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Multiple Myeloma
Published in Pat Price, Karol Sikora, Treatment of Cancer, 2020
Biochemical screening may show abnormalities of urea, creatinine, calcium, uric acid, total protein, and/or albumin. Serum ALP is usually normal but is commonly raised after a fracture. Electrophoresis of serum and serum free light-chain assay or urine testing in all patients to look for monoclonal light chain is important since patients with Bence Jones–only myeloma have no paraprotein in the serum and patients with a serum paraprotein are at greater risk of renal failure if they also produce Bence Jones protein (BJP). The paraprotein in the serum and/or urine is then confirmed and typed by immunofixation. The paraprotein is of IgG subtype in 55–60% of cases and IgA in 20–25%, of whom two-thirds also have BJP in the urine and 95% have a clonal serum free light chain. Rarely, the paraprotein may be of IgD, IgE, or IgM type. Overall two-thirds of paraproteins have kappa light chains and one-third lambda. Twenty percent of cases are Bence Jones–only myeloma. In less than 1% of patients, no paraprotein is detectable in either serum or urine (non-secretory myeloma).
Familial Multiple Myeloma
Published in Dongyou Liu, Handbook of Tumor Syndromes, 2020
Presence of monoclonal (M) protein (consisting of 50% IgG, 20% IgA, 20% immunoglobulin light chain, 2% IgD, and 0.5% IgM) in the serum or urine is suggestive of multiple myeloma, with detection sensitivity ranging from 82% by SPEP and 93% by serum IFE to 97% by either adding SFLC assay or a 24-h urine protein electrophoresis with immunofixation. About 2%–3% of multiple myeloma has no detectable M protein (so-called non-secretory multiple myeloma) [10].
Diagnostic applications of immunology
Published in Gabriel Virella, Medical Immunology, 2019
Ajay Grover, Virginia Litwin, Gabriel Virella
Immunofixation (immunoblotting) is a multistep process (Figure 15.2). In the first step, several aliquots of the patient's serum are simultaneously separated by electrophoresis. One of the separation lanes is stained as reference for the position of the different serum proteins (extreme right lane in the figure), while paper strips embedded with different antibodies are laid over the remaining separation lanes. The antibodies diffuse into the agar and react with the corresponding immunoglobulins. After washing off unbound immunoglobulins and antibodies, the lanes where immunofixation take place are stained, revealing whether the antisera did or did not recognize the proteins they are directed against. In this example, a monoclonal protein reacting with anti-IgG and anti-λ antisera was revealed. This test is useful in diagnosing myeloma, macroglobulinemia of Waldenström, and other plasma cell dyscrasias where the detection and characterization of a monoclonal gammopathy are essential for the diagnosis.
Strong positive light chain immunostaining in a patient with transthyretin amyloidosis
Published in Hematology, 2023
Jiao Chen, Haifei Chen, Lingyun Zhou, Danbo Liu, Fang Du, Hongxian Xiang
Ten months later, he was admitted to third hospital. His laboratory test results were as follows: NT-ProBNP, 682.2 pg/mL; cTnT, 0.054 ng/mL; serum β2-microglobulin, 2.73 Ug/mL; urine kappa free light chain (FLC), 29.9 mg/L and urine lambda FLC, 6.45 mg/L. Further, serum and urine immunofixation electrophoresis (IFE) and urinary protein electrophoresis results were negative. Urinary Bence–Jones protein, 24-h urinary total protein, 24-h urinary albumin and 24-h urinary FLC levels were normal. Notably, no evidence of clonal plasma cells was found in bone marrow morphology and biopsy. Congo red staining of the bone marrow biopsy specimen was negative. Abdominal fat pad biopsy specimen was positive by Congo red staining and exhibited apple-green birefringence under polarised light microscopy (Figure 1C,D). The specimen was positively stained for kappa light chain in mesenchyme and negatively stained for lambda light chain (Figure 1 E,F). Further, owing to the IHC positive results, he was diagnosed with stage 1 systemic AL amyloidosis. Subsequently, he received three cycles of chemotherapy with CyborD regimen (bortezomib 2.2 mg subcutaneous injection; cyclophosphamide 300 mg PO; dexamethasone 40 mg PO, at days 1, 8, 15, 22,and 35 days as one cycle). However, he experienced deteriorating of numbness in all four extremities since the start of chemotherapy.
Therapy-related acute lymphoblastic leukemia following treatment for multiple myeloma – diagnostic and therapeutic dilemma
Published in Acta Oncologica, 2022
Alicja Sadowska-Klasa, Mary Abba, Justyna Gajkowska-Kulik, Jan Maciej Zaucha
He was started on induction chemotherapy according to the Polish Adult Leukemia Group (PALG) protocol for patients above 55 years old (dexamethasone, vincristine, daunorubicin, peg-asparaginase, rituximab), followed by two consolidations mini-FLAM (fludarabine, cytarabine, mitoxantrone) with rituximab intermittent with intermediate-dose methotrexate, cytarabine, and rituximab. He achieved CR with a negative ALL-MRD of 0.003%. but with a residual very small population of abnormal plasmocytes – MM-MRD of 0.05%. The patient was referred for allo-HCT from a matched related donor (sister, 55 years old, two pregnancies, minor ABO incompatibility). He was conditioned with a reduced regimen (thiotepa 10 mg/kg, busulfan 8 × 0.8 mg/kg, and fludarabine 150 mg/m2) together with anti-thymocyte globulin 2.5 mg/kg, and received standard post-transplant immunosuppression with cyclosporine and a short course of methotrexate. Engraftment was reached on day 18. Routine bone marrow examination at day 30 was normal with full donor chimerism and MRD 0% for ALL and 0.016% for MM. Immunofixation remained negative. Immunosuppressive treatment was discontinued 6 months after transplant. At the last follow-up 6 months later (>18 months from tr-ALL diagnosis), the patient remains in deep remission of both diseases with a good performance status without any severe transplant complications.
Bone marrow amyloid: a comprehensive analysis of 1,469 samples, including amyloid type, clinical features, and morphologic distribution
Published in Amyloid, 2022
April Chiu, Surendra Dasari, Paul J. Kurtin, Jason D. Theis, Julie A. Vrana, Angela Dispenzieri, Karen L. Rech, Linda N. Dao, Matthew T. Howard, Martha Grogan, Ellen D. McPhail
Of the intramural cohort, 355 cases had clinical data available to assess all of the following: (i) cardiac involvement by amyloid; (ii) presence of a monoclonal protein of unknown significance (determined via serum/urine protein electrophoresis and/or immunofixation only); and (iii) at least a minimum of 90-days of follow up to perform survival analysis. Bona fide involvement by cardiac amyloidosis was defined as presence by echocardiography of a mean intact ventricular septum (IVS) >12mm, abnormal echocardiography findings consistent with amyloid/infiltrative cardiomyopathy, or N-terminal pro-brain natriuretic peptide (NT-proBNP) > 332pg/mL. In parallel, we utilised serum cardiac troponin T (cTnT) and NT-proBNP measurements to stage the cardiac involvement by the AL and ATTR amyloidosis, as described previously (i.e. modified Mayo 2004 Stage for AL and Grogan’s ATTRwt Stage for ATTR) [20,21].