Hematology, Transfusion and Cell Therapy最新文献

{"title":"Polycythemia and JAK2 variant N1108S: cause-and-effect or coincidence?","authors":"Ana Oliveira e Costa, Ana Barreira, Manuel Cunha, Fernando Salvador","doi":"10.1016/j.htct.2023.01.006","DOIUrl":"10.1016/j.htct.2023.01.006","url":null,"abstract":"","PeriodicalId":12958,"journal":{"name":"Hematology, Transfusion and Cell Therapy","volume":"46 ","pages":"Pages S376-S379"},"PeriodicalIF":1.8,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11726029/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10834264","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"A CASE OF THROMBOTIC THROMBOCYTOPENIC PURPURA RELATED TO MALIGNITY AND CHEMOTHERAPY","authors":"Ebru Kavak Yavuz , Songül Beskisiz Dönen , Etem Özkaya , Esra Pirinççi , Abdullah Karakuş , Orhan Ayyıldız","doi":"10.1016/j.htct.2024.11.061","DOIUrl":"10.1016/j.htct.2024.11.061","url":null,"abstract":"<div><h3>Objective</h3><div>Thrombotic thrombocytopenic purpura (TTP) is a life-threatening multisystem disease. TTP progresses with Microangiopathic hemolytic anemia (MAHA), fever, thrombocytopenia, neurological symptoms, and renal failure. Due to microangiopathic hemolytic anemia, schistocytes are seen in the peripheral blood smear, resulting in thrombocytopenia. Damage to the brain and kidneys occurs due to microvascular thrombosis, and this is how symptoms appear. In pathogenesis, it is caused by the deficiency of ADAMTS 13 (a disintegrin and metalloproteinase with thrombospondin type 1 motif, member 13), which breaks down the von Willebrand factor (VWF) found in the endothelium into multimers, or the development of antibodies against it. Due to ADAMTS 13 deficiency or decrease in its activity, VWF cannot be separated into small pieces and is arranged in large pieces in the endothelium, causing widespread intravascular thrombosis. Many factors can be considered as triggers for the development of TTP, such as pregnancy, malignancy, medications, and autoimmune diseases.</div></div><div><h3>Case Report</h3><div>A 59-year-old female patient was admitted due to thrombocytopenia, epileptic seizure, hematemesis and decreased consciousness while being followed up due to cholangiocellular carcinoma. Due to malignancy, 6 cycles of gemcitabine and carboplatin treatment were applied. The last cure was 6 months ago. In followers, WBC 3.24 10^3/uL (3.7-10 10^3/uL), Hbg 8g/dL(12.9-14.2 g/dL), MCV 84 f/L(81-96fL), platelet 27 10^3/uL (155-356 10^3u/L), total bilirubin 13 mg/dL (0.3-1.2 mg/dL), indirect bilirubin 5.36 mg/dL (0-1.5 mg/dL), LDH 408 U/L (0-247u) /L), creatinine 1.59 mg/dL (0.51-0.95 mg/dl), protein 1+ in full criterion examination, INR 1.36, PT 15.4 sec (10-15 sec), APTT 22.2 sec (21-29 sec) fibrinogen was 1.46 g/L (1.8-3.5 g/L), 3-5 schistocytes were seen in each area in the peripheral smear. Plasmapheresis treatment was started with the preliminary diagnosis of TTP and steroid 80 mg was given. ADAMTS 13 tests were requested. ADAMTS 13 level is 3.78% (40%-130%) low and ADAMTS 13 inhibitor > 80 U/mL (<12U/mL negative, 12-15 U/mL borderline >15U/mL positive), ADAMTS 13 antigen<0.01lU/ mL (0.19-0.81 lU/mL) was seen. As the patient's thrombocytopenia continued, plasmapheresis was started to be performed twice a day after a week. With this treatment, weekly treatment of medicinal rituximab, which could not be treated with platelets, was arranged. However, the patient did not respond to treatment and died.</div></div><div><h3>Conclusion</h3><div>In cancer assosiated TTP, endothelial cells are damaged due to abnormal angiogenesis and tumor cell invasion, and vWF multimers in the endothelial wall are exposed. In addition, ADAMTS 13 activity decreases due to antibodies formed against ADAMTS 13. Some chemotherapeutics such as mitomycin c, gemcitabine can cause TTP. When a diagnosis of TTP is considered, plasma exchange should be started immed","PeriodicalId":12958,"journal":{"name":"Hematology, Transfusion and Cell Therapy","volume":"46 ","pages":"Page S65"},"PeriodicalIF":1.8,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143339830","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"B-LINEAGE PROGENITORS AND CD38-POSİTİVE B CELLS ARE ASSOCIATED WITH SURVIVAL RATES IN BREAST CANCER PATIENTS","authors":"Svetlana Chulkova","doi":"10.1016/j.htct.2024.11.063","DOIUrl":"10.1016/j.htct.2024.11.063","url":null,"abstract":"<div><h3>Objective</h3><div>The immune system plays an increasingly important role in the development of targeted strategies for breast cancer. According to mRNA sequencing data from The Cancer Genome Atlas (TCGA) high expression B cell signatures has beneficial effects on survival rates in many tumors. Bone marrow (BM) is poorly understood from the point of view of the prognostic role of hematopoietic cells and subpopulations of lymphocytes in patients with breast cancer (BC).</div></div><div><h3>Methodology</h3><div>. Study was carried out in 107 BC patients. The immunological and morphological methods were applied. Multiparameter flow cytometry with antibodies to B-cell populations was used (CD19, CD20, CD5, CD38, CD10, CD45, HLA-DR, CD27), FACSCANTO II. Studies of BM lymphocyte subpopulations were carried out in the gate of CD45++ cells. The duration of the follow-up period after surgery was 8 years.</div></div><div><h3>Results</h3><div>The total percentage of B cells in BM was significantly associated with the prognosis of BC. B-1 cells were associated with progression-free and disease-free survival. Disease progression was observed at low levels of B1 cells. In cases more than 10% B-lymphocytes in the BM of BC patients overall survival (OS) rates were more favorable (p = 0.01). Especially for BC with a high Ki-67. Disease progression was observed in 1/3 of BC patients with low levels of B1 cells. CD38 expression on B cells was a prognostically favorable factor: the role is realized during 5–10 years of follow-up after surgery. Level CD38+ B cells more then 10% correlated with high OS, p = 0.02. The presence of CD10+CD19+ B-lineage precursors was associated with a more favorable prognosis (OS, the threshold level 12%, р = 0,04). The prognostic role of the CD10 antigen was realized when patients were observed for more than 5 years.</div></div><div><h3>Conclusion</h3><div>. Total relative number of (more than 10 %) of BM CD19+ cells were significantly related to OS in BC. B-cell precursors and CD38+ B cells were associated with favorable prognosis. Prognositic role of B-lineage precursors and CD38-positive cells was in the periods of 5–10 years after surgery.</div></div>","PeriodicalId":12958,"journal":{"name":"Hematology, Transfusion and Cell Therapy","volume":"46 ","pages":"Page S68"},"PeriodicalIF":1.8,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143339831","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"HEMOPHILIA: ADVANCES IN TREATMENTS","authors":"Nurgül Karakaya","doi":"10.1016/j.htct.2024.11.098","DOIUrl":"10.1016/j.htct.2024.11.098","url":null,"abstract":"&lt;div&gt;&lt;h3&gt;Introduction&lt;/h3&gt;&lt;div&gt;Hemophilia is an X-linked recessive disorder. It is divided into two different subtypes; hemophilia A (HA) and B (HB), which result from the deficiency or complete absence of clotting factors VIII (FVIII) and IX (FIX) respectively. Current management of HA and HB includes prophylactic factor replacement&lt;sup&gt;1&lt;/sup&gt;. Neutralising antibodies, as inhibitors, can develop against the infused factor and that can complicate the management of hemophilia patients. If inhibitors develop, immune tolerance induction can potentially promote tolerance to exogenous FVIII or FIX, and bypassing agents (BPAs) such as recombinant factor VIIa (rFVIIa) and activated prothrombin complex concentrates (aPCC) can be used to circumvent factor use. Inhibitor development impacts negatively upon quality of life and treatment compliance, highlighting the need for improved therapies. Several novel pharmacological therapies developed for hemophilia aim to rebalance the clotting cascade. These therapies utilise a range of different mechanisms, namely: the extension of the circulating half-life of standard recombinant factors; the mimicking of factor VIII cofactor activity; rebalancing of coagulation through targeting of natural anticoagulants such as antithrombin and tissue factor pathway inhibitor; and inducing the production of endogenous factors with gene therapy.&lt;/div&gt;&lt;/div&gt;&lt;div&gt;&lt;h3&gt;Discussion&lt;/h3&gt;&lt;div&gt;Extended half‐life products involves fusing FVIII or FIX to a protein with a long half-life. Albumin and the constant region (Fc) of IgG have long plasma half-lives as they bind to the neonatal Fc receptor, which is critical for the endogenous recycling of both IgG and albumin. Another method is PEGylation, where one or more PEG chains are covalently linked to rFVIII or rFIX. PEG chains interfere with the recombinant factors binding to their clearance receptors, thereby prolonging circulating half-life.&lt;/div&gt;&lt;div&gt;Emicizumab, a recombinant humanised bispecific IgG antibody, mimics the cofactor function of the missing FVIII in HA. It simultaneously binds activated FIX (FIXa) and factor X (FX), bringing them into spatial proximity to promote FIXa-catalysed FX activation, thereby restoring haemostasis.&lt;/div&gt;&lt;div&gt;Fitusiran, a novel therapy applicable to both HA and HB, consists of the amino acid, N-Acetyl- galactosamine, the ligand of the hepatic asialo-glycoprotein receptors, conjugated to a synthetic siRNA. It targets and degrades a region of the SERPINC1 gene mRNA, preventing antithrombin production and enhancing thrombin generation. Antithrombin is a potent anticoagulant which inactivates FIXa, activated factor X (FXa) and activated factor II (FIIa/thrombin). Therefore, fitusiran can correct the coagulation imbalance and prevent the bleeding phenotype.&lt;/div&gt;&lt;div&gt;Concizumab is an IgG4 monoclonal antibody targeting tissue factor pathway inhibitor (TFPI). It presents an alternative therapy for HA and HB patients, both with and without inhibitors. TFP","PeriodicalId":12958,"journal":{"name":"Hematology, Transfusion and Cell Therapy","volume":"46 ","pages":"Pages S8-S9"},"PeriodicalIF":1.8,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143340046","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"A CASE OF MARGINAL ZONE LYMPHOMA PRESENTING WITH DIPLOPIA","authors":"Tansu Koparmal ,&nbsp;Caner Çulha ,&nbsp;Aslı Odabaşı Giden ,&nbsp;Engin Yola ,&nbsp;Özgür Meletli ,&nbsp;Düzgün Özatlı","doi":"10.1016/j.htct.2024.11.048","DOIUrl":"10.1016/j.htct.2024.11.048","url":null,"abstract":"<div><h3>Objective</h3><div>Marginal zone lymphoma (MZL) is characterized by the proliferation of B cells in post-germinal centers located in mucosa-associated lymphoid tissue (MALT), lymph nodes, and the spleen. MZL typically presents with an indolent clinical course. The average age at diagnosis is 60, with a slight female predominance, and it accounts for 5-17% of non-Hodgkin lymphomas (NHL). MZL is categorized into three subtypes based on the site of involvement: extranodal, splenic, and nodal MZL. Although these subtypes share many morphological and immunophenotypic characteristics as well as a slow clinical course, they can differ in terms of frequency, pathogenesis, clinical presentation, and treatment approach. The most common subtype is extranodal MZL, while nodal MZL is the least common.</div></div><div><h3>Case Report</h3><div>A 51-year-old female patient presented to the clinic with a complaint of diplopia that had lasted for the past week. Physical examination revealed limited lateral gaze and anisocoria in the right eye, with other systemic examinations were normal. There were no B symptoms. Complete blood count, biochemical tests, serum electrolytes, and coagulation tests were within normal limits.</div><div>Contrast-enhanced orbital MRI showed a lesion in the right intraorbital intraconal area, adjacent to the lateral aspect of the optic nerve and the medial aspect of the lateral rectus muscle. The lesion extended from the retroocular area to the orbital apex, obliterating intraorbital fat planes. It measured 35 × 13 mm in the axial plane, was hypointense on T2-weighted imaging and T1-weighted imaging, and showed homogeneous diffusion restriction on diffusion-weighted imaging. Post-contrast series revealed intense homogeneous enhancement of the soft tissue. The lesion measured 27 × 17 mm in the coronal plane. The findings were primarily suggestive of lymphoma involvement.</div><div>PET-CT scan identified a hypermetabolic soft tissue lesion in the right intraorbital-retrobulbar area, continuous from the lateral aspect of the lateral rectus muscle to the lateral orbit, consistent with lymphoma. No extraocular nodal or visceral hypermetabolic foci were detected.</div><div>Orbital biopsy results confirmed marginal zone lymphoma. Although radiotherapy could have been considered as a treatment option for localized involvement, the decision was made to administer 6 cycles of RB (Rituximab and Bendamustine) chemotherapy to the patient in order to avoid complications associated with radiotherapy due to the lesion's location in the orbital region. Follow-up PET-CT after 6 cycles of RB showed complete metabolic response with total regression of the hypermetabolic soft tissue lesion in the right retroocular area. The patient is currently in remission.</div><div>This case is discussed due to the rare occurrence of ocular involvement in marginal zone lymphoma.</div></div>","PeriodicalId":12958,"journal":{"name":"Hematology, Transfusion and Cell Therapy","volume":"46 ","pages":"Page S58"},"PeriodicalIF":1.8,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143340169","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"PERITONEAL MESOTHELIOMA AS A CO-MALIGNANCY IN A PATIENT WITH CLL/SLL: CASE REPORT","authors":"Satı Betül Beydilli ,&nbsp;Ennur Ramadan ,&nbsp;Güven Çetin ,&nbsp;Mehmet Aydın","doi":"10.1016/j.htct.2024.11.037","DOIUrl":"10.1016/j.htct.2024.11.037","url":null,"abstract":"<div><h3>Objective</h3><div>Malignant mesothelioma (MM) is an aggressive tumor typically arising from the pleura, with malignant peritoneal mesothelioma (MPM) accounting for 10-15% of cases. The occurrence of MPM alongside hematologic malignancies is rare. Here, we present a case of peritoneal mesothelioma developing synchronously with CLL/SLL.</div></div><div><h3>Case Report</h3><div>A 68-year-old male was referred to our clinic in August 2023 with lymphocytosis, reporting weight loss and night sweats. His medical history included diabetes, hyperlipidemia, and hypertension, and a family history of stomach cancer. The patient had quit smoking 30 years ago and had a history of chronic alcohol use. There was no known asbestos exposure despite his occupation as a construction worker. Physical examination was normal.</div><div>Routine laboratory tests and flow cytometry were conducted. Imaging via thoracic and abdominal USG and PET/CT identified multiple lymphadenopathies and omental thickening indicative of peritoneal infiltration (Image-1). The patient was diagnosed with RAI Stage 3 CLL/SLL.</div><div>In addition to hematological follow-up, the patient was referred to oncology and general surgery. He chose to continue his hematological follow-up in our clinic while receiving oncological and surgical follow-up at an external center. He is treated for CLL with ibrutinib and cisplatin-pemetrexed-altuzan for mesothelioma.</div></div><div><h3>Discussion</h3><div>There is limited knowledge about the epidemiology and treatment of malignant peritoneal mesothelioma due to its rarity. In studies of mesothelioma associated with hematological malignancies, patients published predominantly have pleural mesothelioma.</div></div><div><h3>Conclusion</h3><div>As a result, mesothelioma should be considered as a differential diagnosis in hematological cancer patients with abdominal masses, and further investigation needs to be conducted.</div></div>","PeriodicalId":12958,"journal":{"name":"Hematology, Transfusion and Cell Therapy","volume":"46 ","pages":"Pages S50-S51"},"PeriodicalIF":1.8,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143340181","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"A HEMATOLOGICAL CHAMELEON: THE TRANSITION FROM MDS TO NON-SECRETORY MULTIPLE MYELOMA AND BACK – UNRAVELİNG DIAGNOSTIC COMPLEXITIES AND ADAPTING THERAPEUTIC PATHWAYS","authors":"Candaş Mumcu ,&nbsp;Ceren Kısa ,&nbsp;Birol Güvenç","doi":"10.1016/j.htct.2024.11.022","DOIUrl":"10.1016/j.htct.2024.11.022","url":null,"abstract":"<div><h3>Objective</h3><div>The interplay between myelodysplastic syndrome (MDS) and non-secretory multiple myeloma (MM) can confound even seasoned hematologists, particularly when these conditions shift over time. This case presents a remarkable instance of a patient transitioning from MDS to non-secretory MM and then reverting back to MDS, underscoring the need for meticulous monitoring and adaptable treatment strategies when dealing with complex hematological landscapes.</div></div><div><h3>Case Presentation</h3><div>An 82-year-old patient initially sought care for severe anemia, leading to a diagnosis of MDS based on bone marrow findings. At this point, no signs of MM were present. However, later investigations—specifically urine immunofixation—suggested the emergence of non-secretory MM, which was confirmed through a second bone marrow biopsy.</div><div>The patient began treatment with Velcade, Revlimid, and Dexamethasone (VRD), showing marked improvement in anemia. Yet, given the patient's age and frailty, hematopoietic stem cell transplantation (HSCT) was not considered viable. As treatment progressed, the regimen evolved to ixazomib, lenalidomide, and dexamethasone, achieving remission for several years.</div><div>Despite this stability, a resurgence of anemia signaled a reversion to MDS. A fresh treatment strategy was introduced, combining azacitidine, low-dose lenalidomide, and erythropoietin, aimed at maintaining functionality and quality of life without aggressive interventions.</div></div><div><h3>Discussion</h3><div>This case encapsulates the volatile nature of hematologic disorders, illustrating how diseases like MDS and non-secretory MM can morph and evolve. It emphasizes the importance of adaptive management, especially in elderly patients, where rigid treatment paradigms may fall short. The use of lenalidomide throughout the patient's journey reflects its dual utility in both plasma cell and myeloid disorders, while also sparking questions about whether prolonged exposure could influence secondary disease development.</div></div><div><h3>Conclusion</h3><div>The patient's journey through MDS, MM, and back again underscores the critical need for dynamic reassessment, vigilance, and personalized care. This case exemplifies the blurred boundaries between plasma cell dyscrasias and myeloid neoplasms, raising thought-provoking questions about disease progression and therapeutic strategies. In navigating these complexities, clinicians are reminded of the importance of flexible, patient-centered approaches in managing intricate hematological disorders.</div></div>","PeriodicalId":12958,"journal":{"name":"Hematology, Transfusion and Cell Therapy","volume":"46 ","pages":"Pages S39-S40"},"PeriodicalIF":1.8,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143340354","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"A RARE CASE: NODAL FOLLICULAR T HELPER CELL LYMPHOMA, ANGIOIMMUNOBLASTIC TYPE","authors":"Halil İbrahim Yüksel ,&nbsp;Hatice Asoğlu Rüzgar ,&nbsp;Mehmet Mutlu Kıdı ,&nbsp;Berksoy Şahin","doi":"10.1016/j.htct.2024.11.067","DOIUrl":"10.1016/j.htct.2024.11.067","url":null,"abstract":"<div><h3>Objective</h3><div>Angioimmunoblastic T-cell lymphoma (AITL) is the second most common subtype of mature T-cell lymphoma (MTCL). It is caused by monoclonal proliferation of T-follicular helper (TFH) cells. Although advances have been made in its biological knowledge, its treatment is still an unmet medical need. We would like to present a case of Nodal-TFH; AITL that we followed in our clinic.</div></div><div><h3>Case Report</h3><div>A 67-year-old male patient presented with cough. Thorax CT revealed left supraclavicular-mediastinal multiple lymphadenopathy with pleural effusion. Supraclavicular LN excision was reported as NHL; nodal follicular T helper cell lymphoma, angioimmunoblastic type. Immunohistochemical CD3, PD-1 and CXC13 were positive, CD4, CD8 and CD10 were sparse, CD21 and 23 were positive in increased dentritic cells, CD20, CD30, EBER and IDH-1 were negative. PET-CT revealed Stage 4BS (multiple LNs with FDG uptake in head-neck, thorax-mediastinum and abdominopelvic FDG uptake, increased FDG uptake in bone marrow-spleen; B symptom: positive). Subcutaneous (sc) Azacitidine + intravenous CHOP (cyclophosphamide, doxorubicin, vincristine, prednisone) was started. The 1st course of azacitidine was administered at 75 mg/m2 for 7 days 1 week before CHOP treatment and the following courses were administered at 75 mg/m2 for 14 days 2 weeks before CHOP treatment. After 4 cycles of Azacitidine+CHOP, PET-CT regressed and 2 more cycles of treatment were administered. During the follow-up, the patient's general condition deteriorated and he went into septic shock.</div></div><div><h3>Discussion</h3><div>AITL-containing T-follicular helper; nodal PTCL is characterized by recurrent mutations affecting epigenetic regulators. The association of abnormal DNA methylation with lymphomagenesis provides rationale for the administration of hypomethylating agents. The epigenetic modifier azacitidine, which inhibits DNA methyltransferase, has demonstrated clinical activity alone or in combination in relapsed/refractory PTCL. In a phase-2 clinical trial of 20 patients who experienced oral azacitidine + CHOP as initial treatment for PTCL, CR was 76.5%, 1-year PFS 61.1%, 1-year OS 88.9%. In our case, we added the hypomethylating agent azacitidine to the CHOP protocol and aimed to evaluate the efficacy of this combination in the initial treatment of CD30 negative PTCL.</div></div>","PeriodicalId":12958,"journal":{"name":"Hematology, Transfusion and Cell Therapy","volume":"46 ","pages":"Page S70"},"PeriodicalIF":1.8,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143340369","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"COEXISTENCE OF BREAST CANCER AND MANTLE CELL LYMPHOMA","authors":"Bengü Sezer ,&nbsp;Esra Asarkaya ,&nbsp;Tolga Köseci","doi":"10.1016/j.htct.2024.11.065","DOIUrl":"10.1016/j.htct.2024.11.065","url":null,"abstract":"<div><h3>İntroduction</h3><div>Patients cured of any cancer have an increased risk of developing a new primary malignancy compared to the general population. However, synchronous presentation of two tumours is a very rare condition. Here we aim to review the treatment approach of a case of synchronous mantle cell lymphoma and invasive ductal carcinoma of the breast.</div></div><div><h3>Case Report</h3><div>A 64-year-old woman presented with a right breast mass. Physical examination revealed a 3cm diameter mass lesion in the right breast and lymphadenopathy in the right axilla. Her past medical history was unremarkable except hypertension. In her family history, there was a history of breast cancer in her niece. Breast ultrasonography revealed 3 centimetres (cm) of malignant breast and multiple lymph nodes with thick cortex in bilateral axillae with indistinguishable fatty hilus. Tru-cut biopsy was performed for the mass in the breast and bilateral axilla lymph nodes. The breast biopsy was compatible with invasive ductal carcinoma with ER 90%, PR 10%, her2 negative and Ki67 proliferation index 10%. Bilateral axilla lymph node biopsy was reported as mantle cell lymphoma and immunohistochemically CD20: Positive, CD5: Positive, Cyclin D1: Positive, CD23: Negative, Lef1: Negative, Keratin: Negative, Ki67 proliferation index 25-30%. PET-CT revealed a mass in the right breast, lymph nodes with pathological appearance in the axillae, various lymph node stations in the abdomen and inguinal areas, and diffuse involvement suggestive of lymphoma infiltration in the right lung. Bone marrow aspiration/biopsy revealed mantle cell lymphoma involvement. The patient was discussed in the multidisciplinary tumour council and right axillary lymph node dissection was performed for staging. 5 lymph nodes showed ductal carcinoma metastasis and the rest of the lymph nodes showed mantle cell lymphoma involvement. Stage IV MHL and hormone positive IDC (T2N2) were detected and R-CHOP treatment was applied. PET-CT performed after three cycles of treatment showed complete response. The patient was discussed again in the multidisciplinary tumour council and surgical treatment for the breast was planned after completing 6 cycles of R-CHOP treatment. After treatment, the patient underwent modified radical mastectomy and the pathological stage was T3N3. After adjuvant RT, endocrine therapy was started and the patient is being followed in remission.</div></div><div><h3>Conclusion</h3><div>Coexistence of breast cancer and mantle cell lymphoma is a rare condition. In the few cases reported in the literature, treatment planning was made by considering the stage and treatment priority of both diseases. We planned to prioritise the treatment of lymphoma because our patient had stage 4 mantle cell lymphoma.</div></div>","PeriodicalId":12958,"journal":{"name":"Hematology, Transfusion and Cell Therapy","volume":"46 ","pages":"Page S69"},"PeriodicalIF":1.8,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143339865","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"TREATMENT OF MYELOFIBROSIS: PRESENT AND FUTURE","authors":"Birsen Sahip Yesiralioğlu","doi":"10.1016/j.htct.2024.11.116","DOIUrl":"10.1016/j.htct.2024.11.116","url":null,"abstract":"&lt;div&gt;&lt;div&gt;Primary myelofibrosis (PMF) is a myeloproliferative neoplasm characterised by stem cell-derived clonal myeloproliferation often, but not always, accompanied by JAK2, CALR or MPL mutations. It is associated with bone marrow reticulin/collagen fibrosis, abnormal inflammatory cytokine expression, anaemia, hepatosplenomegaly, extramedullary haematopoiesis (EMH), constitutional symptoms, cachexia, risk of leukaemic transformation and shortened survival. Somatic mutations in MPN are classified as ‘driver’ and ‘other’ mutations. Driver mutations are JAK2, CALR and MPL, other mutations are ASXL1, SRSF2, U2AF1, IDH1/2, SF3B1, TET-2, DNMTA3A. SRSF2, ASXL1, and U2AF1-Q157 mutations indicate poor prognosis in PMF. RAS/CBL mutations predict resistance to ruxolitinib treatment. Type 1/like CALR mutation is associated with better survival. The hallmark of MF is the disruption of the JAK/STAT signalling pathway.&lt;/div&gt;&lt;div&gt;&lt;strong&gt;TREATMENT&lt;/strong&gt;&lt;/div&gt;&lt;div&gt;In the treatment approach, allogeneic stem cell transplantation (ASCT) should first be positioned as a priority option. Then, treatment should be planned according to risk stratification for the control of anaemia and improvement of splenomegaly and related symptoms.&lt;/div&gt;&lt;div&gt;The recommended treatment strategy is what we call risk-adaptive treatment, which is treatment according to risk groups and symptoms/symptoms. The general approach is observation in low-risk asymptomatic patients, treatment selection according to symptoms (constitutional findings, splenomegaly, anaemia) in the medium and low risk group, stem cell transplant-based treatment in the high risk group. If additional risk factors are present in the intermediate risk group, ASCT should be considered as an alternative and a patient-based approach should be taken as basis.&lt;/div&gt;&lt;div&gt;In the absence of symptomatic splenomegaly, non-JAK inhibitor drugs may be preferred as first-line treatment for anaemia. Androgens, prednisone (can be used in addition to androgen therapy or alone), danazol, thymodomide, lenalidomide, erythropoiesis-stimulating agents (ESAs) can be used. Although luspatercept is approved for the treatment of anaemia associated with beta thalassaemia and low/intermediate risk MDS, it has been largely ineffective in MF patients. Response rates to each of these drugs range between 15-25%. In the 2nd step, JAK inhibitors, especially momelotinib and pacritinib, can be considered. These drugs exhibit erythropoietic activity as well as favourable effects on splenomegaly and systemic symptoms. Among the available JAKi, Momelotinib shows activity against all three major complications in MF, including anaemia, splenomegaly and constitutional symptoms.&lt;/div&gt;&lt;div&gt;Ruxolitinib (RUX) is the first oral JAK1-2 inhibitor. It received FDA approval in 2011. Long-term data from the COMFORT-I/II studies showed a 30 per cent mortality reduction in intermediate-2/high-risk patients compared to the control group. COMFORT-I and II analyses found th","PeriodicalId":12958,"journal":{"name":"Hematology, Transfusion and Cell Therapy","volume":"46 ","pages":"Page S19"},"PeriodicalIF":1.8,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143340167","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}