PMC6109557_01
Serum electrophoresis and immunofixation. (a) Case 1: immunofixation shows staining in the gamma region, without a corresponding stain in the light chain region. Two bands are detected in the gamma region, a major and a second minor band. (b) Case 2: immunofixation shows a broad stain in the alpha-beta region, without a corresponding region in the light chain. Immunofixation further identifies an IgMkappa paraprotein. ELP = protein electrophoresis; G: immunofixation with anti-gamma chain; identifying gamma heavy chain; A: anti-alpha chain immunofixation; M: anti-mu chain immunofixation; K: anti-kappa chain immunofixation; L: anti-lambda chain immunofixation.
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fig1
Capillary zone electrophoresis and immunotyping of Case 1. (a) Capillary zone electrophoresis showing a peak in the beta1 region and a minor peak in the alpha region. (b) Immunotyping showing a subtraction by the anti-IgG antibody, without a corresponding region in the light chain region, identifying a gamma chain peak. Ref: protein electrophoresis. IgG: immunosubtraction with anti-IgG antibody; IgA: immunosubtraction with anti-IgA antibody; IgM: immunosubtraction with anti-IgM antibody; K: immunosubtraction with anti-kappa antibody; L: immunosubtraction with anti-lambda antibody.
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fig2
Gel protein electrophoresis and immunofixation (Figure 1(a)) and capillary zone electrophoresis and immunotyping (Figures 2(a) and 2(b)) (Sebia, UK) identified 2 gamma-heavy chains.
Urine electrophoresis and immunofixation. (a) Case 1: urine electrophoresis revealing a broad band and a minor band in the heavy chain region, without a corresponding band in the light chain region. (b) Case 2: urine electrophoresis revealing a broad band in the heavy chain region without a corresponding band in the light chain region. A further kappa light chain band is observed, corresponding to the IgMkappa band identified in the serum. ELP: reference track showing sample proteins; GAM: trivalent antiserum against gamma, alpha, and mu heavy chains; K and L: antisera against bound kappa and lambda light chains; KI and LI: antisera against free kappa and free lambda light chains.
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fig3
UK) and confirmed by a second gel electrophoresis method (Helena, UK). Urine immunofixation identified the gamma-heavy chain and kappa light chain (Figure 3(b)).
Bone marrow stain of Case 1. (a) Giemsa stain. (b) Small lymphocytes and scattered unusually small plasma cells. (c) CD138: plasma cells forming a characteristic corona around a lymphoid nodule. (d) Lambda stain:hardly any positive cells around the lymphoid nodule. (e) Kappa stain:hardly any positive cells around the lymphoid nodule. (f) IgG stain:a rim of strongly staining plasma cells. (g) Same area stained with MUM1 marker. Stains show there are IgG(+), kappa(-) and lambda (-) cells.
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fig4
Bone marrow biopsy showed subtle infiltrates by low-grade B-NHL, associated with groups of clonal plasma cells, which expressed neither kappa nor lambda chain but stained with IgG heavy chain (Figures 4(a)-4(g)).
PMC6109557_02
Serum electrophoresis and immunofixation. (a) Case 1: immunofixation shows staining in the gamma region, without a corresponding stain in the light chain region. Two bands are detected in the gamma region, a major and a second minor band. (b) Case 2: immunofixation shows a broad stain in the alpha-beta region, without a corresponding region in the light chain. Immunofixation further identifies an IgMkappa paraprotein. ELP = protein electrophoresis; G: immunofixation with anti-gamma chain; identifying gamma heavy chain; A: anti-alpha chain immunofixation; M: anti-mu chain immunofixation; K: anti-kappa chain immunofixation; L: anti-lambda chain immunofixation.
CRIHEM2018-4832619.001.jpg
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fig1
Urine electrophoresis and immunofixation. (a) Case 1: urine electrophoresis revealing a broad band and a minor band in the heavy chain region, without a corresponding band in the light chain region. (b) Case 2: urine electrophoresis revealing a broad band in the heavy chain region without a corresponding band in the light chain region. A further kappa light chain band is observed, corresponding to the IgMkappa band identified in the serum. ELP: reference track showing sample proteins; GAM: trivalent antiserum against gamma, alpha, and mu heavy chains; K and L: antisera against bound kappa and lambda light chains; KI and LI: antisera against free kappa and free lambda light chains.
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fig3
UK) and confirmed by a second gel electrophoresis method (Helena, UK). Urine immunofixation identified the gamma-heavy chain and kappa light chain (Figure 3(b)).
Paraprotein levels of Case 2 during treatment. Hospital records for the patient date back to time = 0 after chemotherapy. R-CVP = rituximab with cyclophosphamide, vincristine, and prednisolone.
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fig5
Details of her treatment are given in Figure 5.
Capillary zone electrophoresis and immunotyping of Case 2. (a) Capillary zone electrophoresis prior to the appearance of the gamma heavy chain. A peak identified as the IgMkappa protein is detected in the gamma region. (b) Capillary zone electrophoresis identifies a broad peak in the beta region, identified as gamma heavy chain by immunotyping. (c) Immunotyping identifies a gamma heavy chain without a corresponding light chain and an IgMkappa paraprotein.
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fig6
Capillary zone electrophoresis prior to the development of gamma-HCD is shown in Figure 6(a).
Bone marrow stain of Case 2. (a) CD138 highlights plasma cells:here admixed with lymphocytes (which are CD138 negative). Note also granular golden-brown haemosiderin deposits. (b) CD56:some plasma cells are positive; this is an interesting finding, as plasma cells of lymphoplasmacytic lymphoma usually do not express this marker. It indirectly suggests that the "other clone" may be more of plasma cell myeloma-like (myeloma-like MGUS). Haemosiderin which is naturally yellow/brown is present as well.
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fig7
Her bone marrow results are shown in Figures 7(a) and 7(b) and in the Supplementary Material (available here).