Primary myelofibrosis (PMF) is easily confused with cirrhosis, due to its main clinical manifestations of splenomegaly and the blood cytopenia. This review focuses on clinical studies to identify primary myelofibrosis and cirrhosis related portal hypertension, to analyze the differences between the two diseases, in order to distinguish PMF and cirrhosis from the pathogenesis, clinical manifestations, laboratory examinations and treatment principles, and simultaneously improve clinicians' understanding of PMF, which is a reference for exploring the early screening or diagnostic indicators of PMF, also provides a clinical basis for the application of new targeted drugs such as ruxolitinib.
Humans ; Primary Myelofibrosis/drug therapy* ; Hypertension, Portal/complications* ; Liver Cirrhosis/pathology* ; Splenomegaly/pathology* ; Anemia

Actinin/genetics* ; Anemia ; Blood Platelets/pathology* ; Female ; Humans ; Male ; Megakaryocytes ; Mutation, Missense ; Pedigree ; Thrombocytopenia/genetics*

Anemia of chronic disease (ACD), complicated by various chronic inflammatory diseases, is the second most prevalent type of anemia after iron deficiency anemia in the world. ACD significantly reduces the life quality of patients with chronic diseases, and represents an independent poor prognostic factor in certain chronic diseases. A large body of studies has demonstrated that most of anemia is related to abnormal iron metabolism. In the past decade, hepcidin, as a key factor in regulating iron metabolism, has attracted enormous attention due to its important role in the pathogenesis of ACD. This article reviews the research progress on the role and underlying regulatory mechanisms of hepcidin in ACD. We also discuss the potential of hepcidin as an effective therapeutic target for ACD treatment, in order to provide a new maneuver for improving the quality of ACD patients' life.
Anemia ; Anemia, Iron-Deficiency/pathology* ; Chronic Disease ; Hepcidins ; Humans ; Iron/metabolism*

Objective To explore the clinical and laboratory characteristics and the prognosis of disseminated non-tuberculous mycobacteria(NTM)diseases in human immunodeficiency virus(HIV)negative patients. Methods Cases of disseminated NTM disease were retrospectively collected in Peking Union Medical College Hospital from January 2012 to October 2018.Clinical manifestations,laboratory findings,treatment,and prognosis of these cases were retrieved from the electronic medical record system. Results Among the 23 HIV negative patients with disseminated NTM disease,21 had underlying diseases,with rheumatoid immune disease(n=7)as the most common one.The main clinical manifestation was fever(n=23).Laboratory tests showed anemia [hemoglobin(85.78±25.47)g/L],hypoalbuminemia [albumin 29(27-32)g/L],elevated erythrocyte sedimentation rate [(85.73±43.78)mm/h] and hypersensitive C-reactive protein [(112.00±70.90)mg/L],and reduction of lymphocyte count [0.69(0.29-2.10)×10 /L].Lymphocyte subset analysis indicated reduction in CD4 T cells [213(113-775)/μl],CD8 T cells [267(99-457)/μl],B cells [39(4-165)/μl],and NK cells [88(32-279)/μl] and elevation of human leukocyte antigen-D related(HLA-DR),and CD38 expression in CD8 T cells [HLA-DR CD8 /CD8 ,60(40-68)%;CD38 CD8 /CD8 ,81(65-90)%].The most common species of NTM was Mycobacterium intracellular(n=6).Lymphocyte,CD8 T cell,B cell,and NK cell counts were significantly lower in dead patients than surviving patients(P =0.045,P=0.045,P=0.032,and P=0.010,respectively). Conclusions Disseminated NTM disease in HIV negative patients is mainly manifested as fever,anemia,hypoalbuminemia,and elevated inflammatory indicators.It is more likely to occur in immunocompromised patients.Patients with decreased lymphocytes,CD8 T cells,B cells and NK cells tend to have a poor prognosis.
Anemia ; B-Lymphocytes ; CD4-Positive T-Lymphocytes ; CD8-Positive T-Lymphocytes ; Fever ; HIV Seronegativity ; Humans ; Hypoalbuminemia ; Killer Cells, Natural ; Mycobacterium Infections, Nontuberculous ; diagnosis ; pathology ; Prognosis ; Retrospective Studies

Renal anemia, mainly caused by the deficiencies of erythropoietin (EPO) and iron metabolism disorder, is one of the most common complications of chronic kidney disease. Hypoxia-inducible factor (HIF) is a class of transcription factors responsible for maintaining homeostasis during oxygen deprivation. In normoxia, HIF is degraded by prolyl hydroxylase (PHD). While under hypoxic conditions, the hydroxylation activity of PHD is inhibited, and the cellular concentration of HIF is elevated, resulting in an increase in endogenous EPO production and iron absorption. Therefore, this regulating pathway, also termed as the HIF-PHD axis, has become a promising therapeutic target of treating renal anemia. Several innovative drugs acting as selective HIF-PHD inhibitors have been successfully developed in the past years, and some of them are undergoing clinical trials. In this review, we will introduce the definition and regulatory mechanism of HIF-PHD axis, as well as current insights into its physiologic and therapeutic role in renal anemia.
Anemia ; enzymology ; pathology ; Humans ; Hypoxia ; pathology ; Hypoxia-Inducible Factor 1 ; metabolism ; Kidney Diseases ; enzymology ; pathology ; Oxygen ; Prolyl Hydroxylases ; metabolism

OBJECTIVE: To report on the incidence of nab-paclitaxel hypersensitivity reactions (HSRs) in patients with prior taxane HSR. METHODS: From 2005 to 2015, all patients who received nab-paclitaxel for a gynecologic malignancy were identified. Chart abstraction included pathology, prior therapy, indication for nab-paclitaxel, dosing, response, toxicities including any HSR, and reason for discontinuation of nab-paclitaxel therapy. RESULTS: We identified 37 patients with gynecologic malignancies with a history of paclitaxel HSR who received nab-paclitaxel. Six patients (16.2%) had a prior HSR to both paclitaxel and docetaxel while the other 31 patients had not received docetaxel. No patients experienced a HSR to nab-paclitaxel. Median number of cycles of nab-paclitaxel was 6 (range 2–20). Twelve patients received weekly dosing at 60 to 100 mg/m². The remainder of patients received 135 mg/m² (n=13), 175 mg/m² (n=9), or 225 mg/m² (n=3). Thirty four patients (91.9%) received nab-paclitaxel in combination with carboplatin (n=28, 75.7%), IP cisplatin (n=1, 2.7%), carboplatin and bevacizumab (n=3, 8.1%), or carboplatin and gemcitabine (n=2, 5.4%). Reasons for discontinuing nab-paclitaxel included completion of adjuvant therapy (n=16), progressive disease (n=18), toxicity (n=1), and death (n=1). There were no grade 4 complications identified during nab-paclitaxel administration. Grade 3 complications included: neutropenia (n=9), thrombocytopenia (n=4), anemia (n=1), and neurotoxicity (n=1). CONCLUSION: Nab-paclitaxel is well-tolerated with no HSRs observed in this series of patients with prior taxane HSR. Given the important role of taxane therapy in nearly all gynecologic malignancies, administration of nab-paclitaxel should be considered prior to abandoning taxane therapy.
Albumin-Bound Paclitaxel ; Anemia ; Bevacizumab ; Carboplatin ; Cisplatin ; Drug Hypersensitivity ; Drug Therapy ; Humans ; Hypersensitivity* ; Incidence ; Neutropenia ; Paclitaxel ; Pathology ; Thrombocytopenia

BACKGROUND/AIMS: Videocapsule endoscopy (VCE) and double-balloon endoscopy (DBE) allow deep exploration in patients with suspected small bowel pathology. VCE is often performed as an initial small bowel examination to explore whether an intervention by DBE is indicated and to determine insertion route. The study aim was to evaluate the correlation between DBE and VCE in patients with obscure or overt bleeding or anemia, as well as intervention frequency, and complications. METHODS: Retrospective observational study. RESULTS: DBE procedures (n=205) showed small bowel lesions in 64% cases. Antegrade DBE showed positive results in 79% cases, mostly angiodysplasias (63%). Retrograde DBE showed positive results in 22% cases. An intervention was performed in 64% of DBE procedures. The major complication rate was 0.5%, which was one case of perforation. Pancreatitis did not occur. The overall diagnostic agreement was 66% among the 134 DBEs with preceded VCE. CONCLUSIONS: In cases of overt or occult bleeding or anemia, DBE was positive in 64%, with only a few complications. Positive correlation was 66% among initially performed VCEs and DBEs. Owing to the time-consuming and invasive character of DBE, performing VCE before DBE might still be clinically relevant.
Anemia ; Angiodysplasia ; Capsule Endoscopy ; Endoscopy* ; Hemorrhage* ; Humans ; Intestine, Small ; Observational Study ; Pancreatitis ; Pathology ; Retrospective Studies ; Tertiary Care Centers*

OBJECTIVETo analyze the clinical manifestations and laboratory features of patients with T large granular lymphocytic leukemia (T-LGLL), so as to improve the understanding of this disease.

METHODSThe clinical data of 10 patients with T-LGLL in General Hospital of Chinese PLA from October 2015 to March 2010 were analyzed retrospectively.

RESULTSTheir median age at diagnosis was 51 years old. 9/10 (90%) patients showed symptoms of anemia, with a median Hb level of 82.5 g/L, 5/10 (50%) patients combined with autoimmune disorders and with a median Hb level of 77 g/L. 7/10 (70%) patients had splenomegaly, 2/10 (20%) patients had complex karyotype, 2/10 (20%) patients had gene mutations, the median age of 4 patients with complex karyotype and gene mutation was 49 years old, all of them suffered from splenomegaly. The immunophenotype of 6/10 patients was CD3+ CD4- CD8+ and that of 2/10 patients (20%) was CD3+ CD4- CD8-, that of another 2/10 (20%) was CD3+ CD4+ CD8-, the clinical features between different types of immunization were not statistically different.

CONCLUSIONT-LGLL patients often are old men, combined with anemia and splenomegaly, often associated with autoimmune diseases; the patients with complex karyotype and gene mutation are younger and they are more with hepatosplenomegaly; the guide role of different immunotypes for clinical strategy is no significant.

Anemia ; pathology ; Autoimmune Diseases ; pathology ; Chromosome Aberrations ; Hemoglobins ; analysis ; Humans ; Immunophenotyping ; Leukemia, Large Granular Lymphocytic ; diagnosis ; pathology ; Middle Aged ; Retrospective Studies ; Spleen ; pathology

BACKGROUND/AIMS: The mortality rate of pyogenic liver abscess (PLA) has decreased dramatically, but it remains a potentially life threatening disease. Most cases are cryptogenic or occur in elderly men with underlying biliary tract disease. Although several studies have addressed the characteristics and etiology of PLA, research on factors affecting PLA-associated mortality is lacking. This study intended to identify the clinical and radiological features, pathogens, complications, and predictors of mortality in Korean PLA patients. METHODS: The medical records of 231 PLA patients diagnosed at Yeungnam University Medical Center between January 2010 and January 2014 were analyzed. A diagnosis of PLA was made based on imaging studies and blood and abscess cultures. The clinical, radiological, and laboratory findings of patients were analyzed. RESULTS: The mean patient age was 64.0±12.9 years and the male to female ratio was 1.5:1. Klebsiella pneumoniae was the predominant organism isolated from hepatic abscesses (69.9%) and blood (74.2%). The most common complication was pleural effusion (35.8%) and most common co-infection was cholangitis (8.2%). The overall mortality rate of PLA was 6.9% (16/231), and was significantly higher in patients with a history of liver abscess (OR 5.970, 95% CI 1.207-29.529; p=0.028), bilirubinemia (>2 mg/dL) (OR 9.541, 95% CI 2.382-38.216; p=0.001), thrombocytopenia (<140×10(3)/µL) (OR 4.396, 95% CI 1.130-17.106; p=0.033), or anemia (<12 g/dL) (OR 13.277, 95% CI 1.476-119.423; p=0.021). CONCLUSIONS: The prognosis of PLA appears to be dependent on underlying pathologies and severity of condition. More aggressive treatment should be considered if a poor prognosis is expected.
Abscess ; Academic Medical Centers ; Aged ; Anemia ; Biliary Tract Diseases ; Cholangitis ; Coinfection ; Diagnosis ; Female ; Humans ; Hyperbilirubinemia ; Klebsiella pneumoniae ; Liver Abscess ; Liver Abscess, Pyogenic* ; Male ; Medical Records ; Mortality* ; Pathology ; Pleural Effusion ; Prognosis ; Retrospective Studies* ; Risk Factors ; Thrombocytopenia

No abstract available.
Adolescent ; Anemia, Aplastic/*diagnosis/pathology ; Bone Marrow/pathology ; Child ; Child, Preschool ; Diagnosis, Differential ; Female ; Half-Life ; Humans ; Immunohistochemistry ; Male ; Mutation ; Myelodysplastic Syndromes/*diagnosis/pathology ; Retrospective Studies ; Tumor Suppressor Protein p53/genetics/*metabolism