predominantly affecting individuals over 65 years old with a mean age at diagnosis of 63 years [5]. As a consequence of the lack of specificity of the early clinical manifestations, a delayed diagnosis and treatment often ensues, when treatment is often ineffective.

The clinical spectrum of AL amyloidosis is characterized by a heterogeneous array of symptoms that hinge upon the specific organs affected, with a notable lack of specificity. The kidney and heart are the most commonly affected organs [6]. Renal involvement is observed in approximately 70% of patients [6], manifesting as asymptomatic proteinuria or nephrotic syndrome. Cardiac involvement is present in about 60% of patients, typically characterized by thickening of the interventricular septum and ventricular wall. This can lead to systolic or diastolic dysfunction and heart failure, with some patients experiencing syncope or sudden death. Although rare, angina pectoris or myocardial infarction may also occur. Approximately 25% of patients exhibit neuropathy, with peripheral neuropathy affecting about 20% of them, commonly presenting as numbness, paresthesia, and pain in the extremities. Gastrointestinal tract involvement is uncommon in AL amyloidosis. Hepatomegaly may be present in some patients, while splenomegaly may also occur, though the incidence of these manifestations is uncertain. Amyloid protein infiltration into skeletal muscle can cause visible enlargement, and a scalloped tongue edge, due to a giant tongue or tooth indentation, is a characteristic finding. Cutaneous involvement can manifest as periorbital purpura, known as "raccoon eye," which is precipitated by valsalva maneuvers or minor trauma and is a highly distinctive, though infrequently observed, sign of AL amyloidosis (spontaneous periorbital purpura is virtually unique to this disease) [1]. Other signs of cutaneous involvement include waxy thickening, subcutaneous ecchymosis, and the formation of subcutaneous nodules or plaques.

Research subjects

This study includes a total of 28 patients, comprising 27 cases of immunoglobulin light chain (AL) Amyloidosis characterized by "raccoon eyes," as reported in the existing literature, along with one newly identified case from our institution.

We report the case of a 63-year-old man admitted to our hospital with a one-year history of proteinuria and a 10-day history of nausea accompanied by periorbital purpura. On admission, physical examination revealed a blood pressure of 110/80 mmHg, periorbital purpura around the right eye (Figure 1), and a systolic murmur audible over the aortic valve area. Laboratory investigations showed hypoalbuminemia (albumin 29 g/L), elevated serum creatinine (160 μmol/L), significant proteinuria with a 24-hour urinary protein excretion of 6.02 g, elevated B-type Natriuretic Peptide (BNP) at 909.62 pg/mL, and high-sensitivity cardiac troponin I at 0.025 μg/L. Serum and urine immunofixation electrophoresis and other assessments revealed no significant abnormalities.

Figure 1: Palpebral and periorbital purpura on the right eye.

Electrocardiography demonstrated sinus rhythm, signs indicative of an old inferior myocardial infarction, and ST-T segment changes. Transthoracic echocardiography revealed biatrial enlargement, left ventricular myocardial thickening, reduced left ventricular systolic function with a Fractional Shortening (FS) of 21% and an Ejection Fraction (EF) of 41%, as well as a small pericardial effusion. Renal ultrasonography showed the right kidney measuring 9.5 × 3.9 cm and the left kidney 9.7 × 4.2 cm, with increased cortical echogenicity and indistinct corticomedullary differentiation.

To further clarify the etiological diagnosis, a percutaneous renal biopsy was performed under ultrasound guidance after excluding contraindications. Histopathological examination of the kidney revealed positive Congo red staining, and under polarized light, apple-green birefringent deposits were observed in the vascular walls and mesangial areas. Considering the patient's clinical manifestations and laboratory findings, a diagnosis of renal amyloidosis was established. Unfortunately, the patient experienced sudden cardiac death before the initiation of specific treatment for amyloidosis.

We verbally informed the patient of the intention to publish their case as a case report and sought their confirmation, and the patient gave their consent.

A literature search was conducted in the PubMed database of the U.S. National Library of Medicine up to January 1, 2025, using the search terms [(raccoon eyes) AND (amyloidosis)] or [Amyloid Purpura], restricted to case reports in English. This search yielded 27 case reports of AL amyloidosis patients featuring the characteristic "raccoon eyes," encompassing a total of 27 patients [1,7-32]. For each case, the following variables were recorded: first author and publication year, patient age and sex, triggering factors for "raccoon eyes," involved organs, reported symptoms, treatment received, and patient outcome/prognosis. Including the patient reported in this paper, a total of 28 patients were analyzed (Table 1).

The first case was reported in 1979 and published in the Journal of the American Medical Association (JAMA). The patient was a 75-year-old man who presented with dysphagia, fatigue, weakness, inappetentia, and a weight loss of 15 kg over four months. Physical examination revealed pitting edema of the ankles, muscle atrophy, and orthostatic hypotension. Electrophysiological studies demonstrated peripheral neuropathy. Gastroscopic examination showed absence of peristalsis in the gastric antrum. Urinalysis revealed 4+ proteinuria with hyaline and granular casts. Approximately 18 hours after a percutaneous renal biopsy, significant periorbital purpura was observed. The subsequent treatment and prognosis of this case were not reported [7]. From 2006 to 2024, additional 26 cases of AL amyloidosis characterized by the presence of "raccoon eyes" have been reported.

Including the case, we reported, among the 28 patients, there were 13 males (46.4%) and 15 females (53.6%), with a male-to-female ratio of 0.87:1. The average age for male patients was 66.31 ± 7.239 years, and for female patients, it was 66.00 ± 10.282 years.

Characteristics of "Raccoon Eyes"

In the 28 patients, "raccoon eyes" were predominantly spontaneous rather than trauma-induced. Only one case [22] developed "raccoon eyes" after the patient was asked to hold their breath and perform the Valsalva maneuver during a renal biopsy. Another case [7] observed the onset of "raccoon eyes" 18 hours after renal biopsy. The duration of the "raccoon eyes" was reported in a limited number of cases, lasting for 6 months, 1 year, 1.5 years, 3 years and even 15 years. Additionally, purpura can also appear in other parts of the body, such as the upper limbs, neck, shoulders, chest, and inguinal regions.

Organ and tissue involvement

In terms of systemic involvement, the kidneys were the most commonly affected organs (17/28), presenting as proteinuria with or without renal insufficiency. Cardiac involvement was also common (12/28), mainly manifesting as left ventricular hypertrophy; moreover, several cases reported elevated NT-proBNP levels. Macroglossia (enlarged tongue) was observed in 8 patients [10,12,20,21,23,24,26,30], including one patient with intermittent tongue swelling causing difficulties when speaking. Neurological involvement was noted in five patients [7,10,12,13,26], including depressive syndrome, peripheral neuropathy, bilateral carpal tunnel syndrome and a transient ischemic attack. Liver involvement was observed in one patient [16]; Computed Tomography (CT) showed hepatomegaly and bilateral pleural effusions. This patient also had elevated gamma-glutamyl transferase and aspartate aminotransferase levels, while coagulation profiles and bilirubin levels were normal. Notably, two patients [11,26] exhibited pulmonary involvement, one presenting with exertional dyspnea; lung biopsy indicated amyloid infiltration, and the other indicating increased interstitial lung markings. Pulmonary involvement is very rare in AL amyloidosis, and many physicians may attribute dyspnea to heart failure, potentially overlooking lung involvement. In addition to organ involvement, AL amyloidosis may present with non-specific symptoms such as fatigue, anorexia, and weight loss. Among the 28 cases, 5 patients [7,9,18,29,30] presented with fatigue, and 4 patients [7,11,28,31] experienced unintentional weight loss.

Treatment and prognosis

Among the 28 patients, 10 cases did not mention the prognosis, and 1 patient was lost to follow-up. Of the remaining 17 patients, 8 patients either did not receive treatment or died shortly after initiating treatment. 9 patients achieved disease stabilization after receiving treatment targeting amyloidosis or multiple myeloma, although their long-term prognosis was not detailed.

This integrated analysis of 28 cases underscores that “raccoon eyes,” though rare, constitute a critical clinical indicator of underlying AL amyloidosis. The present case exemplifies both the diagnostic challenge and the profound clinical significance of this sign. Our patient exhibited classic features such as renal and cardiac involvement, yet diagnosis was delayed until the emergence of periorbital purpura, shortly followed by sudden cardiac death. This tragic outcome highlights the aggressive nature of AL amyloidosis and reinforces that “raccoon eyes” often appear in the context of advanced, life-threatening disease.

The pathophysiology of “raccoon eyes” involves amyloid infiltration of dermal blood vessels and periorbital tissues, resulting in increased vascular fragility. Minor elevations in venous pressure, such as those induced by vomiting (as observed in our patient), the Valsalva maneuver, or even postural changes, can precipitate this striking purpura [1]. Its appearance should therefore serve as an urgent clue to systemic amyloid deposition. Notably, in our case, the sign emerged not after trauma, but spontaneously following episodes of vomiting, correlating with elevated intra-thoracic and intra-abdominal pressure which is a mechanism consistent with previous reports [7,22].

A central insight from this review is that the “raccoon eye” sign frequently coincides with multi-organ involvement, particularly renal and cardiac-a pattern clearly illustrated by our case. The patient had documented proteinuria for one year prior to admission, yet the diagnosis of amyloidosis was not pursued until catastrophic cardiac involvement became apparent. This reflects a common diagnostic pitfall: the non-specific early presentation of AL amyloidosis. Importantly, biochemical markers such as NT-proBNP possess high sensitivity for detecting cardiac amyloidosis even before overt heart failure develops [33,34]. Their underutilization in ambulatory settings, as may occurred here, represents a missed opportunity for earlier diagnosis.

Once “raccoon eyes” appear, clinical urgency escalates. Several cases in this review, including our own, suggest a grim short-term prognosis, with death occurring within months of this sign emerging [1,5,15,17,21,28]. Therefore, its recognition should immediately trigger a systematic diagnostic workup. This includes serum and urine immunofixation electrophoresis, serum free light chain assay, cardiac biomarkers (NT-proBNP and troponin), echocardiography, and confirmatory tissue biopsy-adhering to established international diagnostic criteria [35,36]. Early and accurate diagnosis is the cornerstone of management, as it enables initiation of therapy aimed at suppressing the underlying plasma cell clone, such with bortezomib-based regimens or daratumumab combinations, which have shown efficacy in stabilizing disease [37].

Several limitations inherent in this review must be acknowledged. As a synthesis of case reports, it is susceptible to publication bias and incomplete data reporting, particularly regarding treatment responses and long-term outcomes. The true prevalence of “raccoon eyes” in AL amyloidosis and its precise prognostic value remain to be defined through prospective studies.

In conclusion, the “raccoon eye” sign is a red flag for AL amyloidosis. Clinicians must recognize its grave implications. Its appearance, especially in the context of unexplained renal or cardiac abnormalities, should prompt an immediate and comprehensive evaluation to confirm or rule out systemic amyloidosis. Enhanced vigilance can facilitate earlier diagnosis, timely therapeutic intervention, and potentially improved outcomes in this devastating disease [38,39].

The "raccoon eye" sign is a highly specific though infrequent marker of AL amyloidosis. Clinicians must recognize its diagnostic significance. Its appearance, whether spontaneous or after minor strain, should trigger an immediate and comprehensive evaluation for systemic amyloidosis. Heightened vigilance and early diagnosis are essential to guide timely therapy and improve the prognosis of this serious disease.

Not applicable.

This study was supported by the Naval Medical University General Incubation Fund (No: 2022MS027).

There is no conflict of interest.

The study was approved by the Local Ethics Commitee (CHEC 2020-171).

The patient's daughter consented to publication.

All data generated or analyzed during this study are included in this published article and its supplementary information files.

1. Eder L, Bitterman H. Image in clinical medicine. Amyloid purpura. N Engl J Med. 2007 Jun 7;356(23):2406. doi: 10.1056/NEJMicm061510. PMID: 17554122.
2. Milutinovich J, Wu W, Savory J. Periorbital purpura after renal biopsy in primary amyloidosis. JAMA. 1979 Dec 7;242(23):2555. PMID: 490871.
3. Passos Rda H, Pereira A, Neto AC, Ribeiro AF, Kutner J, Hamerschlak N, Scheinberg M. Clinical image: bilateral black eyes (raccoon's eyes) in AL amyloidosis. Arthritis Rheum. 2006 Nov;54(11):3724. doi: 10.1002/art.22184. PMID: 17075892.
4. Lee HJ, Chang SE, Lee MW, Choi JH, Moon KC. Systemic amyloidosis associated with multiple myeloma presenting as periorbital purpura. J Dermatol. 2008 Jun;35(6):371-2. doi: 10.1111/j.1346-8138.2008.00487.x. PMID: 18578717.
5. Lestre S, Gonçalves A, João A, Ferreira A, Apetato M. Púrpura: manifestação de amiloidose sistémica primária [Purpura: primary systemic amyloidosis manifestation]. Acta Med Port. 2009 May-Jun;22(3):307-12. Portuguese. Epub 2009 Jul 15. PMID: 19686633.
6. Nicholson JA, Tappin J. Raccoon eyes in systemic AL amyloidosis. Br J Haematol. 2011 Jun;153(5):543. doi: 10.1111/j.1365-2141.2010.08489.x. Epub 2011 Apr 8. PMID: 21477158.
7. Eldho GC, Gailin BS, Mithun CM, Anoop TM, Sudha R. A case of dyspnea and periorbital rash. Proc (Bayl Univ Med Cent). 2012 Jul;25(3):248-9. doi: 10.1080/08998280.2012.11928842. PMID: 22754127; PMCID: PMC3377293.
8. Lavorato FG, Alves Mde F, Maceira JM, Unterstell N, Serpa LA, Azulay-Abulafia L. Primary systemic amyloidosis, acquired cutis laxa and cutaneous mucinosis in a patient with multiple myeloma. An Bras Dermatol. 2013 Nov-Dec;88(6 Suppl 1):32-5. doi: 10.1590/abd1806-4841.20132531. PMID: 24346874; PMCID: PMC3875965.
9. Colucci G, Alberio L, Demarmels Biasiutti F, Lämmle B. Bilateral periorbital ecchymoses. An often missed sign of amyloid purpura. Hamostaseologie. 2014;34(3):249-52. doi: 10.5482/HAMO-14-03-0018. Epub 2014 Jun 30. PMID: 24975676.
10. Hosoi T, Dhaliwal G, Tokuda Y. Amyloidosis in bilateral external auditory canals. BMJ Case Rep. 2014 May 13;2014:bcr2013200753. doi: 10.1136/bcr-2013-200753. PMID: 24825551; PMCID: PMC4024549.
11. Pereira VG, Jacinto M, Santos J, de Abreu TT. Periorbital purpura (raccoon's eyes). BMJ Case Rep. 2014 Aug 19;2014:bcr2013201407. doi: 10.1136/bcr-2013-201407. PMID: 25139913; PMCID: PMC4139576.
12. Inokuchi R, Tagami S, Maehara H. An elderly woman with bilateral raccoon eyes. Emerg Med J. 2016 Nov;33(11):781. doi: 10.1136/emermed-2015-205071. PMID: 28319930.
13. Kelsey A, Smith DH, Meng J, Murphy M, Rothe MJ. Amyloidosis: A story of how inframammary erosions eclipsed inconspicuous periorbital ecchymoses. Int J Womens Dermatol. 2016 Mar 2;2(1):18-22. doi: 10.1016/j.ijwd.2015.11.001. PMID: 28491996; PMCID: PMC5412114.
14. Forsyth CJ, Tiley CR. Raccoon eyes in systemic light chain amyloidosis. Med J Aust. 2017 May 15;206(9):384. doi: 10.5694/mja16.00848. PMID: 28490298.
15. Matsuura H, Anzai Y, Kuninaga N, Maeda T. Raccoon Eye Appearance: Amyloidosis. Am J Med. 2018 Jul;131(7):e305. doi: 10.1016/j.amjmed.2018.02.009. Epub 2018 Mar 14. PMID: 29550357.
16. Chandra A, Saha SK, Chakraborty U, Karmakar PS, Biswas G, Ray AK, Dutta S. Raccoon Eyes in Amyloidosis. Sultan Qaboos Univ Med J. 2020 Nov;20(4):e399-e400. doi: 10.18295/squmj.2020.20.04.021. Epub 2020 Dec 21. PMID: 33414950; PMCID: PMC7757934.
17. Mei S, Zhao Y, Li L, Mei C, Dai B. A 64-year-old woman with raccoon eyes following kidney biopsy: a case report. BMC Nephrol. 2020 Apr 17;21(1):140. doi: 10.1186/s12882-020-01770-4. PMID: 32303194; PMCID: PMC7165378.
18. Nguyen HT, Nguyen CTH. Cardiac amyloidosis mimicking acute coronary syndrome: a case report and literature review. Eur Heart J Case Rep. 2020 Oct 29;4(6):1-7. doi: 10.1093/ehjcr/ytaa325. PMID: 33442652; PMCID: PMC7793193.
19. Beatty PE, Killion L, Mc Hugh J, Tobin AM. Spontaneous bilateral peri-orbital purpura: an important clinical sign of primary systemic amyloidosis. BMJ Case Rep. 2021 Apr 1;14(4):e239478. doi: 10.1136/bcr-2020-239478. PMID: 33795269; PMCID: PMC8021580.
20. Chen-Xu M, Achilleos K. Periorbital purpura of AL amyloidosis. Rheumatology (Oxford). 2021 Mar 2;60(3):1165. doi: 10.1093/rheumatology/keaa545. PMID: 32944781.
21. Haász C, Kuroli E, Anker P, Márton DF, Szigeti Á, Nagy Z, Demeter J, Sárdy M, Medvecz M. Periorbitalis bőrtünetekkel járó könnyűlánc-amyloidosis [Case report of amyloid light-chain amyloidosis with periocular cutaneous involvement]. Orv Hetil. 2021 Aug 8;162(32):1303-1308. Hungarian. doi: 10.1556/650.2021.32137. PMID: 34370685.
22. LaTour D, Dao H, Limone B, Kivnick AR, Elsensohn A. Oral, periorbital, and inguinal purpura in a patient with paraproteinemia. JAAD Case Rep. 2022 Jul 2;27:79-81. doi: 10.1016/j.jdcr.2022.05.048. PMID: 35990230; PMCID: PMC9388871.
23. Mizuno S. Raccoon Eye Sign in Amyloid Light-chain Amyloidosis. Intern Med. 2022;61(15):2393. doi: 10.2169/internalmedicine.8927-21. Epub 2022 Aug 1. PMID: 35908961; PMCID: PMC9424071.
24. Sapkota S, Kuehl S, Pulluri B. Do Not Ignore Those Raccoon Eyes; They May Indicate Lethal AL Amyloidosis. Case Rep Oncol. 2022 Nov 11;15(3):1039-1048. doi: 10.1159/000527169. PMID: 36636680; PMCID: PMC9830280.
25. Jaber NF, Thelander U, Curman P. Extensive Amyloid Purpura: An Unusual Presentation of Myeloma-associated Light Chain Amyloidosis. Acta Derm Venereol. 2023 Jun 27;103:adv13367. doi: 10.2340/actadv.v103.13367. PMID: 37366561; PMCID: PMC10309058.
26. Mansilla-Polo M, Botella-Estrada R. Raccoon eyes as a diagnostic key for suspected amyloidosis. Rheumatol Adv Pract. 2023 Nov 28;8(1):rkad105. doi: 10.1093/rap/rkad105. PMID: 38076282; PMCID: PMC10710368.
27. Wang XF, Li T, Yang M, Huang Y. Periorbital purpura can be the only initial symptom of primary light chain amyloidosis: A case report. World J Clin Cases. 2024 Sep 16;12(26):5946-5951. doi: 10.12998/wjcc.v12.i26.5946. PMID: 39286381; PMCID: PMC11287502.
28. Senecal JB, Abou-Akl R, Allevato P, Mazzetti I, Hamm C, Parikh R, Woldie I. Amyloidosis: a case series and review of the literature. J Med Case Rep. 2023 Apr 21;17(1):184. doi: 10.1186/s13256-023-03886-1. PMID: 37081462; PMCID: PMC10120233.
29. Nader R, Angel-Korman A, Havasi A. Amyloidosis and the Kidney: An Update. Semin Nephrol. 2022 Nov;42(6):151343. doi: 10.1016/j.semnephrol.2023.151343. Epub 2023 May 4. PMID: 37148782.
30. Wangsa S, Novadian S, Zulkhair A. Renal amyloidosis: a narrative literature review. Bioscientia Medicina: J Biomed Transl Res. 2023;7(11):3753-3766.
31. Kumar N, Zhang NJ, Cherepanov D, Romanus D, Hughes M, Faller DV. Global epidemiology of amyloid light-chain amyloidosis. Orphanet J Rare Dis. 2022 Jul 19;17(1):278. doi: 10.1186/s13023-022-02414-6. PMID: 35854312; PMCID: PMC9295439.
32. Fotiou D, Dimopoulos MA, Kastritis E. Systemic AL Amyloidosis: Current Approaches to Diagnosis and Management. Hemasphere. 2020 Aug 10;4(4):e454. doi: 10.1097/HS9.0000000000000454. PMID: 32885146; PMCID: PMC7430233.
33. Aljama MA, Sidiqi MH, Dispenzieri A, Gertz MA, Lacy MQ, Buadi FK, Dingli D, Muchtar E, Fonder AL, Hayman SR, Hobbs MA, Gonsalves WI, Warsame RM, Kourelis T, Hwa YL, Kapoor P, Leung N, Go RS, Kyle RA, Rajkumar SV, Kumar SK. Comparison of different techniques to identify cardiac involvement in immunoglobulin light chain (AL) amyloidosis. Blood Adv. 2019 Apr 23;3(8):1226-1229. doi: 10.1182/bloodadvances.2019032458. PMID: 30975646; PMCID: PMC6482356.
34. Fotiou D, Theodorakakou F, Kastritis E. Biomarkers in AL Amyloidosis. Int J Mol Sci. 2021 Oct 9;22(20):10916. doi: 10.3390/ijms222010916. PMID: 34681575; PMCID: PMC8536050.
35. Aljama MA, Sidiqi MH, Dispenzieri A, Gertz MA, Lacy MQ, Buadi FK, Dingli D, Muchtar E, Fonder AL, Hayman SR, Hobbs MA, Gonsalves WI, Warsame RM, Kourelis T, Hwa YL, Kapoor P, Leung N, Go RS, Kyle RA, Rajkumar SV, Kumar SK. Comparison of different techniques to identify cardiac involvement in immunoglobulin light chain (AL) amyloidosis. Blood Adv. 2019 Apr 23;3(8):1226-1229. doi: 10.1182/bloodadvances.2019032458. PMID: 30975646; PMCID: PMC6482356.
36. Rajkumar SV, Dimopoulos MA, Palumbo A, Blade J, Merlini G, Mateos MV, Kumar S, Hillengass J, Kastritis E, Richardson P, Landgren O, Paiva B, Dispenzieri A, Weiss B, LeLeu X, Zweegman S, Lonial S, Rosinol L, Zamagni E, Jagannath S, Sezer O, Kristinsson SY, Caers J, Usmani SZ, Lahuerta JJ, Johnsen HE, Beksac M, Cavo M, Goldschmidt H, Terpos E, Kyle RA, Anderson KC, Durie BG, Miguel JF. International Myeloma Working Group updated criteria for the diagnosis of multiple myeloma. Lancet Oncol. 2014 Nov;15(12):e538-48. doi: 10.1016/S1470-2045(14)70442-5. Epub 2014 Oct 26. PMID: 25439696.
37. Rajkumar SV. Multiple myeloma: 2016 update on diagnosis, risk-stratification, and management. Am J Hematol. 2016 Jul;91(7):719-34. doi: 10.1002/ajh.24402. PMID: 27291302; PMCID: PMC5291298.
38. Muchtar E, Gertz MA, Kumar SK, Lacy MQ, Dingli D, Buadi FK, Grogan M, Hayman SR, Kapoor P, Leung N, Fonder A, Hobbs M, Hwa YL, Gonsalves W, Warsame R, Kourelis TV, Russell S, Lust JA, Lin Y, Go RS, Zeldenrust S, Kyle RA, Rajkumar SV, Dispenzieri A. Improved outcomes for newly diagnosed AL amyloidosis between 2000 and 2014: cracking the glass ceiling of early death. Blood. 2017 Apr 13;129(15):2111-2119. doi: 10.1182/blood-2016-11-751628. Epub 2017 Jan 26. PMID: 28126928; PMCID: PMC5391625.
39. Wechalekar AD, Schonland SO, Kastritis E, Gillmore JD, Dimopoulos MA, Lane T, Foli A, Foard D, Milani P, Rannigan L, Hegenbart U, Hawkins PN, Merlini G, Palladini G. A European collaborative study of treatment outcomes in 346 patients with cardiac stage III AL amyloidosis. Blood. 2013 Apr 25;121(17):3420-7. doi: 10.1182/blood-2012-12-473066. Epub 2013 Mar 11. PMID: 23479568.