A retrospective real-world study of the current treatment pathways for myelofibrosis in the United Kingdom: the REALISM UK study.

Background: Myelofibrosis (MF) is a blood cancer associated with splenomegaly, blood count abnormalities, reduced life expectancy and high prevalence of disease-associated symptoms. Current treatment options for MF are diverse, with limited data on management strategies in real-world practice in the United Kingdom.

Methods: The REALISM UK study was a multi-center, retrospective, non-interventional study, which documented the early management of patients with MF.

The not so innocent bystander: an unusual cause of progressive breathlessness.

This case report discusses a 76-year-old man who presented with symptomatic diffuse alveolar-septal and tracheobronchial amyloidosis with a low-grade monoclonal gammopathy. This patient had a combination of both symptomatic diffuse alveolar-septal interstitial disease and tracheobronchial amyloidosis, features that contradict the widely accepted presentations seen in this disease. First, tracheobronchial amyloidosis has been documented as localised disease without systemic involvement.

Recurrent activating STAT5B N642H mutation in myeloid neoplasms with eosinophilia.

Determining the underlying cause of persistent eosinophilia is important for effective clinical management but remains a diagnostic challenge in many cases. We identified STAT5B N642H, an established oncogenic mutation, in 27/1715 (1.6%) cases referred for investigation of eosinophilia.

Toward the harmonization of result presentation for the eosin-5'-maleimide binding test in the diagnosis of hereditary spherocytosis.

Background: The eosin-5'-maleimide (EMA) Binding test measures reduced mean channel fluorescence (MCF) reading of EMA-labeled red cells (EMA-RBCs) from patients with hereditary spherocytosis (HS). Reporting test results can be either in the actual MCF reading or as a ratio by normalization of the test MCF result to the mean MCF value of six normal controls. The latter format has potential for universal reporting.

Towards the harmonization of result presentation for the eosin-5'- maleimide (EMA) binding test in the diagnosis of hereditary spherocytosis.

Background: The EMA Binding test measures reduced mean channel fluorescence (MCF) reading of EMA-labeled red cells (EMA-RBCs) from patients with hereditary spherocytosis (HS). Reporting test results can be either in the actual MCF reading or as a ratio by normalization of the test MCF result to the mean MCF value of 6 normal controls. The latter format has potential for universal reporting.

Mesenteric and coeliac occlusions following heparin-induced thrombocytopenia after aortobiprofunda bypass surgery.

A 56-year-old man developed mid-gut bowel ischaemia following an elective aortobiprofunda bypass for short-distance claudication. The bowel was resected and he was commenced on lifelong total parenteral nutrition. He was found to have developed heparin-induced thrombocytopenia and thrombosis, confirmed by high levels of heparin-platelet factor 4-antibody on enzyme-linked immunosorbent assay (ELISA).

A monoclonal protein identified by an anomalous lipaemia index.

We describe a patient being investigated for anaemia where the lipaemia index on a Beckman Coulter DxC800 analyser was markedly elevated and out of keeping with the visual appearance of the serum. Subsequent investigation revealed a monoclonal IgM kappa immunoglobulin with type I cryoglobulin behaviour. The patient was then diagnosed with a non-Hodgkin B-cell lymphoma.

Lymphomatoid granulomatosis of the uterine cervix.

Lymphomatoid granulomatosis is an Epstein-Barr virus-driven lymphoproliferative disorder, usually with a prominent pulmonary involvement and occasional extrapulmonary manifestations. Here, we present a case of lymphomatoid granulomatosis confined to the uterine cervix at the initial diagnosis. The disease was preceded by an immunosuppressive condition, namely low-grade lymphoplasmacytic lymphoma treated with chemotherapy.

Can serum free light chains replace urine electrophoresis in the detection of monoclonal gammopathies?

Background: We investigated how sensitive serum free light chain (FLC) analysis was for the detection of Bence-Jones protein (BJP) and whether a serum kappa/lambda ratio could replace urine electrophoresis as part of the investigation algorithm for monoclonal gammopathy.

Methods: Serum kappa and lambda FLC analysis was performed on 932 consecutive patients investigated for monoclonal gammopathy, along with serum electrophoresis, serum immunofixation where appropriate, and in 483 individuals urine immunofixation. A reference interval for the kappa/lambda ratio was derived from all patients who had normal serum and urine electrophoresis and no B-cell dyscrasia (n = 312).

Expression of co-stimulatory molecules on acute myeloid leukaemia blasts may effect duration of first remission.

Many solid tumours have been shown to lack expression of either of the immune co-stimulatory molecules CD80 (B7.1) or CD86 (B7.2), which is thought to be one of the ways in which tumours may escape immune recognition.