OBJECTIVE: To explore the effect of Qinghuang Powder (QHP,()combined with Bupi Yishen Decoction (BPYS, ) on myelodysplastic syndromes (MDS) patients with refractory cytopenia with multilineage dysplasia (RCMD) and determine the change of DNA methylation in MDS-RCMD patients after the treatment of Chinese medicine formula. METHODS: All 308 MDS-RCMD patients were treated with QHP combined with BPYS for 2 months at least, absolute neutrophil count (ANC), hemoglobin (Hb), platelets (PLT), primitive bone marrow cells and chromosome karyotype were chosen as the main evaluation indexes to analyze the treatment effect according to criteria from the MDS International Working Group. Then 43 bone marrow samples from 15 MDS-RCMD patients and 28 healthy donors were obtained for the examination of DNA methylation. Gene Ontology (GO) and Pathway analysis were applied to analyze the methylation data. RESULTS: The overall MDS response rate to QHP was 61.68% (190/360) including hematologic improvement-neutrophil (HI-N) or hematologic improvement-erythroid (HI-E) or hematologic improvement-platelet (HI-P). Patients with anemia had a better response rate than patients with neutropenia or thrombocypenia (55.88% vs 31.54% or 55.88% vs. 36.9%). The DNA methylation microarray analysis disclosed that 4,257 hypermethylated genes were demethylated upon the treatment with QHP and BPYS. GO analysis and Pathway analysis showed that these demethylated genes were involved in a lot of tumor-related pathways and functions. CONCLUSIONS QHP combined with BPYS could effectively treat MDS-RCMD patients through hematologic improvement (HI-N, HI-P or HI-E) and PLT and RBC transfusion independence due to the demethylation, thereby providing another choice for the treatment of patients with MDS-RCMD.
Arsenicals ; administration & dosage ; pharmacology ; therapeutic use ; Cell Lineage ; drug effects ; DNA Methylation ; drug effects ; Demethylation ; Drugs, Chinese Herbal ; administration & dosage ; pharmacology ; therapeutic use ; Female ; Gene Ontology ; Humans ; Leukocyte Disorders ; drug therapy ; genetics ; Male ; Middle Aged ; Powders ; Treatment Outcome

Hyperleukocytosis (HL), defined by a peripheral white blood cell (WBC) count exceeding 100,000/mm³, is occasionally observed in childhood acute leukemia. The increased viscosity in the micro-circulation by HL and the interaction between the leukemic blasts and endometrium of blood vessels sometimes result in leukostasis. Leukostasis can incur life-threatening manifestations, such as respiratory distress, brain infarction and hemorrhage, and renal failure, needing an emergency care. Although early stage of leukostasis is difficult to detect due to nonspecific manifestations, an emergency care is mandatory because leukostasis can proceed to a fatal course. Initial management includes an aggressive fluid therapy that can reduce WBC count, and prevent other metabolic complications implicated by HL. Packed red blood cells should be judiciously transfused because it increases blood viscosity. Conversely, transfusion of platelet concentrates or fresh frozen plasma, which does not affect blood viscosity, is recommended for prevention of hemorrhage. To reduce tumor burden, leukapheresis or exchange transfusion is commonly performed. However, the efficacy is still controversial, and technical problems are present. Leukapheresis or exchange transfusion is recommended if WBC count is 200,000–300,000/mm³ or more, especially in acute myelocytic leukemia, or manifestations of leukostasis are present. In addition, early chemotherapy is the definite treatment of leukostasis.
Blood Platelets ; Blood Vessels ; Blood Viscosity ; Brain Infarction ; Disease Management ; Drug Therapy ; Emergencies ; Emergency Medical Services ; Emergency Service, Hospital ; Endometrium ; Erythrocytes ; Female ; Fluid Therapy ; Hemorrhage ; Leukapheresis ; Leukemia ; Leukemia, Myeloid, Acute ; Leukocyte Disorders ; Leukocytes ; Leukocytosis ; Leukostasis ; Plasma ; Renal Insufficiency ; Tumor Burden ; Viscosity

BACKGROUND: Fever is a very common complication that has been related to poor outcomes after aneurysmal subarachnoid hemorrhage (aSAH). The incidence of acalculous cholecystitis is reportedly 0.5%–5% in critically ill patients, and cerebrovascular disease is a risk factor for acute cholecystitis (AC). However, abdominal evaluations are not typically performed for febrile patients who have recently undergone aSAH surgeries. In this study, we discuss our experiences with febrile aSAH patients who were eventually diagnosed with AC. METHODS: We retrospectively reviewed 192 consecutive patients who underwent aSAH from January 2009 to December 2012. We evaluated their characteristics, vital signs, laboratory findings, radiologic images, and pathological data from hospitalization. We defined fever as a body temperature of >38.3℃, according to the Society of Critical Care Medicine guidelines. We categorized the causes of fever and compared them between patients with and without AC. RESULTS: Of the 192 enrolled patients, two had a history of cholecystectomy, and eight (4.2%) were eventually diagnosed with AC. Among them, six patients had undergone laparoscopic cholecystectomy. In their pathological findings, two patients showed findings consistent with coexistent chronic cholecystitis, and two showed necrotic changes to the gall bladder. Patients with AC tended to have higher white blood cell counts, aspartame aminotransferase levels, and C-reactive protein levels than patients with fevers from other causes. Predictors of AC in the aSAH group were diabetes mellitus (odds ratio [OR], 8.758; P = 0.033) and the initial consecutive fasting time (OR, 1.325; P = 0.024). CONCLUSIONS: AC may cause fever in patients with aSAH. When patients with aSAH have a fever, diabetes mellitus and a long fasting time, AC should be suspected. A high degree of suspicion and a thorough abdominal examination of febrile aSAH patients allow for prompt diagnosis and treatment of this condition. Additionally, physicians should attempt to decrease the fasting time in aSAH patients.
Acalculous Cholecystitis ; Aneurysm* ; Aspartame ; Body Temperature ; C-Reactive Protein ; Cerebrovascular Disorders ; Cholecystectomy ; Cholecystectomy, Laparoscopic ; Cholecystitis ; Cholecystitis, Acute* ; Critical Care ; Critical Illness ; Diabetes Mellitus ; Diagnosis ; Fasting ; Fever* ; Hospitalization ; Humans ; Incidence ; Intensive Care Units ; Leukocyte Count ; Prognosis ; Retrospective Studies ; Risk Factors ; Subarachnoid Hemorrhage* ; Urinary Bladder ; Vital Signs

Brain abscess commonly occurs secondary to an adjacent infection (mostly in the middle ear or paranasal sinuses) or due to hematogenous spread from a distant infection or trauma. Pulmonary arteriovenous fistulas (AVFs) are abnormal direct communications between the pulmonary artery and vein. We present two cases of brain abscess associated with asymptomatic pulmonary AVF. A 65-year-old woman was admitted with a headache and cognitive impairment that aggravated 10 days prior. An magnetic resonance (MR) imaging revealed a brain abscess with severe edema in the right frontal lobe. We performed a craniotomy and abscess removal. Bacteriological culture proved negative. Her chest computed tomography (CT) showed multiple AVFs. Therapeutic embolization of multiple pulmonary AVFs was performed and antibiotics were administered for 8 weeks. A 45-year-old woman presented with a 7-day history of progressive left hemiparesis. She had no remarkable past medical history or family history. On admission, blood examination showed a white blood cell count of 6290 cells/uL and a high sensitive C-reactive protein of 2.62 mg/L. CT and MR imaging with MR spectroscopy revealed an enhancing lesion involving the right motor and sensory cortex with marked perilesional edema that suggested a brain abscess. A chest CT revealed a pulmonary AVF in the right upper lung. The pulmonary AVF was obliterated with embolization. There needs to consider pulmonary AVF as an etiology of cerebral abscess when routine investigations fail to detect a source.
Abscess ; Aged ; Anti-Bacterial Agents ; Arteriovenous Fistula* ; Arteriovenous Malformations ; Brain Abscess* ; Brain* ; C-Reactive Protein ; Cognition Disorders ; Craniotomy ; Ear, Middle ; Edema ; Embolization, Therapeutic ; Female ; Frontal Lobe ; Headache ; Humans ; Leukocyte Count ; Lung ; Magnetic Resonance Imaging ; Magnetic Resonance Spectroscopy ; Middle Aged ; Paresis ; Pulmonary Artery ; Thorax ; Tomography, X-Ray Computed ; Veins

BACKGROUND: Fever is a very common complication that has been related to poor outcomes after aneurysmal subarachnoid hemorrhage (aSAH). The incidence of acalculous cholecystitis is reportedly 0.5%–5% in critically ill patients, and cerebrovascular disease is a risk factor for acute cholecystitis (AC). However, abdominal evaluations are not typically performed for febrile patients who have recently undergone aSAH surgeries. In this study, we discuss our experiences with febrile aSAH patients who were eventually diagnosed with AC. METHODS: We retrospectively reviewed 192 consecutive patients who underwent aSAH from January 2009 to December 2012. We evaluated their characteristics, vital signs, laboratory findings, radiologic images, and pathological data from hospitalization. We defined fever as a body temperature of >38.3℃, according to the Society of Critical Care Medicine guidelines. We categorized the causes of fever and compared them between patients with and without AC. RESULTS: Of the 192 enrolled patients, two had a history of cholecystectomy, and eight (4.2%) were eventually diagnosed with AC. Among them, six patients had undergone laparoscopic cholecystectomy. In their pathological findings, two patients showed findings consistent with coexistent chronic cholecystitis, and two showed necrotic changes to the gall bladder. Patients with AC tended to have higher white blood cell counts, aspartame aminotransferase levels, and C-reactive protein levels than patients with fevers from other causes. Predictors of AC in the aSAH group were diabetes mellitus (odds ratio [OR], 8.758; P = 0.033) and the initial consecutive fasting time (OR, 1.325; P = 0.024). CONCLUSIONS: AC may cause fever in patients with aSAH. When patients with aSAH have a fever, diabetes mellitus and a long fasting time, AC should be suspected. A high degree of suspicion and a thorough abdominal examination of febrile aSAH patients allow for prompt diagnosis and treatment of this condition. Additionally, physicians should attempt to decrease the fasting time in aSAH patients.
Acalculous Cholecystitis ; Aneurysm ; Aspartame ; Body Temperature ; C-Reactive Protein ; Cerebrovascular Disorders ; Cholecystectomy ; Cholecystectomy, Laparoscopic ; Cholecystitis ; Cholecystitis, Acute ; Critical Care ; Critical Illness ; Diabetes Mellitus ; Diagnosis ; Fasting ; Fever ; Hospitalization ; Humans ; Incidence ; Intensive Care Units ; Leukocyte Count ; Prognosis ; Retrospective Studies ; Risk Factors ; Subarachnoid Hemorrhage ; Urinary Bladder ; Vital Signs

OBJECTIVETo explore the clinical features, laboratory findings and treatment of infant leukemia.

METHODSA retrospective analysis of the clinical data was performed of the cases with the diagnosis of infant acute leukemia from August 1993 to October 2014 in our hospital.

RESULTSA total of 144 cases of infant leukemia were diagnosed in the defined period, including 83 cases of acute lymphoblastic leukemia, 55 myeloid leukemia, 1 hybrid acute leukaemia and 5 with incompatible cytological and immunophenotyping findings. The patients at the age of 9 to 12 months accounted for the largest proportion (38.2%), and 87.5% of the patients had hepatosplenomegaly; Six patients below 6 months old had skin infiltration. In about 1/3 of the patients, the white blood cells count was no greater than 100 × 10⁹ /L. Ninety-five patients had chromosome examinations, which identified chromosome abnormalities in 67 patients, including 18 positive for t(4;11)or t(9;11)or t(11;19), and younger patients were more likely to have chromosome abnormalities. Thirty-seven patients underwent MLL gene detection and 11 of them had positive results; the positive patients had higher rate of chromosome 11 abnormalities than the negative patients. Most of the patients gave up treatments after diagnosis and only 6 patients older than 6 months completed regular chemotherapeutic treatments and were now in complete remission.

CONCLUSIONInfant leukemia is a rare type of leukemia with different clinical features from other types of leukemia. The patients often present with hepatosplenomegaly, high white blood cell counts, MLL gene fusion, and chromosome 11 abnormalities. The prognosis of infant leukemia is not favorable, and the current treatment still relies on chemotherapy.

Acute Disease ; Chromosome Aberrations ; Chromosome Disorders ; Chromosomes, Human, Pair 11 ; Humans ; Immunophenotyping ; Infant ; Leukemia, Myeloid ; pathology ; Leukocyte Count ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; pathology ; Prognosis ; Retrospective Studies

OBJECTIVETo detect JAK2 V617F mutation burden and its clinical implications in patients with myeloproliferative neoplasm (MPN).

METHODSJAK2 V617F mutation burden were detected by using MGB Taqman probes and its clinical significance were retrospectively studied in 415 MPN patients.

RESULTSJAK2 V617F was found in 56.9% of all patients [83.5% in polycythemia vera (PV), 55.9% in essential thrombocythemia (ET), 41.9% in primary myelofibrosis (PMF) and 64.7% in MPN-unclassifiable)]. The majority of patients carried heterozygous JAK2 V617F mutation and homozygote was found only in 12 cases (4 in PV, 4 in MPN-U, 2 in PMF, 1 in ET, and 1 in chronic neutrophilic leukemia). Most patients (68.8%) were lower mutation burden (mutation burden<50%), but PV had the highest burden, the moderate burden in PMF and the least in ET. The patient's age and WBC count were significantly correlated with higher mutation burden in PV. WBC count was significantly related to higher mutation burden in ET. WBC count, Hb level and the platelet count were significantly related to higher mutation burden in PMF.

CONCLUSIONThe mutation burden of JAK2 V617F from high to low was PV, ET and PMF. The majority of JAK2 V617F mutation was heterozygous. JAK2 V617F mutation burden was positively correlated with age, WBC, Hb and platelet counts.

Homozygote ; Humans ; Janus Kinase 2 ; Leukocyte Count ; Mutation ; Myeloproliferative Disorders ; Platelet Count ; Polycythemia Vera ; Retrospective Studies ; Thrombocythemia, Essential

No abstract available.
Adult ; Bone Marrow/pathology ; DNA Mutational Analysis ; Humans ; Leukemia, Myelomonocytic, Chronic/*diagnosis/genetics/therapy ; Leukemia, Neutrophilic, Chronic/*diagnosis/genetics/therapy ; Leukocyte Disorders/diagnosis ; Male ; Middle Aged ; Mutation ; Prognosis ; Receptors, Colony-Stimulating Factor/*genetics ; Recurrence ; *Stem Cell Transplantation ; Transplantation, Homologous

BACKGROUND: Calreticulin (CALR) mutations were recently discovered in patients with myeloproliferative neoplasms (MPNs). We studied the frequency and type of CALR mutations and their hematological characteristics. METHODS: A total of 168 MPN patients (36 polycythemia vera [PV], 114 essential thrombocythemia [ET], and 18 primary myelofibrosis [PMF] cases) were included in the study. CALR mutation was analyzed by the direct sequencing method. RESULTS: CALR mutations were detected in 21.9% of ET and 16.7% of PMF patients, which accounted for 58.5% and 33.3% of ET and PMF patients without Janus kinase 2 (JAK2) or myeloproliferative leukemia virus oncogenes (MPL) mutations, respectively. A total of five types of mutation were detected, among which, L367fs*46 (53.6%) and K385fs*47 (35.7%) were found to be the most common. ET patients with CALR mutation had lower leukocyte counts and ages compared with JAK2-mutated ET patients. CONCLUSION: Genotyping for CALR could be a useful diagnostic tool for JAK2-or MPL-negative ET or PMF patients. CALR mutation may be a distinct disease group, with different hematological characteristics than that of JAK2-positive patients.
Adolescent ; Adult ; Aged ; Aged, 80 and over ; Amino Acid Sequence ; Base Sequence ; Calreticulin/*genetics ; DNA Mutational Analysis ; Exons ; Female ; Humans ; Janus Kinase 2/genetics ; Leukocyte Count ; Male ; Middle Aged ; Molecular Sequence Data ; Mutation ; Myeloproliferative Disorders/diagnosis/*genetics/pathology ; Receptors, Thrombopoietin/genetics ; Young Adult

PURPOSE: The purpose of this study is to verify the influence of a massive hydrofluoric acid spill on community health through patients who claimed to have been exposed. METHODS: We analyzed 2459 patients who visited our emergency department with the claim of exposure to hydrofluoric acid, and retrospective analyses were performed. We analyzed changes in numbers of visitors per day from the day of the accidental hydrofluoric acid spill, symptoms presented by the 1924 patients, and general characteristics. Comparisons of symptoms and hematologic characteristics were made between the initially set evacuation zone(1.3 km radius parameters from the spill) and the outer zone. RESULTS: A total of 2,459 patients who claimed exposure visited our ED from 27 September 2012 to 23 October 2012, and there was a significant increase in the number of visiting patients from day 8 of the hydrofluoric acid spill. The most common complaints were a sore throat, 729(37.9%) and no specific symptom with health concern, 547(28.4%). Statistically significant findings were pulmonary symptoms (p=0.001), nasal symptoms (p=0.001), diarrhea (p=0.023), and skin symptoms (p=0.007). In hematologic study, a statistically significant difference was observed in white blood cell count (p=0.018), creatine phosphokinase (p<0.001), erythrocyte sediment rate (p=0.013), and phosphorus (p<0.001). CONCLUSION: A significant increase in the number of patients was observed one week after the accidental spill of hydrofluoric acid. The most frequent symptoms were sore throat, headache, cough, and sputum. Statistically significant increase in creatine phosphokinase level and decrease in phosphorus level were noted in patients within the evacuation zone.
Creatine Kinase ; Diarrhea ; Emergencies ; Erythrocytes ; Headache Disorders, Primary ; Humans ; Hydrofluoric Acid* ; Leukocyte Count ; Pharyngitis ; Phosphorus ; Radius ; Retrospective Studies ; Skin ; Sputum