Multiple myeloma (MM) is a plasma cell disorder that is on the rise throughout the world, especially in the US, Australia, and Western Europe. In the US, MM accounts for almost 2% of cancer diagnoses and over 2% of cancer deaths (more than double the global proportion). Incidence has risen by 126% globally and over 40% in the US since 1990, while global mortality has risen by 94% and US mortality has fallen by 18%. The 5 year survival in the US has more than doubled over the past decades with the introduction of new targeted therapies and transplant techniques. Risk factors for MM include age (average age of diagnosis is 69), race (African Americans are over double as likely to be diagnosed), sex (men are at a 1.5× risk), and family history. Diagnosis includes serum or urine electrophoresis and free light-chain assay but requires bone marrow biopsy. It is distinguished from smoldering myeloma and monoclonal gammopathy of undetermined significance by a high (>3 g/dL) level of M-protein (monoclonal light chains) and the presence of CRAB (Hypercalcemia, Renal failure, Anemia, Bone pain) symptoms, which include hypercalcemia, renal failure, anemia, and bone pain, suggesting an end-organ damage. International staging system staging involves beta 2 microglobulin and albumin levels, while the revised system considers prognostic factors such as lactate dehydrogenase levels and chromosomal abnormalities. Front-line management includes induction regimen, maintenance therapy and hematopoietic cell transplantation for eligible patients and bisphosphonates or bone-stimulating agents for the prevention of skeletal events. Treatment for relapsed disease includes newly approved monoclonal antibodies like the CD38-targeting daratumumab, proteasome inhibitors, immunomodulating agents, and investigational therapies such as B cell maturation antigen Chimeric antigen receptor T cells.

Summary Sickle cell disease (SCD) has a distinct pattern of transfusional iron overload (IO) when compared to transfusion‐dependent β‐thalassaemia major (TDT). We conducted a single institution prospective study to evaluate plasma biomarkers of iron regulation and inflammation in patients with SCD with IO (SCD IO cases, n = 22) and without IO (SCD non‐IO cases, n = 11), and non‐SCD controls (n = 13). Hepcidin was found to be inappropriately low, as evidenced by a significantly higher median hepcidin/ferritin ratio in non‐SCD controls compared to SCD IO cases (0·3 vs. 0·02, P < 0·0001) and SCD non‐IO cases (0·3 vs. 0·02, P < 0·0001), suggesting that certain inhibitory mechanism (s) work to suppress hepcidin in SCD. As opposed to the SCD non‐IO state, where hepcidin shows a strong significant positive correlation with ferritin (Spearman ρ = 0·7, P = 0·02), this correlation was lost when IO occurs (Spearman ρ = −0·2, P = 0·4). Although a direct non‐linear correlation between erythroferrone (ERFE) and hepcidin did not reach statistical significance both in the IO (Spearman ρ = −0·4, P = 0·08) and non‐IO state (Spearman ρ = −0·6, P = 0·07), patients with highest ERFE had low hepcidin levels, suggesting that ERFE contributes to hepcidin regulation in some patients. Our results suggest a multifactorial mechanism of hepcidin regulation in SCD.

Lenalidomide is indicated in the front-line management of multiple myeloma. More recently, it has been introduced for use in treating other hematologic malignancies. Although the drug is known to cause myelosuppression, there have been rare reports of lenalidomide-associated immune thrombocytopenia (ITP). Here, we review the literature on lenalidomide-associated ITP and report upon a 59-year-old man who was administered lenalidomide due to concern of progressive multiple myeloma more than a year following his having undergone an autologous hematopoietic stem cell transplant. His platelet count precipitously declined and lead to his hospitalization. Despite our withholding of the drug, he did not respond to platelet transfusions or administration of corticosteroids. He was successfully managed with intermittent immune globulin for several months before definitive treatment with splenectomy, which resulted in the complete resolution of his thrombocytopenia. A literature search identified a total of six additional cases of lenalidomide-associated ITP. Similarly, many of the reported cases were associated with persistent thrombocytopenia after discontinuation of the drug. Furthermore, these patients were generally managed successfully with standard ITP therapies, such as corticosteroids or intravenous immune globulin.

Chemotherapeutic agents and radiation therapy are associated with numerous potential adverse events (AEs). Many of these common AEs, namely chemotherapy- or radiation-induced nausea and vomiting, hypersensitivity reactions, and edema, can lead to deleterious outcomes (such as treatment nonadherence or cessation, or poor clinical outcomes) if not prevented appropriately. The occurrence and severity of these AEs can be prevented with the correct prescribing of prophylactic medications, often called “premedications.” The advanced practitioner in hematology/oncology should have a good understanding of which chemotherapeutic agents are known to place patients at risk for these adverse events as well as be able to determine appropriate prophylactic medications to employ in the prevention of these adverse events. While several guidelines and literature exist regarding best practices for prophylaxis strategies, differences among guidelines and quality of data should be explored in order to accurately implement patient-specific recommendations. Herein, we review the existing literature for prophylaxis and summarize best practices.

Macrothrombocytopenia (MTP) is a group of rare disorders characterized by giant platelets, thrombocytopenia, and variable association with abnormal bleeding. Inherited MTP are frequently misdiagnosed as immune thrombocytopenia. Associated second-organ manifestation can help narrow down syndromic MTPs. We describe a case of autosomal dominant sensorineural hearing loss and MTP caused by a gain of function mutation in DIAPH1. This mutation causes altered megarkaryopoiesis and platelet cytoskeletal deregulation. Although hearing loss and MTP were likely progressive, clinically significant bleeding was not observed. DIAPH1-related MTP can be distinguished clinically from MYH9 mutation by the absence of cataracts and glomerular disease.