Konvansiyonel Tedavilere ve Splenektomiye Refrakter Primer Myelofibrozisli Olguda Ruxolitinib Deneyimi

A 67-year-old male patient who was diagnosed with primary myelofibrosis 4 years ago did not respond to conventional therapies. The splenomegaly progressively increased, which caused spleen infarctions and led to the decision to perform a splenectomy procedure. After splenectomy, the patient started treatment with ruxolitinib. In the first month of ruxolitinib treatment, the patient became transfusion-free and all constitutional symptoms disappeared. However, in the sixth month of ruxolitinib treatment, the disease transformed to acute myeloblastic leukemia, and the patient died 1 month later. This is the first case report that shows the effects of ruxolitinib in a splenectomized patient

Relevance of additional immunohistochemical markers in the differential diagnosis of small B-Cell lymphomas: a case-Control study

Clinical and pathological differential diagnosis of small B-cell lymphomas (SBCLs) is still controversial and may be difficult due to their overlapping morphology, phenotype, and differentiation to plasma cells. We aimed to examine the expression of the immune receptor translocation-associated protein 1 (IRTA1), myeloid cell nuclear differentiation antigen (MNDA), lymphoid enhancer-binding factor-1 (LEF1), and stathmin 1 (STMN1) markers in SBCL cases involving different sites that may have plasma cell differentiation. Materials and Methods: We studied 154 tissue samples with lymphoma involvement from 116 patients and evaluated the staining distribution of the markers. Expressions were evaluated in 21 chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL), 7 follicular lymphoma (FL), 14 nodal marginal zone lymphoma, 17 extranodal marginal zone lymphoma, 55 splenic marginal zone lymphoma, 22 marginal zone lymphoma-not otherwise specified, and 18 lymphoplasmacytic lymphoma/Waldenström macroglobulinemia cases by immunohistochemistry. Results: The results confirmed that LEF1 was the most sensitive and specific marker for CLL/SLL and STMN1 was the most sensitive and specific marker for FL (p<0.001). MNDA and IRTA1 were useful markers to distinguish marginal zone lymphomas. Conclusion: Our results suggest that LEF1 for CLL/SLL and STMN1 for FL are reliable markers. LEF1, MNDA, STMN1, and IRTA1 are helpful with other routinely used immunohistochemical markers in a diagnostic algorithm considering their limitations

Glofitamab in relapsed/refractory diffuse large B cell lymphoma: Real world data

Abstract INTRODUCT ̇ION Glofitamab is a T-cell-engaging bispecific antibody connecting CD20 on B cells and CD3 on T cells. Although, most of the patients with B-cell non-Hodgkin lymphoma (BNHL) achieve complete response (CR) following firstline treatment with rituximab and chemotherapy, about 40% of patients with diffuse large B-cell lymphoma (DLBCL) is refractory or relapse (R/R). Autologous stem-cell transplantation (ASCT) can cure some of these patients but many patients cannot undergo this procedure. CAR-T therapies are a significant advance but not available in many countries like Turkey. In Phase II expansion study, the overall response rate (ORR) was 51.6% and complete remission (CR) rate was 39.4% in R/R DLBCL patients (Dickinson er al. JCO 2022). In this retrospective study, we aimed to report the outcomes of patients who used glofitamab via compessionate use in Turkey

Biomarkers of response to ibrutinib plus nivolumab in relapsed diffuse large B-cell lymphoma, follicular lymphoma, or Richter's transformation

We analyzed potential biomarkers of response to ibrutinib plus nivolumab in biopsies from patients with diffuse large B-cell lymphoma (DLBCL), follicular lymphoma (FL), and Richter's transformation (RT) from the LYM1002 phase I/IIa study, using programmed death ligand 1 (PD-L1) immunohistochemistry, whole exome sequencing (WES), and gene expression profiling (GEP). In DLBCL, PD-L1 elevation was more frequent in responders versus nonresponders (5/8 [62.5%] vs. 3/16 [18.8%]; p = 0.065; complete response 37.5% vs. 0%; p = 0.028). Overall response rates for patients with WES and GEP data, respectively, were: DLBCL (38.5% and 29.6%); FL (46.2% and 43.5%); RT (76.5% and 81.3%). In DLBCL, WES analyses demonstrated that mutations in RNF213 (40.0% vs. 6.2%; p = 0.055), KLHL14 (30.0% vs. 0%; p = 0.046), and LRP1B (30.0% vs. 6.2%; p = 0.264) were more frequent in responders. No responders had mutations in EBF1, ADAMTS20, AKAP9, TP53, MYD88, or TNFRSF14, while the frequency of these mutations in nonresponders ranged from 12.5% to 18.8%. In FL and RT, genes with different mutation frequencies in responders versus nonresponders were: BCL2 (75.0% vs. 28.6%; p = 0.047) and ROS1 (0% vs. 50.0%; p = 0.044), respectively. Per GEP, the most upregulated genes in responders were LEF1 and BTLA (overall), and CRTAM (germinal center B-cell-like DLBCL). Enriched pathways were related to immune activation in responders and resistance-associated proliferation/replication in nonresponders. This preliminary work may help to generate hypotheses regarding genetically defined subsets of DLBCL, FL, and RT patients most likely to benefit from ibrutinib plus nivoluma

Ruxolitinib Treatment in a Patient with Primary Myelofibrosis Resistant to Conventional Therapies and Splenectomy: A Case Report

A 67-year-old male patient who was diagnosed with primary myelofibrosis 4 years ago did not respond to conventional therapies. The splenomegaly progressively increased, which caused spleen infarctions and led to the decision to perform a splenectomy procedure. After splenectomy, the patient started treatment with ruxolitinib. In the first month of ruxolitinib treatment, the patient became transfusion-free and all constitutional symptoms disappeared. However, in the sixth month of ruxolitinib treatment, the disease transformed to acute myeloblastic leukemia, and the patient died 1 month later. This is the first case report that shows the effects of ruxolitinib in a splenectomized patient

Waldenström makroglobulinemili bir hastada mandibuler inatçı enfeksiyon olgusu

Waldenström Makroglobulinemisi WM , farklılaşmış terminal B lenfositlerin neoplastik klonal proliferasyonuyla, aşırı IgM üretimiyle karakterize nadir görülen bir hastalıktır. Bu hastalık, monoklonal IgM proteiniyle karakterize lenfoplasmositik lenfomadır. Osteomiyelit ise; bir mikroorganizmanın infeksiyöz ve inflamatuar süreçler ile kemik dokuda hasar yaratması sonucu ortaya çıkan ilerleyici karakterde bir hastalıktır. Kemiğin sadece bir bölümü tutulabildiği gibi, medüller kanal, periost, korteks, ve çevre yumuşak dokuların tümü bu tabloya katılabilir. WM’li hastalarda muskuloskeletal enfeksiyonlar nadir görülür. Literatürde septik artrit vaka raporları oldukça nadirdir ve yayınlarda sadece bir bölgede lumbar vertebral bölgede osteomiyelit görülmüştür. Biz de bu çalışmada; WM’li bir hastada mandibulada gelişen inatçı enfeksiyon tablosunu, alınan kültürden edinilen bilgilerin ışığında, enfeksiyon kaynağı olan dişlerin çekimi, antibiyoterapi, anti-inflamatuar ilaçların kullandırılması ve hiperbarik oksijen tedavisi uygulaması ile değerlendirdik. 7. Ay sonundaki panoromik filme göre kemik dokuda iyileşmenin olduğu görülmüştür. Hastanın şikayetleri sona ermiş ve olumlu sonuçlar alınmıştı

2nd International Congress on Leukemia, Lymphoma and Myeloma.

The 2nd International Congress on Leukemia, Lymphoma and Myeloma (ICLLM) is a novel initiative of the Turkish Society of Hematology. The first congress was organized in Fethiye in May, 2007. After the success and positive impressions of the participants, the THD decided to organize the ICLLM every other year. The major focus was hematological malignancies, not only LLM, but also myeloproliferative disorders and myelodysplastic syndromes. The target audience was hematologists, oncologists and those involved in stem cell transplantation. The congress was held over 2 and a half days and attended by more than 350 participants from 25 different countries. The scientific program was shaped by 12 distinguished international speakers, who cochaired and designed the sessions. The majority of the hematologists are caring for malignant patients and the Congress was a great opportunity for participants to get the latest update before the American Society of Clinical Oncology and American Society of Hematology 2009 official meeting. The rising interest and encouraging increase in the number of participants geared us to announce the organization of 3rd ICLLM in May 2011 in Istanbul. © 2009 Expert Reviews Ltd