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Hemic and Lymphatic Diseases - Top 30 Publications

Rates and Trends of Pediatric Acute Lymphoblastic Leukemia - United States, 2001-2014.

Acute lymphoblastic leukemia (ALL) is the most prevalent cancer among children and adolescents in the United States, representing 20% of all cancers diagnosed in persons aged <20 years, or >3,000 new cases each year (1). Past studies reported increasing trends of ALL overall and among Hispanics, but these represented ≤28% of the U.S. population and did not provide state-based estimates (1-3). To describe U.S. ALL incidence rates and trends among persons aged <20 years during 2001-2014, CDC analyzed rigorous data (based on established publication criteria) from the United States Cancer Statistics data set, which includes incidence data on approximately 15,000 new cases per year of all types of invasive cancer among children and adolescents aged <20 years (4). The data set represented 98% of the U.S. population during the study period. Overall incidence of pediatric ALL during 2001-2014 was 34.0 cases per 1 million persons and among all racial/ethnic groups was highest among Hispanics (42.9 per 1 million). Both overall and among Hispanics, pediatric ALL incidence increased during 2001-2008 and remained stable during 2008-2014. ALL incidence was higher in the West than in any other U.S. Census region. State-specific data indicated that the highest rates of pediatric ALL incidence were in California, New Mexico, and Vermont. These demographic and geographic ALL incidence data might better inform public health interventions targeting the following areas: exposures to recognized risk factors for leukemia; ALL treatment, including clinical trial enrollment; survivorship care planning; and studies designed to understand the factors affecting changes in pediatric cancer incidence.

Eosinophilic gastroenteritis presenting as upper gastrointestinal hematoma and ulcers after endoscopic biopsy: A case report and literature review.

Eosinphilic gastroenteritis (EG) is a gastrointestinal disorder characterized by eosinophilic infiltration with various manifestations. The diagnosis is usually confirmed by an endoscopic biopsy, which is considered a safe and routine procedure for the majority.

Barriers and facilitators to participation in clinical trial among lymphoma patients from Sun Yat-sen University Cancer Center in China: An observation study.

Recruitment rate of clinical trials in cancer patients is pretty low in China. Little is known about factors influencing trial recruitment in Chinese cancer patients. The aim of present study is to evaluate the barriers and facilitators to participation in clinical trials among lymphoma patients in China.From December 2014 to August 2015, the survey was carried out in the Department of Medical Oncology in Sun Yat-sen University Cancer Center. A self-made questionnaire was used among lymphoma patients (N = 331) to evaluate their attitude toward clinical trials. The questionnaire included 2 parts: patients' basic information and whether they were willing to participate in future clinical trials and their reasons.There were 53.5% patients willing to participate in clinical trials. The most common reasons were thirst for new treatments, trust on hospital and doctors, the idea that clinical trials may be more effective than conventional therapy, and to get more management and monitoring. The following patients are more likely to participate in clinical trials: patients who have children (P = .019) or spouse (P = .037), cannot afford treatment cost (P = .019), have tumor relapse (P = .045), and cared about the medical development (P = .032). Patients who have little knowledge of clinical trials are less likely to participate in clinical trials (P = .047).Popularization of knowledge about clinical trial is helpful to improve clinical trial participation in Chinese lymphoma patients.

Immunomodulatory treatments for persistent and chronic immune thrombocytopenic purpura: A PRISMA-compliant systematic review and meta-analysis of 28 studies.

Corticosteroid sparing is required in 15% to 40% of adults with persistent or chronic primary immune thrombocytopenic purpura (ITP). Herein, the efficacy of immunomodulatory drugs (dapsone, interferon alpha, danazol, and hydroxychloroquine as second-third-line therapies in ITP is investigated.

Intestinal obstruction due to kaposiform hemangioendothelioma in a 1-month-old infant: A case report.

Kaposiform hemangioendothelioma (KHE) is an aggressive vascular tumor, mainly occurring in infants and young children and previously reported cases were mainly cutaneous or visceral form. Intestinal kaposiform hemangioma was first reported in 2012. Intestinal type KHE showed better prognosis if the lesion was limited in the gastrointestinal tract and coagulopathy was not accompanied. Since the number of reported cases is small, further study for treatment options and prognosis need to be done.

Factor V congenital deficiency: about a case.

Factor V congenital deficiency is a rare coagulation disorder initially described by Owren in 1947 and known as para hemophilia. It is transmitted through autosomal-recessive inheritance and homozygous cases are usually symptomatic. Factor V is an essential cofactor in the conversion of prothrombin to thrombin by activated factor X. In the absence of factor V, thrombin generation is slowed down and fibrin formation is delayed. This results in a bleeding tendency. We report a case of factor V congenital deficiency in an infant with recurrent epistaxis.

Epidemiological profile of hemoglobinopathies: a cross-sectional and descriptive index case study.

Hemoglobinopathies are congenital disorders resultimg from hemoglobin abnormalities. Major forms are often severe, their management is difficult and associated with a great psychosocial impact on patients and their families. They are classified as rare diseases and are still insufficiently known by health professionals. This lack of knowledge is at the origin of diagnostic errors, delay in their management and therefore high morbidity and mortality rate for these patients. In 2008, the World Health Organization (WHO) has published data on hemoglobinopathies epidemiology: more than 330.000 cases of hemoglobinopathy occur each year (83% of cases of sickle cell anemia, 17 % of cases of thalassemia). Hemoglobin disorders are responsible for approximately 3.4% of deaths among people under the age of 5. At the global level, approximately 7% of pregnant women would be carriers of a form of thalassemia and 1% of couples are at risk. However, they are relatively frequent in some regions of the globe where consanguineous marriages are common. We conducted a descriptive cross-sectional study based on two surveys, the first in May 2015 and the second in June of the same year. It was performed in the immunization days to deliver pneumococcal vaccine to the index cases and it was aimed to describe the epidemiological features of families at risk of hemoglobinopathies (index case study), whose index cases were treated in the Department of Pediatrics at the Provincial Hospital El Idrisi, Kenitra, Morocco. After having collected the epidemiological data from patients, laboratory tests were performed including: blood count with red blood cells morphological assessment using the MGG assay and automatic numbering of reticulocytes; hemoglobin electrophoresis at alkaline pH (8.8) and then at acid pH (5.4) on agarose gel and densitometric integration. 275 patients had laboratory profiles compatible with hemoglobinopathy. The majority of these patients were born to consanguineous marriages (83.1%) and came from the north regions of Morocco. This family survey allowed to identify families at risk with a high frequency of sickle cell anemia. Our results confirm the existence of hemoglobinopathies variants among Moroccan population.

Von Willebrand's disease: case report and review of literature.

Von Willebrand Disease (VWD) is the most common human inherited bleeding disorder due to a defect of Von Willebrand Factor (VWF), which a glycoprotein crucial for platelet adhesion to the subendothelium after vascular injury. VWD include quantitative defects of VWF, either partial (type 1 with VWF levels < 50 IU/dL) or virtually total (type 3 with undetectable VWF levels) and also qualitative defects of VWF (type 2 variants with discrepant antigenic and functional VWF levels). The most bleeding forms of VWD usually do not concern type 1 patients with the mildest VWF defects (VWF levels between 30 and 50IU/dL). Von willebrand factor is a complex multimeric protein with two functions: it forms a bridge between the platelets and areas of vascular damage and it binds to and stabilizes factor VIII, which is necessary for the clotting cascade. By taking a clinical history of bleeding (mucocutaneous bleeding symptoms suggestive of a primary haemostatic disorder, a quantitative or qualitative abnormality of VWF is possible) it is important to think about VWD and to make the appropriate diagnosis. If the VWD is suspected diagnostic tests should include an activated partial thromboplastin time, bleeding time, factor VIII: C Ristocetin cofactor and vWF antigen. Additional testing of ristocetin induced plattlet adhesion (RIPA) the multimeric structure and collagen binding test and genanalysis allow diagnosing the different types of von. Willebrand Disease. The treatment of choice in mild forms is the synthetic agent desmopressin. In patients with severe type 1, type 2B, 2N and type 3 or in people who do not response to desmopressin, the appropriate treatment is a factor VIII concentrate that is rich of VWF. We report a case of infant in 27-month-old boy who had been referred due to haemorrhagic shock. His birth histories, his familie's social history and developmental milestones were unremarkable. He was born at full term with no antenatal or perinatal complications. Prior to the symptoms, the child was on a normal diet and was thriving appropriately. The child presented one days before his admission trauma to the inner face of the lower lip that caused an external acute bleeding loss. The laboratory data showed unfortunately, the most severe form of Von Willebrand's Disease; Type 3. The management was based on erythrocyte and fresh-frozen plasma (FFP) transfusions with administration of factor VII with good evolution.

Kikuchi-Fujimoto disease: about a case.

Kikuchi-Fujimoto disease or histiocytic necrotizing lymphadenitis is a rare cause of benign cervical adenopathies. It is an anatomoclinic entity of unknown cause. Diagnosis is based on histologic examination of the lymph nodes. Patient's evolution is generally favorable with spontaneous healing after a few weeks. We here report the case of a 9-year old girl presenting with cervical lymphadenopathy associated with fever. Cervical lymph node biopsy showed Kikuchi-Fujimoto disease. Patient's evolution was marked by regression of adenopathies without receiving any treatment.

Angioimmunoblastic T-cell lymphoma and hypereosinophilic syndrome with FIP1L1/PDGFRA fusion gene effectively treated with imatinib: A case report.

Hypereosinophilic syndrome (HES) is a rare disorder characterized by hypereosinophilia and organ damage. Some cases of HES are caused by the FIP1L1/PDGFRA fusion gene and respond to imatinib. FIP1L1/PDGFRA-positive HES occasionally evolves into chronic eosinophilic leukemia or into another form of myeloproliferative neoplasm; however, the development of a malignant lymphoma is very rare. We present a rare case of angioimmunoblastic T-cell lymphoma (AITL) and HES with the FIP1L1/PDGFRA gene rearrangement.

Successful 5-azacytidine treatment of myeloid sarcoma and leukemia cutis associated with myelodysplastic syndrome: A case report and literature review.

Myeloid sarcoma (MS) and leukemia cutis (LC) are extramedullary tumors comprising myeloid blasts. They can occur de novo or concurrently with hematological disorders, usually acute myeloid leukemia (AML). AML chemotherapy is generally the initial therapy for MS and LC, and hematopoietic stem cell transplantation (HSCT) can be considered as additional therapy. However, treatment for older patients who are unable to continue intensive chemotherapy is not currently standardized.

Oral tetrahydrouridine and decitabine for non-cytotoxic epigenetic gene regulation in sickle cell disease: A randomized phase 1 study.

Sickle cell disease (SCD), a congenital hemolytic anemia that exacts terrible global morbidity and mortality, is driven by polymerization of mutated sickle hemoglobin (HbS) in red blood cells (RBCs). Fetal hemoglobin (HbF) interferes with this polymerization, but HbF is epigenetically silenced from infancy onward by DNA methyltransferase 1 (DNMT1).

Connecting the Dots.

Editorial: Widening the Use of the Functional Lumen Imaging Probe to Kids With Eosinophilic Esophagitis: Esophageal Narrowing is not Just an Adult Problem.

The functional lumen imaging probe (FLIP) provides objective measurement of esophageal distensibility, thus offering a unique and potentially valuable assessment in eosinophilic esophagitis (EoE). Menard-Katcher and colleagues report the first study evaluating use of FLIP in pediatric patients that demonstrated a reduction in esophageal distensibility in patients with EoE compared to patients without EoE. An increase in esophageal distensibility was observed with age among pediatric patients without EoE, but not among those with EoE. While this study further supports the promise of esophageal distensibility assessment in EoE, future work remains needed to optimize FLIP utilization in both research and clinical settings.

Cutaneous T-cell lymphoma: 2017 update on diagnosis, risk-stratification, and management.

Cutaneous T-cell lymphomas are a heterogenous group of T-cell lymphoproliferative disorders involving the skin, the majority of which may be classified as Mycosis Fungoides (MF) or Sézary Syndrome (SS).

Comparing the Efficacy of DeVIC Therapy and High-dose Methotrexate Monotherapy with Whole-brain Radiation Therapy for Newly-diagnosed Primary Central Nervous System Lymphoma: A Single Institution Study.

In the current study, we aimed to compare DeVIC (dexamethasone, etoposide, ifosfamide and carboplatin) chemotherapy with high-dose methotrexate (HD-MTX) monotherapy plus whole-brain radiation therapy (WBRT) for newly-diagnosed primary central nervous system lymphoma (PCNSL), in terms of their efficacies and tolerability.

Thrombocytosis Portends Adverse Prognosis in Colorectal Cancer: A Meta-Analysis of 5,619 Patients in 16 Individual Studies.

The current study aimed to determine the prognostic significance of thrombocytosis in patients with colorectal cancer (CRC) by a meta-analysis of the literature.

Significance of Hyperferritinemia in Hospitalized Adults.

Although high ferritin levels are associated with iron overload, it is known that ferritin is also an acute-phase reactant that may be elevated in conditions associated with acute and chronic inflammation. In addition, an elevated ferritin level is a criterion for the diagnosis of hemophagocytic lymphohistiocytosis/macrophage activation syndrome (HLH/MAS). Therefore, the significance of elevated serum ferritin is often unclear. As HLH/MAS is a medical emergency, prompt diagnosis is important to guide appropriate treatment.

Bortezomib-Induced Muscle Toxicity in Multiple Myeloma.

Multiple myeloma (MM) accounts for ∼13% of all hematologic malignancies. Bortezomib treatment is effective in MM, but can be complicated with neurological side effects. We describe a patient with symptomatic MM who had a reversible metabolic myopathy associated with bortezomib administration and pathologically characterized by excessive storage of lipid droplets together with mitochondrial abnormalities. In a single-center prospective study, 14 out of 24 patients with symptomatic MM were treated with bortezomib and, among these, 7 developed muscular signs and/or symptoms. The myopathy was characterized by a proximal muscle weakness involving lower limbs and was an early complication. Complete resolution of muscle weakness occurred after treatment discontinuation. Conversely, none of the patients who received a treatment without bortezomib developed muscular symptoms. Experimental studies demonstrate that in primary human myoblasts bortezomib at low concentrations leads to excessive storage of lipid droplets together with structural mitochondrial abnormalities, recapitulating the pathologic findings observed in patient's muscle. Our data suggest that patients treated with bortezomib should be monitored for muscular signs and/or symptoms and muscle weakness should alert the clinician to the possibility of myopathy. Bortezomib-induced metabolic myopathy is a potentially reversible entity with important implications for management and treatment of patients with MM.

Periorbital ecchymoses and breathlessness.

A 54-year-old man presented at our facility with a 3-month history of exertional breathlessness and purple blotches around his eyes. Examination revealed bilateral periorbital and perioral ecchymosis, purpuric spots along his waist, and waxy papules on his eyelids. In addition, the patient had macroglossia with nodular infiltration and irregular indentations at the lateral margin of his tongue. The patient also had a raised jugular venous pressure and prominent atrial and ventricular waves. Further examination revealed a fourth heart sound over the left ventricular apex, as well as bilateral basal rales. All other systems were normal except for mild hepatomegaly. Routine hematologic and biochemical lab work was unremarkable. X-rays of the spine and skull were normal, but a chest x-ray showed mild cardiomegaly. An electrocardiogram showed a QS complex from leads V1 to V4 (a pseudo-infarction pattern). An echocardiogram showed biatrial enlargement, left ventricular hypertrophy with a left ventricular ejection fraction of 48%, a speckled pattern on the myocardium, a thickened interatrial septum, and mild pericardial effusion. A color Doppler revealed mild mitral and tricuspid regurgitation with a restrictive pattern of mitral valve flow. Serum protein electrophoresis was normal. WHAT IS YOUR DIAGNOSIS? HOW WOULD YOU TREAT THIS PATIENT?

Myeloproliferative Neoplasms.

Model-based meta-analysis of progression-free survival in non-Hodgkin lymphoma patients.

Non-Hodgkin lymphoma (NHL) is a group of lymphoproliferative malignancies with varying treatment responses and progression-free survival (PFS) times. The objective of this study was to quantify the effect of treatment and patient-population characteristics on PFS in patients with NHL.

Langerhans cell histiocytosis of the thyroid complicated by papillary thyroid carcinoma: A case report and brief literature review.

Langerhans cell histiocytosis (LCH) involves mainly the skin and bone and rarely the thyroid. Meanwhile, papillary thyroid carcinoma (PTC) is the most common subtype of thyroid cancer. Both LCH and PTC could make the thyroid enlarged and hypermetabolic. The coincidence of these 2 events in a patient is rare, and this paper aimed to report such case.

Exacerbation of primary intestinal lymphangiectasia during late pregnancy and recovery after delivery: A case report and literature review.

Primary intestinal lymphangiectasia (PIL) is a rare disease characterized by dilated intestinal lacteals resulting in lymph leakage into the small bowel lumen. Main clinical features include intermittent diarrhea, hypoproteinemia. Scattered case reports suggested that PIL is compatible to pregnancy, but with increased complications.

Oral and maxillofacial non-Hodgkin lymphomas: Case report with review of literature.

Lymphomas take up about 14% of all head-neck malignancies, out of which 97% are non-Hodgkin lymphomas (NHL). The clinical courses, treatment responses, and prognoses of NHLs vary with different subtypes and anatomic sites. In the Chinese population (including the Taiwanese), head-neck NHLs are often seen with the tonsils, nasal cavity, nasal sinus, and the nasopharynx. However, oral NHLs are relatively rare. Delay of diagnosis is also often seen in clinical practice. Thus, we present 4 cases with delayed diagnosis of oral maxillofacial NHLs and discuss their clinical manifestations so as to draw a clue that can remind the doctors to take biopsies in time.

Myeloproliferative Neoplasms.

Myeloproliferative Neoplasms.

Eosinophilic Bronchitis.

An Aγ-globin G->A gene polymorphism associated with β(0)39 thalassemia globin gene and high fetal hemoglobin production.

Increase of the expression of γ-globin gene and high production of fetal hemoglobin (HbF) in β-thalassemia patients is widely accepted as associated with a milder or even asymptomatic disease. The search for HbF-associated polymorphisms (such as the XmnI, BCL11A and MYB polymorphisms) has recently gained great attention, in order to stratify β-thalassemia patients with respect to expectancy of the first transfusion, need for annual intake of blood, response to HbF inducers (the most studied of which is hydroxyurea).

Multiple Hemorrhagic Skin Lesions in a 52-Year-Old Patient with Liver Cirrhosis.