Clinical boundaries in adult cases of large B cell lymphoma with IRF4 rearrangement.

AIMS: Large B-cell lymphoma with IRF4 rearrangement (LBCL-IRF4) is a recently described entity included in the revised 4th edition of the WHO Classification of Haematolymphoid Tumours (2017). Here we highlight the difficulties in classification of those cases which arise in adult patients with unusual clinical features. RESULTS: We present three cases with morphological and immunohistochemical features consistent with large B-cell lymphoma arising in adult patients, which were found to have isolated IRF4 rearrangements on FISH analysis. Each patient presented with advanced-stage disease and had a history of immunosuppression; clinical features that are not typical of LBCL-IRF4 and which make the distinction from DLBCL, not otherwise specified (NOS) challenging. CONCLUSION: We propose that the clinical boundaries of LBCL-IRF4 arising in adult patients need further delineation to allow distinction from true cases of DLBCL, NOS.

Remission of chronic actinic dermatitis on baricitinib: A case report.

Chronic actinic dermatitis (CAD) is an immune-mediated photodermatosis characterised by eczematous, pruritic changes to sun-exposed skin. The pathophysiology of CAD is poorly understood, with current explanations including a hypersensitivity reaction and cross-reactivity to contact allergens. The disease is often refractory to immunosuppressive treatment and has a marked impact on patient quality of life. Janus kinase inhibitors (JAKi) are a novel class of small molecules licenced for the management of certain inflammatory conditions, including atopic dermatitis We present the case of a 69-year-old gentleman with a history of severe CAD, unresponsive to standard therapies, who was prescribed baricitinib, a janus kinase (JAK) inhibitor as a single agent treatment for his disease. The patient experienced a dramatic clinical improvement with this therapy. In addition, normalisation of photo test and improvement of patch test results following treatment were observed. There is one previous case report in the literature describing the clinical response of patients with CAD to JAK inhibitor therapy, but no comment on pre or post treatment photo testing, patch testing or photo-patch testing results was made. In this case report, we discuss our understanding of the role of JAK inhibitors in CAD and highlight a potential new therapeutic avenue for this disabling disease.

Clinical response of myelodysplastic syndromes patients to treatment with coenzyme Q10.

The myelodysplastic syndromes (MDS) are a collection of hematopoietic disorders with varying degrees of mono- to trilineage cytopenias and bone marrow dysplasia. In recent years much progress has been made in the treatment of MDS and there are now several therapeutic compounds used with varying levels of success. These compounds typically cause side effects that make them unattractive for treatment of patients in the early stages of MDS. Naturally occurring compounds that are not toxic may provide a means to treat patients in the initial stages of disease. We conducted a pilot study to test the efficacy of coenzyme Q10 (coQ10) in MDS patients with low to intermediate-2 risk disease. A variety of responses were observed in 7 of 29 patients including two trilineage and two cytogenetic responses. Sequencing mitochondrial DNA (mtDNA) from pretreatment bone marrows showed multiple mutations, some resulting in amino acid changes, in 3/5 nonresponders, 1/4 responders and in two control samples. We conclude that coQ10 may be of clinical benefit in a subset of MDS patients, but responders cannot be easily pre-selected on the basis of either the conventional clinical and pathologic characteristics or mtDNA mutations.