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Eur J Intern Med 2020 Jul 1. Epub 2020 Jul 1.

Université Mohamed V, Rabat, Morocco; Hematology Laboratory, Mohammed V Military Teaching Hospital, Rabat, Morocco.

J Cancer Res Clin Oncol 2020 Jul 2. Epub 2020 Jul 2.

Department of Hematology, The First Affiliated Hospital of Guangdong Pharmaceutical University, Guangdong, China.

Purpose: Case reports suggest that ruxolitinib-containing treatment could increase the clinical response rate of patients with hemophagocytic syndrome (HPS). This study aimed to explore the effect of ruxolitinib-containing treatment for patients with lymphoma-associated hemophagocytic syndrome (LAHS).

Methods: This was a retrospective study of patients with LAHS hospitalized at the First Affiliated Hospital of Guangdong Pharmaceutical University between October 2017 and September 2019. Read More

Mol Genet Metab 2020 Jun 24. Epub 2020 Jun 24.

Children's Hospital at Montefiore, Albert Einstein College of Medicine, Bronx, NY, USA.

Background: Acid sphingomyelinase deficiency (ASMD) also known as Niemann-Pick disease, is a rare lysosomal storage disorder with a diverse disease spectrum that includes slowly progressive, chronic visceral (type B) and neurovisceral forms (intermediate type A/B), in addition to infantile, rapidly progressive fatal neurovisceral disease (type A).

Purpose And Methods: We review the published evidence on the relevance of splenomegaly and reduced lung diffusion capacity to the clinical burden of chronic forms of ASMD. Targeted literature searches were conducted to identify relevant ASMD and non-ASMD studies for associations between diffusing capacity of the lungs for carbon monoxide (DL) and splenomegaly, with clinical parameters and outcome measures. Read More

Intest Res 2020 Jul 2. Epub 2020 Jul 2.

Department of Internal Medicine, School of Medicine, Kyungpook National University, Daegu, Korea.

Azathioprine is widely used for the treatment of Crohn's disease (CD). Few cases from Western countries have reported idiopathic non-cirrhotic portal hypertension (NCPH) related to thiopurine therapy in patients with inflammatory bowel disease. Idiopathic NCPH is a rare hepatic condition with intrahepatic portal hypertension but no evidence of cirrhosis or chronic liver disease. Read More

Pediatric Health Med Ther 2020 8;11:161-169. Epub 2020 Jun 8.

Department of Nursing, College of Health Science, Woldia University, Woldia, Ethiopia.

Background: Intestinal parasites (IP) are a major public health problem in several developing countries. It accounts for 1.5 billion infections with one or more intestinal parasitic agents. Read More

Surg Case Rep 2020 Jun 29;6(1):153. Epub 2020 Jun 29.

Department of Surgery, Nagasaki University Graduate School of Biomedical Science, 1-7-1 Sakamoto, Nagasaki, 852-8102, Japan.

Background: Congenital absence of portal vein (CAPV) is a rare structural anomaly in which the portal vein (PV) blood that normally flow into the liver directly drains into the systemic venous system through other collateral circulation. Congenital portal vein shunts (CPSs) is classified into types I and II according to the absence or presence of the intrahepatic portal vein, respectively. The CPS type I is also known as CAPV. Read More

Surgery 2020 Jun 26. Epub 2020 Jun 26.

Department of Medical and Surgical Sciences and Translational Medicine, St Andrea University Hospital, Sapienza University, Rome, Italy.

Background: Pancreatoduodenectomy with synchronous resection of the portal vein/superior mesenteric vein confluence may result in the development of left-sided portal hypertension. Left-sided portal hypertension presents with splenomegaly and varices and may cause severe gastrointestinal bleeding. The aim of the study is to review the incidence, treatment, and preventive strategies of left-sided portal hypertension. Read More

Iran J Parasitol 2020 Apr-Jun;15(2):278-281

Pediatric's Center of Excellence, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran.

Visceral leishmaniasis is a common parasitic disease between humans and animals, transmitted by sandflies () in the Mediterranean countries, including Iran. The statistics have been reported less than real due to errors in the diagnosis and reporting of affected cases. In this report, we will present the symptoms and manifestations of this disease to reduce late detection and exacerbating factors. Read More

Biol Blood Marrow Transplant 2020 Jun 24. Epub 2020 Jun 24.

People Tree Hospitals, Bangalore, India; Sankalp India Foundation, Bangalore, India; Cure2Children Foundation, Florence, Italy.

Severe thalassemia syndromes (ST) are highly curable by BMT but rejection may still occur. We retrospectively analyzed our fully matched related donor transplants to establish if isolated splenomegaly is an independent risk factor for rejection and if this risk can be reduced by modifying the conditioning protocol. In this study we compared rejection rates between patients with and without splenomegaly in 189 consecutive low risk ST transplants across two sequential conditioning regimens: Regimen A (Aug 2013 and Dec 2016) - busulfan (14 mg/kg oral, not adjusted to serum levels), cyclophosphamide (200 mg/kg) and anti-thymocyte globulin (ATG) (Genzyme 4 mg/kg or Fresenius 16 mg/kg on days -12 to -10) and Regimen B - same backbone as Regimen A except Fludarabine total dose of 150 mg was added upfront and ATG dose was increased to 7 mg/kg in case of splenomegaly and/or sex mismatched transplants (Jan 2017 to Sep 2018). Read More

J Pediatr Hematol Oncol 2020 Jun 23. Epub 2020 Jun 23.

Epidemiology Unit, Faculty of Medicine, Prince of Songkla University, Hat Yai, Thailand.

In childhood, Hodgkin disease (HD) has an excellent outcome in developed countries. There are few studies on outcomes of HD from resource-limited countries. This study aimed to assess clinical outcomes and factors associated with survival rates of childhood HD in a tertiary care center in Thailand. Read More

Clin Chem Lab Med 2020 Jun 25. Epub 2020 Jun 25.

Molecular Diagnosis and Rare Diseases Laboratory, Department of Clinical Biochemistry, Hospital Universitario Virgen del Rocío, Seville, Spain.

Objectives Gaucher disease (GD) is the most common inherited lysosomal storage disease, caused by mutations in acid β-glucosidase (GBA) gene. This study aimed to identify mutations in Andalusia patients with GD and their genotype-phenotype correlation. Methods Descriptive observational study. Read More

Hepatol Int 2020 Jun 25. Epub 2020 Jun 25.

Department of Obstetrics& Gynaecology, Jawaharlal Institute of Postgraduate Medical Education & Research, Dhanvantri Nagar, Pondicherry, 605006, India.

Objective: To assess the maternal and perinatal outcomes of pregnant women with non-cirrhotic portal hypertension (NCPH).

Methods: This was an observational study done by retrieving the records of pregnant women with non-cirrhotic portal hypertension admitted to a tertiary hospital in South India, over a 9-year study period. Data regarding the clinical course, complications during pregnancy, labor, and delivery details were reviewed. Read More

Ann Glob Health 2020 Jun 17;86(1):62. Epub 2020 Jun 17.

University of Benin/University of Benin Teaching Hospital, Benin City Nigeria, NG.

Background: Malaria is commonly associated with alteration in haematologic cells of infected individuals in both the acute uncomplicated and severe phases. Whether this alteration occurs in the asymptomatic phase of the disease is still being investigated.

Objectives: To examine the haematocrit, thrombocytes, and monocytes levels of children with asymptomatic malaria compared with age/sex-matched controls who are malaria parasite negative and living in a stable malaria endemic region. Read More

Antibiotics (Basel) 2020 Jun 23;9(6). Epub 2020 Jun 23.

Área Académica de Medicina Veterinaria y Zootecnia, Instituto de Ciencias Agropecuarias, Universidad Autónoma del Estado de Hidalgo, Rancho Universitario Av. Universidad km 1, EX-Hda de Aquetzalpa, 43600 Tulancingo, Hgo, Mexico.

Plants have been used since ancient times to cure certain infectious diseases, and some of them are now standard treatments for several diseases. Due to the side effects and resistance of pathogenic microorganisms to antibiotics and most drugs on the market, a great deal of attention has been paid to extracts and biologically active compounds isolated from plant species used in herbal medicine. is an important perennial shrubby plant that has been widely used for the treatment of several ailments. Read More

J Dig Dis 2020 Jun 25. Epub 2020 Jun 25.

Department of Gastroenterology, Digestive Endoscopy Center, Changhai Hospital, the Second Military Medical University, Shanghai, China.

Aim: Sinistral portal hypertension (SPH) is an uncommon complication of chronic pancreatitis (CP) and can result in severe gastrointestinal bleeding. The aim of this study was to determine the prevalence and the potential risk factors for SPH and related gastrointestinal variceal bleeding in CP patients.

Methods: This is a case-control study. Read More

Vet Radiol Ultrasound 2020 Jun 24. Epub 2020 Jun 24.

Diagnostic and Interventional Radiology Division, San Marco, Veterinary Clinic and Laboratory, Veggiano, Italy.

Extramedullary hematopoiesis (EMH) is the formation and development of blood cells outside the bone marrow, and in dogs it frequently occurs in the spleen. Although splenic EMH is a relatively common condition, data regarding its appearance in veterinary medicine are lacking. Our aim was to describe different multidetector computed tomographic (MDCT) features of splenic EMH in dogs. Read More

World J Surg Oncol 2020 Jun 23;18(1):139. Epub 2020 Jun 23.

Department of General Surgery, Chiba University Graduate School of Medicine, 1-8-1 Inohana, Chuo-Ku, Chiba, 260-8670, Japan.

Background: Conversion chemotherapy may downsize unresectable colorectal liver metastases (CRLMs), but may cause liver injury and splenic enlargement. The effect of preoperative chemotherapy on liver regeneration after liver resection remains undetermined. The aim of this study was to examine whether splenic enlargement induced by preoperative chemotherapy is an indicator to identify high-risk patients for impaired liver regeneration and liver dysfunction after resection. Read More

J Ethnopharmacol 2020 Jun 20:112951. Epub 2020 Jun 20.

Department of Pharmacy, Wuhan No.1 Hospital (Wuhan Hospital of Traditional and Western Medicine), Wuhan, China. Electronic address:

Ethnopharmacological Relevance: Fuzi and ganjiang are widely used as traditional Chinese medicines (TCM) in China, Korea, Japan, and many other southeast Asian countries for treating ulcerative colitis (UC), emesis and heart failure for more than 1800 years. However, the underlying mechanism of fuzi, ganjiang and fuzi-ganjiang herb pair is still unclear. In our study, we explored the therapeutic effects of fuzi, ganjiang and fuzi-ganjiang herb pair against dextran sulfate sodium (DSS)-induced UC in mice model, along with the relevant mechanism. Read More

Pediatr Blood Cancer 2020 Jun 22:e28483. Epub 2020 Jun 22.

Division of Pediatric Hematology and Oncology, Department of Pediatrics, Faculty of Medicine Siriraj Hospital, Mahidol University, Salaya, Thailand.

Background: The role of splenectomy prior to hematopoietic stem cell transplantation (HSCT) is controversial. Only few studies compared the outcomes of splenectomized and nonsplenectomized children with transfusion-dependent thalassemia (TDT) undergoing allogeneic HSCTs.

Methods: A retrospective analysis was undertaken on a transplantation cohort of TDT patients; August 1987-December 2014 to compare transplant outcomes between splenectomized and nonsplenectomized groups. Read More

Cureus 2020 Jun 17;12(6):c32. Epub 2020 Jun 17.

Radiology, Ankara City Hospital, Ankara, TUR.

[This corrects the article DOI: 10.7759/cureus.7230. Read More

Case Rep Gastrointest Med 2020 10;2020:8509591. Epub 2020 Jun 10.

Department of Medicine, BronxCare Hospital Center, Bronx, NY 10457, USA.

Introduction: Human immunodeficiency virus (HIV) positive individuals with the CD4 count less than 200 cells/mm are at risk for opportunistic infections. , a fungal pathogen, is a common cause of opportunistic infections with predominantly pulmonary involvement. Disseminated infection presenting with hepatosplenic lesion is extremely rare. Read More

Can J Infect Dis Med Microbiol 2020 27;2020:6934149. Epub 2020 May 27.

Division of Infectious Disease, University of Colorado, Denver, CO, USA.

Splenic complications of acute infection include splenomegaly, splenic infarct, and splenic rupture. These complications are relatively rarely reported, and the aim of this research was to synthetize data on this topic according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines using the PubMed database. In this review, we find that unlike other severe complications of babesiosis, splenic infarct and rupture occur in younger and immunocompetent patients, and they do not correlate with parasitemia level. Read More

BMJ Case Rep 2020 Jun 21;13(6). Epub 2020 Jun 21.

Department of General Surgery, Cork University Hospital Group, Cork, Ireland.

An unusual presentation of sclerosing angiomatoid nodular transformation in a 42-year-old man who was admitted with jaundice, deranged liver function tests and subsequently diagnosed with acute hepatitis C infection in the context of recent intravenous drug use. During his admission, he had an ultrasound of the abdomen followed by a CT thorax, abdomen and pelvis which showed splenomegaly and a large splenic lower pole mass that was hypoechoic and concerning for lymphoma. A bone marrow biopsy showed no evidence of lymphoma and an ultrasound-guided biopsy of the splenic mass suggested unusual features with vascular proliferation, either neoplastic or reactive, with no evidence of lymphoma or high-grade sarcoma. Read More

J Med Case Rep 2020 Jun 22;14(1):75. Epub 2020 Jun 22.

Azienza Ospedaliera Santa Croce e Carle, Via Michele Coppino, 26, 12100, Cuneo, CN, Italy.

Background: Immunoglobulin M multiple myeloma and Waldenström macroglobulinemia are two different hematological diseases with the common finding of an immunoglobulin M monoclonal gammopathy of unknown significance. However, clinical characteristics of the two entities can overlap.

Case Presentation: In this report, we describe two cases of immunoglobulin M neoplasm with the same histological bone marrow presentation but with different clinical behavior, cytogenetics, and biological assessment. Read More

Int Immunopharmacol 2020 Jun 17;86:106553. Epub 2020 Jun 17.

College of Medicine, Dongguk University, Goyang 410-773, South Korea. Electronic address:

Although tacrolimus (FK-506) has been shown to be an effective monotherapy for psoriasis, it does not always work well. Currently, combination therapy is frequently used to manage psoriasis because clinical trials have shown it may provide additive or synergistic benefits and reduce risks of adverse effects. Myeloid-derived suppressor cells (MDSCs) have potent immunomodulatory and anti-inflammatory properties in autoimmune diseases. Read More

Mol Immunol 2020 Jun 16;124:117-124. Epub 2020 Jun 16.

College of Animal Science and Technology, Shihezi University, Shihezi 832003, Xinjiang Province, People's Republic of China. Electronic address:

Brucellosis, which is caused by Brucella spp., is an important zoonotic infectious disease that can cause great hazard to public health and safety. However, the current vaccines have several drawbacks, including residual virulence for animals and humans. Read More

Hepatol Res 2020 Jun 19. Epub 2020 Jun 19.

Department of Gastroenterology and Hepatology, Osaka University Graduate School of Medicine, Suita, Osaka, Japan.

Background And Aim: Preserved liver function may be an important factor affecting therapeutic efficacy in hepatocellular carcinoma (HCC) patients treated with lenvatinib, but not all patients can be treated while preserving liver function. This study evaluated the therapeutic efficacy of lenvatinib in patients with poor liver function with and without portal hypertension.

Methods: This prospectively registered multicenter study analyzed 93 patients treated with lenvatinib. Read More

J Med Virol 2020 Jun 19. Epub 2020 Jun 19.

Department of Neurology, The Children's Hospital, Zhejiang University School of Medicine, National Clinical Research Center for Child Health, Hangzhou, China.

Epstein-Barr virus (EBV) infection occurs commonly in children and presents as a primary or reactivated infection, which are difficult for clinicians to distinguish. This study investigated the clinical characteristics of the two types of infections. Children with detectable plasma EBV-DNA were retrospectively enrolled and divided into primary and reactivated infection group by EBV-specific antibody. Read More

CPT Pharmacometrics Syst Pharmacol 2020 Jun 19. Epub 2020 Jun 19.

Translational Disease Modelling, Digital Data Science, Sanofi, Bridgewater, New Jersey, USA.

Gaucher's disease type 1 (GD1) leads to significant morbidity and mortality through clinical manifestations, such as splenomegaly, hematological complications, and bone disease. Two types of therapies are currently approved for GD1: enzyme replacement therapy (ERT), and substrate reduction therapy (SRT). In this study, we have developed a quantitative systems pharmacology (QSP) model, which recapitulates the effects of eliglustat, the only first-line SRT approved for GD1, on treatment-naïve or patients with ERT-stabilized adult GD1. Read More

Blood 2020 Jun 17. Epub 2020 Jun 17.

Perelman School of Medicine, Philadelphia, United States.

ß-thalassemia intermedia is a disorder characterized by ineffective erythropoiesis (IE), anemia, splenomegaly and systemic iron overload. Novel approaches are being explored based on the modulation of pathways that reduce iron absorption (i.e. Read More

Zhongguo Shi Yan Xue Ye Xue Za Zhi 2020 Jun;28(3):1006-1011

Department of Geriatric Hematology, The First Affiliated Hospital of Nanjing Medical University, Jiangsu Province Hospital. Nanjing 210029, Jiangsu province,

Objective: To investigate the expression and clinical significance of soluble B7-H3 (sB7-H3) in patients with secondary hemophagocytic lymphohistiocytosis (sHLH).

Methods: The plasma samples of 85 newly diagnosed sHLH patients from December 2012 to April 2018 were collected. The patients were divided into lymphoma-related HLH（LHLH）group and infection-related HLH（IHLH）group. Read More

BMC Geriatr 2020 Jun 17;20(1):213. Epub 2020 Jun 17.

Department of Geriatric Medicine, Vrije Universiteit Brussel (VUB), Universitair Ziekenhuis Brussel (UZ Brussel), Laarbeeklaan 101, 1090, Brussels, Belgium.

Background: Hematologic malignancies can spread to the central nervous system (CNS), either as focal lesions or as leptomeningeal disease. Marginal zone lymphoma (MZL) is a low-grade non-Hodgkin lymphoma and generally presents as an indolent disease. This case report illustrates an unexpected diagnosis of leptomeningeal metastasis in an MZL, presenting as a delirium without B symptoms, pronounced hematologic progression or abnormalities on cerebral imaging. Read More

Cureus 2020 May 15;12(5):e8130. Epub 2020 May 15.

Rheumatology, Temple University Hospital, Philadelphia, USA.

Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening condition caused by overproduction of inflammatory cytokines and overactivation of macrophages that can progress to multiorgan dysfunction and failure. Although there are guidelines that attempt to recognize the condition in its early stage, diagnosis can be very challenging due to heterogeneous presentations of HLH. Symptoms and clinical findings include fever, neurologic complaints, respiratory issues, liver dysfunction, cytopenias, amongst others most of which are not specific to HLH. Read More

World J Hepatol 2020 May;12(5):184-206

Departments of Pharmacology and Pathology, School of Medicine, University of Zagreb, Zagreb 10000, Croatia.

Background: The Pringle maneuver [portal triad obstruction(PTO)] provides huge disturbances during ischemia and even more thereafter in reperfusion. Contrarily, a possible solution may be stable gastric pentadecapeptide BPC 157, with already documented beneficial effects in ischemia/reperfusion conditions. Recently, BPC 157, as a cytoprotective agent, successfully resolved vessel occlusions in rats (ischemic colitis; deep vein thrombosis, superior anterior pancreaticoduodenal vein; bile duct cirrhosis) through rapid collateral vessel recruitment to circumvent vessel occlusion. Read More

Int J Lab Hematol 2020 Jun;42 Suppl 1:90-98

Department of Pathology and Immunology, Washington University, St. Louis, MO, USA.

T-cell prolymphocytic leukemia (T-PLL) is a rare T-cell leukemia characterized in many patients by marked peripheral lymphocytosis, prominent splenomegaly, and skin lesions. The differential diagnosis is broad and includes other T-cell disorders presenting with similar clinical findings. This review addresses (a) the natural history, demographics, and genetic features of T-PLL; (b) clinical and pathologic differential diagnostic considerations; and (c) recent developments in the T-PLL literature relevant to laboratory professionals. Read More

Leukemia 2020 Jun 14. Epub 2020 Jun 14.

Center for the Study of Myelofibrosis, Laboratory of Biochemistry, Biotechnology and Advanced Diagnostics, Istituto di Ricovero e Cura a Carattere Scientifico Policlinico S. Matteo Foundation, Pavia, Italy.

The expression of the CXCR4 chemokine receptor on CD34-positive blood cells is reduced in persons with primary myelofibrosis (PMF). We analyzed the relevance of cytofluorimetric assessment of the percentage of CD34-positive blood cells that had a positive CXCR4 surface expression (CD34/CXCR4-se) in a large cohort of subjects with myeloproliferative neoplasms. Mean CD34/CXCR4-se was lower in subjects with PMF compared with those with essential thrombocythemia (ET) or polycythemia vera (PV). Read More

Oncogene 2020 Jun 12. Epub 2020 Jun 12.

Division of Hematology, Department of Internal Medicine, National Taiwan University Hospital, Taipei, Taiwan.

HOPX is a stem cell marker in hair follicles and intestines. It was shown critical for primitive hematopoiesis. We previously showed an association between higher HOPX expression and clinical characteristics related to stemness and quiescence of leukemic cells in acute myeloid leukemia (AML) patients. Read More

Pediatrics 2020 Jul 12;146(1). Epub 2020 Jun 12.

The Children's Hospital at Montefiore, Bronx, New York; and.

An 8-year-old boy with no significant past medical history presented to his pediatrician with 5 days of fever, diffuse abdominal pain, and pallor. The pediatrician referred the patient to the emergency department (ED), out of concern for possible malignancy. Initial vital signs indicated fever, tachypnea, and tachycardia. Read More

J Vet Diagn Invest 2020 Jun 12:1040638720927669. Epub 2020 Jun 12.

Department of Veterinary Diagnostic and Production Animal Medicine, College of Veterinary Medicine, Iowa State University, Ames, IA.

Investigations of 2 cases of high mortality in cull sows and feeder pigs from a buying station in Ohio and cull sows at an abattoir in Tennessee were conducted at the Iowa State University Veterinary Diagnostic Laboratory. The animals were presented as weak, lethargic, and some with high fever. Rapidly escalating mortality was reported to be as high as 30-50% within groups at the buying station over 8-10 d, and 30-40% over 5-7 d at the abattoir. Read More

J Assoc Genet Technol 2020 ;46(2):74-91

The International Circle of Genetic Studies, Los Angeles, CA.

Objectives: Transient myeloproliferative disorder (TMD), now more commonly known as transient abnormal myelopoiesis (TAM), is a condition closely associated with Down syndrome. Ninety-five percent of Down syndrome cases occur as a result of chromosomal nondisjunction and are rarely due to mosaicism or translocation. TMD is found exclusively in neonates and is most commonly characterized by trisomy 21, somatic GATA1 mutation, and the increased presence of megakaryoblasts. Read More

Ann Hematol 2020 Jun 9. Epub 2020 Jun 9.

Bone Marrow Transplantation Center, the First Affiliated Hospital, School of Medicine, Zhejiang University, 79 Qingchun Road, Hangzhou, 310003, Zhejiang, People's Republic of China.

Post-transplantation thrombocytopenia (PT) is a common and severe complication which usually leads to poor prognosis. Eltrombopag (EPAG), a novel oral thrombopoietin (TPO) receptor agonist, has shown promising effects in thrombocytopenia due to immune thrombocytopenic purpura (ITP) and refractory severe aplastic anemia (rSAA), while the effectiveness of EPAG for PT patients still needs to be evaluated. A total of 32 PT patients receiving EPAG were retrospectively analyzed between September 2017 and July 2019, including 15 patients with poor graft function (PGF) and 17 patients with secondary failure of platelet recovery (SFPR). Read More

Int J Hematol 2020 Jun 8. Epub 2020 Jun 8.

Department of Internal Medicine, Šibenik-Knin County General Hospital, Stjepana Radića 83, 22000, Šibenik, Croatia.

Chronic kidney disease (CKD) is a well-known risk factor for venous thromboembolism and cardiovascular (CV) disease development in the general population, but its role in thrombotic risk in essential thrombocythemia (ET) and polycythemia vera (PV) remains poorly understood. This retrospective multicenter study analyzed clinical correlations and the potential impact of CKD on thrombosis development in ET and PV patients. We included 167 patients (76 ET and 91 PV); 25. Read More

Cell Death Dis 2020 Jun 8;11(6):430. Epub 2020 Jun 8.

Department of Immunology, 2nd Faculty of Medicine Charles University, University Hospital in Motol, V Uvalu 84, Prague, Czech Republic.

X-linked inhibitor of apoptosis (XIAP) is the most potent human inhibitor of apoptosis, and is also involved in NOD2-dependent NFκB and MAPK signalling cascade activation. The absence or defective function of XIAP leads to the development of a rare and severe primary immunodeficiency known as X-linked lymphoproliferative syndrome type 2 (XLP-2), which is characterized by a triad of clinical manifestations, including a high incidence of haemophagocytic lymphohistiocytosis (HLH), lymphoproliferation and inflammatory bowel disease (IBD), usually with very early onset. Here, we present a novel XIAP mutation identified in a patient with atypical adult-onset IBD complicated by relapsing HLH, splenomegaly and sarcoid-like disease. Read More

BMC Pediatr 2020 Jun 9;20(1):284. Epub 2020 Jun 9.

Bangabandhu Sheikh Mujib Medical University, Dhaka, Bangladesh.

Background: Histoplasmosis is a rare infectious condition with mainly pulmonary involvement. Disseminated histoplasmosis may occur in immunocompromised condition. It can present in different ways but jaundice and ascites is very uncommon. Read More

Prenat Diagn 2020 Jun 7. Epub 2020 Jun 7.

Assistance Publique-Hôpitaux de Paris, Service de Gynécologie-Obstétrique, Hôpital Louis Mourier, APHP Nord Université de Paris, Colombes, France.

Objective: To describe the lesions detected by prenatal ultrasound examination in congenital toxoplasmosis (CT).

Methods: We retrospectively analyzed all cases of fetal infection with Toxoplasma gondii with ultrasound anomalies described by fetal medicine experts in 2009 to 2019 in 30 French centers.

Results: Eighty-eight cases of CT were included. Read More

Biol Blood Marrow Transplant 2020 Jun 4. Epub 2020 Jun 4.

Department of Hematology, Toranomon Hospital, Tokyo, Japan; Okinaka Memorial Institute for Medical Research, Tokyo, Japan.

Delayed neutrophil engraftment (NE) has been reported in cord blood transplantation (CBT) compared with other stem cell transplantation methods. The numbers of total nucleated cells (TNCs), CD34 cells (generally ≥ 1 × 10/kg), and granulocyte/macrophage colony-forming units (CFU-GM) significantly impact NE. Splenomegaly exerts negative effects on NE, but the appropriate cell dose for the patients with splenomegaly has not yet been determined, especially in CBT. Read More

Transplant Proc 2020 Jun 4;52(5):1617-1618. Epub 2020 Jun 4.

Surgical, Medical and Dental Department of Morphologic Sciences, Section of Nephrology, University of Modena and Reggio Emilia, Modena, Italy; Nephrology Dialysis and Transplant Unit, University Hospital of Modena, Modena, Italy.

T-cell large granular lymphocytic (T-LGL) leukemia is a rare clonal proliferation presenting with cytopenia, splenomegaly, and autoimmune manifestations. It has rarely been described in recipients of solid organ transplants. We report the clinical case of a young kidney transplant recipient that developed T-LGL leukemia 3 years after kidney transplantation. Read More

Chest 2020 Jun;157(6):e197-e201

Department of Respiratory Medicine, Nanjing Drum Tower Hospital, The Affiliated Hospital of Nanjing University Medical School, Nanjing, Jiangsu, China. Electronic address:

Case Presentation: A 52-year-old man presented with repeated cough and worsening exertional dyspnea for 5 years. Long-term oral prednisone had been administered with little effect. He denied chest pain, hemoptysis, or nighttime paroxysmal dyspnea. Read More

Immunol Lett 2020 Aug 3;224:30-37. Epub 2020 Jun 3.

Faculty of Life Sciences and Biotechnology, South Asian University, Akbar Bhawan, Chanakyapuri, New Delhi, 110021, India. Electronic address:

Graft versus host disease (GVHD) results from hyper-activation of transplanted lymphocytes against the host antigens. Bone marrow transplantation in humans as well as some cases of blood transfusion and organ transplantation are associated with a strong GVH reaction resulting in GVHD that in many cases may be fatal. We had previously shown that poly-dispersed acid-functionalized single-walled carbon nanotubes (AF-SWCNTs) specifically target activated T and B lymphocytes and kill them. Read More

BMC Gastroenterol 2020 Jun 5;20(1):176. Epub 2020 Jun 5.

Department and Clinic of Tropical and Parasitic Diseases, Poznan University of Medical Sciences, Przybyszewskiego Street 49, 60-355, Poznań, Poland.

Background: Infection of Echinococcus multilocularis causes in humans the alveolar echinococcosis. Although the infection has world-wide distribution it is rarely detected. Diagnosis of alveococcosis is difficult because of not typical clinical picture and irregular results of radiological examinations suggesting neoplasmatic process which begins in the liver tissue or in the biliary tracts. Read More