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Summary: Treatment advances have greatly improved survival, but myeloma is among the worst of all cancers for delayed diagnosis, causing serious morbidities and early deaths. This delay is largely because the symptom profile of myeloma has very low specificity, and in primary care, myeloma is rare. However, initiating the journey to diagnosis simply requires considering myeloma and sending blood to test for monoclonal immunoglobulin. Laboratory tests reliably detect monoclonal immunoglobulin, which is present in 99% of myeloma cases, so why do health care systems have such a problem with delayed diagnosis? The Myeloma UK early diagnosis programme has brought together diverse expertise to investigate this problem, and this article was prepared by the programme's working group for laboratory best practice. It reviews evidence for test requesting, analysis and reporting, for which there is large variation in practice across the United Kingdom. It presents a 'GP Myeloma diagnostic tool' and how it can be integrated into laboratory practice alongside a laboratory best practice tool. It proposes improved requesting and integration with haematology services for reporting and interpretation. Here the laboratory has a central role in creating efficient and cost‐effective pathways for appropriate and timely bone marrow examination for myeloma diagnosis. [ABSTRACT FROM AUTHOR]

Summary: This Good Practice Paper provides recommendations for the diagnosis, risk stratification and management of the monoclonal gammopathy of undetermined significance (MGUS). It describes the recently recognised entity of the monoclonal gammopathy of clinical significance (MGCS), and recommends how it should be managed. The potential for targeted population screening for MGUS is also discussed. [ABSTRACT FROM AUTHOR]

Multiple myeloma is a haematological cancer caused by malignant plasma cells in the bone marrow that can result in organ dysfunction and death. Recent novel treatments have contributed to improved survival rates, including monoclonal antibody therapies that target the CD38 protein on the surface of plasma cells. Anti-CD38 therapies are IgG kappa monoclonal antibodies that are given in doses high enough for the drug to be visible on serum protein electrophoresis as a small paraprotein. We present a case where isatuximab, the most recent anti-CD38 monoclonal antibody to be approved for treatment of myeloma, obscured the patient's paraprotein on gel immunofixation, so that complete remission could not be demonstrated. This was resolved using the isatuximab Hydrashift assay. The interference on gel immunofixation was unexpected because isatuximab migrated in a position distinct from the patient's paraprotein on capillary zone electrophoresis. We demonstrate the surprising finding that isatuximab migrates in a different position on gel electrophoresis compared to capillary zone electrophoresis. It is vital that laboratories are aware of the possible interference on electrophoresis from anti-CD38 monoclonal antibody therapies, and are able to recognise these drugs on protein electrophoresis. The difference in isatuximab's electrophoretic mobility on capillary and gel protein electrophoresis makes this particularly challenging. Laboratories should have a strategy for alternative analyses in the event that the drugs interfere with assessment of the patient's paraprotein. [ABSTRACT FROM AUTHOR]

Summary: Management of chronic myeloid leukaemia (CML) has recently undergone dramatic changes, prompting the European LeukemiaNet (ELN) to issue recommendations in 2013; however, it remains unclear whether real‐world CML management is consistent with these goals. We report results of UK TARGET CML, a retrospective observational study of 257 patients with chronic‐phase CML who had been prescribed a first‐line TKI between 2013 and 2017, most of whom received first‐line imatinib (n = 203). Although 44% of patients required ≥1 change of TKI, these real‐world data revealed that molecular assessments were frequently missed, 23% of patients with ELN‐defined treatment failure did not switch TKI, and kinase domain mutation analysis was performed in only 49% of patients who switched TKI for resistance. Major molecular response (MMR; BCR‐ABL1IS ≤0·1%) and deep molecular response (DMR; BCR‐ABL1IS ≤0·01%) were observed in 50% and 29%, respectively, of patients treated with first‐line imatinib, and 63% and 54%, respectively, receiving a second‐generation TKI first line. MMR and DMR were also observed in 77% and 44% of evaluable patients with ≥13 months follow‐up, receiving a second‐generation TKI second line. We found little evidence that cardiovascular risk factors were considered during TKI management. These findings highlight key areas for improvement in providing optimal care to patients with CML. [ABSTRACT FROM AUTHOR]

Background/purpose: Amidst the emergence of new therapeutic options, traditional therapeutic plasmapheresis (TPE) used in diseases involving a toxic substance in the plasma, remains a viable alternative for cases of recalcitrant solar urticaria (SU). We emphasize the importance of documenting successful experience with repeated plasmapheresis to increase awareness amongst physicians and dermatologists regarding this effective treatment option., Material and Method: We reported a case of recalcitrant SU that had not responded to a combination of H1-antihistamines, immunosuppressants, omalizumab and intravenous immunoglobulin. We introduced serial TPE, which involved two consecutive days of procedures for each course was introduced. We detailed the regimen and highlighted the clinical and objective benefits observed with multiple treatments. Additionally, we compared this to other plasmapheresis regimens and their treatment responses previously reported for solar urticaria., Results: Our patient underwent serial TPE, totaling 42 procedures over five years. Following the last TPE session, phototesting showed a sustained prolongation of minimal urticating doses (MUDS), which exceeded the maximum tested doses across nearly all ultraviolet (UV) and visible light ranges, with the exception of the two short ultraviolet B (UVB) wavelengths. MUDs increased to 25 from 6 mj/cm2 at 307.5± 5nm, and to 500 from 15 mj/cm2 at 320 ± 10nm, before the initial TPE. In our review, we included five articles covering eight SU patients who received TPE. Of these, the five patients with positive intradermal tests responded particularly well immediately after treatment. However, the condition relapsed within two weeks in one patient and within two months in another. In contrast, the other three patients with negative intradermal tests, showed no significant benefits from the treatment. No serious side effects from TPE were reported amongst the patients., Conclusions: This review underscores the efficacy of serial plasmapheresis procedures in treating refractory cases of SU, high3lighting the robust results observed.

Competing Interests: The authors declare that there is no conflict of interest.

Persistent thrombocytopenia may be the consequence of chronic infections with hepatitis C virus (HCV), human immunodeficiency virus (HIV), and Helicobacter pylori, and should be considered in the differential diagnosis of primary immune thrombocytopenia (ITP). Studies have shown that on diagnosis of infections, treatment of the primary disease often results in substantial improvement or complete recovery of the thrombocytopenia. In patients with thrombocytopenia due to HCV-related chronic liver disease, the use of eltrombopag, a thrombopoietin receptor agonist, normalizes platelet levels, thereby permitting the initiation of antiviral therapy. Antiviral therapy with highly active antiretroviral therapy for HIV has aided in platelet recovery, with a corresponding decrease in circulating viral load. Thrombocytopenia in the absence of other disease symptoms requires screening for H. pylori, especially in countries such as Japan, where there is a high prevalence of the disease and the chances of a platelet response to eradication therapy are high.

Granulocyte colony stimulating factor (G-CSF) has proven efficacy in the prophylaxis of chemotherapy induced neutropenia and is associated with a reduction in the duration of neutropenia, febrile neutropenia, hospitalisation and intravenous antibiotic use. It is an effective mobiliser of peripheral blood progenitor cells and is used in many countries to mobilise and provide a source of stem cells for autologous and allogeneic bone marrow transplantation. The longevity of G-CSF action is limited by its short half-life, necessitating daily injections. Pegfilgrastim (Neulasta, Amgen, Inc.) is a novel form of filgrastim (G-CSF) with a sustained duration of action. Single dose pegfilgrastim has been shown to be as safe and effective as daily filgrastim in reducing the incidence of chemotherapy induced neutropenia. Pegfilgrastim provides clinical and quality of life benefits for patients as a result of its once per cycle administration. It is licenced in the US for use in the prophylaxis and treatment of chemotherapy induced neutropenia. Clinical trials to evaluate its ability to mobilise peripheral blood progenitor cells are ongoing.