Celiac disease is a systemic disease in which the natural and adaptive immune system is affected by the effect of gluten exposure and environmental factors in individuals with genetic ...predisposition. Multiple myeloma; is characterized by an increase in clonal plasma cells. It is the most common hematological malignancy after lymphomas.We aimed to present a case siagnosed with celiac disease and multipl myeloma
A 56-year-old female patient with a diagnosis of asthma and celiac disease for 1 year was referred to the Hematology department because her refractory anemia. Serum IgA level of the patient was 4490 mg/dl without renal failure and hypercalcemia.bone marrow biopsy compatible with myeloma. The patient received 6 cycles of bortezomib, cyclophosphamide, and dexamethasone and 3 cycles lenalidomid dexametazon chemotherapy.After chemotherapy, Autologous stem cell transplantation was performed.
Celiac disease is an autoimmune disease, characterized by inflammation and villus atrophy in the small intestine mucosa as a result of sensitivity to gluten, resulting in malabsorption. The incidence of lymphoma and gastrointestinal system malignancy is increased in individuals with celiac disease. Multiple myeloma may also be accompanied by autoimmune diseases such as ankylosing spondylitis, scleroderma, and sjögren's syndrome. Coexistence of multiple myeloma and celiac disease is rare.
•Fundus examination is mostly normal in cases of iron deficiency anemia. In our study, we found that both macular and optic disc small vessel density decreased significantly in patients with iron ...deficiency anemia on optical coherence tomography angiography. Optical coherence tomography angiography may be valuable in monitoring the risk of developing retinopathy associated with anemia.
To investigate effect of iron deficiency anemia (IDA) on macular and radial peripapillary capillary (RPC) vascular changes by optical coherence tomography angiography (OCTA).
Thirty-three patients with IDA and 33 healthy controls were enrolled in the study. Foveal avascular zone (FAZ) area, macular superficial capillary plexus (SCP) and deep capillary plexus (DCP) vessel density and RPC vessel density were evaluated by the AngioVue Imaging System. Hemoglobin (Hb), hematocrit (Hct), mean corpuscular volume (MCV), mean corpuscular hemoglobin (MCH), red cell distribution width (RDW), serum iron, total iron-binding capacity (TIBC), serum ferritin and transferrin saturation values were also recorded.
There were no statistically significant differences between the two groups in terms of FAZ area and FAZ perimeter while foveal density (FD) was significantly decreased in the IDA group. Compared to control group, IDA group revealed significantly decreased macular vessel density in all macular regions except fovea in both SCP and DCP. RPC vessel density was significantly decreased in whole image, peripapillary and superior-hemifield area wihout RNFL thinning. Hemoglobin level was positively correlated with SCP whole and RPC whole vessel density and serum iron level was also positively correlated with SCP whole vessel density.
Macular and optic disc vessel density were reduced in IDA patients. OCTA may be useful in detecting retinal ischemia before clinically visible signs of retinopathy associated with IDA appear.
Deletion of 13q14 del(13q) is the most common cytogenetic change (50%) in chronic lymphoblastic leukemia (CLL), and it is a good prognostic factor if it is detected as a sole aberration by FISH. ...However, it is observed the clinical course of CLL cases with del(13q) are quite heterogeneous and the responsible for this clinical heterogeneity has not been established yet. Some investigators suggest type II deletion (include RB1 gene) is associated with more aggressive clinical course. Also, it is suggested that the deletion burden and the deletion type have a prognostic effect. In this study, we aimed to investigate the effect of RB1 gene deletion, deletion burden and deletion type on overall survival (OS), disease stage and time to first treatment (TTFT) in patients with isolated del(3q). Sixty eight cases, detected isolated del(13q) were included in the study. Also, RB1 deletion was analyzed from peripheral blood of them using FISH.
RB1 deletion was detected in 41% of patients, but there was no statistically significant difference between RB1 deletion and TTFT, stage and OS (p > 0.05). At same time, statistically significant difference was detected between high del(13q) (> 80%) and TTFT (p < 0.05).
The statistical analysis of our data regarding to the association between RB1 deletion and deletion type, TTFT, disease stage, and OS has not confirmed type II deletion or biallelic deletion cause poor prognosis. However, our data supports the deletion burden has a prognostic effect. More studies are needed to elucidate the cause of the clinical heterogeneity of CLL cases with del(13q).
Aim: Classical Hodgkin lymphoma is common, it is one of the lymphomas whose differential diagnosis can be difficult. It is thought that Hodgkin cell may originate from the germinal center. Our aim in ...this study was to determine the germinal center transformation markers OCT-2, BOB.1, BCL-6, PAX-5, CD20 and MUM-1 in Classic Hodgkin Lymphoma (CHL), Nodular Lymphocyte Predominant Hodgkin Lymphoma (NLPHL) and Diffuse Large B-cell Lymphoma (DLBCL) to evaluate the expressions of by immunohistochemical method and chromogenic in-situ hybridization (ISH) of EBV early RNAs (EBER).
Material and methods: 49 biopsies diagnosed with Hodgkin lymphoma (HL), 5 with NLPHL and 19 with DLBCL were evaluated for CD30, PAX-5, OCT-2, BOB.1, MUM-1, BCL-6, and CD20, and EBER positivity. SPSS 18 was used for statistical analysis.
Results: 73 lymphoma cases were included in the study, 61.6% males and 38.4% females. The median age of patients was 50 years. CHL (67.1%) was the most common lymphoma type, and mixed cellular Hodgkin lymphoma (MSHL) was the most common subtype. There was a statistically significant difference in CD30, OCT-2, BOB.1, MUM-1, PAX-5, CD20, BCL-6, EBER expression between CHL and DLBCL cases (p
ÖZ
Amaç: Klasik Hodgkin Lenfoma sık görülmek ile birlikte ayrıcı tanısı zor olabilen lenfomalardandır. Hodgkin hücresinin germinal merkezli olabileceği düşünülmektedir. Bu çalışmadaki amacımız, Classic Hodgkin Lymphoma (CHL), Nodular Lymphocyte Predominant Hodgkin Lymphoma (NLPHL) ve diffuse large B-cell lymphoma (DLBCL)’da germinal merkez transformasyon belirteçleri olan OCT-2, BOB.1, BCL-6, PAX-5, CD20 ve MUM-1’in ekspresyonlarını immünohistokimyasal yöntem ile, EBV early RNAs (EBER)’in chromogenic in-situ hybridization (ISH) ile değerlendirmektir.
Gereç ve yöntemler: 49 Hodgkin lenfoma (HL), 5'i NLPHL ve 19'u DLBCL tanılı biyopsi CD30, PAX-5, OCT-2, BOB.1, MUM-1, BCL-6 ve CD20 ve EBER pozitifliği açısından değerlendirildi. İstatistiksel analiz için SPSS 18 kullanıldı.
Bulgular: Çalışmaya %61.6 erkek ve %38.4 kadın olmak üzere 73 lenfoma olgusu dahil edildi. Hastaların medyan yaşı 50 idi. En sık görülen lenfoma tipi CHL (%67,1), en sık görülen alt tip mikst hücreli Hodgkin lenfoma (MSHL) idi. CHL ve DLBCL olguları arasında CD30, OCT-2, BOB.1, MUM-1, PAX-5, CD20, BCL-6, EBER ekspresyonu açısından istatistiksel olarak anlamlı fark vardı (p
Aim
To evaluate the inflammatory parameters and oxygenation in severe coronavirus disease‐19 patients who underwent extracorporeal cytokine adsorption (CA).
Methods
Patients who underwent ...extracorporeal CA for cytokine storm were included in the study. The changes in oxygenation, laboratory parameters, and mortality rates were investigated.
Results
Thirty‐six patients were included in the study. The hemoglobin, thrombocyte, and C‐reactive protein (CRP) decreased, and PaO2/FiO2 ratio increased (p < 0.001; p < 0.01; p < 0.001; p = 0.04, respectively). Twelve (33.3%) patients received a single session, 24 (66.6%) received 2 or more sessions. CRP and fibrinogen levels decreased, and PaO2/FIO2 ratio increased in the single session group (p = 0.04; p = 0.04; p = 0.01, respectively). In the multi‐session group, the hemoglobin, platelet, procalcitonin, and CRP levels decreased, and PaO2/FIO2 ratio increased (p < 0.01; p = 0.02; p = 0.02; p < 0.01; p = 0.01, respectively). Day 15, 30, and 90 mortality rates were 61.1%, 83.3%, and 88.9%.
Conclusion
CA with hemoperfusion reduced CRP and improved oxygenation; however, mortality rates were high.
To evaluate the macular and optic disc vascular changes in vitamin B12 deficiency anemia.
A total of 24 patients with vitamin B12 deficiency anemia and 24 healthy controls were involved in this ...study. All participants were evaluated for central macular thickness (CMT), peripapillary retina nerve fiber layer (RNFL) thickness, foveal avascular zone (FAZ) area, macular vessel density (VD) in superficial capillary plexus (SCP) and deep capillary plexus (DCP), choriocapillaris flow area, and optic disc radial peripapillary capillary (RPC) VD using optic coherence tomography (OCT) and optic coherence tomography angiography (OCTA). Metabolic parameters were also noted.
Temporal RNFL thickness significantly decreased in the B12 deficiency anemia group (p = 0.04). Choriocapillaris flow area (p = 0.045) and macular vessel density in both SCP (p = 0.022) and DCP (p = 0.018) markedly declined in the study group. Optic disc RPC VD in the B12 deficiency anemia group was lower in all regions compared to that of the control group, but the difference was not statistically significant (p > 0.05). There were significant positive correlations between choriocapillaris flow area, macular VD, vitamin B12, and hemoglobin.
Retinal vascular alterations were observed in B12 deficiency anemia, and OCTA may be beneficial in the diagnosis and follow-up of ocular complications in these cases.
Thrombocytopenia is among the most common complications following hematopoietic stem cell transplantation and is associated with increased mortality and morbidity with no standard treatment yet. In ...this multicenter and retrospective study, we aim to present our multi-center experience of Eltrombopag treatment in patients with isolated thrombocytopenia following HSCT.
A total of 73 patients from 5 centers who underwent autologous or allogeneic stem cell transplantation, had no primary disease relapse, all of whom had neutrophil engraftment, complete chimerism, and who were diagnosed with Prolonged Isolated Thrombocytopenia (PIT) or Secondary Failure Of Platelet Recovery (SFPR) were included in the study. The patients were initiated on Eltrombopag at a dose of 50–150 mg. Complete response was defined as a platelet count >50×109/L for 7 consecutive days with no transfusion support.
A total of 50.3% of the patients underwent Autologous and 49.7% Allogeneic Stem Cell Transplantation, 54.8% were diagnosed with PIT, and 45.2% were diagnosed with SFPR, and the treatment with 50–150 mg/day Eltrombopag was initiated on the median day +42. Complete response was achieved in 71.2% of these patients on the median day 23 of the treatment. No significant effects of the initial dose (50–150 mg/day) were detected in the Complete Response in the multivariate analysis on response. An insufficient number of Megakaryocytes in the bone marrow before Eltrombopag treatment was determined as an independent risk factor in determining the response (OR 3.57, 95% CI 1.21–10.55). The overall survival of the patients who did not respond to Eltrombopag was found to be significantly worse than that of patients who responded (p=0.022, HR:2.74, 95% CI 1.12–6.54).
As a result of the present study, Eltrombopag treatment was found to be effective and safe in thrombocytopenia that develops following hematopoietic stem cell transplantation. It was concluded that its use may be more effective in patients with sufficient bone marrow megakaryocytes before the treatment and an initial dose of 50 mg/day may be appropriate in terms of cost, effectiveness, and toxicity. Large-scale randomized and controlled prospective studies are needed to determine the roles of Eltrombopag treatment in patients with post-transplant PIT and SFPR.
The incidence of cancer is increasing in the world. With the developments in cancer treatment, the life expectancy of patients is prolonged and the incidence of secondary malignancies is increasing. ...We retrospetively patients with syncronous / metachronous oncological malignancies accompanying hematological malignancies in a newly established hematology center. Data were obtained from the medical records. Demographic data, treatments and overall survival of the patients were evaluated. Twenty eight (6%) of 433 patients hematological malignancies were included in the study. 12 patients (42.9) were diagnosed with syncronous and 16 (57.1%) patients with metachronous hematologic-oncologic tumors. Sixteen of the patients were male,twelve were female. In syncronous tumors, the most common hematologic malignancy was Non-hodgkın lymphoma (NHL), while the most common oncologic malignancies were thyroid papillary cancer and colon cancer. In metachronous tumors, the most common malignancies were NHL and breast cancer. The median time between diagnosis of metachronous tumors was 49.5 months (8-192 months). The median survival of patients with syncronous malignancies was 19 months (SE=12.19) (95% CI 0-42.89), with metachronous malignancies was 22 months (SE=14.0) (95% CI 0-49.44). There was no statistically significant difference in the comparison of survival curves of patients with syncronous and metachronous malignancies (p=0.382). Oncological malignancies accompanying hematological malignancies are not uncommon. There is no standart treatment for syncronous / metachronous hematologic malignancies. In the presence of syncronous multipl malignancies should be evaluated individually.
Dünyada kanser görülme sıklığı giderek artmaktadır. Kanser tedavisindeki gelişmelerle birlikte hastaların ortalama yaşam süreleri uzamakta ve sekonder malignitelerin görülme sıklığı artmaktadır. Yeni kurulan bir hematoloji merkezinde hematolojik malignitelere eşlik eden senkron / metakron maligniteleri retrospektif olarak inceledik.Veriler tıbbi kayıtlardan elde edildi.Hastaların demografik verileri, tedavileri ve genel sağ kalımları değerlendirildi. Hematolojik maligniteli 433 hastanın 28’i (%6) çalışmaya devam edildi. 12 hasta (%42,9) senkron, 16 (%57,1) hasta ise metakron hematolojik-onkolojik tümör tanısı almıştır. Hastaların 16’sı erkek, 12’si kadındı. Senkron tümörlerde en sık görülen hematolojik malignite non-hodgkın lenfoma (NHL), en sık görülen onkolojik maligniteler ise tiroid papiller kanseri ve kolon kanseri idi. Metakron tümörlerde en sık görülen maligniteler NHL ve meme kanseri idi. Metakron tümörlerin tanısı arasındaki medyan süre 49,5 aydı (8-192 ay). Senkron malignitesi olan hastalrın medyan sağkalımı 19 aydı (SE=12,19) )%95 CI 0-42,89), metakron maligniteleri olan hastaların medyan sağkalımı 22 aydı (SE=14,9= (%95 CI 0-49,44). Senkron ve metakron maligniteleri olan hastaların sağkalım eğrilerinin karşılaştırılmasında istatistiksel olarak anlanmlı fark saptanmadı (p=0,382). Hematolojik malignitelere eşlik eden onkolojik maligniteler nadir değildir. Senkron / metakron hematolojik maligniteler için standart bir tedavi yoktur. Senkron multipl malignite varlığında, malignitelerin her biri ayrı ayrı değerlendirilmelidir.
Objective: Chronic myeloid leukemia (CML) is one of the most common hematological malignancies derived from the BCR/ABL1 fusion gene. Patients with CML generally manifest leukocytosis with basophilia ...and neutrophilia. The verification of CML is often based on the detection of BCR/ABL1 fusion. We aimed to investigate the impact of peripheral blood (PB) differential and complete blood count (CBC) on BCR/ABL1 p210 test ordering in patients with suspected CML.
Methods: We performed a retrospective assessment of patients tested for the first time for BCR/ABL1 p210 fusion. We obtained clinical and laboratory findings of 235 patients from the database of our clinic. BCR/ABL1 p210 fusion was detected by quantitative real-time polymerase chain reaction (RT-qPCR). We implemented t-tests or Mann–Whitney U tests for the comparison of continuous data. We plotted the receiver operating characteristic (ROC curves) and calculated the area under the ROC curve (AUC) for each parameter.
Results: Among 235 patients, 25 (%10.6) received a new diagnosis of CML. CML patients had significantly increased white blood cell count (WBC) with differential. Absolute basophil count showed the highest area under the ROC curve (AUC) value of 0.829, which had a cut-off value of 0.3 × 103/ µL. 76.00% of CML cases had an absolute basophil count of ≥0.3 × 103/µL, while 95.24% of the non-CML cases had an absolute basophil count of
Amaç: Kronik miyeloid lösemi (KML), BCR/ABL1 füzyon geninden kaynaklanan en yaygın hematolojik malignitelerden biridir. KML'li hastalar genellikle bazofili ve nötrofili içeren lökositoz gösterirler. KML'nin doğrulanması genellikle BCR/ABL1 füzyonunun saptanmasına dayanır. KML şüphesi olan hastalarda BCR/ABL1 p210 testi için periferik kan (PK) diferansiyeli ve tam kan sayımının (TKS) etkisini araştırmayı amaçlıyoruz.
Metot: BCR/ABL1 p210 füzyonu için ilk kez test edilen hastaların retrospektif bir değerlendirmesini yaptık. Kliniğimiz veri tabanından 235 hastanın klinik ve laboratuvar bulgularını elde ettik. BCR/ABL1 p210 füzyonu, kantitatif Gerçek Zamanlı Polimeraz Zincir Reaksiyonu (RT-qPCR) ile tespit edilmiştir. Sürekli verilerin karşılaştırılması için t-testi veya Mann-Whitney U testleri uyguladık. Her parametre için alıcı çalışma karakteristiğini (ROC eğrileri) çizdik ve ROC eğrisi altındaki alanı (AUC) hesapladık.
Bulgular: 235 hastanın 25'ine (%10.6) yeni KML tanısı kondu. KML hastalarında, diferansiyel ile önemli ölçüde artmış beyaz kan hücresi sayısı (WBC) vardı. Mutlak bazofil sayısı, 0,3 × 103/uL'lik bir kesme değerine sahip olan 0,829'luk ROC eğrisi (AUC) değerinin altındaki en yüksek alanı gösterdi. KML vakalarının %76,00'ında mutlak bazofil sayısı ≥0,3 × 103/uL iken, KML olmayan vakaların %95,24'ünde mutlak bazofil sayısı
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