Browsing by Author "Ekinci, Omer"

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Acute Hepatitis-A Virus Infection as a Rare Cause of Hemophagocytic Lymphohistiocytosis
(Coll Physicians & Surgeons Pakistan, 2021) Dogan, Ali; Demircioglu, Sinan; Ekinci, Omer
Hemophagocytic lymphohistiocytosis (HLH) is a rare disorder of the mononuclear phagocytic system, characterised by histiocyte and lymphocyte activation. It can be classified as primary and secondary HLH. Primary HLH usually presents in childhood, and is associated with gene mutations. Secondary HLH usually presents in adulthood, and is due to an underlying infection, autoimmune disease or malignancy. We describe a case of HLH secondary to acute hepatitis-A virus infection, which was characterised by persistent fever, pancytopenia, splenomegaly, hyperferritinemia, and hemophagocytosis observed in the bone marrow.
Apheresis in Patients With Sepsis: a Multicenter Retrospective Study
(Pergamon-elsevier Science Ltd, 2021) Aydin, Kaniye; Korkmaz, Serdal; Erkurt, Mehmet Ali; Sarici, Ahmet; Ekinci, Omer; Baysal, Nuran Ahu; Altuntas, Fevzi
Background and objectives: To consider the effectiveness of apheresis, which is a supportive treatment method, in sepsis. Materials and methods: A hundred and eleven adults with sepsis or septic shock were included in this retrospective study. The demographic characteristics of the patients, the focus and source of infection causing sepsis or septic shock, characteristics of the pathogen, Acute Physiological and Chronic Health Assessment (APACHE) II score, routine laboratory values, which apheresis method was used, the characteristics of the replacement fluids used during the apheresis procedure, the number of apheresis procedures, complications related to the apheresis procedure, the follow-up time after the procedure, and mortality were recorded. The primary outcome was 28day mortality. Results: Sixty-nine (62.2 %) of the patients were male. The mean age of the patients was 47.7 +/- 18.6 years. The most common source of sepsis was hospital-acquired (79.3 %), the most common pathogen causing sepsis was gram-negative bacteria (41.4 %), and the most common infection site was the respiratory tract (58.7 %). The median APACHE II score was 19 (13-24). 92 (82.9 %) of the patients had septic shock. Theropeutic plasma exchange (TPE) was performed in 11.7 % of the patients and immunoabsorbtion IA in 88.3 %. The median number of sessions was 3 (3-5). No procedure-related fatal complication was observed in the study. While 28day mortality was 61.3 % in all patients, when the mortality according to the apheresis procedures was examined, it was 11.3 % and 88.2 % in the patients who underwent TPE and IA, respectively. The most common cause of mortality was multiorgan failure. Conclusions: Apheresis in sepsis can be considered as a salvage treatment. The indication for apheresis in sepsis is still at the level of patient-based individualized decision in line with the studies done so far, including our study. However, there is a need for a multicenter randomized controlled study with a large number of patients in order to give positive or negative recommendations about its effectiveness.
Clinical and Laboratory Features of Cd5-Negative Chronic Lymphocytic Leukemia
(int Scientific Literature, inc, 2017) Demir, Cengiz; Kara, Erdal; Ekinci, Omer; Ebinc, Senar
Background: Chronic lymphocytic leukemia (CLL) usually expresses CD5 antigen. However, 7-20% of patients are CD5 negative. We report here a series of 19 CD5-negative B-CLL cases. Material/Methods: We reviewed 19 consecutive CD5-negative B-CLL cases seen in our medical center from 2009 to 2015 and compared them with 105 CD5-positive B-CLL cases. The two groups were compared in terms of clinical parameters, laboratory parameters, and survival characteristics. Results: Lymphadenopathy was present in 31.5% of the CD5-negative group and 51.4% of the CD5-positive group (p=0.029). Splenomegaly was present in 42.1% of the CD5-negative group and 16.1% of the CD5-positive group (p=0.029). There was no difference between the groups in terms of Binet A, B, and C stages (p=0.118, p=0.051, and p=0.882, respectively). The median thrombocyte count was 144x10(9)/L and 160x10(9)/L in the CD5- negative and CD5- positive groups, respectively (p=0.044).There was no difference between the two groups in terms of median neutrophil count (p=0.169). The mean lymphocyte count was 43.2 +/- 4.0x10(9)/L and 36.7 +/- 3.2x10(9)/L in the CD5-negative and CD5-positive groups, respectively (p=0.001). There was no difference between the groups in terms of autoimmune hemolytic anemia and autoimmune thrombocytopenia. In five-year follow-up, 84.2% of CD5-negative patients and 90.5% of CD5-positive patients were alive (p=0.393). Conclusions: We found more isolated splenomegaly, less lymphadenopathy, a higher lymphocyte count, and a lower thrombocyte count in the CD5-negative group. There was no difference between the groups in terms of clinical stage, autoimmune phenomena, hemoglobin and neutrophil count, and survival.
Clinical and Serological Autoimmune Complications in Chronic Lymphocytic Leukemia
(Springer Wien, 2017) Demir, Cengiz; Ekinci, Omer
Autoimmune disorders often develop during the course of chronic lymphocytic leukemia (CLL). The aim of our study was to investigate the incidence of autoimmune complications (AIC) and serological autoantibodies, and to assess the relationship of these to patient characteristics. We prospectively collected screenings of AIC and serological markers from a total of 192 patients. AIC was observed in 18 (9.4%) patients. Autoimmune hemolytic anemia (AIHA) was observed in 8 patients. Autoimmune thrombocytopenia (AITP) was observed in 3 patients. Other various types of AIC were observed in the remaining 7 patients. Serological autoantibodies were positive in 17.2% of patients with CLL. The mean age of patients with AIC was higher than the control group (p = 0.036). Patients with AIC were mostly in advanced disease stage (p = 0.004), and they had received more first-line treatments than the control group (p = 0.003). Patients with AIC had a higher mean age and more advanced disease stage than patients with positive serological autoantibodies (p = 0.020 and p = 0.009; respectively). In addition, patients with AIC had also received more first-line treatment than the patients with positive serological autoantibodies (p = 0.015). Hematologic AIC was associated with older age, advanced disease stage, and treatment. Conversely, non-hematological AIC and serological autoantibodies are generally observed in early stages. Our study has established a coexistence of CLL and autoimmune complications. Hematologists are usually familiar with AIHA and AITP, but less so with non-hematologic AIC. The latter complications should be carefully searched for, particularly in patients with early CLL.
Clinical Features and Responses To Eculizumab of Paroxysmal Nocturnal Hemoglobinuria Patients: a Single-Center Experience
(2019) Sonmez, Gulcin Miyase; Demir, Cengiz; Demircioğlu, Sinan; Ekinci, Omer
Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired clonal hematopoietic stem cell disease classically characterized by chronic intravascular hemolysis, bone marrow failure, and thrombosis. Eculizumab is an anti-C5 monoclonal antibody proven to reduce hemolysis and thrombotic attacks in the treatment of PNH. We aimed to present our data on PNH, a rare disease, and to share our experiences treating PNH with eculizumab. Demographic data, clinical features, history of thrombosis, responses to eculizumab treatment, and survival rates of 9 patients diagnosed with PNH at our hematology center were retrospectively analyzed. The median follow-up period was 46 months (range: 25-62). Five of the patients were female (55.5%), and 4 were male (44.5%), with a median age of 33.5±12.3 years. The mean hemoglobin level was 8.2 g/dL (5.7–10.1 g/dL), mean leukocyte count was 5.80x103/μL (1.72x103/μL–8.30x103/μL), mean platelet count was 96.6x103/L (42x103/μL–214x103/μL), mean lactate dehydrogenase level was 1312 U/L (423–2690 U/L), and mean reticulocyte level was 3.76% (1.1–6.3%). In our study, all cases received eculizumab therapy, of which 8 exhibited full or partial responses, while one was unresponsive to treatment. All patients were alive after the follow-up period. In 8 of the nine patients treated with eculizumab, hemolysis decreased following treatment and blood transfusion was not necessary. The quality of life experienced by all patients was improved. Following determination of the pathogenesis of the PNH, besides the classical treatment methods, eculizumab is a popular treatment option.
Decreased Bone Mineral Density and Associated Factors in Severe Haemophilia a Patients: a Case-Control Study
(Wiley, 2019) Ekinci, Omer; Demircioglu, Sinan; Dogan, Ali; Merter, Mustafa; Yildiz, Saliha; Demir, Cengiz
Introduction Haemophilia patients may exhibit lower levels of bone mineral density (BMD) than the general population for a variety of reasons. Aim We aimed to investigate decreased BMD in people with severe adult haemophilia A (PWH) living in eastern Turkey, and to evaluate the related potential risk factors. Methods The study included 41 PWH and 40 healthy volunteers. Dual-energy x-ray absorptiometry (DXA) was used to measure the BMD. Blood tests and body mass index (BMI) were recorded. The Functional Independence Score in Hemophilia (FISH) test was used to measure functional ability status. Results There was a significant difference between the PWH and control groups with respect to femoral neck and total hip BMD (in g/cm(2)), but the difference for lumbar spine was not significant (P = .017, P < .001, P = .071, respectively). In PWH, patients under 50 years of age, 19.4% were found to have "lower than expected" BMD levels for their age, while 27.8% showed "low normal" levels. In PWH, osteoporosis was found in 60% of the patients over 50 and osteopenia in 20%. Vitamin D insufficiency and deficiency were present in 63.4% of the PWH, significantly higher than the control group (37.5%; P < .001). Conclusion The results indicated that the decrease in BMD was significantly greater in patients with severe haemophilia A than in the normal healthy population. This reduction was correlated with BMI, vitamin D and low functional ability status. However, in multivariate analysis, none of these was a strong independent risk factor.
Endobronchial Plasmacytoma in Patient With Multiple Myeloma
(Wiley, 2017) Sunnetcioglu, Aysel; Ekin, Selami; Bayram, Irfan; Ekinci, Omer; Bugday, Inci Bahadir
Endobronchial plasmacytoma is a rare manifestation of extramedullary plasmacytoma. A 49-year-old woman with a history of multiple myeloma consulted to our pulmonary service with progressive dyspnoea and cough and abnormal chest X-ray. A lesion measuring 6 x 5 cm in size existed in anterior baseline of the right lung's lower lobe in thoracic computed tomography in addition to right bronchial narrowing and atelectasis distal in lesions. Diagnostic bronchoscopy was performed previously for the obstruction and biopsy was taken from the lesion in the right middle lobe bronchus. Endobronchial biopsies showed extensive tumour infiltration with plasmocytoid cells. Immunohistochemistry was positive CD138 and Lamda. Microscopic and immunohistochemical findings supported the diagnosis of extramedullary endobronchial plasmacytoma.
Evaluation of Clinical and Prognostic Features and Treatment Outcomes in Patients With Chronic Lymphocytic Leukemia
(Mattioli 1885, 2021) Ekinci, Omer; Turgut, Ergin
Objective: We aimed to investigate the demographic and clinicopathologic characteristics, treatment responses, survival rates, and prognostic factors affecting survival in patients with chronic lymphocytic leukemia (CLL). Material and Methods: We retrospectively evaluated a total of 131 patients with CLL and-divided into two groups, alive and deceased, based on their situation at the time the data were collected for comparison. Results: The majority of the patients were male (n = 95; 72.5%) and the median age was 62 (35-82) at disease baseline. The mean follow-up time was 31.7 months and overall 3- and 5-year survival rates (OS) were 93.4% and 87.4%, respectively, for all patients. There were significant differences between the alive and deceased group with respect to age, platelet count, hemoglobin level, lactate dehydrogenase, albumin, Rai, modified Rai, and Binet stages, B symptoms, splenomegaly, hepatomegaly and autoimmune hemolytic anemia (AIHA) < 0.05). Regardless of treatment regimen, the treatment response rate in patients receiving first-line treatment was better in alive than in deceased (p < 0.001). Multivariate Cox regression analysis showed the following independent prognostic factors to affect both overall survival (OS) and treatment-free survival (TFS): age <= 64, Binet <= stage B, B symptoms, albumin > 4.1 g/dL, and presence of hepatomegaly. Also, AIHA was an independent prognostic factor affecting only TFS rates. Conclusion: The demographic characteristics of our patients were consistent with the literature, while our 3- and 5-year survival rates were higher. Notably, hepatomegaly and hypoalbuminemia were associated with low OS and TFS. The limitation of the study was the lack of a clear comparison between treatment regimens due to the uneven distribution of the number of patients receiving treatment.
Infective Endocarditis Caused by Gemella Sanguinis: a Case With Fever of Unknown Origin and Anemia
(Coll Physicians & Surgeons Pakistan, 2021) Ekinci, Omer; Ozbek, Emrah
Kronik Lenfositik Lösemi Hastalarında Bruselloz ve Hepatit B Virüsü Seropozitiflik Sıklığı
(2020) Ekinci, Omer; Doğan, Ali; Demir, Cengiz; Ebinç, Senar
Amaç: Kronik lenfositik lösemi (KLL) hastalarında hücresel ve humoral immün yanıtta defektler görülmektedir. Bu immun yetmezlik durumda özellikle gram pozitif ve negatif bakteriyel enfeksiyon sıklığının arttığı bilinmekte, ancak bruselloz gibi zoonozların ve hepatit B virüsü (HBV) gibi viral enfeksiyonların artışı ile ilgili bilgiler net değildir. Bu çalışmada KLL tanılı hastalarda bruselloz ve HBV seropozitiflik sıklığını araştırmayı amaçladık. Yöntemler: 2005–2019 yılları arasında takip edilen KLL tanılı hastalar değerlendirildi. Tüm hastalar içerisinde; HBsAg ve anti–HBs serolojisi için ELISA testi, bruselloz için serum aglütinasyon (Wright) testi kullanılan hastaların test sonuçları kaydedildi. Çalışmaya alınan tüm hastaların demografik verileri ve laboratuvar sonuçları değerlendirildi. Bulgular: KLL tanılı 188 hasta çalışmaya dâhil edildi. Bunların 56 (%29,8)’sı kadın, 132 (%70,2)’si erkek idi. Hastaların medyan yaşı 62 (33–92) idi. Tanı anındaki tam kan sayım parametreleri olarak; medyan lökosit sayısı 54.4×109/L (5.1–312.3×109/L); medyan lenfosit sayısı 42.3×109/L (2.8–296.8×109/L); medyan trombosit sayısı 148×109/L (86.3–342.3×109/L); medyan hemoglobin düzeyi 13.4 g/dL (8.5-16.9 g/dL) idi. Hastaların 142’sine HBsAg ve anti–HBs bakılmıştı. HBsAg kadınların 5(%3,52)’inde, erkeklerin 11 (%7,75)’inde, toplamda 16 (%11,27) kişide pozitif idi. Anti–HBs antikoru kadınların 32 (%22,54)’sinde, erkeklerin 73 (%51,41)’ünde, toplamda ise 105 (73,95) kişide pozitifti. Wright aglütinasyon testi 82 kişide bakılmıştı. Kadınların 3 (%3,66)’ünde, erkeklerin 1 (%1,22)’inde, toplamda 4 (%4,88) kişide Wright testi pozitif saptandı. Sonuç: Aynı bölgede yapılan epidemiyolojik çalışmalar ile karşılaştırıldığında; Wright aglütinasyon testi pozitiflik oranı literatürde sunulan veriler ile uyumlu idi, ancak HBsAg pozitiflik oranı ise çalışmalarda sunulandan daha yüksek olarak izlendi. Bu durumun KLL nedeni ile oluşan immün defekt veya tedavide kullanılan ilaçların oluşturdukları immünsüpresif tablonun HBV bulaş riskini artırması veya viral reaktivasyon ile ilişkili olabileceği düşünüldü.
Measurement of Spleen Stiffness by Shear-Wave Elastography for Prediction of Splenomegaly Etiology
(Lippincott Williams & Wilkins, 2019) Batur, Abdussamet; Alagoz, Sumeyra; Durmaz, Fatma; Baran, Ali Irfan; Ekinci, Omer
Objective The aim of this study was to evaluate the reproducibility of measurement of spleen stiffness at the time of the initial detection of splenomegaly, whether it is found incidentally or not, in determining the etiology of splenomegaly. Methods The pathologies that brought about the diffuse splenomegaly were evaluated in 3 main groups as follows: hepatoportal, myeloproliferative, and infectious causes. In addition, 17 healthy control patients were recruited. All patients were examined with acoustic radiation force impulse imaging with VTQ. Results The difference between the splenic parenchymal elasticity values in the hepatoportal group (3.27 +/- 0.36 m/s), in the myeloproliferative disease group (2.98 +/- 0.33 m/s), in the infectious disease group (2.44 +/- 0.21 m/s), and in the control group (2.08 +/- 0.19 m/s) was found to be statistically significant (P = 0.001). The intraclass correlation coefficient for shear wave velocity measurement between hepatoportal causes and myeloproliferative causes was 71.2% (95% confidence interval [CI], 54.9%-87.4%), between hepatoportal causes and infective causes was 99.7% (95% CI, 98.6%-100.0%), and between myeloproliferative causes and infective causes was 83.3% (95% CI, 68.8%-97.9%). In the same patient groups, spleen volumes were measured as 64.08 +/- 9.66, 78.18 +/- 18.52, and 51.57 +/- 7.44 cm(2), respectively; in the control group, it was 26.75 +/- 6.57 cm(2). The difference between spleen volumes was found to be statistically significant (P = 0.001). Conclusions Distinguishing the causes of splenomegaly is important because the disorders require different management strategies. In diseases that cause splenomegaly, tissue content may change according to pathogenesis. Such changes in the spleen are mechanical properties that can be quantified by elastography.
A Multicenter Retrospective Analysis on Therapeutic Plasma Exchange in Immune Thrombocytopenic Purpura
(Pergamon-elsevier Science Ltd, 2021) Basturk, Abdulkadir; Sayin, Serhat; Erkurt, Mehmet Ali; Sarici, Ahmet; Ekinci, Omer; Kum, Asli; Altuntas, Fevzi
Immune Thrombocytopenia (ITP) is an autoimmune disease characterized by thrombocytopenia and skin and mucosal bleeding. In patients with an indication for treatment, corticosteroids, intravenous immunoglobulin (IVIg) and anti-D are recommended as the first line, while splenectomy, thrombopoietin receptor agonists or rituximab are recommended second line options. Approximately 10 % of adult patients with ITP fall into the chronic refractory ITP group. Therapeutic plasma exchange (TPE) has generally been tested in patients with refractory ITP, who have failed to respond to conventional treatments, in case of bleeding or prior to surgical interventions. It has been stated that elimination of the antibodies that are held responsible in the pathogenesis of the disease has an effective role in the treatment. In this article, we present the results of 17 patients, who underwent TPE for refractory ITP, together with the literature data.
Nadir Bir Pansitopeni Sebebi: Transfüzyona Bağlı Graft Versus Host Hastalığı
(2020) Doğan, Ali; Demir, Cengiz; Demircioğlu, Sinan; Ekinci, Omer; Mamiş, Yasin
Transfüzyonla ilişkili greft versus-host hastalığı (Ta-GVHD) nadir görülen fakat mortal seyirli bir transfüzyon reaksiyonudur. Genellikle son bir ayiçinde kan transfüzyonu yapılmış kişilerde cilt döküntüleri, sarılık, ishal ve pansitopeni bulguları ile ortaya çıkar. Herhangi bir tedavisi olmadığı içingereksiz kan transfüzyonundan kaçınılması veya transfüze edilecek kan ürünlerini ışınlaması en önemli korunma yöntemleridir. Pansitopeni etyolojisinde transfüzyon öyküsünün sorgulanmasının önemini vurgulamak amacıyla vücudunda yaygın döküntüler, ateş, sarılık ve pansitopeni ile başvuran bir hastada tanı konulan Ta-GVHD olgusu sunuldu
Presence of Paroxysmal Nocturnal Hemoglobinuria in Patients With Idiopathic Portal Vein Thrombosis: a Single-Center Study
(Tubitak Scientific & Technological Research Council Turkey, 2020) Demir, Cengiz; Ebinc, Senar; Ekinci, Omer
Background/aim: Paroxysmal nocturnal hemoglobinuria (PNH) is a very rare clonal hematopoietic stem cell disease characterized by chronic hemolytic anemia and thrombosis. We report data from a study of the occurrence of PNH among patients with idiopathic portal vein thrombosis (PVT). Materials and methods: Patients who were followed up with the diagnosis of idiopathic PVT were enrolled into this study. Those with laboratory and/or imaging evidence of any local or systemic factor that could lead to PVT were excluded. PNH clone was examined in all patients using established FLAER methodology. Results: A total of 112 patients (42 males and 70 females), none of them had a markedly PNH clone, but 4 patients (3.6%) with confirmed tests two times had small PNH clones (size between 3.02% and 4.62%). The median ages of PNH clone (-) and PNH clone (+) patients were 42 (range; 24-59) vs 39 (range; 36-42) years, respectively. The median hemoglobin concentration, platelet count and leukocyte count were lower in the PNH clone (+) group than the PNH clone (-) group. Anemia, thrombocytopenia, and leukopenia were detected in all PNH clone (+) patients. In addition, the PNH clone positivity size in monocytes was higher than erythrocytes in all of 4 patients. Conclusions: PNH should be considered during differential diagnosis among patients with idiopathic PVT. Small PNH clones can be detected in PVT patients that we cannot clearly determine its relationship with PVT. We need furthermore studies to explore the potential role of this finding.
Prognostic Significance of Flow Cytometric Immunophenotyping in Patients With Acute Myeloid Leukemia
(Galenos Publ House, 2022) Demircioglu, Sinan; Ekinci, Omer; Dogan, Ali; Ulas, Turgay
Objective: Chromosomal abnormalities are one of the most important prognostic factors in acute myeloid leukemia (AML). However, not all patients may have such informative chromosomal abnormalities. Although there are many studies on the prognostic value of immunophenotyping in AML, it is still not used as a prognostic marker. In this study, we aimed to investigate the effects of CD13, CD33, CD34, CD117, MPO and HLADR expressions on prognosis of non-acute promyelocytic leukemia AML.Methods: One hundred thirteen patients diagnosed as having non -acute promyelocytic leukemia AML and followed up between 2010 and 2018 were included in this study. The associations of CD13, CD33, CD34, CD117, MPO and HLA DR expressions with chemotherapy response, progression free survival (PFS) and overall survival (OS) were statistically analyzed.Results: It was seen that response to chemotherapy was achieved in 67.3% of the patients. Median PFS duration was 9 months and median OS duration was found as 13 months. Of the immunophenotypic characteristics, only MPO expression was determined to be an independent risk factor for PFS and OS.Conclusion: Immunophenotypic features may be helpful in the diagnosis of AML as well as give an idea about prognosis. In this study, MPO expression was shown to be an independent risk factor for PFS and OS in our own patient population.
Relaps / Refrakter T Hücreli Lenfomada Brentuximab Vedotin Monoterapisi- Gerçek Yaşam Verisi
(2024) Darçın, Tahir; Serın, Istemi; Ugur, Mehmet Can; Ekinci, Omer; Yonal-hindilerden, Ipek; Akpınar, Seval; Demircioğlu, Sinan
Amaç: Brentuksimab vedotin (BV) ile tedavi edilen Relaps /Refrakter (R/R) THL’li hastaların gerçek yaşam verilerini sunmayı amaçladık. Gereç ve Yöntem: Bu çalışma gözlemsel, çok merkezli, retrospektif bir çalışmadır. Ocak 2014’ten Temmuz 2020’ye kadar Türkiye’deki on üç merkezde yalnızca BV ile tedavi edilen tüm hastaların (n=17) verileri toplandı. Bulgular: Ortanca yaşı 53 olan 17 hastaya kurtarma kemoterapisi olarak BV verildi. Dokuz (%52,9) hastaya periferik T hücreli lenfoma, diğer türlü sınıflandırılamayan tanısı konurken, 8 (%47,1) hastaya anaplastik büyük T hücreli lenfoma tanısı konuldu. Kohortun ortanca takip süresi 20 aydı. Dokuz (%52,9) hastada tam yanıt, 5 (%29,5) hastada kısmi yanıt, 3 (%17,6) hastada ilerleyici hastalık görüldü. Güvenlik verileri BV bilinen profiliyle tutarlıydı, 2 pnömoni ve 4. dereceli 1 trombositopeniyi içeriyordu. Grubun medyan progresyonsuz sağkalımı 10 aydı. Siklus sayısının BV tedavisine yanıtın üzerinde etkili olduğu değişkenli analiz ile bulundu. Sonuç: R/R THL’leri olan hastalarda BV olumlu güvenlik profili ile tatmin edici antitümör aktivitesine sahip olduğu görülmektedir.
Relapsed/Refractory Thrombotic Thrombocytopenic Purpura Treated With N-Acetylcysteine: a Case Report
(Sage Publications Ltd, 2018) Demircioglu, Sinan; Ekinci, Omer; Dogan, Ali; Demir, Cengiz
Thrombotic thrombocytopenic purpura is a rare condition that presents with microangiopathic haemolytic anaemia, thrombocytopaenia, fever, renal impairment and neurological symptoms. Plasma exchange is a lifesaving treatment for this condition. However, some cases may be non-responsive to plasma exchange, or loss of response may occur. Treatment options for refractory cases include high-dose corticosteroids, rituximab, vincristine, cyclophosphamide, splenectomy, bortezomib and N-acetylcysteine. We present a refractory case of thrombotic thrombocytopenic purpura responding to the last of these therapies.
Spleen Stiffness Measurement by Using Shear-Wave Elastography as a Predictor of Progression To Secondary Myelofibrosis
(Lippincott Williams & Wilkins, 2021) Ekinci, Omer; Ozgokce, Mesut; Turko, Ensar; Merter, Mustafa
Objective Polycythemia vera (PV) and essential thrombocythemia (ET) are chronic myeloproliferative diseases that can transform to secondary myelofibrosis (SMF). In this study, we evaluated spleen stiffness using shear-wave elastography (SWE) as a predictor of progression to SMF. Methods Participants were grouped as healthy volunteers (HVs), PV/ET patients, and SMF patients. Participants' spleen sizes, spleen stiffness values, bone marrow fibrosis degrees, and the other parameters were evaluated. Spleen stiffness values and spleen sizes were compared between groups. Results Of the 121 participants included in this study, 52 patients were HVs, 52 patients were PV and/or ET patients, and 17 patients were SMF patients. In terms of age and sex, there was no difference between groups. Splenic parenchymal stiffness median values by using SWE were found to be 0.82 m/s in HVs, 1.41 m/s in PV/ET patients, and 2.32 m/s in SMF patients (P < 0.001). In terms of median length of the spleen, the difference between groups was significant (P < 0.001). In addition, we found a significant positive correlation between spleen stiffness and bone marrow fibrosis degree (P < 0.001, r = 0.757). However, in multivariate analysis, there was no strong independent risk factor for spleen stiffness. Conclusion In this study, we showed that measurement of spleen stiffness using SWE can distinguish SMF from PV/ET patients and HVs. Therefore, we believe that SWE may be used as a noninvasive and easily accessible method to check the fibrotic progression of bone marrow in PV and ET patients to monitor the transformation to SMF, and enables to detect fibrosis in early phase.
Therapeutic Plasma Exchange in Gastric Signet Ring Cell Carcinoma Presenting as Microangiopathic Hemolytic Anemia: a Rare Case Report
(Wiley, 2022) Candar, Omer; Ekinci, Omer; Merter, Mustafa; Aslan, Mehmet; Aras, Ibrahim
Microangiopathic hemolytic anemia (MAHA) defines a group of disorders characterized by the formation of microthrombi in capillaries and arterioles and the fragmentation of erythrocytes that pass through. Cancer-related MAHA is a rare but serious condition that is encountered in patients diagnosed with a malignancy. This clinical picture is thought to be linked to certain tumor characteristics; particularly, adenocarcinoma histology, vascular invasion, and bone marrow infiltration. MAHA is most commonly associated with tumors of gastric, prostate, and breast origin. The optimal treatment is not clear; however, there is evidence for the importance of promptly starting an effective antineoplastic regimen and it was also reported that administering therapeutic plasma exchange (TPE) therapy for immunocomplex removal could be beneficial for patients with symptoms of bleeding and thrombosis. Here, we present a case that presented a picture of MAHA secondary to gastric signet-ring cell adenocarcinoma (SRCC). The clinical picture was initially evaluated as thrombotic thrombocytopenic purpura and the patient benefited significantly from the TPE treatment administered before the adenocarcinoma diagnosis was confirmed. In this period, epistaxis stopped, platelet count increased from 25 x 10(9)/L to 162 x 10(9)/L, fragmented erythrocyte rate in the peripheral smear decreased by more than 75% and other laboratory findings of hemolysis (LDH, bilirubin, etc.) significantly improved.