The frequency of sarcopenia in haemophilia patients: Effects on musculoskeletal health and functional performance.

INTRODUCTION: In patients with haemophilia A, chronic arthropathy develops over time as a result of recurrent joint bleeds, which leads to restricted mobility and disability in the affected joints. There are limited studies in the literature evaluating sarcopenia in patients with haemophilia. The present study aims to determine the prevalence of sarcopenia in severe haemophilia-A patients and to evaluate musculoskeletal health and functional performance. METHODS: Thirty haemophilia-A patients and 26 adult male volunteers were enrolled in the study. For detection of sarcopenia, the appendicular skeletal muscle mass (ASM) obtained by bioelectrical impedance analysis (BIA) was divided by height squared (m2 ) to obtain the appendicular skeletal muscle mass index (ASMI) value. The thighs of both lower extremities were measured using the Modified Sonographic Tight Adjustment Ratio (STAR) method, which was obtained by adding the bilateral rectus femoris and vastus intermedius muscle thicknesses measured by ultrasound. Hand and quadriceps muscle strength (MS) were measured with a dynamometer. Physical performance was determined using the walking speed (WS), timed up-and-go test (TUGT), 5-repetition sit-to-stand test (5RSTS), and 6-min walk test (6MWT). Haemophilia Joint Health Score (HJHS) and Haemophilia Early Arthropathy Detection-Ultrasonography (HEAD-US) were also used to assess the musculoskeletal system. RESULTS: According to the modified STAR values calculated based on body mass index, sarcopenia was present in 15 (50%) of 30 patients. However, based on the ASMI-BIA values, sarcopenia was present in only two (6.6%) patients. A weak correlation was found between ASMI and HJHS, HEAD-US, WS, TUGT, and hand MS (left), while a moderate correlation was found with knee MS (right), knee MS (left), and 5RSTS. A strong correlation was found between the modified STAR score and HEAD-US, HJHS, knee MS (left), 5RSTS, TUGT, and WS, while a moderate correlation was found with hand MS (left), hand MS (right), and knee MS (right). CONCLUSION: This study showed muscle loss, joint mobility restrictions, and decreased functional capacity in haemophilia patients and demonstrated the presence of sarcopenia in these patients. The STAR measurement method showed stronger relationships with MS and functional performance values than ASMI measurements in terms of evaluating sarcopenia.

Attention! Cardiac tamponade may be caused by underlying Castleman's disease.

Castleman's disease is a rarely observed lymphoproliferative disease. In the literature, various signs and symptoms of the disease have been reported; one of these is secondary cardiac tamponade. We describe the case of a 41-year-old man who developed cardiac tamponade during examination, and who was later diagnosed with Castleman's disease, based on his lymph node biopsies.

The relationship of T helper-2 pathway components interleukin-4, interleukin-10, immunoglobulin e, and eosinophils with prognostic markers in non-hodgkin lymphoma: a case-control study.

OBJECTIVE: Increased risk for non-Hodgkin lymphoma (NHL) is associated with infections and environmental agents. We hypothesized that these factors chronically trigger the T helper-2 (Th2) pathway and result in lymphoma. We investigated the role of the Th2 pathway by exploring the relationships between components of the Th2 pathway, interleukin (IL)-10, IL-4, immunoglobulin E (IgE), and eosinophils, and prognostic markers of NHL. MATERIALS AND METHODS: Thirty-one NHL patients and 27 healthy controls were enrolled. IL-10, IL-4, IgE, and eosinophils were measured. IL-4 and IL-10 were analyzed with the enzyme amplified sensitivity immunoassay method. RESULTS: High IL-10 levels were correlated with several poor prognostic features, short early survival, and lymphopenia. There was a positive correlation between albumin and IL-4 levels and a negative correlation between IL-10 and albumin. There was no relationship related with eosinophils and IgE. We found remnant increased IL-4, which could be a clue for the triggering of the Th2 pathway in the background. CONCLUSION: There is a need for differently designed studies to detect the place of the Th2 pathway in NHL.

A tuberculosis case mimicking lymphoma.

Tuberculosis remains a worldwide health problem causing morbidity and mortality. Abdominal tuberculosis is a rare form of the disease. Abdominal form of tuberculosis can mimic other non-infectious diseases. In this report, we presented an abdominal tuberculosis presenting with an intra-abdominal mass lesion and multiple lymphadenopathies that mimics lymphoma.

Mantle cell lymphoma mimicking rectal carcinoma.

Mantle cell lymphoma (MCL) is a mature B-cell non-Hodgkin lymphoma. After the (11;14) translocation was identified as its constant finding in 1992, MCL was recognized as a separate subgroup of non-Hodgkin lymphoma (NHL). In MCL, extranodal involvement may be observed in the bone marrow, the spleen, the liver, and the gastrointestinal system (GIS). Cases of MCL that present with a massive and solitary rectal mass are rare in the literature. In this case report, our aim was to present an MCL patient with a rarely observed solitary rectal involvement mimicking rectal carcinoma and to discuss treatment options for this patient.

Predicting survival for multiple myeloma patients using baseline neutrophil/lymphocyte ratio.

The neutrophil/lymphocyte ratio (NLR) at diagnosis has been shown to be a prognostic factor for survival in solid tumors. The NLR at diagnosis as a prognostic factor for multiple myeloma (MM) has not been studied. Therefore, the focus of the study was the correlation of NLR with the proven prognostic parameters in patients with MM. A total of 151 MM patients who fulfilled the International Myeloma Working Group (IMWG) criteria were enrolled in the study by a retrospective review of the patients' records. One hundred fifty-one age- and gender-matched healthy controls were also included in the study. NLR was calculated using data obtained from the complete blood count (CBC). NLR was significantly higher in MM patients than the control group (2.79 ± 1.82 vs. 1.9 ± 0.61, respectively; p < 0.0001). The median follow-up on living patients in this study was 41 months. NLR at the diagnosis was found to be an independent predictor for overall survival (OS) and event-free survival (EFS) by univariate and multivariate analysis. Patients with a NLR <2 at diagnosis experienced superior OS compared with patients with a NLR ≥2 (5-year OS rates were 87.5 and 42.4 %, respectively; p < 0.0001). In a similar fashion, superior EFS was observed in patients with a NLR <2 at the diagnosis compared with patients with a NLR ≥2 (5-year EFS rates were 88.4 and 41.8 %, respectively, p < 0.0001). This study suggests that NLR at the diagnosis is a simple, inexpensive, possible prognostic factor to assess clinical outcomes in MM patients.

Hyper-CVAD regimen in routine management of adult acute lymphoblastic leukemia: a retrospective multicenter study.

Treatment of acute lymphoblastic leukemia is unsatisfactory in adults due to disease and patient-related factors and probably because adult chemotherapy regimens are weaker than pediatric protocols. Worries about inadequacy of adult regimens urged many hematologists, including us, to reconsider their routine treatment practices. In this retrospective multicenter study, we aimed to evaluate results of hyper-CVAD treatment in comparison to other intensive protocols. All patients aged ≤65 years who were commenced on intensive induction chemotherapy between 1999 and 2011 were included in the study. Sixty-eight of 166 patients received hyper-CVAD, 65 were treated with CALGB-8811 regimen and 33 with multiple other protocols. Limited number of patients who were treated with other intensive protocols and mature B-acute lymphoblastic leukemia cases who were mostly given hyper-CVAD were eliminated from the statistical analyses. In spite of a favorable complete remission rate (84.2%), overall (26.3 vs. 44.2% at 5 years, p = 0.05) and disease-free (24.9 vs. 48.2%, p = 0.001) survival rates were inferior with hyper-CVAD compared to CALGB-8811 due to higher cumulative nonrelapse mortality risk (29.7 vs. 5.9%, p = 0.003) and no superiority in cumulative relapse incidence comparison (45% for both arms, p = 0.44). Hyper-CVAD, in its original form, was a less favorable regimen in our practice.

EUTOS CML prognostic scoring system predicts ELN-based 'event-free survival' better than Euro/Hasford and Sokal systems in CML patients receiving front-line imatinib mesylate.

OBJECTIVES: The validity of the three currently used chronic myeloid leukemia (CML) scoring systems (Sokal CML prognostic scoring system, Euro/Hasford CML scoring system, and the EUTOS CML prognostic scoring system) were compared in the CML patients receiving frontline imatinib mesylate. PATIENTS AND METHODS: One hundred and fourty-three chronic phase CML patients (71 males, 72 females) taking imatinib as frontline treatment were included in the study. The median age was 44 (16-82) years. Median total and on-imatinib follow-up durations were 29 (3.8-130) months and 25 (3-125) months, respectively. RESULTS: The complete hematological response (CHR) rate at 3 months was 95%. The best cumulative complete cytogenetic response (CCyR) rate at 24 months was 79.6%. Euro/Hasford scoring system was well-correlated with both Sokal and EUTOS scores (r = 0.6, P < 0.001 and r = 0.455, P < 0.001). However, there was only a weak correlation between Sokal and EUTOS scores (r = 0.2, P = 0.03). The 5-year median estimated event-free survival for low and high EUTOS risk patients were 62.6 (25.7-99.5) and 15.3 (7.4-23.2) months, respectively (P < 0.001). This performance was better than Sokal (P = 0.3) and Euro/Hasford (P = 0.04) scoring systems. Overall survival and CCyR rates were also better predicted by the EUTOS score. DISCUSSION: EUTOS CML prognostic scoring system, which is the only prognostic system developed during the imatinib era, predicts European LeukemiaNet (ELN)-based event-free survival better than Euro/Hasford and Sokal systems in CML patients receiving frontline imatinib mesylate. This observation might have important clinical implications.

Turkish Chronic Myeloid Leukemia Study: Retrospective Sectional Analysis of CML Patients.

OBJECTIVE: here have been tremendous changes in treatment and follow-up of patients with chronic myeloid leukemia (CML) in the last decade. Especially, regular publication and updating of NCCN and ELN guidelines have provided enermous rationale and base for close monitorization of patients with CML. But, it is stil needed to have registry results retrospectively to evaluate daily CML practices. MATERIALS AND METHODS: In this article, we have evaluated 1133 patients' results with CML in terms of demographical features, disease status, response, resistance and use of second-generation TKIs. RESULTS: The response rate has been found relatively high in comparison with previously published articles, and we detected that there was a lack of appropriate and adequate molecular response assessment. CONCLUSION: We concluded that we need to improve registry systems and increase the availability of molecular response assessment to provide high-quality patient care. CONFLICT OF INTEREST: None declared.