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BLOOD COMMENTARIES

PLENARY PAPER

Kaposiform hemagioendothelioma (KHE) is a rare, pediatric vascular neoplasm frequently associated with thrombocytopenia and consumptive coagulopathy, termed Kasabach-Merritt phenomenon (KMP), predicting a poor prognosis. In this Plenary paper, Ji and colleagues present results of a trial of 73 patients with KHE randomized to sirolimus plus or minus prednisolone for the treatment of KFE/ KMP, indicating that patients receiving combined therapy have markedly improved platelet and tumor responses.

PERSPECTIVE

In this timely Perspective, Trotman and Pettitt discuss the role of FDG-PET in the management of follicular lymphoma. They review its role in diagnosis, for end-of-induction assessment of response and follow-up post-remission. They outline the path forward for integrating PET into treatment decision-making to more effectively treat patients with poor prognosis and to consider limiting therapy in patients with good prognosis at risk of being overtreated.

BLOOD SPOTLIGHT

Chronic graft-versus-host disease (cGVHD) is the major complication of allogeneic stem cell transplantation, and effective therapies are limited. In this Blood Spotlight, Zeiser and Lee review three drugs that have been approved by the FDA in the past 4 years: ibrutinib, belumosudil, and ruxolitinib. They summarize the development and clinical testing of these drugs and discuss their role in the treatment of cGVHD.

CLINICAL TRIALS AND OBSERVATIONS

Older adults with acute myeloid leukemia (AML) have worse outcomes than their younger counterparts, and the impact of geriatric assessment in predicting outcomes is controversial. In this month's CME article, Min et al report on extended geriatric assessment in over 100 older patients with AML, demonstrating that assessment of physical function, depression, and cognitive impairment may improve survival prediction.

HEMATOPOIESIS AND STEM CELLS

IMMUNOBIOLOGY AND IMMUNOTHERAPY

The prognosis of relapsed acute myeloid leukemia (AML) following allogeneic stem cell transplant is very poor, and salvage chemotherapy and donor lymphocyte infusion (DLI) are rarely curative. Previous studies suggest that ex vivo cytokine-stimulated natural killer (NK) cells become memory-like (ML) NK cells with enhanced anti-leukemia activity. Bednarski and colleagues report promising Phase 1 results in 9 pediatric/young adult patients treated with DLI and donor ML NK cells, suggesting a novel treatment approach for relapsed AML following allogeneic transplant that should be further explored.

LYMPHOID NEOPLASIA

Patients with follicular lymphoma (FL) who progress within 24 months of front-line therapy (POD24) have poor outcomes, but clinical factors predicting early relapse are not established. Casulo et al analyzed pooled data from 13 clinical trials and validated POD24 as a predictor of poor prognosis and identified male gender, unfavorable performance status, elevated β-2 microglobulin, and high FLIPI score as baseline predictors of POD24, laying the groundwork for the development of better predictive models at diagnosis to guide therapy.

MYELOID NEOPLASIA

PHAGOCYTES, GRANULOCYTES, AND MYELOPOIESIS

Chronic granulomatous disease (CGD) results from loss of NADPH oxidase and is associated with altered phagocyte function and exaggerated inflammation. Gibbings et al demonstrate that monocyte-derived macrophages (MoMacs) from CGD mice are recruited normally following peritoneal zymogen injection but fail to mature, maintaining an inflammatory phenotype and forming pyrogranulomata. Interestingly, this is not cell-intrinsic but appears to be driven by the milieu, as CGD MoMacs transferred into inflamed peritoneum of wildtype mice mature normally.

THROMBOSIS AND HEMOSTASIS

TRANSPLANTATION

VASCULAR BIOLOGY

Dosunmu-Ogunbi and colleagues report that a common genetic variant in mitochondrial superoxide dismutase 2 (SOD2) is associated with increased cardiac dysfunction in patients with sickle cell disease (SCD). This SOD2 variant increases production of reactive oxygen species leading to increased oxidant stress. This could be an important biomarker indicating predilection to cardiovascular dysfunction in patients with SCD.

LETTER TO BLOOD

BLOOD WORK

ERRATA

CONTINUING MEDICAL EDUCATION (CME) QUESTIONS

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