Hepatosplenic gammadelta T-cell lymphoma is a rare clinicopathologic entity with poor outcome: report on a series of 21 patients

We report on the characteristics of 21 patients with hepatosplenic gammadelta T-cell lymphoma (HSgammadeltaTCL), an entity recognized since 1994 in the Revised European American Lymphoma (REAL) classification. Median age was 34 years. Patients had splenomegaly (n = 21), hepatomegaly (n = 15), and thrombocytopenia (n = 20). Histopathologic findings were homogeneous and showed the presence of medium-sized lymphoma cells within the sinusoids of splenic red pulp, liver, and bone marrow. Marrow involvement was usually mild but could be demonstrated by phenotyping in all patients. Cells were CD3+CD5-, expressed the gammadelta T-cell receptor, and had a nonactivated cytotoxic cell phenotype (TIA-1+, granzyme B-). Most patients were CD4-/CD8- (16 of 18); CD56+ (15 of 18), expressed the Vdelta1epitope (Vd1+/Vd2-/Vd3-) (9 of 12); and were negative for Epstein-Barr virus (EBV) (18 of 20). Isochromosome arm 7q was documented in 9 of 13 patients. Eight patients had previously undergone kidney transplantation or had a history of systemic lupus, Hodgkin disease, or malaria. Prognosis was poor; median survival time was 16 months, and all but 2 patients ultimately died despite consolidative or salvage high-dose therapy. In conclusion, HSgammadeltaTCL is a disease with distinctive clinical, histopathologic, and phenotypic characteristics. Bone marrow biopsy with combined phenotyping is sufficient for diagnosis, and splenectomy is therefore unwarranted. Current treatment modalities appear to be ineffective in most patients.     

The impact of the new scene drug “crystal meth” on oral health: a case–control study

Objective

The use of methamphetamine (MA), a highly addictive stimulant, is rapidly increasing, with MA being widely abused as the scene drug “Crystal Meth” (CM). CM has been associated with severe oral health effects, resulting in so-called “Meth mouth”. This term appeared for the first time in 2005 in the literature and describes the final complex of symptoms including rampant caries, periodontal diseases and excessive tooth wear. The aim of this study was to investigate the effects of chronic CM abuse on teeth and intraoral tissue with respect to potential symptoms of Meth mouth.

Materials and methods

In cooperation with two centres for addiction medicine, we performed clinical intraoral investigations in 100 chronic CM users and 100 matched-pair controls. We undertook a caries and periodontal examination by using the clinical parameters DMF-T/DMF-S, bleeding on probing index (BOP) and periodontal screening index (PSI) and tested individual oral hygiene by using approximal space plaque index (API). All clinical data were analysed by the t test for independent samples.

Results

We found significantly larger numbers of caries (p < 0.001) and higher levels of gingival bleeding (p < 0.001) and periodontal disease (p < 0.001) among CM users. Oral hygiene was significantly lower in CM users (p < 0.024).

Conclusion

Chronic CM use can lead to extensive potential damage within the intraoral cavity. When CM is used over a long period of time and in the absence of treatment, clinical symptoms in terms of Meth mouth syndrome cannot be excluded.

Clinical relevance

Based on our results, we recommend a specific prevention and therapeutic concept including educational campaigns for MA users and specialized dental care for CM patients.

     

Management of Patients With Giant Cell Myocarditis: JACC Review Topic of the Week

Giant cell myocarditis is a rare, often rapidly progressive and potentially fatal, disease due to T-cell lymphocyte-mediated inflammation of the myocardium that typically affects young and middle-aged adults. Frequently, the disease course is marked by acute heart failure, cardiogenic shock, intractable ventricular arrhythmias, and/or heart block. Diagnosis is often difficult due to its varied clinical presentation and overlap with other cardiovascular conditions. Although cardiac biomarkers and multimodality imaging are often used as initial diagnostic tests, endomyocardial biopsy is required for definitive diagnosis. Combination immunosuppressive therapy, along with guideline-directed medical therapy, has led to a paradigm shift in the management of giant cell myocarditis resulting in an improvement in overall and transplant-free survival. Early diagnosis and prompt management can decrease the risk of transplantation or death, which remain common in patients who present with cardiogenic shock.     

A simple mechanism underlying the behavior of reentrant atrial tachycardia during ablation

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Device-Related Thrombosis After Percutaneous Left Atrial Appendage Occlusion for Atrial Fibrillation

Background

Transcatheter left atrial appendage (LAA) occlusion is an alternative strategy for stroke prevention in patients with atrial fibrillation (AF).

Impact of frontline fludarabine and cyclophosphamide combined treatment on peripheral blood stem cell mobilization in B-cell chronic lymphocytic leukemia

Ongoing studies in B-cell chronic lymphocytic leukemia are evaluating autologous peripheral blood stem cell (PBSC) transplantation in first remission following fludarabine therapy. However, fludarabine could impair PBSC harvest. In 38 patients after frontline oral fludarabine and cyclophosphamide (FDR-CY) therapy, we prospectively evaluated steady state filgrastim- or lenograstim-primed PBSC mobilization to collect 2.0 x 106/kg or more CD34 cells. The first mobilization, performed a median of 178 days (range, 69-377 days) from the last FDR-CY course, was unsuccessful in 32 patients. This result was significantly associated with a low platelet count before mobilization but not with age, interval from last FDR-CY course, initial stage, remission status, or other blood parameters. Finally, after 1, 2, and 3 mobilizations in 27, 10, and 1 patients, 2.0 x 106/kg or more CD34 cells were collected in only 12. Explorations of the mechanism of poor mobilization and adaptation of PBSC harvest policies after fludarabine treatment are therefore warranted.     

Refining the audiological assessment in children using narrow-band CE-Chirp-evoked auditory steady state responses

Objective: To demonstrate the feasibility and reliability of simultaneous binaural recording of auditory steady-state responses (ASSR) in young children using narrow-band CE-Chirps as stimuli. Design: Prospective cohort study comparing ASSR thresholds to four frequency stimuli (0.5, 1, 2, and 4 kHz), with click-evoked auditory brainstem responses (ABR) and behavioral response audiometry. Study sample: Thirty-two young children (mean age 7.4 ± 5.2 months) referred for auditory assessment were evaluated. Results: The mean duration for ABR recordings was 13.3 ± 7.2 min versus 22.9 ± 15.8 min for ASSR (p < 0.01). ASSR (means of 2 and 4 kHz thresholds) were highly correlated with ABR thresholds (R² = 0.935, p < 0.001), though significantly different (3 ± 10.7 dB, p = 0.02). ASSR (means of 0.5, 1, 2, and 4 kHz thresholds) were highly correlated with mean behavioral response audiometry thresholds (R² = 0.968, p < 0.001). ASSRs were highly and significantly correlated with behavioral response audiometry at 0.5, 1, 2, and 4 kHz (R² = 0.845, 0.907, 0.929, and 0.859 respectively, p < 0.001). 87.5% and 90.7% ASSR thresholds were within a ± 10 dB range around their corresponding ABR and mean behavioral response audiometry thresholds. Conclusions: Narrow-band CE-Chirps allow a fast and reliable assessment of auditory thresholds in children, especially in the low-frequency range, by comparison with other stimuli.