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Objective: Familial hypercholesterolemia (FH) is an autosomal dominant inherited genetic disorder that causes a significant increase in low-density lipoprotein (LDL) cholesterol levels and leads to early coronary heart disease and cardiac mortality. Although this disease has a heterozygous (HeFH) and homozygous (HoFH) form, the incidence of HeFH is reported to be 1: 500, while HoFH is reported to be 1: 1 000 000. This disease is often caused by LDL receptor (most common), apolipoprotein B (Apo B), Proprotein Convertase Subtilisin/Kexin 9 (PCSK9), and LDL receptor adaptor protein (LDLRAP) gene mutations. Patients with FH do not respond well to lipid-lowering therapies such as a statin, and so lipoprotein apheresis is the treatment of choice. Material and Methods: We compared the results of lipoprotein apheresis in a 20-year-old female patient diagnosed with HoFH with two different methods [double-filtration plasmapheresis (DFPP) and dextran sulfate column (DSC) methods]. 40 lipoprotein apheresis procedures including 20 sessions of DFPP and 20 sessions of DSC were evaluated. Results: When the two methods were compared the changes in high-density lipoprotein, white blood cells, platelets, potassium, calcium, prothrombin time (INR) and activated partial thromboplastin time values were statistically significant. Conclusions: In conclusion, our study suggests that it would be more appropriate to choose the apheresis method according to the clinical and laboratory findings of the patient since it shows that two different methods have different effects on serum electrolyte values, hemostasis criteria and leukocyte and platelet counts.

Thrombotic microangiopathy (TMA) associated with hematopoietic stem cell transplantation, remains a difficult complication due to its high mortality rate, lack of standardised diagnostic criteria, and limited treatment options. Although the etiology of the disease is not clear, medications, radiotherapy, graft-versus-host disease (GVHD), angioinvasive fungal, viral infections and complement activation have been implicated. Agents such as therapeutic plasma exchange (TPE), steroids, rituximab, defibrotide, eculizumab are used in its treatment. The use of treatments such as TPE, steroids, defibrotide, and rituximab did not yield satisfactory results in transplant-associated TMA (TA-TMA). Recent studies have reported successful results with eculizumab in the treatment of TA-TMA. As there is no standard dose for eculizumab in the treatment of TA-TMA, doses used in atypical hemolytic uremic syndrome (aHUS) were administered. In this paper, we present a case that was unresponsive to TPE and steroids, but which was successfully treated with eculizumab. Key Words: Acute myeloid leukemia, Stem cell transplantation, Thrombotic microangiopathy, Apheresis, Eculizumab.

Objective: Pomalidomide is a new generation thalidomide analogue. Effectiveness as a single agent or combination with low dose dexamethasone has been in the treatment of relapse/refractory Multiple Myeloma (MM). The aim of the present study was to share the experience of different oncology centres with pomalidomide treatment in patients with relapsed/refractory MM. Materials and Methods: Seventy-three patients from 16 centres were enrolled into the study. The patients were followed for a median of 6 months. Relapsed/refractory MM patients who received at least one line of treatment before pomalidomide were included into the study. ISS, R-ISS and Eastern Cooperative Oncology Group (ECOG) scores of the patients and treatment-related side effects were evaluated. Results: As a result of the median follow-up for 6 months, 36% (26/72) of the patients presented progression. The estimated median PFS was found 29 months. The Cox regression analysis revealed that ECOG affected PFS only, myeloma subtype; ISS and R-ISS scores did not affect PFS. The most common side effects with pomalidomide treatment in our population include neutropenia, infections, anaemia and thrombocytopenia. Conclusion: In our study, it was statistically shown that the ECOG score was effective in survival in relapsed / refractory MM patients treated by pomalidomide. Therefore, we recommend evaluation of the ECOG score for each patient before treatment in eligible cases.

Introduction Bing-Neel syndrome (BNS) is a rare complication of of Waldenström macroglobulinemia (WM) identified by involvement of central nervous system (CNS) lymphoplasmacytic cells. Case report We present a patient who was diagnosed with Bing-Neel syndrome four years after the diagnosis of Waldenström macroglobulinemia. Management & outcome The patient was admitted with neurological symptoms. There were lesions associated with WM involvement on brain imaging. The diagnosis was made by brain biopsy. High dose methotrexate treatment was given. Discussion CNS infiltrating agents such as fludarabine, methotrexate and cytarabine are often used in BNS treatment. Ibrutinib, which is a new bruton tyrosine kinase inhibitor, has recently started to be used in BNS treatment, as it has been shown to be effective and penetrate the CNS.

Patients with solid malignancies are more vulnerable to severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) infection than the healthy population. The outcome of SARS-CoV-2 infection in highly immunosuppressed populations, such as in patients with hematological malignancies, is a point of interest. We aimed to analyze the symptoms, complications, intensive care unit admissions, and mortality rates of patients with hematological malignancies infected with SARS-CoV-2 in Turkey.In this multicenter study, we included 340 adult and pediatric patients diagnosed with SARS-CoV-2 from March to November 2020. Diagnosis and status of primary disease, treatment schedules for hematological malignancies, time from last treatment, life expectancy related to the hematological disease, and comorbidities were recorded, together with data regarding symptoms, treatment, and outcome of SARS-CoV-2 infection.Forty four patients were asymptomatic at diagnosis of SARS-CoV- 2 infection. Among symptomatic patients, fever, cough, and dyspnea were observed in 62.6%, 48.8%, and 41.8%, respectively. Sixty-nine (20%) patients had mild SARS-CoV-2 disease, whereas moderate, severe, and critical disease was reported in 101 (29%), 71 (20%), and 55 (16%) patients, respectively. Of the entire cohort, 251 (73.8%) patients were hospitalized for SARS-CoV-2. Mortality related to SARS-CoV-2 infection was 26.5% in the entire cohort; this comprised 4.4% of those patients with mild disease, 12.4% of those with moderate disease, and 83% of those with severe or critical disease. Active hematological disease, lower life expectancy related to primary hematological disease, neutropenia at diagnosis of SARS-CoV-2, ICU admission, and first-line therapy used for coronavirus disease-2019 treatment were found to be related to higher mortality rates. Treatments with hydroxychloroquine alone or in combination with azithromycin were associated with a higher rate of mortality in comparison to favipiravir use.Patients with hematological malignancy infected with SARS-CoV-2 have an increased risk of severe disease and mortality.Solid malignite hastalarının şiddetli akut solunum yolu enfeksiyonu-koronavirüs-2 (SARS-CoV-2) enfeksiyonuna sağlıklı bireylerden daha yatkın oldukları gösterilmiştir. Bu verilerin ardından oldukça yoğun immünosupresif olan hematolojik malignite hastalarında SARS-CoV-2 infeksiyonu sonuçları ilgi konusu olmuştur. Biz de bu makalede Türkiye’de hematolojik malignite tanısı ile takip ve tedavi edilirken SARS-CoV-2 enfeksiyonu saptanan hastaların semptom, komplikasyon, yoğun bakım ünitesine yatış ve mortalite oranlarını değerlendirmeyi amaçladık.Çok merkezli çalışmamıza Mart-Kasım 2020 tarihleri arasında SARS-CoV-2 enfeksiyonu tanısı alan erişkin ve pediatrik 340 hastayı dahil ettik. Hastaların hematolojik malignite tanıları, hastalık statusları, tedavileri, son tedaviden enfeksiyona kadar geçen süre, komorbiditeleri, yaşam beklentileri değerlendirildi. Semptomları, oluşan komplikasyonlar ve sonuçlar analiz edildi.Kırk-dört hasta semptomsuz olarak enfeksiyonu geçirirken, semptomatik hastaların ateş, öksürük, ve dispne oranları sırasıyla %62,6, %48,8 ve %41,8 idi. Altmış altı hasta (%20) hastalığı hafif geçirirken, orta, ciddi ve kritik hastalık tanısı alanların oranı sırasıyla %29, %20 ve %16 idi. Tüm kohortta ölüm oranı %26,6 idi; ölüm hafif hastalığı olanlarda %4,4 , orta derece hastalık geçirenlerde %12 ve ciddi/kritik hastalık geçirenlerde ise %83 olarak saptandı. Aktif hematolojik hastalık olması, primer hematolojik hastalığa bağlı düşük hayat beklentisi, SARS-CoV-2 tanısı aldığında nötropenik olmak, yoğun bakıma alınmış olmak ve ilk sıra koronavirüs hastalığı-2019 tedavisi yüksek ölüm riski ile ilişkilendirilen faktörlerdendi. Tek başına veya azitromisin ile kombine olarak hidroksiklorokin kullanan hastaların sadece favipiravir kullananlara göre ölüm riskleri daha yüksek saptandı.SARS-CoV-2 infeksiyonu geçiren hematolojik malignite hastalarında daha yüksek oranda ciddi hastalık ve ölüm riski gözlenmektedir.

Introduction Cardiac involvement in diffuse large B-cell lymphoma is a rare entity in non-Hodgkin lymphomas. Symptoms are usually related to heart failure. Patients who are severely symptomatic due to cardiac mass could be considered treatment as soon as possible. In this report, we present a patient diagnosed with diffuse large B-cell lymphoma with cardiac involvement. Case Report A 61-year-old female patient was admitted to our unit with gastric biopsy diffuse large B-cell lymphoma. Computerized tomography of the chest and positron emission tomography/computed tomography demonstrated a neoplastic mass in the intra-atrial septum extended to inferior vena cava (5 × 4 cm in size and standardized uptake value maximum 24.6). She was in stage III and in the high-risk group. Because of pronounced heart failure findings associated with the mass-specific chemotherapy was planned early. Management & Outcome Although a fraction of ejection was 60% by echocardiography before the treatment, she had a cardiac risk for doxorubicin due to being over 60 years old and hypertension. Complete remission was achieved after three cycles of rituximab–cyclophosphamide–doxorubicin–vincristine and methylprednisolone protocol including doxorubicin. Treatment was completed with six cycles and she was followed up for three months. Discussion Because of the cardiotoxicity of doxorubicin-based protocols, patients should be evaluated according to cardiac functions before and during the chemotherapy.

Objective: Primary Immune Thrombocytopenia (ITP, idiopathic thrombocytopenic purpura, immune thrombocytopenic purpura) is an acquired thrombocytopenia caused by anti-platelet antibodies. The diagnosis of ITP may be challenging due to the various potential causes of thrombocytopenia, some of which are overlooked. Immunodeficiencies are also a rare cause of ITP. Although autoimmunity and, therefore, ITP are a common complication in primary immunodeficiency (PI) patients, there are not many publications in the literature that examine the frequency of PI in ITP patients and the immune abnormalities in these patients. Materials and Methods: Forty-five patients with ITP (F: 37 [78.7%], M: 10 [21.3%]) were included in the study (age 42.9 ± 15.9). Results: At least one antibody deficiency was detected in 7 patients (14.9%), and following further investigations, 2 patients (4.3%) were diagnosed with CVID, 3 (6.4%) with IgG deficiency, 1 (2.1%) with selective IgA deficiency and 1 (2.1%) with possible IgM deficiency. Immunoglobulin levels were normal while at least one abnormality was detected in 20 patients (42.6%) in peripheral lymphocyte subset analyses. The most common abnormality in this patient group was a reduced percentage of CD4+ T-cells (9 patients, 45% of patients with PLS abnormalities, 19.1% of all patients). CD3+ T-cell rates in 8 patients (17.8%), CD19+ B-cell rates in 6 (12.8%), CD3-CD16+56+ natural killer cell rates in 4 (8.5%), and CD4/CD8 cell ratios in 7 (14.9%) were reduced. In addition, the CD8+ T-cell rate in 8 patients (17%) was above the reference ranges. Conclusion: Adult patients who are diagnosed with ITP may develop a variety of immunological abnormalities in addition to hypogammaglobulinemia. Therefore, clinicians should not overlook immunological evaluation in the etiological investigation of ITP and should closely monitor patients with immunological abnormalities.

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. Key Words: Hemophagocytic lymphohistiocytosis, Hepatitis-A, Mononuclear phagocytes.

Introduction: Passive antibody therapy has been used to immunize vulnerable people against infectious agents. In this study, we aim to investigate the efficacy of convalescent plasma (CP) in the treatment of severe and critically ill patients diagnosed with COVID-19. Method: The data of severe or critically ill COVID-19 patients who received anti-SARS-CoV-2 antibody-containing CP along with the antiviral treatment (n = 888) and an age-gender, comorbidity, and other COVID-19 treatments matched severe or critically ill COVID-19 patients at 1:1 ratio (n = 888) were analyzed retrospectively. Results: Duration in the intensive care unit (ICU), the rate of mechanical ventilation (MV) support and vasopressor support were lower in CP group compared with the control group (p = 0.001, p = 0.02, p = 0.001, respectively). The case fatality rate (CFR) was 24.7 % in the CP group, and it was 27.7 % in the control group. Administration of CP 20 days after the COVID-19 diagnosis or COVID-19 related symptoms were associated with a higher rate of MV support compared with the first 3 interval groups (?5 days, 6-10 days, 11-15 days) (p=0.001). Conclusion: CP therapy seems to be effective for a better course of COVID-19 in severe and critically ill patients.

Objective: This study aimed to retrospectively evaluate the efficacy, safety, and survival outcome of single-agent ibrutinib therapy in chronic lymphocytic leukemia patients. Materials and Methods: A total of 136 patients (mean age +/- standard deviation: 64.6 +/- 10.3 years, 66.9% males) who had received at least one dose of ibrutinib were included in this retrospective multicenter, noninterventional hospital-registry study conducted at 33 centers across Turkey. Data on patient demographics, baseline characteristics, laboratory findings, and leukemia-cell cytogenetics were retrieved. Treatment response, survival outcome including overall survival (OS) and progression-free survival (PFS), and safety data were analyzed. Results: Overall, 36.7% of patients were categorized as Eastern Cooperative Oncology Group (ECOG) class 2-3, while 44.9% were in Rai stage 4. Fluorescence in situ hybridization revealed the presence of del(17p) in 39.8% of the patients. Patients received a median of 2.0 (range: 0-7) lines of pre-ibrutinib therapy. Median duration of therapy was 8.8 months (range: 0.4-58.0 months). The 1-year PFS and OS rates were 82.2% and 84.6%, respectively, while median PFS time was 30.0 (standard error, 95% confidence interval: 5.1, 20.0-40.0) months and median OS time was 37.9 (3.2, 31.5-44.2) months. Treatment response (complete or partial response), PFS time, and OS time were better with 0-2 lines versus 3-7 lines of prior therapy (p, Janssen Pharmaceutica Turkey, This study was supported by Janssen Pharmaceutica Turkey. The authors would like to thank Prof. Sule Oktay, MD, PhD, and Cala Ayhan, MD, from KAPPA Consultancy Training Research Ltd. (stanbul, Turkey) , who provided editorial support.

This study aims to evaluate the COVID-19 pandemic consultations requested from the division of hematology in the hospital. We recorded the following features of the patients: demographic information, the status of outpatient or inpatient, the clinic requested consultation, the result of the consultation, having a hematological disease, diagnosis and COVID-19 suspicion. In this study, 82 cases were evaluated. The median age of the patients was 49,5. Females were mild higher (56,1% vs. 43,9%). Eight of the patients was suspected with COVID-19. The frequency of suspicion in patients with the previously diagnosed hematological disorder was higher significantly than patients without previously diagnosed (42,9% vs. 6,7% p=0,018). The frequency of suspicion was higher significantly inpatients than outpatients (19,4% vs. 2,2%, p=0,019). Only two of the patients who was suspected with COVID-19 were PCR-positive and these patients were diagnosed with concomitant hematologic malignancy; CLL and MCL. The most common clinic and cause of this consultation of this clinic were gynecology and thrombocytopenia, respectively, and most of the patients were diagnosed with pseudothrombocytopenia. After the consultation for all patients, the diagnosis was drug-related cytopenia; however, detailed evaluation for cytopenia was delayed to period which after the pandemic because any patients had no deep levels of cytopenia. Although our study has some limitations, this study is remarkable because this is the first study that has investigated hematology consultation during the pandemic. We think that more significant results will be achieved as new data become available, as long as the pandemic continues.

Sarcoidosis is a rare disease characterized by granulomatous inflammation in affected organs, primarily in lungs. Neutrophil/lymphocyte ratio (NLR) and platelet/lymphocyte ratio (PLR) are easy and practical methods providing valuable information in diagnosis, severity, and prognosis of various diseases. Here, we aimed to investigate the association between NLR, PLR, and hematological parameters in sarcoidosis. The study was performed with 75 sarcoidosis patients and 92 controls. Patients’ NLR, PLR, and hematological parameters were compared with those of controls. Additionally, while differences between NLR and PLR were investigated in sarcoidosis patients, differences of extrapulmonary involvement, pulmonary hypertension (PH), and spontaneous remission between those with and without responses to treatment concerning stages were also assessed. NLR and PLR were significantly higher in sarcoidosis patients than controls. For NLR, sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV) were found as 68, 61, 58, and 70% respectively, while sensitivity, specificity, PPV, and NPV for PLR were found as 72, 67, 63, and 74%, respectively. In sarcoidosis patients, NLR and PLR were significantly higher at stage-2 and -3 than at stage -1 and -4. There was a significant weak positive correlation between C-reactive protein (CRP) and NLR and PLR. Mean platelet volume (MPV), hemoglobin (Hgb), and mean corpuscular volume (MCV) were lower among patients than controls. A positive moderate correlation was detected between NLR and CD4/CD8 in blood, while there was a strong positive correlation between CD4/CD8 in bronchoalveolar lavage (BAL) and positive moderate correlation between PLR and CD4/CD8 in BAL. High NLR and PLR values were not significantly associated with pulmonary PH, spontaneous remission, response to treatment, and prognosis. The increase in PLR and NLR may be a guide for diagnoses of both sarcoidosis and lung parenchymal involvement. To use these entities as markers, our findings should be supported with prospective studies with larger samples.

Objective: Chronic antigenic stimulation is frequently blamed in the pathogenesis of extranodal marginal zone lymphomas including splenic marginal zone lymphoma (SMZL). Chronic hepatitis C is frequently observed in SMZL patients in some geographical regions. However, these reports are largely from North America and Europe, and data from other countries are insufficient. In this multicenter study we aimed to identify the clinical characteristics of SMZL patients in Turkey, including viral hepatitis status and treatment details. Materials and Methods: Data were gathered from participating centers from different regions of Turkey using IBM SPSS Statistics 23 for Windows. Hepatitis B virus surface antigen (HBsAg), anti-HBs antibody, anti-HB core antigen antibody (anti-HBcAg), HB viral load, anti-hepatitis C virus (HCV) antibody, HCV viral load results were analyzed. Results: One hundred and four patients were reported. Hepatitis C virus positivity was observed in only one patient. However, hepatitis B virus surface antigen (HBsAg) positivity was observed in 11.2% and HBsAg and/or anti-HB core antigen antibody (anti-HBcAg) positivities were seen in 34.2% of the patients. The median age was 60 years (range=35-87). Median follow-up duration was 21.2 months (range=00.2-212; 23.2 months for surviving patients). Median overall survival was not reached. Estimated 3-year and 10-year survival rates were 84.8% and 68.9%, respectively. Older age, no splenectomy during follow-up, platelet count of, Amaç: Splenik marjinal zon lenfoma’yı da (SMZL) içeren ekstranodal marjinal zon lenfomaların patogenezinde, kronik antijenik stimülasyon sorumlu olan mekanizmadır. Bazı coğrafik bölgelerde SMZL hastalarında kronik hepatit C sıklıkla gözlenir. Fakat bu çalışmalar sıklıkla kuzey Amerika ve Avrupa’dandır. Diğer ülkelerden veriler sınırlıdır. Bu çalışma ile Türkiye’deki SMZL hastalarının hepatit serolojisi durumları ve tedavi yöntemlerinin ortaya konulması amaçlanmıştır. Gereç ve Yöntemler: Türkiye’de farklı merkezlerden IBM SPSS Statistics for Windows v23 elektronik tablo kullanılarak veriler toplanmıştır. Hepatit B virüs yüzey antijeni (HBsAg), Anti-HBs antikor, Anti-HB kor antijen antikoru (anti-HBcAg), HB virüs yükü, anti-hepatit C virüs (HCV) antikoru, HCV virüs yükü sonuçları analiz edilmiştir. Bulgular: Yüz dört hastanın verilerine ulaşılmıştır. Hepatit C virüs pozitifliği sadece 1 hastada tespit edilmiştir. Hepatit B virüs yüzey antijeni (HBsAg) pozitifliği %11,2, HBsAg ve/veya hepatit B kor antijen antikoru (anti-HBcAg) pozitifliği %34,2 oranında tespit edilmiştir. Ortanca yaş 60 yıl (35-87) olarak saptanmıştır. Ortanca izlem süresi 21,2 (0,2-212) ay (yaşayan hastalar için 23,2 ay) olarak bulunmuştur. Ortanca genel sağkalım (GS) süresine ulaşılamamıştır. Tahmini 3-yıl ve 10-yıl GS oranları %84,8 ve %68,9 olarak bulunmuştur. İleri yaş, splenektomi yapılmaması

PubMed: 32423761 Background: Homozygous familial hypercholesterolemia (HoFH) is a rare, life-threatening disease due to high serum low-density lipoprotein (LDL) cholesterol levels. LDL cholesterol–lowering interventions are fundamental for patients with HoFH. Objective: It was aimed to investigate the association between the mental status of patients with HoFH and healthy lifestyle behaviors. Methods: This subgroup analysis of the A-HIT1 population included the data of patients aged ?18 years with a clinical diagnosis of HoFH undergoing therapeutic LDL apheresis. Besides the demographic and clinical characteristics of patients, healthy lifestyle behaviors were assessed, and psychiatric symptoms were screened by Symptom Check List (SCL-90-R). Results: The highest percentage for pathology was observed in dimensions of obsessive-compulsive, somatization, interpersonal sensitivity, and depression in SCL-90-R. Patients with any cardiovascular condition have more psychiatric symptoms in different fields of SCL-90-R. The outcomes of the correlative analysis indicated that lower the age of the first coronary event better the psychiatric status, probably denoting a better adaptation to disease and its treatment. Among 68 patients, 36 patients were not exercising regularly. Patients with regular physical activity had significantly lower scores in most dimensions of SCL-90-R and there was no association between regular physical activity and other investigated variables. The strongest predictor of regular exercising was global severity index of SCL-90-R. Conclusion: In the HoFH population, there was a high prevalence of mental disturbances. Better psychiatric status was associated with regular exercising. Therefore, assessing the mental status of patients with HoFH and referring patients in need, to a psychiatrist, may improve the outcome of patients. © 2020 National Lipid Association Data collection of this investigator-initiated study was funded by Aegerion pharmaceuticals.

Yenercag, Mustafa/0000-0002-0933-7852; YILMAZ, MEHMET BIRHAN/0000-0002-8169-8628; Demircioglu, Sinan/0000-0003-1277-5105; pekkolay, zafer/0000-0002-5323-2257; Kaynar, Leylagul/0000-0002-2035-9462; durmus, rana berru/0000-0001-9182-2760 WOS: 000427639800007 PubMed: 29407887 Background and aims: Homozygous familial hypercholesterolemia (HoFH) is a genetic condition characterized by lethally high levels of low-density lipoprotein cholesterol (LDL-C) from birth, and requires rapid and aggressive intervention to prevent death due to coronary heart disease and/or atherosclerosis. Where available, lipoprotein apheresis (LA) is the mainstay of treatment to promote survival. Methods: A-HIT1 registry was conducted with the aim of providing insight to the real-life management of HoFH patients undergoing LA in Turkey, where LA procedures are fully reimbursed and widely available. Participating centers provided patient information, including family history, treatment patterns and relevant laboratory values, via a standard questionnaire. Results: The study evaluated 88 patients (mean age: 27 +/- 11 years, 41 women) in 19 centers. All patients were receiving regular LA with a clinical diagnosis of HoFH. Mean age at first symptom disease was 10 +/- 10 years, and at diagnosis it was 12 +/- 11 years; 74.7% were diagnosed before age 15 years; and only 31% before the age of 7. First referral of most patients was to pediatricians. Early onset coronary artery disease was present in 57.8% of patients. Mean age at first LA was 21 +/- 12 years. Only 11 (12.5%) patients were undergoing LA weekly. Mean frequency of apheresis sessions was 19 +/- 13 days. For the last four LA sessions, LDL-C levels reached the target in only in 5.7% of patients. Conclusions: Diagnosis of HoFH is delayed, and LDL targets are not reached. LA frequencies are not optimal. Urgent attention is needed to support the survival of patients with HoFH. (c) 2018 Elsevier B.V. All rights reserved. Aegerion pharmaceuticals; Amryt Pharmaceuticals This investigator initiated study was funded by Aegerion pharmaceuticals, and authors wish to thank Nigel Eastmond of Eastmond Medicomm Ltd for assistance in editing the final manuscript, which was funded by Amryt Pharmaceuticals.

Diffuse large B-cell lymphoma is the most common subtype of non-Hodgkin lymphoma. Approximately 40% of cases have extranodalinvolvement. Renal involvement is rare, however, it is associated with poor prognosis. Furthermore, renal involvement increasesthe risk of central nervous system (CNS) recurrence. Therefore, it is recommended that CNS prophylaxis should be included inlymphoma treatment in cases of renal involvement.

Thrombotic thrombocytopenic purpura (TTP) is a rare disease associated with microangiopathic hemolytic anemia, thrombocytopenia, fever, neurological disorders, and renal insufficiency pentad. It is a fatal hematologic emergency if left untreated. If plasma exchange is feasible, treatment is easy and comfortable. It should be kept in mind that such as in our patient may be acquired at all congenital illnesses.

Chronic lymphocytic leukemia (CLL) is a neoplastic condition of B cells that frequently affects the lymph nodes, liver, spleen and bone marrow. The extranodal involvement of CLL is most commonly observed in the skin, whereas gastrointestinal and genitourinary involvement is rare. Renal involvement may not always present with renal failure. In this study, we aimed to present a patient with CLL infiltration in the kidney without renal failure and proteinuria.

Objective The aim of the present study was to evaluate the efficacy and safety of eltrombopag, an oral thrombopoietin receptor agonist, in patients with chronic immune thrombocytopenia (ITP). Materials and Methods A total of 285 chronic ITP patients (187 women, 65.6%; 98 men, 34.4%) followed in 55 centers were enrolled in this retrospective cohort. Response to treatment was assessed according to platelet count (/mm3) and defined as complete (platelet count of >100,000/mm3), partial (30,000-100,000/mm3 or doubling of platelet count after treatment), or unresponsive (

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.

BACKGROUND It is known that daily divided doses and high doses of iron increase hepcidin and reduce iron absorption. AIM This study aimed to compare treatments of iron replacement every other day at low doses, once a day and twice a day in terms of their effectiveness and frequencies of side effects. METHODS For a month, Group I received 270 mg ferrous sulphate twice a day (total elemental iron dose: 160 mg/day), Group II received 270 mg ferrous sulphate once a day (total elemental iron dose: 80 mg/day), and Group III received 270 mg ferrous sulphate every other day (total elemental iron dose: 80 mg/every other day). Intragroup and intergroup statistical analyses were carried out. RESULTS Haemoglobin (Hb) increased significantly in all three groups (P = 0.00). The increase in Hb levels was similar among the groups (P = 0.09). Ferritin significantly increased in all three groups after the treatment (P = 0.00). The increase in ferritin in Group I was significantly higher than those in Groups II and III (P

WOS: 000475544900016, PubMed: 30928440, BACKGROUND: Homozygous familial hypercholesterolemia (HoFH) is a rare, life-threatening inherited disease leading to early-onset atherosclerosis and associated morbidity. Because of its rarity, longitudinal data on the management of HoFH in the real world are lacking, particularly on the impact the condition has on quality of life (QoL), including the impact of the extracorporeal lipid removal procedure apheresis (LA). METHODS: The A-HIT1 study included 88 patients with HoFH aged >= 12 years receiving regular LA in 19 centers in Turkey. Demographic and disease characteristics data were obtained. For patients aged >= 18 years, additional data on psychosocial status were obtained via the SF-36 score, the Hospital Anxiety and Depression Scale, and a HoFH-specific questionnaire. RESULTS: There was no standardized approach to therapy between centers. Mean (+/-SD) frequency of LA sessions was every 19.9 (+/-14) days, with only 11.6% receiving LA weekly, and 85% of patients were not willing to increase LA frequency. The most common concerns of patients were disease prognosis (31%), and physical, aesthetic, and psychological problems (27.5%, 15.9%, and 11.6%, respectively). Lower age at diagnosis was associated with better QoL, lower anxiety, improved functioning, and greater emotional well-being compared to later diagnosis. CONCLUSIONS: These findings demonstrate that adult patients with HoFH undergoing LA, experience significant impairment of QoL with an increased risk of depression. From patients' point of view, LA is time-consuming, uncomfortable, and difficult to cope with. The speed of diagnosis and referral has a considerable impact on patient well-being. (C) 2019 National Lipid Association. All rights reserved., Aegerion pharmaceuticals; Amryt Pharmaceuticals DAC, This investigator initiated study was funded by Aegerion pharmaceuticals, and authors wish to thank Nigel Eastmond of Eastmond Medicomm Ltd for assistance in editing the final article, which was funded by Amryt Pharmaceuticals DAC.

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.

Objective: The secondary primary malignancy frequencies have seen increased in chronic lymphocytic leukemia (CLL) regardless of therapy. The aim of this study was to investigate the frequency of secondary primary malignancy in patients followed with the diagnosis of chronic lymphocytic leukemia. Materials and Methods: The 183 patients with diagnosed of CLL were enrolled into this study. The data of the patients were evaluated retrospectively. Patients diagnosed with CLL were categorized according to age, gender and presence or absence of additional malignancy. Patients with CLL and concomitant malignancy were compared with other patients. Results: Fifty four patients (29.5%) were female and 129 (70.5%) were male. Secondary primary malignancy was detected in 9 (%4,9) patients. CD5 positivity was found in all of the patients with malignancy and in 91% of whole patients. 5.4% of males and 3.7% of females had solid organ tumors with CLL. Although the proportion of solid tumors was higher in males, this difference was not statistically significant (p = 0.847). The mean age of cases with secondary malignancy was statistically significantly higher than that without secondary malignancy (p

Some rare malignant diseases exhibit clinical features and bone marrow aspirate morphology similar to that of acute leukemia. For instance, rhabdomyosarcoma, neuroblastoma, medulloblastoma, anaplastic oligodendroglioma, small cell carcinoma, Ewings sarcoma and neuroendocrine tumors have been reported to display an acute leukemia-like morphology in bone marrow aspirates after metastasizing to the bone marrow. Rhabdomyosarcoma (RMS), a malignant tumour of mesenchymal origin which can occur at various sites in the body, is one of the most common soft tissue sarcomas in both children and adolescents, but is rare in adults with a prevalence of less than 1 %. Bone marrow metastases associated with this condition may be readily confused with acute leukemia or lymphoma. Diagnostic confirmation requires immunohistochemical and flowcytometric examinations. In patients with positive CD56 and negative CD45, rhabdomyosarcoma should be included in the differential diagnosis. Here, we report an unusual case of RMS confined to the bone marrow in an older adult. [Med-Science 2018; 7(4.000): 946-8]

61st Annual Meeting and Exposition of the American-Society-of-Hematology (ASH) -- DEC 07-10, 2019 -- Orlando, FL, BERBER, Ilhami/0000-0003-3312-8476, WOS:000577164606194, [No Abstract Available], Amer Soc Hematol

Heparin-induced thrombocytopenia (HIT) is caused by heparin exposure and presents with reduced platelet count. Patients undergoing hemodialysis (HD) treatment have increased risk of developing HIT due to prolonged exposure to unfractionated heparin or low-molecular weight heparin. We report a 79-year-old male patient with end-stage renal disease who developed type-II HIT during maintenance HD. Platelet count of the patient decreased gradually and antiplatelet factor IV antibody was found to be positive. The patient was treated with fondaparinux and continued heparin-free HD. Unfortunately, despite favorable initial response without any thrombotic episodes, the patient died due to severe sepsis complicated by gastrointestinal hemorrhage.

Brucellosis is a zoonotic infection that humans contract usually by ingestion of unpasteurized milk and milk products or by direct contact with raw infected animal products. Infection is endemic in many countries, including Turkey. Being a systemic disease, brucellosis may affect almost any part of the body. The peritoneum is a site rarely involved in brucellosis. Most peritonitis episodes involving Brucella species have been spontaneous cases reported in cirrhotic patients with ascites. To our knowledge, the literature contains only 5 cases of Brucella peritonitis related to continuous ambulatory peritoneal dialysis. Here, we report Brucella peritonitis in a continuous ambulatory peritoneal dialysis patient, and we discuss the relevant literature.