Engin Kelkitli

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

Abstract 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.

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.

Ultrasonic Gallbladder Function in Chronic Kidney Disease: Does Predialysis, Hemodialysis, or CAPD Affect it?

Background. There are contradictory reports about the prevalence of cholelithiasis in chronic kidney disease (CKD). The pathogenesis of gallstones is associated with the lithogenic changes of bile composition, increased tendency to nucleation, and decreased gallbladder motility. The studies related to these factors can predict the development of cholelithiasis. The aim of this study was to evaluate the ultrasonic gallbladder function in CKD and to compare it in predialysis (PreD), hemodialysis (HD), and continuous ambulatory peritoneal dialysis (CAPD) patients. Methods. Age, gender, and body mass index matched 49 CKD patients (14 PreD, 19 HD, 16 CAPD), and 17 control individuals were included in the study. Diabetic and cirrhotic patients were not included. Ultrasonic gallbladder volume was evaluated in pre- and postprandial period, and ejection fraction was calculated. We also measured several biochemical parameters (cholesterol, triglyceride, blood urea nitrogen (BUN), creatinine, calcium, Phosphorus, parathormone, albumin, total protein) in blood. Results. Preprandial gallbladder volume in PreD, HD, CAPD, and control groups were 26.7 ± 13.6, 20.8 ± 10.4, 23.2 ± 14.7, and 26.4 ± 14.8 mL, respectively (p > 0.05). Ejection fractions were 54.1 ± 22.9%, 54.9 ± 23.9%, 48.6 ± 15.9%, and 51.8 ± 19.2% in PreD, HD, CAPD, and control groups, respectively (p > 0.05). Serum triglyceride was higher in PreD patients than control group (207 ± 144 vs. 110 ± 48 mg/dL) (p < 0.05). Serum BUN, Cre, P, and PTH levels were higher in CKD groups than the control group, whereas serum total protein and albumin levels were higher in the control group (p < 0.05). Serum Ca was lower in PreD and HD patients than in the controls (p < 0.05). Conclusions. In conclusion, CKD and renal replacement therapy (HD and CAPD) do not affect gallbladder functions, but more studies are needed to evaluate prevalence of gallstones, gallbladder motility, and the composition of bile in CKD.

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.

Hematopoietic stem cell transplantation in geriatric patients in Turkey

The incidence of most hematologic malignancies increases with age. Physicians increasingly refer older patients for hematopoietic stem cell transplantation (HSCT) due to more experience and improved supportive care in HSCT. This article discusses the available data regarding the feasibility, tolerability, toxicity, and effectiveness of autologous and allogeneic HSCT in older adults.

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.

An unusual cause of thigh swelling: extramedullary myeloid tumor.

Memis Hilmi Atay1, Engin Kelkitli2, Piltan Buyukkaya3, Kubilay Ekiz4, Levent yildiz5, Mehmet Turgut3 1Van Training and Research Hospital, Department of Internal Medicine, Division of Hematology, Van, Turkey 2Erzurum Training and Research Hospital, Department of Internal Medicine, Division of Hematology, Erzurum, Turkey 3Ondokuz Mayis University Faculty of Medicine, Department of Internal Medicine, Division of Hematology, Samsun, Turkey 4Ondokuz Mayis University Faculty of Medicine, Department of Internal Medicine, Samsun, Turkey 5Ondokuz Mayis University Faculty of Medicine, Department of Pathology, Samsun, Turkey

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.

Prognostic impact of LIM domain only 2 (LMO2) in chronic myelogenous leukaemia

Sir: We read with great interest the recent analysis performed by Agostinelli et al. They reported that LIM domain only 2 (LMO2) expression was detected in five out of a total of five patients with chronic myelogenous leukaemia (CML). We also conducted an analysis of LMO2 expression in CML patients. Our secondary aim was to explore its prognostic impact on disease outcome. CML is a haematopoietic stem cell (HSC) disease, characterized by a reciprocal translocation between chromosomes 9 and 22, resulting in the formation of Philadelphia chromosome. The product of BCR–ABL fusion plays a central role in the pathogenesis of the disease. The ABL protein becomes constitutively active as a protein tyrosine kinase enzyme; the DNA–protein binding activity of ABL is attenuated; and the binding of ABL to cytoskeletal actin microfilaments is enhanced. These effects increase proliferation, affect differentiation, and block apoptosis. LMO2 [also known as rhombotin like-2 (RBTN2) or TTG2] is a cysteine-rich protein containing two zinc-binding LIM domains and a short N-terminal domain with potential transcriptional transactivational activity. It indirectly mediates gene expression, by mediating protein–protein interactions with other transcriptional factors, facilitating the formation of DNAbinding complexes. It is mainly expressed in endothelial and haematopoietic cells, and is implicated in angiogenesis, haematopoiesis, HSC maintenance, neovascularization, and angiogenesis. Activation of LMO2 has been widely studied in lymphoid malignancies. It initiates T-acute lymphoblastic lymphoma/leukaemia (ALL) as a consequence of translocations or deletions. LMO2 has been associated with a favourable outcome in B-ALL and diffuse large B-cell lymphoma. Although its expression in myeloid disorders was reported to be high, the prognostic significance of this association still remains to be elucidated.