Table 1

Clinical and pathologic characteristics of study patients

Patient no.Age (y)/ sexMYD88 L265P mutationOrgans involved2012 Revised Mayo AL amyloidosis stageMonoclonal protein in serumUrine total protein (g/24 h)Clonal cells in bone marrow*OS from diagnosis (mo)Progression
Isotypeg/dLY/NPFS (mo)
68/F Positive Peripheral nervous system II IgM λ 2.4 0.05 Clonal B lymphocytes and clonal plasma cells 109 76 
70/M Positive Peripheral and autonomic nervous system IgM λ 0.9 0.98 Clonal B lymphocytes and clonal plasma cells 43 43 
63/F Positive Spleen and kidney IgM λ IgA λ 0.3 8.87 Clonal plasma cells 83 28 
52/M Positive Peripheral and central nervous system IgM κ 0.5 0.08 Clonal B lymphocytes 78 67 
48/F Positive Kidney and autonomic nervous system II IgM κ 1.5 3.65 Clonal B lymphocytes and clonal plasma cells 62 48 
65/M Positive Lungs and lymph node IgM λ 1.4 0.06 Clonal B lymphocytes 41 33 
56/M Positive Lymph nodes and soft tissue IgM κ 1.6 0.12 Clonal plasma cells and clonal B lymphocytes 26 26 
65/M Positive Kidney IgM κ 2.4 9.59 Clonal plasma cells and clonal B lymphocytes 30 30 
60/M Positive Kidney IgM κ IgM λ 1.1 1.66 Clonal plasma cells and clonal B lymphocytes 12 12 
10 60/M Positive Heart and tongue III IgM κ 1.1 0.07 Clonal B lymphocytes and clonal plasma cells 21 21 
11 62/F Negative Kidney IgM κ 0.6 5.90 Clonal plasma cells 104 104 
12 53/M Negative Kidney, heart and gastrointestinal tract III IgM κ 0.1 34.9 Clonal plasma cells 16 14 
13 60/M Negative Heart, lymph nodes, and tongue II IgM κ IgM λ 0.5 0.20 Clonal plasma cells 120 120 
14 56/M Negative Kidney IgM λ 0.9 6.19 Clonal plasma cells and clonal B lymphocytes 55 55 
Patient no.Age (y)/ sexMYD88 L265P mutationOrgans involved2012 Revised Mayo AL amyloidosis stageMonoclonal protein in serumUrine total protein (g/24 h)Clonal cells in bone marrow*OS from diagnosis (mo)Progression
Isotypeg/dLY/NPFS (mo)
68/F Positive Peripheral nervous system II IgM λ 2.4 0.05 Clonal B lymphocytes and clonal plasma cells 109 76 
70/M Positive Peripheral and autonomic nervous system IgM λ 0.9 0.98 Clonal B lymphocytes and clonal plasma cells 43 43 
63/F Positive Spleen and kidney IgM λ IgA λ 0.3 8.87 Clonal plasma cells 83 28 
52/M Positive Peripheral and central nervous system IgM κ 0.5 0.08 Clonal B lymphocytes 78 67 
48/F Positive Kidney and autonomic nervous system II IgM κ 1.5 3.65 Clonal B lymphocytes and clonal plasma cells 62 48 
65/M Positive Lungs and lymph node IgM λ 1.4 0.06 Clonal B lymphocytes 41 33 
56/M Positive Lymph nodes and soft tissue IgM κ 1.6 0.12 Clonal plasma cells and clonal B lymphocytes 26 26 
65/M Positive Kidney IgM κ 2.4 9.59 Clonal plasma cells and clonal B lymphocytes 30 30 
60/M Positive Kidney IgM κ IgM λ 1.1 1.66 Clonal plasma cells and clonal B lymphocytes 12 12 
10 60/M Positive Heart and tongue III IgM κ 1.1 0.07 Clonal B lymphocytes and clonal plasma cells 21 21 
11 62/F Negative Kidney IgM κ 0.6 5.90 Clonal plasma cells 104 104 
12 53/M Negative Kidney, heart and gastrointestinal tract III IgM κ 0.1 34.9 Clonal plasma cells 16 14 
13 60/M Negative Heart, lymph nodes, and tongue II IgM κ IgM λ 0.5 0.20 Clonal plasma cells 120 120 
14 56/M Negative Kidney IgM λ 0.9 6.19 Clonal plasma cells and clonal B lymphocytes 55 55 

F, female; M, male.

*

Clonality was assessed by immunohistochemistry and flow cytometry from bone marrow specimens.

Calculated using serum troponin T, N-terminal pro–brain natriuretic peptide, and difference between involved and uninvolved free light chains.

Patients alive.

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