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.

Assessing Safety of Pneumatic Tube System (PTS) for Patients with Very Low Hematologic Parameters.

BACKGROUND Preventive interventions save lives during the process of chemotherapy for hematologic malignancies, when a hematology laboratory can ensure accurate results. The use of a pneumatic tube system (PTS) is associated with measurement errors and unnecessary transfusions. The aim of this study was to evaluate pre-analytical errors associated with transportation method (PTS versus hand-delivered) and to investigate whether there are unnecessary transfusion events in pancytopenia leukemia patients with very low hematological parameters. MATERIAL AND METHODS A total of 140 paired blood collections were performed for hemogram and biochemistry assays. Paired EDTA and serum gel blood samples were collected from 58 cases with acute leukemia on different days. For each pair, one sample was hand-delivered by a courier (Group 1) while the other sample was transported through a PTS (Group 2). RESULTS The hand-delivered method showed that some platelet transfusions were unnecessary for different thrombocyte cut-off values. Calculated unnecessary platelet (PLT) transfusion ratios when using PTS (PLT <30×10³/µL, 16.3%; PLT <25×10³/µL, 16.4%; PLT <20×10³/µL, 80.3%; PLT <15×10³/µL, 48.6%; and PLT <10×10³/µL, 150.0%) were found to be statistically significant (p=0.002, p=0.046, p<0.000, p=0.028, and p<0.000, respectively). In contrast, for RBC transfusion ratios, although the ratios were high in Group 2, we found no significant difference between the two groups; (HGB <8.0 g/dL, 23.3%; HGB <9.0 g/dL, 25.0%, HGB<10.0 g/dL, 19.3%) and (p=0.002, p=0.085, p<0.160, and p=0.235, respectively). CONCLUSIONS Although our results cannot be universally applied, physicians should be careful, skeptical, and suspicious of transfusion decisions in hematology clinics and consider potential analytical and pre-analytical errors in cases of severe cytopenia when using PTS.

DHAP plus filgrastim as an effective peripheral stem cell mobilization regimen for autologous stem-cell transplantation in patients with relapsed/refractory lymphoma: A single center experience.

This study aimed to evaluate the efficiency of DHAP regimen plus filgrastim for mobilization of stem cells in patients with recurrent and/or refractory lymphoma. Thirty-four patients who took DHAP as salvage therapy prior to autologous stem cell transplantation were included. After chemotherapies, 2 cycles of DHAP plus filgrastim were administered to the patients. Stem cells from 32 patients (94%) were collected on median 11th day (8-12), and the median collected CD34(+) cell dose was 9.7 × 10(6)/kg (range 3.8-41.6). DHAP plus filgrastim was found to be an effective chemotherapy regimen in mobilizing CD34(+) stem cells into the peripheral.

Leukapheresis treatment in elderly acute leukemia patients with hyperleukocytosis: A single center experience.

AIM: Leukapheresis is an invasive treatment modality used for hyperleukocytosis. Various drugs and fluids are used during the leukapheresis. Aging itself and associated factors such as increased comorbidity, decreased tolerance to drugs, increased drug toxicity give rise to the application of other treatment modalities in elderly patients. Treatment of acute leukemia in the elderly differs from young patients. Consequently, we assumed that outcome, effectiveness, and side effects of leukapheresis treatment used for acute leukemia patients with hyperleukocytosis may be different in elderly compared to younger patients. METHODS: We retrospectively evaluated a total of 39 patients. Eighteen patients were 65 years and older. Indications for leukapheresis were determined as symptoms of leukostasis and prophylaxis. Acid citrate dextrose-A, calcium gluconate, and plasma were used during the leukapheresis. Age, sex, diagnosis, count, and indications of leukapheresis procedures, leukocyte count, and lactate dehydrogenase level were analyzed at the onset of and after leukapheresis; side effects, causes of death, early and total mortality rates were also analyzed. We compared the two groups with regard to effectiveness, clinical outcomes, and side effects. RESULTS: There were no statistically significant differences between the two groups with respect to sex, diagnosis, initial leukocyte count, lactate dehydrogenase level, number of leukapheresis procedures, rates of side effects, or early and total mortality (P > 0.05). Leukapheresis treatment was effective in both groups (P < 0.05) and no significant difference was found in its effectiveness between two groups (P > 0.05). CONCLUSION: Leukapheresis is an effective and safe treatment modality in elderly acute leukemia patients with hyperleukocytosis.

Can BuCyE conditioning regimen be an alternative treatment to BEAM at autologous transplantation in malignant lymphoma patients?: a single center experience.

High-dose chemotherapy (HDC) applied together with autologous stem cell transplantation (ASCT) is a commonly used treatment modality in patients with malignant lymphoma. At present, there is a limited number of studies which compare toxicity and efficacy of various high-dose regimens applied in the treatment of malignant lymphoma. For this reason, the aim of this study was to investigate the efficacy and toxicity of BuCyE (busulfan, cyclophosphamide and etoposide) and BEAM (carmustine, etoposide, cytarabine and melphalan) preparative regimens in the patients with malignant lymphoma scheduled for autologous stem cell transplantation. Between November, 2010 and April, 2015, 42 patients with relapsed or refractory malignant lymphoma who underwent autologous stem cell transplantation following BEAM (n=11) and BuCyE (n=31) preparative regimens were analyzed at Bone Marrow Transplantation Unit of TurgutOzal Medicine Center in Turkey. The groups were compared in terms of patient characteristics, hematopoietic engraftment time, toxicity profiles and survival. No significant differences were detected between the groups with regard to age, gender distribution, international prognostic index, ASCT indications, disease status at the time of ASCT and type of lymphoma (P>0.05). Median number of infused CD34+ cells/kg, neutrophil and platelet engraftment statuses of BuCyE and BEAM groups were found to be similar (P>0.05). More patients in BuCyE group developed mucositis and nausea, but this difference was not statistically significant (P>0.05). A similar statistically insignificant difference was seen in that infectious complications occurred more commonly in BEAM group (P>0.05). Overall survival and event-free survival rates were not significantly different between the groups (P>0.05). BuCyE is a conditioning regimen which can be effectively used as an alternative to BEAM in the patients with malignant lymphoma undergoing ASCT. Moreover, toxicity rates of both regimens are similar. In order to comprehend the effect of each HDC regimen, further evidence-based data obtained from the studies involving larger sample sizes are required.

Leukapheresis in acute myeloid leukemia patients with hyperleukocytosis: A single center experience.

Hyperleukocytosis is defined as WBC count above 100,000/mm(3) in peripheral blood. Increased WBC count leads to leukocyte aggregation, increased blood viscosity, and consequently results in stasis in small blood vessels. Ultimate neurological, pulmonary, gastrointestinal complications, coagulopathy, and tumor lysis syndrome cause increase in morbidity and mortality. Leukapheresis is a treatment modality used for hyperleukocytosis. In patients presenting with hyperleukocytosis the indications for leukapheresis were accepted as having symptoms of leukostasis and prophylactic. Indications for leukapheresis in prophylactic group evaluated according to WBC count. We report a single center experience about leukapheresis in managing 31 AML patients with hyperleukocytosis. In addition to demographic characteristics, disease-related clinical and laboratory findings of the patients were recorded. Survival rates were also calculated. Ten patients were female. The most common of AML subtype was AML-M2. The median number of leukapheresis per patient was 2 and totally 60 leukapheresis cycles were performed in all patients. There was a significant decrease in WBC count and LDH level after leukapheresis as compared with the baseline values (p < 0.05). Early and total mortality were 16.1% and 58.0%, respectively. Alive and died patients were evaluated according to baseline WBC, LDH; increased WBC count and LDH level were found in died patients (p < 0.05). According to leukapheresis indications, patients were divided into two groups: 14 patients in symptomatic leukostasis, 17 patients in prophylaxis. No statistically significant differences were noted between both groups in leukapheresis effectiveness, mean survival time, early and total mortality rate (p > 0.05). None of our patients suffered serious side effects and tumor lysis syndrome during or after apheresis. Leukapheresis is an effective and safe approach to reduce WBC counts in patients with AML with hyperleukocytosis. Further evidence-based data obtained from larger sample sizes are required to better understand the impact of prophylaxis leukapheresis on early and total mortality of AML patients with hyperleukocytosis.

A life-saving therapy in Class I HELLP syndrome: Therapeutic plasma exchange.

HELLP syndrome, which can affect multiple organ systems and cause maternal and fetal mortality, is a serious complication of pregnancy characterized by microangiopathic hemolytic anemia, elevation of liver enzymes, and thrombocytopenia. Delivering the infant usually suffices for the treatment of this syndrome. In cases with Class I HELLP syndrome, however, the clinical picture may rapidly deteriorate despite delivery. In this paper we presented the outcomes with the use of therapeutic plasma exchange in cases with class I HELLP syndrome. This study included 21 patients diagnosed with the Class I HELLP syndrome at Inonu University Faculty of Medicine, Department of Hematology between 2011 and 2014. A central venous catheter was placed and plasma exchange therapy was begun in patients unresponsive to delivery, steroid, and supportive therapy (blood and blood products, antihypertensive therapy, intravenous fluid administration, and antibiotics) within 24 hours after the diagnosis of Class I HELLP syndrome according to the Mississippi Criteria. All patients underwent therapeutic plasma exchange for three sessions each with a 1:1 volume. Hemogram and biochemical parameters of the patients were evaluated before and after the procedure. According to results, there was a statistically significant decrease in total bilirubin, LDH, AST, and ALT levels whereas a significant increase in platelet count was observed. Hemoglobin levels were increased, although this increase was not statistically significant. HELLP syndrome is primarily treated with the delivery of infant; however, some cases may show disease progression despite completion of delivery. As a potential cause of both maternal and fetal mortality, HELLP syndrome condition should be aggressively treated. Therapeutic plasma exchange is one of the available treatment options. Our study has found that postpartum use of plasma exchange therapy within 24 hours is an efficient and lifesaving treatment choice in Class I HELLP syndrome.

Clinical characteristics and therapeutic outcomes of elderly patients with chronic myeloid leukemia: A retrospective multicenter study.

AIMS: We aimed to investigate whether older age leads to limitations in the starting dose of imatinib in daily treatment of chronic myeloid leukemia, and to determine the compliance of elderly patients with tyrosine kinase inhibitors (TKI) therapy. METHODS: Data including the clinical characteristics, therapeutic outcomes and compliance with TKI therapy of elderly patients with chronic myeloid leukemia aged >65 years were collected from 13 institutions in Turkey, retrospectively. RESULTS: A total of 69 patients (27 [39%] men, 42 [61%] women) were evaluated retrospectively. The median age of the patients was 71 years (range 66-85 years). Of the patients, 66 (96%) were in the chronic phase and three (4.3%) were in the accelerated phase when diagnosed. A total of 63 (91.3%) patients were receiving imatinib as the first-line therapy. The initial dose of imatinib was 400 mg/day in 59 patients (93.6%). Imatinib treatment induced 57 (90.5%) complete hematological responses at 3 months, 29 (46%) complete cytogenetic responses at 6 months and 49 (77.7%) major molecular responses at 12 months. As a result, nilotinib and dasatinib were used in 14 patients as second-line therapy. Second-line TKI induced nine complete hematological responses (64.3%) at 3 months, four complete cytogenetic responses (28.6%) at 12 months and seven major molecular responses (50%) at 18 months. A total of 56 of the patients (81.2%) are still alive. The median overall survival and progression-free survival rates were 35 months (range 1-95 months) and 17 months (range 0.8-95 months), respectively. CONCLUSION: Elderly patients should receive TKI according to the same guidelines that apply to younger patients.

Thrombotic thrombocytopenic purpura presenting with pathologic fracture: a case report.

Thrombotic thrombocytopenic purpura is an acute syndrome with abnormalities in multiple organ systems, which becomes manifest with microangiopathic hemolytic anemia and thrombocytopenia. The hereditary or acquired deficiency of ADAMTS-13 activity leads to an excess of high molecular weight von Willebrand factor multimers in plasma, leading to platelet aggregation and diffuse intravascular thrombus formation, resulting in thrombotic thrombocytopenic purpura. Thrombotic lesions occurring in TTP leads to ischemia and convulsion. Depending on the properties of the bony tissue, fractures are divided into three groups as traumatic, pathological, and stress fractures. A pathologic fracture is a broken bone caused by disease leading to weakness of the bone. This process is most commonly due to osteoporosis, but may also be due to other pathologies such as cancer, infections, inherited bone disorders, or a bone cyst. We herein report a case with a pathologic fracture due to convulsion secondary to thrombotic thrombocytopenic pupura. Thrombotic lesions occurring in TTP may lead to ischemia and convulsion, as in our patient and pathological fractures presented in our case report may occur as a result of severe muscle contractions associated with convulsive activity. Thrombotic thrombocytopenic pupura is a disease that involves many organ systems and thus may have a very wide spectrum of clinical presentations.

Hermansky-pudlak syndrome: a case report.

Objective. The aim of this paper is to report the case of a patient diagnosed with Hermansky-Pudlak syndrome, as a result of bleeding diathesis. Clinical Presentation and Intervention. A 23-year-old male presented with recurrent epistaxis and, upon physical examination, was found to be remarkable for albinism and suborbital ecchymosis. The absence of dense bodies in the platelets was demonstrated using electron microscopy. This patient was (slowly) administered one unit of a platelet suspension, and his bleeding decreased considerably. Conclusion. This case shows that Hermansky-Pudlak syndrome should be considered in the differential diagnosis of a patient presenting with bleeding diathesis, when the clinical presentation also includes oculocutaneous albinism and visual problems.

Pulmonary embolism as the initial presentation of testicular carcinoma.

Objective. The risk of pulmonary embolism is well recognized as showing an increase in oncological patients. We report a case presenting with pulmonary embolism initially, which was then diagnosed with testicular cancer. Clinical Presentation and Intervention. A 25-year-old man was admitted to the emergency department with a complaint of dyspnoea. Thoracic tomography, lung ventilation/perfusion scintigraphy, and an increased D-dimer level revealed pulmonary embolism. For the aetiology of pulmonary embolism, a left orchiectomy was performed and the patient was diagnosed with a germinal cell tumour of the testicle. Conclusion. In this paper, we present a patient for whom pulmonary embolism was the initial presentation, and a germinal cell tumour was diagnosed later during the search for the aetiology.

The association of low-grade systemic inflammation with hypertensive retinopathy.

High-sensitivity C-reactive protein (hs-CRP) is a marker of systemic low-grade inflammation. The pathophysiologic mechanism of hypertensive retiopathy (HR) is not fully established. Elevated blood pressure (BP) alone does not fully account for the extent of retinopathy, other pathogenic mechanisms may be involved, such as low-grade inflammation. Therefore, this study was designed to answer the following questions. (i) Do hs-CRP levels change in HR? (ii) Is there any relation between degree of HR and hs-CRP levels? This study included 84 hypertensive patients with HR. The hypertensive patients were divided into two groups according to the Keith-Wagener classification. Group 1 comprised 42 patients with grade I HR, and Group 2 comprised 42 patients with grade II HR. We selected 42 healthy subjects matched for age, sex, and body mass index (BMI) for control group. The level of hs-CRP in group 2 was significantly higher than in group 1 group (p = 0.018) and control group (p = 0.001), it was also higher in group 1 than in control group (p = 0.002). Also, hs-CRP showed positive correlations with degree of HR (r = 0.29, p = 0.017). Our study suggests that there is a relationship between HR and hs-CRP levels, which may be associated with systemic low- grade inflammation.

A case of primary Sjögren's syndrome with pulmonary-limited Wegener's granulomatosis.

A 60-year-old woman had a history of dyspnea for 5-6 weeks. The chest radiograph and computed tomography scans revealed bilateral patchy reticulonodular pattern. The patient had positive test results for antineutrophil cytoplasmic antibody against proteinase-3 (c-ANCA), antinuclear antibody and anti-Ro antibody. According to European Study Group on Classification Criteria for Sjögren's Syndrome, the patient was diagnosed as primary Sjögren's syndrome based on the presence of clinical features, positive findings on Schirmer's test and parotis scintigraphy. Lung biopsy obtained by wedge resection showed granulomatous inflammation with extensive multinuclear giant cells involving the lung parenchyma and vascular structures. There was neither upper airway nor renal involvement. Thus, the patient was simultaneously diagnosed as pulmonary-limited Wegener's granulomatosis. With this unique case, we would like to emphasize that the awareness of ANCA-associated vasculitis as a diagnostic possibility in primary Sjögren's syndrome is important during the work-up of lung lesions.