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23 August 2021: Articles

Hepatic AL Amyloidosis without Significant Light Chain Elevation in a Patient Treated with CyBorD Plus Daratumumab

Unusual clinical course, Rare disease

Brad Rybinski A* , Mehmet Kocoglu A

DOI: 10.12659/AJCR.933241

Am J Case Rep 2021; 22:e933241

Table 1. Results obtained during admission confirming diagnosis of AL amyloidosis with amyloid deposition in the liver and bone marrow. All results were obtained prior to the initiation of treatment. Normal values, when applicable, are provided in the left-hand column.

Bone marrow biopsyThe marrow is mostly replaced by pink, amorphous proteinaceous material, consistent with amyloid. The Congo Red stain is positive for amyloid deposition in the interstitial areas as well as in vascular walls.
Liver biopsyThe liver shows marked amyloid deposition within sinusoidal spaces, highlighted on Congo Red and trichrome stains. This is associated with extensive loss of hepatic parenchyma. Liquid chromatography tandem mass spectrometry (LC MS/MS) was performed on peptides extracted from Congo Red-positive, micro-dissected areas of the paraffin-embedded specimen. LC MS/MS detected a peptide profile consistent with AL (lambda)-type amyloid deposition.
Alkaline phosphatase (U/L)Normal: 38–126309 day of admission, 357 day of discharge, maximum during admission 379
Bilirubin (total) (mg/dL)Normal: 0.3–1.20.9 day of admission, 11 day of discharge, maximum during admission 17.3
Bone marrow biopsyHypocellular marrow (5%) with tri-linage hypoplasia
Platelets (K/mcL)Normal: 153–367Platelets: 294 day of admission, 522 day of discharge, minimum during admission 20
PT (seconds)Normal: 12.1–15.0PT: 15.6 day of admission, 15.6 day of discharge, maximum during admission 18.8
Transaminases (U/L)Normal AST: 14–36Normal ALT: 0–3475 day of admission, 131 day of discharge, maximum during admission 1441 42 day of admission, 64 day of discharge, maximum during admission 903
Bone marrow biopsyBased on CD138 and CD38, flow cytometric studies show a population of plasma cells (2% of the leukocytes in the flow cytometry preparation) which are lambda-restricted. CD138 immunohistochemistry was difficult to interpret due to diffuse staining within the amyloid deposits.In-situ hybridization studies for Kappa and Lambda light chains reveal that the plasma cells are lambda light chain-restricted (kappa: lambda ratio of 1: 4). MUM-1 stain: Plasma cells account for approximately 5% of the cellularity.
Bone marrow biopsy cytogeneticsThree copies of 1q21 (17%), 3 copies of intact MYC (33%), and 3 copies of IGH with one copy missing the 5’-IGH (46%). A hyperdiploid subclone showing the same abnormalities was noted in a small number of cells. There was no evidence of loss of 13q or TP53, and no FGFR3-IGH or CCND1-IGH rearrangement in this specimen.
Free light chains (mg/L)Normal kappa: 3.30–19.40Normal lambda: 5.70–26.30Normal kappa/lambda ratio: 0.26–1.65Kappa/lambda: 12.11/15.81, Ratio=0.77
Serum protein electrophoresis, monoclonal protein (g/dL)Normal: 00.63
Urine protein electrophoresisNormal: 0+ for monoclonal free Lambda light chains

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American Journal of Case Reports eISSN: 1941-5923
American Journal of Case Reports eISSN: 1941-5923