Issue Archive

The treatment paradigms for multiple myeloma and amyloidosis have been transformed by novel therapies and continue to be modified in the face of new immunologic agents. In this How I Treat series on plasma cell dyscrasias edited by Associate Editor Hervé Avet-Loiseau, experts in the field discuss the rapidly changing treatment landscape for multiple myeloma and light-chain amyloidosis. Using illustrative cases, they review the role of autologous transplant, novel combination therapies, and the impact of new immunotherapy approaches to these complex and heterogeneous diseases.

Chronic granulomatous disease (CGD) is caused by mutations of the leukocyte NADPH oxidase 2 (NOX2) and is associated with increased infections. It is also characterized by sterile inflammation, the mechanism of which is poorly understood. In this Plenary paper, Bhattacharya et al demonstrate in a mouse model of CGD that normal alveolar macrophages (AMs) have an anti-inflammatory influence on lung inflammation and that AMs arising from NOX2-null monocytes stimulate inflammatory pathways, suggesting an explanation for excess non-infectious inflammation.

Therapy for essential thrombocythemia (ET) and polycythemia vera (PV) is aimed at reducing thrombotic risk through normalizing blood counts. Mascarenhas et al report on a randomized trial of hydroxyurea (HU) vs pegylated interferon α (PEG) in 168 patients with ET and PV who are at a high risk for vascular complications. They found that there is no significant difference between the 2 agents in CR rates at 12 months or in the rates of thrombotic events and disease progression. In an accompanying Perspective, Jason Gotlib places these results in the context of other studies, emphasizing the place of standard aspirin and phlebotomy, and speculating on the best use of HU, PEG, ruxolitinib, and novel agents in the treatment of ET and PV, while outlining gaps in our understanding that limit the certainty of management decisions.

The treatment paradigms for multiple myeloma and amyloidosis have been transformed by novel therapies and continue to be modified in the face of new immunologic agents. In this How I Treat series on plasma cell dyscrasias edited by Associate Editor Hervé Avet-Loiseau, experts in the field discuss the rapidly changing treatment landscape for multiple myeloma and light-chain amyloidosis. Using illustrative cases, they review the role of autologous transplant, novel combination therapies, and the impact of new immunotherapy approaches to these complex and heterogeneous diseases.

The treatment paradigms for multiple myeloma and amyloidosis have been transformed by novel therapies and continue to be modified in the face of new immunologic agents. In this How I Treat series on plasma cell dyscrasias edited by Associate Editor Hervé Avet-Loiseau, experts in the field discuss the rapidly changing treatment landscape for multiple myeloma and light-chain amyloidosis. Using illustrative cases, they review the role of autologous transplant, novel combination therapies, and the impact of new immunotherapy approaches to these complex and heterogeneous diseases.

The treatment paradigms for multiple myeloma and amyloidosis have been transformed by novel therapies and continue to be modified in the face of new immunologic agents. In this How I Treat series on plasma cell dyscrasias edited by Associate Editor Hervé Avet-Loiseau, experts in the field discuss the rapidly changing treatment landscape for multiple myeloma and light-chain amyloidosis. Using illustrative cases, they review the role of autologous transplant, novel combination therapies, and the impact of new immunotherapy approaches to these complex and heterogeneous diseases.

The treatment paradigms for multiple myeloma and amyloidosis have been transformed by novel therapies and continue to be modified in the face of new immunologic agents. In this How I Treat series on plasma cell dyscrasias edited by Associate Editor Hervé Avet-Loiseau, experts in the field discuss the rapidly changing treatment landscape for multiple myeloma and light-chain amyloidosis. Using illustrative cases, they review the role of autologous transplant, novel combination therapies, and the impact of new immunotherapy approaches to these complex and heterogeneous diseases.

Therapy for essential thrombocythemia (ET) and polycythemia vera (PV) is aimed at reducing thrombotic risk through normalizing blood counts. Mascarenhas et al report on a randomized trial of hydroxyurea (HU) vs pegylated interferon α (PEG) in 168 patients with ET and PV who are at a high risk for vascular complications. They found that there is no significant difference between the 2 agents in CR rates at 12 months or in the rates of thrombotic events and disease progression. In an accompanying Perspective, Jason Gotlib places these results in the context of other studies, emphasizing the place of standard aspirin and phlebotomy, and speculating on the best use of HU, PEG, ruxolitinib, and novel agents in the treatment of ET and PV, while outlining gaps in our understanding that limit the certainty of management decisions.

Chen et al describe a surprising role for lactate dehydrogenase A (LDHA) in regulating translation of platelet-specific proteins that influence megakaryocyte maturation and platelet number. This pathway may offer a novel target for the treatment of thrombocytopenia.

Sclerodermatous and pulmonary chronic graft-versus-host disease (cGVHD) is highly resistant to therapy. Previous studies suggest these fibrotic syndromes are germinal center dependent. Zaiken and colleages report that blocking germinal center development with BET bromodomain and EZH2 inhibitors improves these cGVHD syndromes in a murine model, setting the stage for clinical trials of these agents for the treatment of human cGVHD.