Development of Multiple Myeloma of the IgA Type in a Patient with Cold Agglutinin Disease: Transformation or Coincidence?

Sefland, Øystein; Randen, Ulla; Berentsen, Sigbjørn

doi:10.1155/2019/1610632

Cited by 8 publications

(8 citation statements)

References 32 publications

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“…Only ∼7% of the cases show λ light chain restriction, while CA of the IgG class occurs in less than 5% (18,57). Monoclonal IgA is even rarer and may not be identical to the CA but rather a bystander (58)(59)(60). In CAS secondary to aggressive B-cell lymphoma, the light chain phenotype can be λ as well as κ (61).…”

Section: Immunological Properties Of Cold Agglutininsmentioning

confidence: 99%

New Insights in the Pathogenesis and Therapy of Cold Agglutinin-Mediated Autoimmune Hemolytic Anemia

2020

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Autoimmune hemolytic anemias mediated by cold agglutinins can be divided into cold agglutinin disease (CAD), which is a well-defined clinicopathologic entity and a clonal lymphoproliferative disorder, and secondary cold agglutinin syndrome (CAS), in which a similar picture of cold-hemolytic anemia occurs secondary to another distinct clinical disease. Thus, the pathogenesis in CAD is quite different from that of polyclonal autoimmune diseases such as warm-antibody AIHA. In both CAD and CAS, hemolysis is mediated by the classical complement pathway and therefore can result in generation of anaphylotoxins, such as complement split product 3a (C3a) and, to some extent, C5a. On the other hand, infection and inflammation can act as triggers and drivers of hemolysis, exemplified by exacerbation of CAD in situations with acute phase reaction and the role of specific infections (particularly Mycoplasma pneumoniae and Epstein-Barr virus) as causes of CAS. In this review, the putative mechanisms behind these phenomena will be explained along with other recent achievements in the understanding of pathogenesis in these disorders. Therapeutic approaches have been directed against the clonal lymphoproliferation in CAD or the underlying disease in CAS. Currently, novel targeted treatments, in particular complement-directed therapies, are also being rapidly developed and will be reviewed.

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Section: Immunological Properties Of Cold Agglutininsmentioning

confidence: 99%

New Insights in the Pathogenesis and Therapy of Cold Agglutinin-Mediated Autoimmune Hemolytic Anemia

2020

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“…31 Another patient had clinical CAD together with monoclonal IgA but turned out to have two independent clonal disorders, indicating that the CA was not identical to the clonal IgA. 60 This indicates that IgA-mediated CAD does not exist.…”

Section: Waihamentioning

confidence: 99%

Novel insights into the treatment of complement-mediated hemolytic anemias

Berentsen

Hill

et al. 2019

Therapeutic Advances in Hematology

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Complement-mediated hemolytic anemias can either be caused by deficiencies in regulatory complement components or by autoimmune pathogenesis that triggers inappropriate complement activation. In paroxysmal nocturnal hemoglobinuria (PNH) hemolysis is entirely complement-driven. Hemolysis is also thought to be complement-dependent in cold agglutinin disease (CAD) and in paroxysmal cold hemoglobinuria (PCH), whereas warm antibody autoimmune hemolytic anemia (wAIHA) is a partially complement-mediated disorder, depending on the subtype of wAIHA and the extent of complement activation. The pathophysiology, clinical presentation, and current therapies for these diseases are reviewed in this article. Novel, complement-directed therapies are being rapidly developed. Therapeutic terminal complement inhibition using eculizumab has revolutionized the therapy and prognosis in PNH but has proved less efficacious in CAD. Upstream complement modulation is currently being investigated and appears to be a highly promising therapy, and two such agents have entered phase II and III trials. Of these, the anti-C1s monoclonal antibody sutimlimab has shown favorable activity in CAD, while the anti-C3 cyclic peptide pegcetacoplan appears to be promising in PNH as well as CAD, and may also have a therapeutic potential in wAIHA.

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“…27,28 In our patient, the identification of the anti-JK a antibody and subsequent transfusion of Jk a -negative PRCs led to improvement and stabilization of hemoglobin. There was no further drop in hemoglobin, indicating that the presence of cold antibody was a silent bystander, 29 despite requiring multiple transfusions during diagnosis and peri-transplant period.…”

Section: Discussionmentioning

confidence: 88%

A Rare Case of Acute Hemolytic Anemia in a Patient with Newly Diagnosed Multiple Myeloma: Maintaining a Fine Balance between Occam's Razor and Hickam's Dictum

Ovett

Karunakaran

Kalaiyarasi

et al. 2022

Indian J Med Paediatr Oncol

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Anemia is a common feature in multiple myeloma and is multifactorial. A 52-year-old lady was admitted to our hospital with complaints of fatigue, exertional dyspnea, paresthesia, and a recent-onset confusion state. Fundus examination revealed features of hyperviscosity. The patient received 2 units of packed red blood cell transfusion (PRBC) before the present hospital admission. Laboratory investigations revealed severe anemia and thrombocytopenia. The M-protein was 5.8 g/dL. Bone marrow showed sheets of plasma cells. Immunofixation electrophoresis confirmed the presence of an IgAλ band. FISH was positive for IgH-FGFR3 fusion. The investigations confirmed multiple myeloma R-ISS stage III. The patient was immediately started on CyBorD chemotherapy regimen. The patient had indirect hyperbilirubinemia and symptomatic anemia. Initial testing of the patient's sample showed blood grouping discrepancy with DCT, ICT, and auto control positive. The symptomatic anemia persisted requiring PRC transfusions. Further antibody study revealed the presence of anti-Jka antibody—a warm IgG antibody and cold antibody. Subsequently, the patient received Jka antigen-negative B-positive compatible PRBC transfusions and the hemoglobin normalized. Our patient had transfusion-associated alloimmunization along with hyperviscosity. The timely recognition and early institution of plasmapheresis and myeloma-directed therapy along with transfusion of compatible Jka antigen-negative PRBC lead to rapid improvement.

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Development of Multiple Myeloma of the IgA Type in a Patient with Cold Agglutinin Disease: Transformation or Coincidence?

Cited by 8 publications

References 32 publications

New Insights in the Pathogenesis and Therapy of Cold Agglutinin-Mediated Autoimmune Hemolytic Anemia

New Insights in the Pathogenesis and Therapy of Cold Agglutinin-Mediated Autoimmune Hemolytic Anemia

Novel insights into the treatment of complement-mediated hemolytic anemias

A Rare Case of Acute Hemolytic Anemia in a Patient with Newly Diagnosed Multiple Myeloma: Maintaining a Fine Balance between Occam's Razor and Hickam's Dictum

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