Clinically occult amyloidosis derived from leukocyte chemotactic factor 2 (ALECT 2) with cardiac involvement complicating renal transplantation: case report and literature review

Eletta, Olanrewaju; Ali, Mahmoud; Grieff, Anthony N.; Puri, Sonika; Matsuda, Kyle; Bongu, Advaith; Fyfe, Billie

doi:10.1016/j.carpath.2021.107375

Cited by 7 publications

(5 citation statements)

References 18 publications

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“…Unlike AL and ATTR, cardiac involvement by ALECT2 amyloidosis is exceedingly rare. 29 Therefore, despite the lack of specific treatment, the prognosis of ALECT2 amyloidosis is generally excellent. In contrast to AL amyloidosis, atraumatic splenic rupture is a rare event in ALECT2; perhaps this could be attributed to the relatively intact architecture in involved spleens.…”

Section: Discussionmentioning

confidence: 99%

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Proteomic Identification and Clinicopathologic Characterization of Splenic Amyloidosis

Chiu¹,

Dasari

Kurtin³

et al. 2022

American Journal of Surgical Pathology

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The spleen is a commonly encountered specimen in surgical pathology. However, little is known about the incidence, morphologic pattern, and clinical features of spleens involved by amyloidosis. We retrospectively identified 69 spleen amyloid cases typed using a proteomics-based method between 2008 and 2020. The frequency of amyloid types, clinicopathologic features, and distribution of amyloid deposits were assessed. Four amyloid types were detected: immunoglobulin light chain (AL) (N=30; 43.5%); leukocyte chemotactic factor 2 amyloidosis (ALECT2) (N=30; 43.5%); amyloid A (AA) (N=8; 11.6%); and fibrinogen alpha (AFib) (N=1; 1.4%). The splenic amyloid showed 5 distinct distribution patterns: (1) diffuse pattern, exhibited by most AL cases; (2) red pulp pattern, exhibited by most ALECT2 cases; (3) multinodular pattern, seen in subsets of AA and AL-kappa cases; (4) mass-forming pattern, seen in the AFib case; and (5) vascular only, seen in a subset of AA cases. Atraumatic splenic rupture was the most common reason for splenectomy in AL cases, while most ALECT2 spleens were removed incidentally during an unrelated abdominal surgery. Splenomegaly was significantly more common in AA spleens than in AL or ALECT2 spleens and was often the reason for splenectomy in this group. In conclusion, splenic amyloid may be underrecognized as it is often an incidental finding. Although, as expected, many of the spleens were involved by AL amyloidosis, ALECT2 emerged as another common spleen amyloid type. Although the spleen amyloid types exhibited characteristic distribution patterns, proteomics-based typing is warranted as some morphologic overlap still exists. Awareness of ALECT2 as a major spleen amyloid type is important for appropriate diagnostic workup and patient management.

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Section: Discussionmentioning

confidence: 99%

“…Also similar to hepatic ALECT2 amyloidosis, the clinical significance of splenic ALECT2 amyloidosis is unclear. Unlike AL and ATTR, cardiac involvement by ALECT2 amyloidosis is exceedingly rare 29. Therefore, despite the lack of specific treatment, the prognosis of ALECT2 amyloidosis is generally excellent.…”

Section: Discussionmentioning

confidence: 99%

Proteomic Identification and Clinicopathologic Characterization of Splenic Amyloidosis

Chiu¹,

Dasari

Kurtin³

et al. 2022

American Journal of Surgical Pathology

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Section: Other Organ System Involvementmentioning

confidence: 99%

“…None had deposition in the cardiac myocardium, brain, skin, or fibroadipose tissue. Only one case has been reported in the literature describing cardiac ALECT2 38 and another reported a rare presentation of pulmonary-renal syndrome. 39 It is conceivable that other Review Table 1 The pioneer cases or case series published on ALECT2 amyloidosis are enlisted with description of findings of histopathology, immunochemistry, immunofluorescence and tandem mass spectrometry…”

Section: Other Organ System Involvementmentioning

confidence: 99%

“…None had deposition in the cardiac myocardium, brain, skin, or fibroadipose tissue. Only one case has been reported in the literature describing cardiac ALECT238 and another reported a rare presentation of pulmonary-renal syndrome 39. It is conceivable that other clinically significant organ involvement aside from renal or hepatic is under-recognized.…”

Section: Introductionmentioning

confidence: 99%

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LECT-2 Amyloidosis: What do we Know?

Mann

Bhandohal

Cobos

et al. 2022

Journal of Investigative Medicine

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Amyloidosis is a rare group of diseases characterized by abnormal folding of proteins and extracellular deposition of insoluble fibrils. It can be localized to one organ system or can have systemic involvement. The kidney is the most common organ to be involved in systemic amyloidosis often leading to renal failure and the nephrotic syndrome. The two most common types of renal amyloidosis are immunoglobulin light chain-derived amyloidosis (AL) and reactive amyloidosis (AA). A novel form of amyloidosis (ALECT2) derived from leukocyte chemotactic factor 2 (LECT-2) and primarily involving the kidneys was first described by Benson et al in 2008. The liver was subsequently identified as the second most common organ involved in ALECT2 amyloidosis. LECT-2 is a unique protein that can form amyloid deposits even in its unmutated form. Patients with ALECT2 present with minimal proteinuria in contrast to other forms of amyloidosis especially AL and AA. They may present with slightly elevated serum creatinine. Nephrotic syndrome and hematuria are rare. ALECT2 can be found in association with other types of amyloidosis as well as malignancies or autoimmune diseases. ALECT2 may be confused with amyloidosis associated with light and heavy chain monoclonal gammopathy if the immunofluorescence is positive with anti-light chain and anti-AA sera. The other organs involved are the duodenum, adrenal gland, spleen, prostate, gall bladder, pancreas, small bowel, parathyroid gland, heart, and pulmonary alveolar septa, but consistently uninvolved organs included brain and fibroadipose tissue. A renal biopsy along with characteristic features found on immunohistochemistry and mass spectrometry is diagnostic of ALECT2. ALECT2 should be suspected when all markers for AL and AA are negative. Proper diagnosis of ALECT2 can determine need for supportive care versus more aggressive interventions.

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ALECT2 amyloidosis with concurrent IgG4-related interstitial nephritis, membranous nephropathy and diabetic kidney disease: a case report and literature review

Shaheen,

Bharat,

Friedman

et al. 2024

J Nephrol

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Clinically occult amyloidosis derived from leukocyte chemotactic factor 2 (ALECT 2) with cardiac involvement complicating renal transplantation: case report and literature review

Cited by 7 publications

References 18 publications

Proteomic Identification and Clinicopathologic Characterization of Splenic Amyloidosis

Proteomic Identification and Clinicopathologic Characterization of Splenic Amyloidosis

LECT-2 Amyloidosis: What do we Know?

ALECT2 amyloidosis with concurrent IgG4-related interstitial nephritis, membranous nephropathy and diabetic kidney disease: a case report and literature review

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