Lymphoid follicular hyperplasia (LFH) is a benign lymphoproliferative disease. Although it can occur within the thoracic cavity, LFH originating from the chest wall has not been reported. A 79-year-old woman was incidentally found to have a well-defined mass on the left posterior chest wall during a preoperative examination for aortic valve replacement. The mass had slowly grown over 6 years. Thoracoscopic surgical resection was performed without complications. Pathological examination ruled out lymphoproliferative diseases, such as Castleman disease or malignant lymphoma, and a diagnosis of LFH was made. Although LFH generally has a good prognosis, surgical resection is recommended for diagnostic and therapeutic purposes owing to the possibility of malignancy masquerading as a reactive lesion. This is the first report of an LFH arising from the chest wall with imaging findings similar to other benign tumours. Its potential as a differential diagnosis for tumours with similar imaging findings is highlighted.

Large granular lymphocytic (LGL) leukaemia is a rare chronic lymphoproliferative disorder characterized by an expansion of cytotoxic T or NK cells. Despite a usually indolent evolution, most patients will require a treatment over the course of the disease because of cytopenia or symptomatic associated autoimmune disorders. First-line treatment is based on immunosuppressive agents, namely cyclophosphamide, methotrexate and ciclosporin. However, relapses are frequent, and there is no consensus on the management of relapsed/refractory patients. The implication of the JAK/STAT pathway in the pathogenesis of this disease has prompted our group to propose treatment with ruxolitinib. A series of 21 patients who received this regimen is reported here. Ten patients (47.6%) were refractory to the three main immunosuppressive drugs at the time of ruxolitinib initiation. Ruxolitinib yielded an overall response rate of 86% (n = 18/21), including 3 complete responses and 15 partial responses. With a median follow-up of 9 months, the median response duration was 4 months. One-year event-free survival and 1-year overall survival were 57% and 83% respectively. Mild side effects were observed. Biological parameters, notably neutropenia and anaemia, improved significantly, and complete molecular responses were evidenced. This study supports ruxolitinib as a valid option for the treatment of relapsed/refractory LGL leukaemia.

A 10-year-old neutered male Maltese dog was presented for an investigation of lymphocytosis. The dog was up-to-date on vaccinations and deworming. Physical examination did not reveal any significant abnormalities. A complete blood cell count (CBC) showed mild leukocytosis with moderate lymphocytosis, basophilia, and moderate neutropenia, but no significant left shift or toxic change. Serum biochemistry and urinalysis were unremarkable. All performed tests for infectious agents common in this geographical region were negative. No significant abnormalities were found on abdominal ultrasound examination. Multiparametric flow cytometry of peripheral blood showed a CD8+ T-cell lymphocytosis, and PCR for antigen receptor rearrangement revealed a clonal expansion of the T-cell receptor gamma chain genes. A clinical diagnosis of chronic lymphocytic leukemia (CLL) was made, and follow-up was recommended. On Day 48 post-presentation, the CBC showed mild non-regenerative anemia (NRA), moderate leucocytosis due to moderate to marked lymphocytosis, basophilia, and a marked increase in hyposegmented neutrophils with mild toxic change in the absence of neutrophilia or neutropenia. Treatment with chlorambucil and prednisolone was initiated. On Days 87 and 197 post-presentation, the CBC showed mild NRA, with progressively decreasing numbers of hyposegmented neutrophils. The dog remained without clinical signs. Basophilia and probable pseudo-Pelger-Huët anomaly were possibly secondary to CLL. To the authors\' knowledge, this is the first report of these two hematologic conditions secondary to CLL in dogs. Recognition of a pseudo-Pelger-Huët anomaly is clinically relevant to avoid misinterpretation as a marked left shift due to severe inflammation and prevent unnecessary urgent therapeutic actions.

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METHODS: This retrospective analysis aimed to assess whether a 12-hour mean temperature (measured around either diagnosis of HLH or peak ferritin value) has value as a quick and simple diagnostic test for HLH in people with lymphoproliferative disease (LPD). Hospital records from 2018 to 2022 were retrospectively screened for patients with LPD and peak ferritin during admission to hospital >3000ng/mL. Patients were grouped as either HLH or non-HLH after consensus discussion at a multi-disciplinary meeting with access to full, detailed patient records and H-scores.
RESULTS: The total cohort of 23 patients consisted of 12 with HLH and 11 grouped as non-HLH. 12-hour mean temperature at HLH diagnosis was 38.6 °C in the HLH cohort and 37.5 °C measured at the point of peak ferritin measurement in non-HLH groups. It was also positively correlated with HLH status (P = 0.001) and showed high retrospective sensitivity and specificity for HLH above 37.7 °C.
CONCLUSIONS: These results demonstrate that a 12-hour mean temperature may add value and diagnostic certainty to the first-line investigations for HLH associated with LPD. The moderately high sensitivity and specificity achieved with this dataset supports the need for further research into whether the test retains validity in larger patient groups.

T-large granular lymphocytic leukemia (T-LGLL) is a rare lymphoproliferative disorder. The diagnosis is established by identifying an abnormally high number of clonal granular T lymphocytes in the peripheral blood and eventually in the bone marrow, in cases with medullary infiltration. The majority of patients present with symptoms related to neutropenia and this condition may be associated with autoimmune diseases in up to a third of cases. The authors describe the case of a 26-year-old patient admitted with subacute high fever and bullous dermatitis with necrotic lesions with central bullae. Analytically, she presented anemia and leukopenia with severe neutropenia of 200 cells/L. Skin lesions were compatible with ecthyma and the skin biopsy revealed aspects compatible with leukocytoclastic vasculitis. The myelogram and bone biopsy revealed hypoplasia of the myeloid line and a pathological T population of CD8+, TIA-1+ and granzyme B+, which were associated with compatible flow cytometry (CD3+, T-cell receptor (TCR) Alpha-Beta+, CD5+, CD2+, with loss of CD7 antigen expression) established the diagnosis of T-LGLL. The patient had a favorable evolution, with cytopenias almost returning to normal after two months. She began follow-up at a Hematology Reference Center, remaining asymptomatic without specific treatment considering the indolent course of the disease.

BACKGROUND: In the last decade, Ibrutinib has become the standard of care in the treatment of several lymphoproliferative diseases such as chronic lymphocytic leukemia (CLL) and several non-Hodgkin lymphoma. Beyond Bruton tyrosine kinase inhibition, Ibrutinib shows broad immunomodulatory effects that may promote the occurrence of infectious complications, including opportunistic infections. The infectious burden has been shown to vary by disease status, neutropenia, and prior therapy but data focusing on severe infections requiring intensive care unit (ICU) admission remain scarce. We sought to investigate features and outcomes of severe infections in a multicenter cohort of 69 patients receiving ibrutinib admitted to 10 French intensive care units (ICU) from 1 January 2015 to 31 December 2020.
RESULTS: Median time from ibrutinib initiation was 6.6 [3-18] months. Invasive fungal infections (IFI) accounted for 19% (n = 13/69) of severe infections, including 9 (69%; n = 9/13) invasive aspergillosis, 3 (23%; n = 3/13) Pneumocystis pneumonia, and 1 (8%; n = 1/13) cryptococcosis. Most common organ injury was acute respiratory failure (ARF) (71%; n = 49/69) and 41% (n = 28/69) of patients required mechanical ventilation. Twenty (29%; n = 20/69) patients died in the ICU while day-90 mortality reached 55% (n = 35/64). In comparison with survivors, decedents displayed more severe organ dysfunctions (SOFA 7 [5-11] vs. 4 [3-7], p = 0.004) and were more likely to undergo mechanical ventilation (68% vs. 31%, p = 0.010). Sixty-three ibrutinib-treated patients were matched based on age and underlying malignancy with 63 controls receiving conventional chemotherapy from an historic cohort. Despite a higher median number of prior chemotherapy lines (2 [1-2] vs. 0 [0-2]; p < 0.001) and higher rates of fungal [21% vs. 8%, p = 0.001] and viral [17% vs. 5%, p = 0.027] infections in patients receiving ibrutinib, ICU (27% vs. 38%, p = 0.254) and day-90 mortality (52% vs. 48%, p = 0.785) were similar between the two groups.
CONCLUSIONS: In ibrutinib-treated patients, severe infections requiring ICU admission were associated with a dismal prognosis, mostly impacted by initial organ failures. Opportunistic agents should be systematically screened by ICU clinicians in this immunocompromised population.

The recent introduction of targeted therapies, including monoclonal antibodies, tyrosine-kinase inhibitors, and immunotherapies has improved the cure rate of hematologic patients. The implication of personalized treatment on primary antifungal prophylaxis will be discussed.
We reviewed the literature for clinical trials reporting the rate of invasive fungal infections during targeted and cellular therapies and stem cell transplant, and the most recent international guidelines for primary antifungal prophylaxis.
As the use of personalized therapies is growing, the risk of invasive fungal infection has emerged in various clinical settings. Therefore, it is possible that the use of mold-active antifungal prophylaxis would spread in the next years and the risk of breakthrough infections would increase. The introduction of new antifungal agents in the clinical armamentarium is expected to reduce clinical unmet needs concerning the management of primary antifungal prophylaxis and improve outcome of patients.

T-cell lymphoma in the gastrointestinal tract (intestinal T-cell lymphoma, [ITCL]) is rare. ITCL, not otherwise specified (ITCL, NOS) which is a type of ITCL is particularly rare. There are few case reports of ITCL, NOS but no previous reports describe its endoscopic features. In this report, the 69-year-old man was diagnosed with ITCL, NOS. Colonoscopy revealed the elevated legion and edematous mucosa with focal depressions in the lower rectum. On the depressed legions, magnifying endoscopy combined with narrow-band imaging detected the disappearance of glandular structure and branching abnormal blood vessels like a tree. These findings were similar to the tree-like appearance, which has been described as a unique feature of gastric mucosal-associated lymphoid tissue lymphoma. The targeted biopsy of the tree-like appearance showed abnormal histopathological findings which fit the definition of ITCL, NOS. He was treated with chemotherapy and achieved complete remission. As is the case of gastric mucosal-associated lymphoid tissue lymphoma, the tree-like appearance is possibly the unique sign of ITCL, NOS. We report the endoscopic features of ITCL, NOS and show characteristic findings by magnifying endoscopy combined with narrow-band imaging.

OBJECTIVE: Epstein-Barr virus (EBV) causes multiple human cancers, including B-cell lymphomas. In cell culture, EBV converts healthy human B-cells into immortalized ones that grow continuously, which model post-transplant lymphomas. Constitutive signaling from two cytoplasmic tail domains of the EBV oncogene latent membrane protein 1 (LMP1) is required for this transformation, yet there has not been systematic analysis of their host gene targets. We identified that only signaling from the membrane proximal domain is required for survival of these EBV-immortalized cells and that its loss triggers apoptosis. We identified key LMP1 target genes, whose abundance changed significantly with loss of LMP1 signals, or that were instead upregulated in response to switching on signaling by one or both LMP1 domains in an EBV-uninfected human B-cell model. These included major anti-apoptotic factors necessary for EBV-infected B-cell survival. Bioinformatics analyses identified clusters of B-cell genes that respond differently to signaling by either or both domains.