A COVID-19 mystery: multisystem inflammatory syndrome in adults (MIS-A) associated with splenic rupture.

Severe inflammation and one or more extrapulmonary organ dysfunctions have been observed in those who had recently developed COVID-19, except for a macrophage activation syndrome-like picture. A 50-year-old female patient was admitted to the emergency department with fever and a history of COVID-19 infection. More than one area of hemophagocytosis was found in the bone marrow aspiration. The HLH-2004 protocol was started with neurological involvement and she underwent splenectomy due to massive intra-abdominal bleeding secondary to splenic laceration on the 3rd day. Multiple microthrombosis and infarcts were observed in the splenectomy specimen. At the 4th week of the treatment, she was discharged with oral agents. Splenic microthrombosis and splenic rupture due to "multisystem inflammatory syndrome in adults" are the most important findings of this report.

Beta Thalassemia Minor: Patients Are Not Tired but Depressed and Anxious.

OBJECTIVE: We aimed to investigate whether the severity of fatigue and the incidences of depression and anxiety of patients with beta thalassemia minor (BTm) are different from healthy individuals using the Fatigue Severity Scale (FSS) and Hospital Anxiety and Depression Scale (HADS). SUBJECTS AND METHODS: BTm patients who were followed at the University of Health Sciences Istanbul Training and Research Hospital Hematology Clinic between 2016 and 2017 and who had normal biochemical parameters, thyroid function tests and C-reactive protein levels, and did not use any medications, consume alcohol or tobacco, have any chronic diseases or sleep disturbances were included in the study. Healthy control subjects who were matched with age, sex, marital status, educational status, and body mass index (BMI) were also included for comparison. RESULTS: Thirty-nine BTm patients and 25 healthy controls were included in the study. The BTm and the control groups were comparable in terms of gender, age, BMI, educational status and marital status (p = 0.368, 0.755, 0.851, 0.785, and 0.709, respectively). FSS score was ≥4 in 23 (59.0%) BTm subjects and in 15 (60%) control subjects (p = 1.0). HADS anxiety score was ≥10 in 20 (51.3%) BTm subjects and in 5 (20.0%) control subjects (p = 0.018), and HADS depression score was ≥7 in 20 (51.3%) BTm subjects and in 6 (24.0%) healthy control subjects (p = 0.039).There was no correlation of hemoglobin with FSS score (p = 0.526, r = -0.105), HADS anxiety score (p = 0.703, r = -0.063), or HADS depression score (p = 0.718, r = -0.06) in the BTm group. CONCLUSION: We found that both depression and anxiety were higher in BTm patients than in healthy individuals, but this difference was not feasible for fatigue.

The impact of transfusion burden and comorbidities on the prognosis of patients with myelodysplastic syndromes.

PURPOSE: Early comorbidity detection has been reported to be associated with treatment-related outcomes in several diseases. Two main goals of the present study were to investigate both the impact of comorbidities and transfusion frequencies on the survival and quality of life of patients with myelodysplastic syndromes (MDS). METHODS: One hundred and four MDS patients with a median International Prognostic Scoring System (IPSS) score of 0.5 (range: 0-3) were included in the study. Almost half of the patients had more than one comorbidity. RESULTS: Median short form health surveys (SF)-36 mental and physical scores were 42.1 (range: 20.6-66.1) and 38.7 (range: 18-59.7), respectively. Mean scores of the Eastern Cooperative Oncology Group (ECOG) performance scales at diagnosis and during recruitment were 1.0 (1.4 ± 1.0) and 2.0 (1.8 ± 1.1), respectively. The mean Charlson Comorbidity Index (CCI) score was 1.0 (1.4 ± 1.5). In the model that was constructed using variables with a p value < 0.100 in the univariate analysis, factors that predicted death were refractory anemia with excess blasts (RAEB) and ECOG scores at recruitment. When ECOG was removed from the model, RAEB and CCI at diagnosis moved to the forefront as mortality predictors. CONCLUSION: This study demonstrated that both CCI and ECOG performance status had an impact on survival in MDS patients who had low IPSS scores. ECOG stood out as a better and more practical predictor of survival than CCI, especially after considering its (ECOG) ease of use.

Evaluation of the Reliability of Interim PET/CT in the Hodgkin Lymphoma.

INTRODUCTION: Positron-emission tomography (PET)/computerized tomography (CT) with 18F-fludeoxyglucose (FDG) has been come into use for risk assessment of Hodgkin lymphoma (HL) patients in recent years. The aim of our study is to evaluate the reliability of interim PET results according to Deauville score (DS), and also to compared PET findings with tumor reduction on CT. METHODS: Forty-two HL patients (median 39, range 19-75 y, 27 M, 15 F) were retrospectively evaluated with pre, interim and post-treatment PET/CT imaging. PET/CT imaging was obtained 60 min after the intravenous administration of 3.7-5.2 MBq/kg 18F-FDG. RESULTS: The negative predictive value of the interim PET was 89%. Four (10.5%) of the 38 interim PET-negative patients became post-treatment PET-positive. According to CT, 15 patients were in complete remission (CR), 27 (64.6%) patients were in partial remission (PR) or stable disease (SD). CONCLUSION: The negative predictive value of interim PET was not satisfactory considering the treatment rate of over 80% of HL. Additionally, high rate of interim PET-negative patients' conversion to PET-positive post-treatment state was considered as unexpected.

An uncommon neurological manifestation of chronic lymphocytic leukemia: Longitudinally extensive transverse myelitis.

We describe a 50-year-old male patient who was admitted to the emergency department with complaints of fever and fatigue that had suddenly started two weeks ago. In the laboratory evaluation, a white blood cell count of 131.000/mm3 was detected. The patient was hospitalized and developed fecal incontinence on the first day of hospitalization. Detailed neurological examination revealed the patient had tetraparesis. Long segment high signal intensity was observed on spinal MRI. Flow cytometry examination of the CSF and biopsy findings of the bone marrow were compatible with Chronic Lymphocytic Leukemia (CLL). The patient's MRI appearances resolved after treatment. The tetraparesis resolved partially. There was no motor deficit in upper extremities and the patient was able to walk without aid or rest for 100 m. Clinical manifestation of central nervous system (CNS) involvement in CLL is heterogeneous and therefore may be difficult to pinpoint. We have described an uncommon occurrence of CNS involvement in CLL.

Comparison of clinical features and survival between young and old chronic lymphocytic leukemia patients: a single center study from Turkey.

We aimed to investigate whether the clinical characteristics, the rate of treatment demand and survival differ between chronic lymphocytic leukemia (CLL) patients <65 years (y) and ≥65 y. Sixty three (46%) patients were <65 y and 74 (54%) were ≥65 y. 28.6% (18/63) of the patients <65 required treatment, while this rate was 44.6% (33/74) for patients ≥65 y (p > .05). The probability of overall survival (OS) was 90 vs. 51% in patients <65 y and ≥65 y (p = .016). In univariate analysis, the age affected OS (p = .04, HR: 0.212, CI: 0.047-0.946). In subgroup analysis, in treated patients, the probability of OS was 73 vs. 49% in patients <65 y and ≥65 y (p = .389). The survival difference between the young and old patients is too high to be ignored and age seems to affect the outcome of CLL patients.

A diffuse large B cell lymphoma emerging with breast cancer relapse.

The prevalence of secondary cancers associated with the breast cancer treatment has increased, which is due to the administration of cytotoxic/hormonal drugs as well as radiotherapy. A 54-year-old female patient with a history of breast cancer for 4 years and receiving tamoxifen the hematology clinic with fatigue and nosebleed. Laboratory parameters were revealed pancytopenia. The bone marrow biopsy finding was compatible with CD20 positive high-grade B cell lymphoma resembling diffuse large B cell lymphoma. The patient started to receive a chemotherapy. Her hemogram values displayed an improvement after the second cycle. However, interim PET-BT, performed after the fourth cycle, showed an incomplete response in cervical lymphatic nodes. Then, a tru-cut biopsy was performed resulting in breast cancer metastasis. This is an unusual case of secondary-DLBCL presenting with pancytopenia and occuring 4 years after the diagnosis of breast cancer. In conclusion, clinicians should carefully set the dosage of chemotherapy drugs to avoid the long-term side effects associated with such drugs.

Rethinking the usefulness of bone marrow biopsy on treatment decision in CLL patients at diagnosis.

We aimed to investigate the role of bone marrow infiltration pattern (BMIP) and bone marrow reticulin fibrosis (BMRF) in determining treatment demand in patients with diagnosis of chronic lymphocytic leukemia (CLL). We retrospectively evaluated the data of 65 patients, who were followed with the diagnosis of CLL at Istanbul Training and Research Hospital, Department of Hematology, between July 2007 and June 2016. The median age of the patients was 64 years (range, 32-83). Twenty-three (35.4%) patients were female, and 42 (64.6%) were male. Early/mild grade BMRF was observed in 46 (70.8%) patients and advanced grade BMRF in 19 (29.2%) patients. Eleven (23.9%) of 46 patients with early/mild grade BMRF and 10 (52.9%) of 19 patients with advanced grade BMRF required treatment during follow-up (p = 0.04). According to the BMIP, 14 (21.5%) patients had diffuse and 51 (78.5%) patients had non-diffuse BMIP. Eleven (78.6%) of 14 patients with diffuse BMIP and 10 (19.6%) of 51 patients with non-diffuse BMIP required treatment during follow-up (p < 0.001). In univariate analysis, both advanced grade BMRF and diffuse BMIP had an impact on occurrence of treatment demand (p = 0.028, HR = 3.535 vs. p < 0.01 HR = 15.033). Multivariate analysis also revealed diffuse BMIP to be effective (p < 0.001, HR 13.089), while advanced grade BMRF failed to significantly influence treatment demand (p = 0.140, HR 2.664). In conclusion, in the light of our findings, it is reasonable to consider that bone marrow biopsy at the time of diagnosis might provide a preliminary information about treatment demand in patients with CLL.

Adverse impact of hyperferritinemia and transfusion dependency on treatment success in myelodysplastic syndrome.

BACKGROUND: Myelodysplastic syndrome (MDS) is characterized by peripheral cytopenias and dysplasia in one or more cell lines in the bone marrow. A significant proportion of patients require blood product support due to symptomatic anemia and/or thrombocytopenia during the course of their disease. This retrospective study was planned to evaluate the transfusion requirement of MDS patients and the role of ferritin in predicting transfusion requirement and response to treatment. METHODS: We retrospectively reviewed the records of 35 MDS patients [median age: 66 (22-84); male/female: 21/14]. The World Health Organization (WHO) criteria was used for disease classification and International Prognostic Scoring System (IPSS) for risk stratification. RESULTS: A total of 22 patients (62.8%) required transfusions during follow-up. While all the 22 patients received packed red blood cells (PRBCs), only 8 patients (22.9%) required platelet transfusion(s). Although no significant relationship was demonstrated between transfusion dependency and disease progression, patients who responded to disease-specific treatment were exposed to less PRBC transfusions compared to non-responders (p=0.04). Treatment response was found to be better in patients who had lower serum ferritin levels at diagnosis (p=0.004). A total of 11 patients were followed for a minimum of 24months. Transfusion load was not different among these patients with respect to disease subtype, IPSS risk score and treatment protocol in the first and second 12-month interval. Median overall survival of the cohort was 26.3 (0.4-160.3) months and median progression free survival was 24.9 (0.4-160.3) months. CONCLUSION: The present report underlines the association of baseline hyperferritinemia and transfusion dependency with treatment success in MDS. Even in patients treated with new generation agents, the vicious impact of transfusion load seems to be the tender spot of the MDS puzzle. The prognostic impact of baseline hyperferritinemia should be validated with further studies.

Outcome of autologous stem-cell transplantation in relapsed or refractory Hodgkin lymphoma patients in a centre from Turkey.

PURPOSE: The aim of this study is to assess the predictors of outcome in patients with relapsed or refractory Hodgkin's lymphoma (HL) receiving autologous stem-cell transplantation (ASCT) MATERIALS AND METHODS: Fifty-two consecutive patients who received ASCT at the Stem Cell Transplantation Unit of Gazi University Hospital from February 2005 through June 2011 for relapsed or refractory HL were analysed retrospectively RESULTS: Fifty-one patients could be evaluated after transplantation, as one of the patients died in the early post-transplantation period. Complete remission was obtained in 36 (71%), partial remission in 9 (18%), stable disease in 4 (8%), and progressive disease in 2 (3%) patients. After a median follow-up of 22 (range, 0.5-75) months, 46 (88%) patients were alive. The probability of overall survival (OS), progression free survival (PFS) and transplantation related mortality at 5 years were 87, 53, and 2%, respectively. Chemosensitive relapse had a positive impact on both OS and PFS CONCLUSION: ASCT remains to be the standard treatment of relapsed or refractory HL patients. Chemosensitive relapse is the most important prognostic factor determining the outcome of the ASCT.

HBV-related events after allogeneic hematopoetic stem cell transplantation in a center from Turkey.

To investigate the frequency of hepatitis B virus (HBV)-related events after allogeneic HCT in a moderate endemic area for HBV infection. The data of 197 patients who underwent allogeneic hematopoetic stem cell transplatation (HCT) from September 2003 through December 2010 were reviewed retrospectively with respect to HBV-related events. Resolved HBV infection was described as negative HBsAg, positive HBcAb, and positive HBsAb. Latent HBV infection was defined in patients with HBcAb positivity in the abscence of HBV DNA and HBsAb. Hepatitis B naive patients are defined as the patiens with no serological or molecular marker related to HBV. Seropositive patients were the patients with positive HBsAg and HBV-DNA. Median age was 28 (range, 15-64) years, with 128 male and 69 female patients. Median follow-up of the cohort was 8 (range, 0.5-78) months. We detected HBV-related events in 7 (3.6 %) recipients after allogeneic HCT. Five (71.4 %) of these events were HBV reactivation, while two cases (28.6 %) had acute hepatitis B infection. Four of the five reactivations were in the seropositive group (80 %), while one ocurred in a patient with resolved hepatitis. Two patients who developed acute hepatitis B were HBV naive and previously immunized patients, respectively. Hepatitis B virus reactivation remains a problem in seropositive patients and might require more effective treatment strategies.

The role of body mass index and other body composition parameters in early post-transplant complications in patients undergoing allogeneic stem cell transplantation with busulfan-cyclophosphamide conditioning.

Patients with impaired nutritional status may show increased risk of hematopoietic stem cell transplantation (HSCT)-related complications. This study was conducted to determine whether body mass index (BMI) and other body composition parameters, such as lean body mass index (LBMI) and body fat mass (BFM), are associated with early post-transplantation toxicity and mortality in allogeneic HSCT recipients. The records of 71 patients diagnosed with acute myeloid leukemia (AML), acute lymphoblastic leukemia (ALL), chronic myeloid leukemia (CML), or myelodysplastic leukemia (MDS) who had undergone allogeneic HSCT with a conditioning regimen of busulfan-cyclophosphamide (Bu-Cy), between September 2003 and January 2009 at the Stem Cell Transplantation Unit of Gazi University Hospital were retrospectively evaluated. BMI was found to be negatively correlated with the NCI grade of mucositis, cardiotoxicity, emesis, and hyperglycemia, and with the number of erythrocyte transfusions. LBMI was also negatively correlated with the number of erythrocyte transfusions, cardiotoxicity, emesis, and hyperglycemia. BFM was negatively correlated with the day of neutrophil engraftment, and NCI grade of mucositis. Nutritional status did not have an impact on overall survival (OS), progression-free survival (PFS), or 100-day transplant related mortality (TRM).