Lower Body Mass Index and Prognostic Nutritional Index Are Associated with Poor Posttransplant Outcomes in Lymphoma Patients Undergoing Autologous Stem Cell Transplantation.

INTRODUCTION: Pretransplant inflammatory and nutritional status has not been widely explored in terms of its impact on autologous hematopoietic stem cell transplantation (auto-HSCT) outcomes in lymphoma patients. We aimed to evaluate the impact of body mass index (BMI), prognostic nutritional index (PNI), and C-reactive protein to albumin ratio (CAR) on auto-HSCT outcomes. METHODS: We retrospectively analyzed 87 consecutive lymphoma patients who underwent their first auto-HSCT at the Adult Hematopoietic Stem Cell Transplantation Unit at Akdeniz University Hospital. RESULTS: The CAR had no impact on posttransplant outcomes. PNI ≤50 was an independent prognostic factor for both shorter progression-free survival (PFS) (hazard ratio [HR] = 2.43, p = 0.025) and worse overall survival (OS) (HR = 2.93, p = 0.021), respectively. The 5-year PFS rate was significantly lower in patients with PNI ≤50 than in patients with PNI >50 (37.3% vs. 59.9%, p = 0.003). The 5-year OS rate in patients with PNI ≤50 was significantly low when compared with patients who had PNI >50 as well (45.5% vs. 67.2%, p = 0.011). Patients with BMI <25 had higher 100-day transplant-related mortality compared with patients with BMI ≥25 (14.7% vs. 1.9%, p = 0.020). BMI <25 was an independent prognostic factor associated with shorter PFS and OS (HR = 2.98 [p = 0.003], HR = 5.06 [p < 0.001], respectively). The 5-year PFS rate was significantly lower in patients with BMI <25 than patients with BMI ≥25 (40.2% vs. 53.7%, p = 0.037). Similarly, the 5-year OS rate in patients with BMI <25 was significantly inferior compared to patients with BMI ≥25 (42.7% vs. 64.7%, p = 0.002). CONCLUSION: Our study confirms that lower BMI and CAR have negative impacts on auto-HSCT outcomes in lymphoma patients. Furthermore, higher BMI should not be considered an obstacle for lymphoma patients who need auto-HSCT; conversely, it could be an advantage for posttransplant outcomes.

The Prognostic Impact of Pretreatment Geriatric Nutritional Risk Index in Patients with Diffuse Large B-Cell Lymphoma.

The prognostic value of the geriatric nutritional risk index (GNRI) is not clear in patients with diffuse large B-cell lymphoma (DLBCL). This study was designed to analyze the GNRI in DLBCL patients and to investigate its prognostic value in DLBCL. The archive records of DLBCL patients between 2008 and 2020 at the Akdeniz University Hospital were retrospectively analyzed. A total of 206 patients with DLBCL were recruited and classified into two GNRI-based groups based on nutrition status. The GNRI cut off value was determined by ROC analysis. In the univariate Cox regression analysis for overall survival (OS), age, lactate dehydrogenase, B symptoms, infiltration of bone marrow, and the GNRI were determined as prognostic factors for mortality. The OS of patients with a GNRI ≤104.238 was significantly lower than that of patients with a GNRI >104.238 (p = 0.001). The progression-free survival (PFS) of patients with GNRI ≤104.238 was significantly lower compared to the patients with GNRI >104.238 (p = 0.010). Based on the results of the present study with a relatively large hospital-based cohort, the GNRI can be recommended for use as an independent prognostic marker for OS and PFS in patients with DLBCL.

Long-Term Complete Clinical and Hematological Response With Bortezomib: The Report of a Case With TEM(P)I Syndrome and a Review of the Literature.

TEMPI syndrome was first defined in 2011 and classified as a plasma cell neoplasm with associated paraneoplastic syndrome in 2016. The pathogenesis of the syndrome is not well understood. Recognition of a combination of telangiectasia, erythrocytosis with a high erythropoietin level, monoclonal gammopathy, perinephric fluid collection, and intrapulmonary shunt is the first step in managing the disease. Diagnoses are often delayed because the syndrome is rare and can be mistaken for other dermatological, renal, and pulmonary disorders. Without early diagnosis significant disability results from the pulmonary damage. The article we present here describes a clinical case of TEMPI-syndrome in a 58-year-old woman, which illustrates the difficulties associated with the timely recognition of this unusual disease. Here, we also review the clinical features of TEMPI syndrome, differential diagnosis and available treatment options, based on current literature. Although limited in number, with the addition of new patients to the literature, TEMPI syndrome is evolving into a well characterized multisystem syndrome. This rare disorder should not be missed, especially if the patient has a putative diagnosis of essential telangiectasia with a monoclonal gammopathy and polistemia. Increasing the awareness of clinicians about the disease and adding new patient data to the literature may contribute to a better understanding of the pathophysiology of the disease and standardization of treatment.

Papilledema and idiopathic intracranial hypertension due to the possible potentiation of ATRA by posaconazole in a case of acute promyelocytic leukemia.

INTRODUCTION: Idiopathic intracranial hypertension (IIH) (pseudotumor cerebri) is a rare side effect of all-trans retinoic acid (ATRA). IIH cases have been observed after the concomitant use of ATRA with azole group antimicrobials such as fluconazole and voriconazole. Here, we discuss about the diagnosis and treatment process of the IIH emerging in a young acute promyelocytic leukemia (APL) case with the ATRA impact, which can be increased by posaconazole. CASE: A 19-year-old male patient was diagnosed with APL. Headache and blurred vision were developed on the 12th day of the AIDA (ATRA, 45 mg/m2/day, oral and idarubicin 12 mg/m2, on days 2, 4, 6, 8, intravenous) protocol and posaconazole proflaxis. He was diagnosed IIH along with the existing eye findings and imagings. MANAGEMENT & OUTCOME: ATRA treatment and posaconazole were interrupted. Systemic acetazolamide and dexamethasone treatment were initiated. After significant clinical response was observed, ATRA treatment was resumed without posaconazole and a similar clinical condition did not recur. DISCUSSION: The combined use of ATRA and azole group drugs increases the risk of developing IIH. Patients with APL who developed IIH during the concomitant use of ATRA and fluconazole or voriconazole have been reported. To the best of our knowledge, our case is the first APL case with a IIH who treated with ATRA-based therapy and used posaconazole. In case of development of side effects, drugs should be interrupted and this combination should be avoided if possible after appropriate approach and clinical improvement.

Outcomes of stem cell mobilization and engraftment in patients with multiple myeloma according to CD56 expression status.

BACKGROUND: The molecular mechanism underlying the mobilization and engraftment of CD34+ cells is poorly understood. The most relevant factors in the regulation of stem cell release and engraftment include chemokines, adhesion molecules, and chemokine receptors. Previously, it was suggested that the absence of CD56 expression could be used as a predictive factor for mobilization failure at the time of diagnosis. Here, we investigated the effect of CD56 expression status on both mobilization and engraftment processes. Additionally, other factors affecting mobilization and engraftment efficacy were investigated. METHODS: Data from 79 multiple myeloma patients undergoing autologous stem cell transplantation between 2015 and 2020 were analyzed for peripheral stem cell mobilization and posttransplant neutrophil and platelet engraftment according to CD56 expression on myeloma cells. RESULTS: No difference in either the median number of CD34+ cells collected or time to engraftment was found between the CD56+ and CD56- groups. The age of the patients (p = 0.025) and peak number of circulating CD34+ cells in peripheral blood (p = 0.005) were important predictors for a higher number of collected CD34+ cells. The average time to recovery of leukocytes and platelets after transplantation was markedly correlated with the number of transplanted stem cells and peak number of circulating CD34+ cells in peripheral blood, respectively (p = 0.049 and p = 0.003). CONCLUSIONS: Our results indicated no effect of CD56 expression status on the mobilization and engraftment of PBSCs. Our results also support the notion that the peak number of circulating CD34+ cells in peripheral blood is clinically important for rapid platelet engraftment following HPC transplantation.

Recurrent arthritis as an unexpected side effect associated with azacitidine in a patient with myelodysplastic syndrome.

INTRODUCTION: Hypomethylating agents have confirmed efficacy for myelodysplastic syndrome and acute myeloid leukemia and are widely used. Although arthralgia is common side effect associated with hypomethylating agents, arthritis has not been reported previously. CASE REPORT: We present the first recorded patient with arthritis after azacitidine treatment. The patient we presented here had severe cytopenias requiring transfusion with erythrocyte and platelet suspensions, and a complete hematological response was obtained for myelodysplastic syndrome after three cycles of azacitidine (AZA) treatment. However, interestingly, after each AZA treatment cycle, the patient had recurrent attacks of arthritis. MANAGEMENT AND OUTCOMES: The episodes of arthritis were possibly acute flares of pre-existing crystal-induced arthritis, as exhibited with azacitidine treatments and were managed effectively with nonsteroidal anti-inflammatory drugs. DISCUSSION: Because it is a rare condition, clinicians should not overlook AZA as a possible cause of arthritis exacerbations when arthritis of unknown etiology develops in patients treated with AZA.

Prognostic impact of the psoas muscle index, a parameter of sarcopenia, in patients with diffuse large B-cell lymphoma treated with rituximab-based chemoimmunotherapy.

Muscle mass, defined as the psoas muscle index (PMI), is an important parameter of sarcopenia and it has been shown to be a prognostic factor of non-hematological cancers. This study aimed to investigate the prognostic impact of sarcopenia defined using PMI measurement in patients with diffuse large B-cell lymphoma treated with R-CHOP. We retrospectively investigated the impact of pretreatment PMI on survival and response to treatment. One hundred and twenty patients with DLBCL were included in the study, of whom 65 had baseline sarcopenia according to the defined PMI cutoffs. Sarcopenic patients displayed a worse response to treatment compared with non-sarcopenic patients. In a multivariate analysis, sarcopenia remained predictive of outcomes for overall survival (p = .009), progression free survival (p = .028), and response to treatment (p = .006). Sarcopenia defined by evaluating PMI is a simple and routinely applicable method that can predict poor outcomes in patients with DLBCL.

[A Case of Simultaneous Acute Lymphoblastic Leukemia Diagnosis with Crimean-Congo Hemorrhagic Fever]. / Kirim-Kongo Kanamali Atesi ile Es Zamanli Akut Lenfoblastik Lösemi Tanisi Alan Olgu.

Crimean-Congo hemorrhagic fever (CCHF) is a zoonotic disease that can be presented with fever, fatigue, generalized joint/body pain, diarrhea and bleeding in various parts of the body. The risk of developing a severe fatal disease in humans, the possibility of being infected with aerosols and the risk of being used as a biological weapon make the disease still an important health problem all over the world as there is no a specific treatment and vaccine that has proven effective againt the virus today. The pathogenesis of the disease is not known, but vascular endothelial damage is prominent. Therefore, it progresses with thrombocytopenia, anemia, leukopenia and this hematological findings can be confused with hematological malignancies. Acute lymphoblastic leukemia (ALL) is a malignancy included in differential diagnoses and occurs as a result of mutations occuring at a stage of differentiation in the lymphoid precursor cells in the bone marrow. In this study, we present a case of ALL who was diagnosed with CCHF simultaneously. A 43-year old female patient who works in the library and does not have a chronic disease other than asthma and thyroid disorder, has admitted to our hospital with the complaints of intermittent fever, weakness, generalized joint and body pain for about 3 weeks. She had fever and the physical examination revealed bilateral cervical and right postauricular lymphadenopathies. Her aspartate aminotransferase: 77 U/L, alanine aminotransferase: 117 U/L, lactate dehydrogenase: 616 U/L, hemoglobin: 8.27 g/dl, leukocyte count: 15.690/mm3 , neutrophil count: 550/mm3 (%3.5), lymphocyte count: 6690/mm3 (%42.6), platelet count: 102.100/mm3 , C-reactive protein: 163.6 mg/L was detected and the patient was hospitalized on 5 August 2019 for further examination and treatment. Considering that the patient may have viral infection in the foreground the requested test results were detected as; anti-CMV IgM negative, anti-CMV IgG positive, anti-toxoplasma IgM negative, anti-toxoplasma IgG positive, anti-rubella IgM negative, anti-rubella IgG positive, HBsAg negative, anti-HBc IgM negative, antiHBs positive, anti-HAV IgM negative, anti-HAV IgG positive, anti-HCV negative, anti-HIV negative, EpsteinBarr virus (EBV) VCA IgM negative, EBV VCA IgG positive, EBV EBNA IgG positive. Brucella Rose Bengal and Coombs tube agglutination was found be negative. As the cytopenia of the patient deepened, the patient was accepted to have neutropenic fever and it was planned to start piperacillin-tazobactam 4 x 4.5 g/day and two units of erythrocyte replacement therapy. When the patient's history was questioned again, it was learned that she had a tick on her neck about three weeks ago and she had removed the tick herself; 4-5 days later she had the complaints of fever and flu like symptoms and also diarrhea complaints lasting for 3-4 days. Considering the current anamnesis and laboratory findings, the patient was thought to have CCHF and the patient was isolated. The serum sample taken from patient with an initial diagnosis of CCHF and sent to Department of Microbiology Reference Laboratory Public Health Agency of Turkey. The patient was referred to the Antalya Training and Research Hospital. The patient's CCHF serum result was positive. Ribavirin treatment was not initiated in the patient who was accepted to be in the convalescence period, piperacillin-tazobactam 4 x 4.5 g/day treatment was continued and supportive treatment was given. In the follow-up, as the patient's neutropenia, thrombocytopenia and lymphocytopenia still continuing, she was transferred to hematology clinic for malignancy examination and bone marrow biopsy performed by hematology and B cell ALL was diagnosed. She was accepted to be convalescent in terms of CCHF and chemotherapy was started for ALL treatment by hematology. The patient is still being followed up by the hematology clinic and allogenic hematopoietic stem cell tranplantation is planned for the patient. As a result, CCHF is a disease that can be confused with many differential diagnosis. With this case, it is aimed to draw attention to the diagnostic difficulties of CCHF and ALL and to be the first case in the literature.

Conventional Cytogenetics and Interphase Fluorescence In Situ Hybridization Results in Multiple Myeloma: A Turkey Laboratory Analysis of 381 Cases.

Multiple myeloma (MM) is an uncontrolled proliferation of plasma cells and these cells play an important role in the immune system. In this research, we retrospectively analyzed cytogenetic abnormalities in 381 patients with MM. Conventional cytogenetic analysis was successful in 354 patients (92.9%). Chromosomal abnormalities were detected in 31.9% (113/354) and 45.8% (116/253) of patients screened with conventional cytogenetics and FISH, respectively. Of 113 patients with chromosomal abnormalities, 31 patients (27.4%) had hyperdiploid and 26 of 31 patients with hyperdiploidy had both numerical and structural anomalies. On the other hand, non-hyperdiploidy was observed in 62 patients (54.8%). The most common gains of chromosomes were 15, 9, 19 followed by 3, 5, 11, and 21. Whole chromosome losses were also frequent involving Y, 13 and 22 chromosomes. In our patients, 1q gain was determined in a total of 25 patients (22%), including 7 structural abnormalities and 19 unbalanced translocations causing complete or partial duplication of the long arm of chromosome 1. Although the breakpoints were different, t(1;5) balanced translocation and unbalanced translocations of t(1;2), t(1;3), t(1;7), t(1;16) and t(1;19) were observed twice. The most common structural abnormality was the deletion of the short arm of chromosome 13 (13q) or monosomy of chromosome 13 (-13) (24.1%, 61/253) in patients evaluated by FISH. Deletion involving chromosome 17p (del 17p) or monosomy of chromosome 17 (-17) were found in 31 (12.3%) patients. Translocations involving IgH regions were as follows: t(11;14)(q13;q32.33) in 22 (8.7%), t(4;14)(p16.3;q32.33) in 22 (8.7%) and t(14;16)(q32.33;q23.1) in 2 (0.8%) patients. In addition, t(14;17)(q32;q21) translocation was detected in a multiple myeloma patient for the first time in this study. There are a limited number of large study groups including both cytogenetic and FISH findings in MM patients. As the number of these studies increases, it is thought that new cytogenetic data can be guiding especially in clinical risk determination.

Thrombotic Thrombocytopenic Purpura Cases and Consumption of Large Quantities of Energy Drink: Related or Coincidence?

The vast majority of cases of thrombotic thrombocytopenic purpura (TTP) are the result of acquired antibodies which inhibit the activity of the ADAMTS13 enzyme. Acquired TTP is more frequently seen in young females or in individuals with autoimmune disease. The development of antibodies against ADAMTS13 may also result from the administration or consumption of drugs and other substances. However, specific laboratory tests to identify the pathogenic mechanism of a particular drug may not be available, and the role of a potentially implicated drug or other ingested substance may not be clear. In this report we present 2 acquired TTP cases involving the consumption of a large amount of energy drink.

Comparison of Various Hematopoietic Stem Cell Mobilization Regimens in Patients with Lymphoma and Myeloma.

BACKGROUND: The aim of this study was to evaluate the efficacy of cyclophosphamide-based (CB) and platinum-based (PB) chemotherapy regimens for hematopoietic stem cell mobilization in patients with Multiple Myeloma (MM), Hodgkin's lymphoma (HL), and non-Hodgkin's lymphoma (NHL) and the less well-known IEV (iphosphamide, epirubicin, etoposide) regimen in terms of stem cell harvesting competence, factors affecting stem cell com-petence, and toxicity. METHODS: A retrospective evaluation was made of 203 patients (94 MM, 37 HL, and 72 NHL) with peripheral blood stem cell mobilization in different chemotherapy regimens between 2000 and 2010 at the Department of He-matology, Faculty of Medicine, Akdeniz University. RESULTS: There were no differences between CB or PB mobilization regimens and IEV chemotherapy schema in terms of sufficiency of peripheral stem cell harvest, which was predefined as the collected number of peripheral stem cells ≥ 3 x 106/kg for single hematopoietic stem cell transplantation (HSCT) and ≥ 5 x 106/kg for tandem HSCT. There were also no significant differences between low dose cyclophosphamide, high dose cyclophosphami-de, and HCVAD (cyclophosphamide, vincristine, dexamethasone, methotrexate, cytosine arabinoside [ARA-C]) among the subgroups of cyclophosphamide-based regimens. The number of peripheral blood stem cells collected using ESHAP (ethoposide, methylprednisolone, ARA-C, cisplatin), a platin-based regimen, was significantly higher than the other platin-based regimens including DHAP (dexamethasone, ARA-C, cisplatin), ICE (iphosphamide, carboplatin, ethopocide), and EDAP (etoposide, dexamethasone, ARA-C, cisplatin). The toxicity profiles of CB, PB, and IEV chemotherapies were similar. To determine the independent predictors of the efficacy of the stem cell harvest procedure and collected stem cell count, age, diagnosis, duration of disease, number of treatment sequences before mobilization, and number of rescue regimens were included into multiple logistic regression analysis. However, no correlations were determined. CONCLUSIONS: The results of this study provide information on the effectiveness of different stem cell mobilization regimens. Although no significant difference was determined between the three major chemotherapy regimens, the ESHAP regimen appears to be the preferred treatment regimen for stem cell mobilization in selected lymphomas.

Investigation of seasonal frequency and pathogens in febrile neutropenia.

BACKGROUND: In patients with hematological malignancies, febrile neutropenia (FEN) is the most frequent complication and the most important cause of mortality. Various risk factors have been identified for severe infection in neutropenic patients. However, to the best of our knowledge, it is not defined whether there is a change in the risk of febrile neutropenia according to seasons. The first aim of study was to determine the difference in frequency of febrile neutropenic episodes (FNEs) according to months and seasons. The second aim was to document isolated pathogens, as well as demographical and clinical characteristics of patients. METHODS: In the study, 194 FNEs of 105 patients who have been followed with hematological malignancies between June 2013 and May 2014 were evaluated retrospectively. RESULTS: Although the number of FNEs increased in autumn, there was no significant difference in frequency of FNEs between months (p = 0.564) and seasons (p = 0.345). There was no isolated pathogen in 54.6% of FNEs. In 45.4% of 194 FNEs, pathogens were isolated. Of all pathogens, 50.4% were gram negative bacteria, 29.2% were gram positive bacteria, 13.3% were viruses, 5.3% were fungi, and 1.8% were parasites. CONCLUSIONS: The frequency of FEN does not change according to months or seasons. Also, the relative proportions of different pathogens in the cause of FEN do not vary according to seasons.