ATTR-CM in Patients With Clinically Significant Plasma Cell Dyscrasias

Figures

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  Visual Summary. Diversity in Presentation of Cardiac Amyloidosis Cardiac amyloidosis can present across a diverse clinical spectrum, including ATTR-CM, ATTR-CM with concomitant plasma cell dyscrasias, AL-CM, and dual AL and ATTR pathology. AL = light chain; AL-CM = light chain amyloidosis; ATTR-CM = transthyretin amyloidosis; MGUS = monoclonal gammopathy of undetermined significance; MM = multiple myeloma; sMM = smoldering multiple myeloma; TTR = transthyretin.

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  Figure 1. Cardiac Imaging and Biopsy Pathology From a Patient With Smoldering Multiple Myeloma and Hereditary (V122I) ATTR-CM Images from patient 2 in our case series, including (A) LGE on CMR in a nonischemic pattern at the base with multifocal subendocardial (white arrow), transmural inferolateral (open arrow), and right-ventricular side of the septum (white arrowhead). (B) CMR 4-chamber cine at end-diastole showing basal septal hypertrophy of 20 mm (double-headed white arrow, confirmed on short axis) with relative apical sparing. (C) SPECT-CT fusion short-axis image with PYP uptake in the inferoseptum (white arrowhead) and inferolateral wall (open arrow) in the same segments as LGE. (D) Second endomyocardial biopsy with Masson trichrome staining demonstrating a small amount of perimyocytic collagen (blue). No definite amyloid is identified. (E) Second endomyocardial biopsy with H&E staining demonstrating mild interstitial fibrosis and mild myocyte hypertrophy. CMR = cardiac magnetic resonance; H&E = hematoxylin and eosin; LGE = late gadolinium enhancement; PYP = technetium-99m pyrophosphate; SPECT-CT = single-photon emission computed tomography–computed tomography.

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  Figure 2. Cardiac Imaging and Skin Biopsy Pathology From a Patient Diagnosed With Multiple Myeloma and TTR on Skin Biopsy Images from patient 4 in our case series, including (A) CMR 4-chamber image showing left atrial wall enhancement (white arrow), typical of amyloidosis. (B) Short-axis blood null PSIR LGE image showing nonspecific nonischemic pattern of the subepicardial and midwall LGE in the basal inferoseptal wall extending into the right ventricle (white arrow). LGE in the anterior right ventricle insertion point (white arrowhead). (C) Skin biopsy demonstrating Congo red–positive amyloid deposition within the dermis under light microscopy (×400). (D) Immunohistochemistry with anti-TTR showing expression in dermis (×200). CMR = cardiac magnetic resonance; LGE = late gadolinium enhancement; PSIR = phase sensitive inversion recovery; TTR = transthyretin.