Ilknur Nizam

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.

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.

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/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.

Leukapheresis Treatment in Elderly Acute Leukemia Patients With Hyperleukocytosis: A Single Center Experience

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.

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

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.

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.

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.

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.

Etiologic evaluation of 1012 patients admitted with thrombocytopenia.

Introduction: Thrombocytopenia is the situation where the number of thrombocytes is less than 150.000/ μL. This may result due to decreased thrombocyte production, increase in the destruction of thrombocytes and differentiation in distribution of thrombocytes. Hereditary and acquired diseases both contribute to the condition, but the acquired reasons are more common in older patients. In this study we aimed to present the patients’ data with thrombocytopenia in the eastern Turkey and the underlying diseases that cause thrombocytopenia in order to contribute to literature. Material and Methods: In this study, we retrospectively aimed to evaluate the etiology of thrombocytopenia at 1012 patients who admitted to Inonu University Medical Faculty Turgut Ozal Medical Center Hematology outpatient clinic, hematology service, emergency department and the ones who were consulted from other departments in the hospital. We collected data of patients for whom we searched the aetiology of thrombocytopenia as Department of Hematology. Results: Total of 1012 patients, of whom 508 were female and 504 were male, were included to the study. The mean age was 51±20 years. The mean number of the thrombocytes was 64.28±43.05 /mL. The mean number of leucocytes was 13.89±32.71 /μL. The mean level of hemoglobin was 11.22±3.03 g/dL. Leukaemia, infections and the immune thrombocytopenias represented most of the cases, and the other reasons of Research Article British Journal of Medicine & Medical Research, 4(1): 104-113, 2014 105 thrombocytopenia were drugs, chronic liver diseases, megaloblastic anemias, pseudothrombocytopenia, thrombotic microangiopathies and other diseases. Conclusion: The reasons of thrombocytopenia may differ according to geographic distribution and step level of health centers. It’s an expected situation for thrombocytopenia reasons that the high rate of infections can be accused in developing countries and malignant diseases to be the first reason in developed countries.

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.