Table 1.

Summary of Clinical and Laboratory Data for the Described Clinical Cases^a

Characteristic	Case 1	Case 2	Case 3	Case 4
Age (y)	46	75	71	54
Sex	Male	Male	Female	Female
Race	Black	White	White	Black
Onset of symptoms before the diagnosis of plasma cell dyscrasia	2 y (Dyspnea) Pulmonary artery hypertension	2 y (Dyspnea) Pulmonary artery hypertension	1 y (Dyspnea) Pulmonary artery hypertension	4 mo (Dyspnea, lower extremity edema) Restrictive cardiomyopathy Pulmonary artery hypertension
Diagnosis of MGUS	No	No	Yes	No
Laboratory data
BNP level (<100 pg/mL)	1433	979	1125	805
Troponin I level (<0.02 ng/mL)	0.05	<0.02	0.085	0.074
NT-proBNP level (<300 pg/mL)	NA	NA	23,899	NA
Troponin T level (<0.01 ng/mL)	<0.01	NA	0.08	NA
Hemoglobin level (g/dL)	13	11.7	13.3	10.6
Calcium level (mg/dL)	9.7	9.4	9.8	9.6
Serum creatinine level (mg/dL)	1.5	2.2	1.7	0.8
Serum monoclonal-spike	Present (1.81 g/dL)	Present (4.1 g/dL)	Absent	Present (2 g/dL)
Kappa light chain (mg/L)	184	2266	19.7	1.5
Lambda light chain (mg/L)	23.8	3.1	948	519
Serum free light chain ratio	7.76	731.23	0.02	0.002
Urine monoclonal-spike	Present	Present	Present	Present
Urine protein level (24 h)	10 g	937 mg	248 mg	NA
Immunoglobulins
IgA (66-436)	150	22	129	24
IgG (694-1618)	2693	5326	647	3067
IgM (45-281)	72	<10	29	<25
CRAB criteria	Absent	Present Renal insufficiency	Present Renal insufficiency (eGFR <30 mL/min per 1.73 m2)	Present Anemia
Electrocardiography	Normal sinus rhythm RVH	Atrial fibrillation Right bundle branch block	Normal sinus rhythm (82 beats/min) Low QRS voltage Right axis deviation Poor R-wave progression	Normal sinus rhythm Poor R-wave progression
2D echocardiography	Normal LV systolic function Moderate concentric LVH RV dilated and severely depressed function	Normal LV systolic function and wall thickness RV dilated and function severely depressed	Moderate concentric LVH LVEF 35%-40% Small pericardial effusion	Mild concentric LVH LVEF >55% RVSP 72 mm Hg Normal RV systolic function
Right heart catheterization
RA pressure (mm Hg)	19	21	20	11
PA pressure (systolic/diastolic/mean)	87/30/50	88/41/57	79/34/50	61/25/40
PCWP (mm Hg)	10	16	26	18
Fick cardiac index (L/min per m2)	2.46	1.43	1.8	2.16
PVR (WU)	7.6 Precapillary	14 Combined pre- and postcapillary	5.7 Combined pre- and postcapillary	6 Combined pre- and postcapillary
Bone marrow biopsy
Plasma cells	20% monoclonal plasma cells	30% monoclonal plasma cells	10%-15% monoclonal plasma cells	90% plasma cells
Congo red stain	Negative	Negative	Negative	Not performed
Endomyocardial biopsy	Not done	Not done	Congo red–positive deposits Mass spectrometry: AL (lambda) type	Not performed
Renal biopsy	Global and segmental glomerulosclerosis, interstitial fibrosis, tubular atrophy Immunofluorescence staining positive for kappa LC; Congo red staining negative for amyloid	NA	NA	NA
Treatment	Bortezomib	Bortezomib	Bortezomib	Induction chemotherapy (RVD) Autologous bone marrow transplant

^a2D = 2-dimensional; BNP = brain natriuretic peptide; CRAB = criteria hypercalcemia (>11.5 mg/dL), renal insufficiency (serum creatinine level > 2 mg/dL or eGFR < 40 mL/min per 1.73 m², anemia (hemoglobin level < 10 g/dL), bony lesion >1 site osteolytic lesions on skeletal survey; D = diastolic; eGFR = estimated glomerular filtration rate; LC = light chain; LV = left ventricular; LVEF = left ventricular ejection fraction; LVH = left ventricular hypertrophy; M = mean; MGUS = monoclonal gammopathy of undetermined significance; NA = not available; NT-proBNP = N-terminal pro–hormone brain natriuretic peptide; PA = pulmonary artery; PCWP = pulmonary capillary wedge pressure; PVR = pulmonary vascular resistance; RA = right atrium; RV = right ventricular; RVD = Revlimid + Velcade + dexamethasone; RVH = right ventricular hypertrophy; RVSP = right ventricular systolic pressure; S = systolic; WU = wood unit.