The Impact of Cytogenetic Aberrations in the Clonal Evolution of Chronic Myeloid Leukemia: A Single-Center Experience Among 450 Turkish Patients (Cohort Study)

Objective: Chronic myeloid leukemia (CML) is a clonal hematologic disorder characterized by t(9;22) translocation, in which cytogenetic aberrations can occur in Ph(+) and (-) clones. These aberrations develop due to clonal evolution as well as treatment and they have prognostic significance. They are grouped as major and minor route anomalies in terms of their effects on prognostic parameters, such as treatment response, overall survival (OS), disease stage, complete cytogenetic response (CCyR), and major molecular response (MMR). It is stated that major route anomalies have unfavorable prognostic effects compared to minor route anomalies. We aimed to investigate the frequency and prognostic effects of cytogenetic anomalies detected in Ph(+) and (-) clones. Materials and Methods: In this study, we retrospectively analyzed the cytogenetic results of 450 patients diagnosed with CML between 2005 and 2020. Results: We detected cytogenetic aberrations in Ph-positive and negative clones in 41 of 450 patients. The most common anomalies were trisomy 8 (+8), additional Ph chromosome (+Ph), and loss of chromosome Y. Rarely, aneuploidy of the Y chromosome, dup (22), +11, and +6 were seen in CML patients. We observed that these identified aberrations negatively affected MMR and CCyR, and generally resulted in changing imatinib treatment for second-generation tyrosine kinase activity inhibitors. Our results are compatible with the literature. Conclusion: We suggest that cytogenetic aberrations detected in Ph(+) and (-) clones should be a warning sign in terms of treatment and require close observation. The use of cytogenetic methods for the identification of these anomalies is also important.

Clinical Characteristics and Outcomes of COVID-19 in Turkish Patients with Hematological Malignancies

Objective: 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. Materials and Methods: 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. Results: 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. Conclusion: Patients with hematological malignancy infected with SARS-CoV-2 have an increased risk of severe disease and mortality.

Evaluation and Differential Diagnosis of Hypereosinophilia in Rheumatology Practice.

BACKGROUND: There has been no investigation so far on the prevalence or causes of hypereosinophilia during rheumatic diseases. OBJECTIVES: The study aimed to identify the prevalence and causes of hypereosinophilia among the patients followed in a rheumatology department. METHODS: The patients aged 18 years or over followed in our rheumatology department between January 2010 and December 2019 who had at least one AEC ≥1,500/µL measurement in their peripheral blood count were identified retrospectively. RESULTS: Over the 10 years, a total of 130,769 peripheral blood counts were performed, of which 3.9% showed eosinophilia and 0.065% showed hypereosinophilia. Hypereosinophilia was identified in 85 patients. The underlying rheumatic disease was determined in 89.4% (n = 76) of patients. Of these, the most frequent one was rheumatoid arthritis at a ratio of 40.8%, followed by eosinophilic granulomatosis with polyangiitis (EGPA) at a ratio of 10.5%. Hypereosinophilia was in primary form in 3.5% of the patients, whereas secondary to another condition in 91.8% (n = 78) of the cases and idiopathic in 4.7% (n = 4) of patients. The most common cause of secondary hypereosinophilia was drug induced, as detected in 61.2%, followed by allergic conditions in 11.5% and EGPA in 9.4%. In 15.2% (n = 13) of the cases, hypereosinophilia was associated with an underlying rheumatic disease. In the cases with drug-induced hypereosinophilia, most often (in 28.8%) methotrexate was the offending agent. CONCLUSIONS: Rheumatologists should be cognizant that hypereosinophilia concurrent to rheumatic diseases is usually not due to the underlying rheumatic disease, except for the conventional eosinophil-related rheumatic diseases.

Evaluation of hyperferritinemia causes in rheumatology practice: a retrospective, single-center experience.

Hyperferritinemia may develop due to various reasons such as inflammation, infection, or malignancy. The purpose of the study to explore the prevalence and to figure out the causes of general hyperferritinemia and extreme hyperferritinemia as detected through the ferritin measurements requested by the rheumatology department. Adult patients at the age of 18 years and older with at least one serum ferritin level measurement at or above 500 ng/mL as requested by the rheumatology department between January 2010 and December 2019 were evaluated retrospectively. Hyperferritinemia was detected in 4.7% of 11,498 serum ferritin tests. The mean age of 242 patients found to have hyperferritinemia was 53.7 ± 17.1 years; of the patients, 63.2% were female, and the mean serum ferritin value was 2820 ± 5080 ng/mL. The most common cause of hyperferritinemia was rheumatological diseases with a ratio of 59.1%, which was followed by infections, iron overload, and solid malignancy. Among the rheumatologic diseases, adult-onset Still's disease (AOSD), rheumatoid arthritis, and vasculitis were the cause accounting for hyperferritinemia. Ferritin levels were significantly higher in the AOSD group compared to the other rheumatologic disease groups (p < 0.0001). While extreme hyperferritinemia (ferritin ≥ 10,000 ng/mL) rate in our cohort was 0.2%, the most common cause was AOSD (15/17). In patients with hyperferritinemia, 3 month mortality was found to be 8.7%. CRP level was identified as the only independent predictor for the 3 month mortality in all patients [OR 1.088 (95% CI 1.004-1.178), p = 0.039]. Although rheumatologic disease activation and infections are the most common causes, the other causes should also be considered for the differential diagnosis.

Weekly low-dose recombinant factor VIIa prophylaxis in Glanzmann thrombasthenia.

Glanzmann thrombasthenia is an inherited disease causing bleeding episodes due to platelet dysfunction. The standard treatment for moderate-severe bleeding is platelet transfusion. Recombinant factor VIIa (rFVIIa) is successfully used in bleeding episodes and invasive procedures. Here, we present a patient with Glanzmann thrombasthenia, whose bleeding episodes could only be controlled by rFVIIa. The patient is a 28 years old male, who has had frequent bleeding episodes unresponsive to local hemostatic agents and tranexamic acid and had an anaphylactoid reaction to platelet transfusion. We started the patient on a low-dose (20 µg/kg) rFVIIa once a week. The patient has no spontaneous bleeding since then. This is the first case report of a Glanzmann thrombasthenia patient on routine prophylaxis with low-dose rFVIIa.

A new four-way complex translocation variant involving the t(8;5;21;4)(q21;q13,q22,q31) and the relocalization of AML1/ETO fusion gene.

In acute myeloid leukemia, t(8;21) detected with a frequency of 10% is associated with good prognosis. However, variant t(8;21) is observed in 4% of these cases, and although the prognostic effects of these variant translocations have not been clearly revealed, there are findings that they affect the prognosis poorly. Here, we report on a 39 years old man, detected 4-way varyant t(8;21) which include relocalization of RUNX1/RUNX1T1 fusion gene, and loss of Y chromosome. RT-PCR also confirmed RUNX1/RUNX1T1 fusion transcript. Additionally, D820G and N822K mutations on KIT gene and mut B on NMP1 gene were detected. A complete remission could not achieved after first chemotherapy treatment. Due to primary resistance and variant of t(8;21), stem cell transplantation was performed. The variant translocation we have reported is unique and also the case is the second case that was reported in the literature in terms of the relocation of the AML1/ETO fusion gene. Since c-KIT mutations and LOY were also observed, it is not possible to predict the prognosis. To highlight the importance of variant translocations and relocalization of fusion gene, more cytogenetic and molecular data are needed.

Which prognostic marker is responsible for the clinical heterogeneity in CLL with 13q deletion?

BACKGROUND: 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. RESULTS: 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). CONCLUSION: 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).

Use of rotational thromboelastometry for a global screening of coagulation profile in patients of myeloproliferative neoplasms.

Chronic myeloproliferative neoplasms (MPN) are clonal disorders of hematopoietic stem/progenitor cell characterized by thrombohemorrhagic complications and a tendency to transform into acute leukemia. The pathogenesis of thrombosis in MPN is complex and results from a multifaceted interplay of clinical and disease-related factors. Rotational thromboelastometry (ROTEM) provides the complete and rapid information about all stages of the coagulation process. Here, we assess ROTEM parameters as a screening of coagulation profile in patients with MPNs. In particular, higher mean maximum clot firmness values were found in Essential thrombocythemia and Polycythemia vera patients when compared to healthy controls. Rotational thromboelastometry may be able to detect MPN patients who are susceptible to thrombotic and/or hemorrhagic complications. The predictive value of ROTEM for thrombosis remains to be established to classify subsets of patients at prominent risk who may benefit from prophylaxis with antithrombotic drugs.

Temporal artery biopsy for suspected giant cell arteritis: a retrospective analysis.

Temporal artery biopsy (TAB) is one of the diagnostic criteria of giant cell arteritis (GCA) according to 1990 ACR criteria and remains a tool for diagnosis. Although clinicians perform TAB with an intent to confirm suspected GCA, some biopsies result in negative and some lead to non-GCA diagnoses. We aim to review the diagnoses after TAB biopsy performed for suspected GCA and also wanted to evaluate the diagnostic changes and concomitant diseases that develop over time. The patients who had undergone TAB for suspected GCA were identified using the record entry code for TAB. Patients meeting the classification criteria for GCA were designated as the GCA group and not meeting criteria were designated as a non-GCA group. Other classification criteria were implemented for the non-GCA group diseases. A total of 51 patients (Female: 62.7%, median age: 72.1 ± 7.4 years) who had undergone TAB for suspected GCA were evaluated. TAB was positive in 23 (69.6%) of the 33 patients who met the GCA classification criteria. No significant difference was found between TAB-positive and TAB-negative GCA patients in terms of clinical and laboratory parameters. In the non-GCA group, 12 patients had isolated polymyalgia rheumatica (PMR), and the diagnoses of the remaining six patients were as follows: four large vessel vasculitis (LVV) not satisfying GCA diagnostic criteria, one chronic myelomonocytic leukemia (CMML), and one amyloidosis. TAB was negative in all patients with isolated PMR. TAB showed primary amyloidosis in one patient. Out of 33 GCA patients, 21 had "isolated" GCA, four had GCA + Rheumatoid arthritis (RA), seven had GCA + PMR, and one had GCA + polymyositis. RA was diagnosed antecedent to GCA in two patients, and after GCA in the other two patients. One of the patients had developed GCA 20 years after polymyositis had been diagnosed. TAB was found to be positive in two-thirds of patients with suspected GCA. Late-onset RA and rarely other inflammatory rheumatic diseases may develop in the course of GCA.

A new approach for diagnosing hematological malignancies using monocytosis workflow optimization and abnormal lymphocyte/blast flag of Sysmex XN series of blood count analyzers.

INTRODUCTION: Monocytosis Workflow Optimization rule set has been developed by using mono-dysplasia-score to determine reactive monocytosis and prevent unnecessary blood smear of these patients and for detection of chronic myelomonocytic leukemia cases during complete blood count. In our study, we aimed to examine the contribution of Monocytosis Workflow Optimization rule set. METHODS: Adult patients with monocyte count ≥1.0 103 /µL and monocyte percentage ≥10% were included in our study. Blood smears were made from the samples in our laboratory. These smears were examined and patients were divided into two groups as reactive monocytosis or hematological malignancy. The groups were compared in terms of Monocytosis Workflow Optimization rule set and device flags. RESULTS: Twenty-one patients had hematological malignancies of 155 patients who were included in our study. Monocytosis Workflow Optimization rule set suggested performing blood smear in 19 of the patients with hematological malignancy, and evaluated two patients as reactive monocytosis with 90.5% sensitivity and 76.9% specificity. There was an "abnormal lymphocyte/ blast" flag in 90.5% of patients with hematological malignancies and in patients whose Monocytosis Workflow Optimization rule set defined as reactive monocytosis and it was found that sensitivity and negative predictive value reached 100%. CONCLUSION: Automated validation support systems and softwares developed especially for these systems make it possible to classify patients with their non-specific findings, as a result both contributing to the reduction of laboratory workload and costs and assisting laboratory specialists and clinicians with adding value to laboratory results.

The Turkish experience with therapeutic plasma exchange: A national survey.

Therapeutic plasma exchange (TPE) is used to treat more than 60 diseases worldwide and has drawn growing interest. Little is known about the current situation of TPE activity in Turkey, so we developed a survey to obtain information about this timely topic. We collected data on TPE from 28 apheresis units throughout Turkey. We performed a total of 24,912 TPE procedures with 3203 patients over the past decade. Twenty years ago, the majority of procedures were performed for neurological and hematological disorders, and today, most TPE procedures are done for the same reasons. The only historical change has been an increase in TPE procedures in renal conditions. Currently, renal conditions were more frequently an indication for TPE than rheumatic conditions. Fresh frozen plasma was the most frequently used replacement fluid, followed by 5% albumin, used in 57.9% and 34.6% of procedures, respectively. The most frequently used anticoagulants in TPE were ACD-A and heparin/ACD-A, used with 1671 (52.2%) and 1164 (36.4%) patients, respectively. The frequency of adverse events (AEs) was 12.6%. The most common AEs were hypocalcemia-related symptoms, hypotension, and urticaria. We encountered no severe AEs that led to severe morbidity and mortality. Overall, more than two thirds of the patients showed improvement in the underlying disease. Here, we report on a nationwide survey on TPE activity in Turkey. We conclude that there has been a great increase in apheresis science, and the number of TPE procedures conducted in Turkey has increased steadily over time. Finally, we would like to point out that our past experiences and published international guidelines were the most important tools in gaining expertise regarding TPE.

HLA-DQ2/DQ8 frequency in adult patients with celiac disease, their first-degree relatives, and normal population in Turkey.

BACKGROUND/AIMS: Celiac disease is an autoimmune, familial disease that results in susceptibility to gluten in cereal and cereal products in genetically susceptible individuals. The aim of the present study was to investigate the presence of HLA-DQ2/DQ8 in patients with celiac disease, their first-degree relatives, and healthy community. MATERIALS AND METHODS: HLA-DQ2/DQ8 analysis was performed in adult patients with celiac disease >18 years old (94 patients), their first-degree relatives (89 people), and healthy group (102 individuals). Anemia, osteoporosis, and diarrhea were interrogated in the celiac patient group and also anti-tissue transglutaminase, anti-endomysium, and anti-gliadin antibodies were recorded. RESULTS: There was a significant relationship between HLA-DQ2/DQ8 presence in all groups, and the distribution of HLA-DQ2/DQ8 in all groups was different (p=0.000). No statistically significant correlation was found between the HLA tissue groups and diarrhea (p=0.087), osteoporosis (p=0.215), anemia (p=1.000), tissue transglutaminase antibodies (p=0.295), anti-gliadin antibodies (p=0.104), and anti-endomysium antibodies (p=0.243) in the celiac patient group. CONCLUSION: HLA-DQ2/DQ8 can be used to diagnose celiac disease particularly when the tests are useless and to screen first-degree relatives.

Is it required to routinely check fibrinogen level in patients with rheumatic diseases on tocilizumab? Case-based review.

Tocilizumab (TCZ) may rarely cause hematological side effects including neutropenia and thrombocytopenia. TCZ is essentially expected to lower the fibrinogen levels to stay within the normal range, but TCZ-induced hypofibrinogenemia has been rarely reported in literature. Although it may remain asymptomatic, hypofibrinogenemia has clinical significance owing to the tendency of the condition to result in bleeding. A 65-year-old female patient with known polymyositis was, approximately 20 years after the diagnosis was made, examined due to elevated acute phase reactants leading to the diagnosis of giant cell arteritis (GCA) and TCZ treatment was initiated as she had former steroid-induced osteoporotic fractures. 1 month after the initial dose of intravenous (IV) TCZ, she presented with ecchymosis and was detected to have hypofibrinogenemia. Following the administration of the second dose, hypofibrinogenemia was detected again. In this review, we have analyzed this patient in addition to the cases in six other articles of TCZ induced hypofibrinogenemia which we found out based on our search strategy. Our aim is to point out a rare side effect of TCZ, hypofibrinogenemia, thus to emphasize a possible bleeding disorder and discuss probable underlying mechanisms.

Do Anti-TNF Agents Increase the Risk of Inflammatory Bowel Disease Evolution in Patients with Ankylosing Spondylitis? Real Life Data.

OBJECTIVE: The aim of this study was to determine whether there is any association with anti-tumor necrosis factor (TNF) agent administration and development of new-onset inflammatory bowel disease (IBD) in ankylosing spondylitis (AS) patients. METHODS: Records of the patients who met 1984 modified New York criteria for AS between 1998 and 2016 at Rheumatology Department were evaluated retrospectively and data about the patients, IBD properties and medication were obtained. RESULTS: Among 420 patients, 310 were male, the average age was 42.9 ± 1.3 years, average disease duration was 16.7 ± 10.4 years. Anti-TNF agents were in use by 154 patients, 52 patients were receiving etanercept (ETN), infliximab (INF), adalimumab (ADA), and golimumab (GO) treatments were ongoing in 50, 41, and 11 patients, respectively. New-onset IBD developed in 10 patients; 3 from the group treated with non-anti-TNF drugs (1.1%) and 7 from the group treated with anti-TNF agents (4.5%) (p = 0.042). No significant difference was detected between three anti-TNF agent forms in relation with the risk of IBD onset. In AS patients, existence of familial AS (OR 4.69 (95%CI 1.28-17.19, p = 0.020) and anti-TNF agent treatment (OR 4.17 (95%CI 1.06-16.38, p = 0.041) were independent risk factors for new-onset IBD development. CONCLUSION: Despite the increased risk of new-onset IBD development during the course of AS, paradoxical response to anti-TNF drugs must also be considered as a source that triggers onset of IBD.

Bone marrow as a target organ of systemic lupus erythematosus: analysis of cases with myelofibrosis.

AIM: Cytopenia in the course of systemic lupus erythematosus (SLE) may be due to multiple factors. In this study, we aimed primarily to evaluate the detailed results of bone marrow (BM) biopsies of SLE patients, secondly to determine the myelofibrosis (MF) frequency and thirdly to compare BM morphologic findings as well as the clinical and laboratory parameters between groups (with MF and without MF) in cytopenic SLE patients. METHODS: We retrospectively analyzed 224 SLE patients' files. Patients were divided into two groups according to whether they had MF or not. Concurrent SLE organ involvements, medical therapy and detailed BM findings were recorded. RESULTS: Forty-five (20%) of 224 SLE patients were found to have undergone BM biopsy due to cytopenia. Four patients were excluded (two drug-induced cytopenia, one lymphoma, one insufficient BM biopsy samples). While MF was detected in 29 (70.7%) of the 41 patients, 12 patients did not have MF. Between the two groups, no differences were identified in terms Systemic Lupus Erythematosus Disease Activity Index, BM cellularity, or BM dysplastic changes (P = 0.788, P = 0.672 and P = 0.494, respectively). In the SLE-associated MF group, 27 patients responded to immunosuppressive therapy and corticosteroids, but two patients were unresponsive. The response time was longer for the SLE-associated MF group compared to the without MF group (3.3 ± 3.1 months vs. 1.7 ± 1.2 months, P = 0.091). Correlation analysis revealed that increased degree of BM fibrosis delayed the response time (r = 0.471, P = 0.002). CONCLUSIONS: MF is common in SLE patients. SLE-associated MF as an additional factor for cytopenia in SLE patients may lead to delayed response to appropriate therapy, which may be dependent on the increased grade of BM fibrosis.

Is thrombocytosis always an indicator of autosplenectomy in patients with systemic lupus erythematosus?

In systemic lupus erythematosus (SLE), the most commonly encountered finding related to platelets is thrombocytopenia whereas thrombocytosis is rarely reported. Our aim here was to reveal the type and the frequency of thrombocytosis in SLE patients along with its causes. Data of patients were evaluated retrospectively. Patients who had a platelet count of > 450,000/mm3 (> 450 × 109/L) in at least two subsequent counts and lasting more than 6 months during the follow-up were considered to have "persistent thrombocytosis". Peripheral smear results of patients with thrombocytosis were analyzed, and spleen imaging was performed for autosplenectomy/hyposplenism to patients with persistent thrombocytosis. A total of 205 patients with SLE were included in the study [196 (95.6%) female, mean age 41.5 years]. Out of 12 patients (5.9%) with thrombocytosis, 9 (4.3%) had transient thrombocytosis and 3 patients (1.4%) had persistent thrombocytosis. Of those with transient thrombocytosis, 5 were associated with iron deficiency anemia (IDA), 2 to polyarthritis, and the remaining 2 to digital ischemia and/or cutaneous vasculitis. Of three patients with persistent thrombocytosis, one was identified to have had splenectomy due to resistant immune thrombocytopenic purpura, and the other two (0.9%) patients had autosplenectomy. The only independent risk factor for the development of thrombocytosis was the presence of cutaneous vasculitis (OR 10.79 (95% CI 2.14-54.47), p = 0.0004). During the course of SLE, frequency of thrombocytosis is similar to that of the general population and the most common cause is reactive thrombocytosis. If the thrombocytosis was persistent, rheumatologist must consider that the patient may have autosplenectomy/asplenia/hyposplenism.

Survival Analysis of Turkish Patients With Systemic Lupus Erythematosus: Older Age at Diagnosis Affects Mortality.

OBJECTIVES: This study aims to determine the rate, causes, and risk factors of mortality in Turkish systemic lupus erythematosus (SLE) patients and whether age at diagnosis has an effect on mortality or not. PATIENTS AND METHODS: We retrospectively analyzed the data from 221 patients (10 males, 211 females; mean age 41.5±13.5 years; range 18 to 74 years) who were followed-up due to the diagnosis of SLE in our department between January 1998 and March 2016. Detailed clinical findings, organ involvements, autoantibodies, and complement levels of the patients were recorded. RESULTS: Of the entire group, 19 patients (8.6%) died. Mortality incidence rate was 1.05/100 patient-years. Most frequent causes of death were infections, ischemic cardiovascular and cerebrovascular diseases. Through univariate analysis, older age at diagnosis and a short duration of follow- up were identified as the only factors with an influence on mortality (p=0.004 and p=0.002, respectively). Beyond the mentioned factors, organ involvements in SLE, autoantibodies or antiphospholipid antibody syndrome were not found to have a relationship with mortality. Further analysis conducted on late-onset, defined as the patient age of 50 or above at diagnosis, versus adult-onset, defined as the diagnosis at an age of younger than 50 years, revealed a remarkably shorter survival in patients diagnosed after age of 50 (p=0.003). Cumulative five-year, 10-year, and 15-year survivals of patients were 91.9%, 90%, and 88.2%, respectively. CONCLUSION: We identified older age at diagnosis as an effective factor on mortality. SLE patients who are diagnosed at an older age should be more closely and meticulously followed-up than those diagnosed earlier in terms of their mortality risk.

Utility of flow cytometric κ and λ light chain analysis of peripheral blood.

PURPOSE: Monoclonality in the peripheral blood can be shown by flow cytometric analysis of kappa (κ) and lambda (λ) light chain ratio of B lymphocytes. We aimed to show the utility of this method in patients with unknown causes of lymphadenopathy and/or splenomegaly. METHODS: This method was performed in 81 adult patients with undefined causes of lymphadenopathy and splenomegaly. RESULTS: 18 (22%) of these patients had clonality and all of them were diagnosed as B cell lymphoma later. None of the patients with benign causes had clonality in the peripheral blood. We could not find any relationship between presence of clonality and type and stage of lymphoma and bone marrow involvement. CONCLUSION: This method is easy to perform, cheap and non-invasive and yet it can give valuable information about the malignant nature of a suspected disease. If there is a sign of clonality in the peripheral blood, more invasive diagnostic procedures should be performed rather than watch and wait.