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Med Clin (Barc) 2019 Mar 11. Epub 2019 Mar 11.

Servicio de Hematología, Hospital Virgen de las Nieves, Granada, España.

Exp Ther Med 2019 Mar 23;17(3):2137-2142. Epub 2019 Jan 23.

Ward Pharmacy, Qingdao Municipal Hospital, Qingdao, Shandong 266000, P.R. China.

Therapeutic effects of rituximab combined with cyclophosphamide on refractory idiopathic thrombocytopenic purpura (ITP) were investigated. We retrospectively analyzed 249 patients with refractory ITP who were admitted to Qingdao Hiser Medical Group between March 2013 and March 2017. Curative effects of patients treated with rituximab, cyclophosphamide, and combination therapy were observed and the changes of platelet count, PA IgG, and lymphocyte CD20 before and after treatment, as well as the incidence of adverse reactions after treatment, were compared. Read More

Anasthesiol Intensivmed Notfallmed Schmerzther 2019 Mar 13;54(3):194-205. Epub 2019 Mar 13.

Nowadays, management of hemotherapy is regulated in Germany by the transfusion act and several guidelines while the transfusing physician is responsible for correct implementation at the bedside. Indications for blood products have to be carefully adapted to the patient's current clinical situation and pre-existing diseases have to be considered as well. Today, for most perioperative elective surgeries, evidence-based transfusion thresholds for packed red blood cell concentrates (RBC) have been defined and should be considered. Read More

Curr Opin Nephrol Hypertens 2019 Mar 6. Epub 2019 Mar 6.

Department of Obstetrics and Gynecology, Division of Maternal Fetal Medicine, Cedars Sinai Medical Center, Los Angeles, California, USA.

Purpose Of Review: Atypical hemolytic uremic syndrome (aHUS) is a diagnosis that has captured the interest of specialists across multiple fields. The hallmark features of aHUS are microangiopathic hemolysis and thrombocytopenia, which creates a diagnostic dilemma because of the occurrence of these findings in a wide variety of clinical disorders.

Recent Findings: In most of the instances, aHUS is a diagnosis of exclusion after ruling out causes such as Shigella toxin, acquired or genetic a disintegrin and metalloproteinase thrombospondin motif 13 deficiency (thrombotic thrombocytopenic purpura), and vitamin B12 deficiency. Read More

Clin J Am Soc Nephrol 2019 Mar 12. Epub 2019 Mar 12.

Service de Néphrologie-hypertension, Dialyses, Transplantation Rénale, Hôpital Bretonneau et hôpital Clocheville,

Background And Objectives: Thrombotic microangiopathies constitute a diagnostic and therapeutic challenge. Secondary thrombotic microangiopathies are less characterized than primary thrombotic microangiopathies (thrombotic thrombocytopenic purpura and atypical hemolytic and uremic syndrome). The relative frequencies and outcomes of secondary and primary thrombotic microangiopathies are unknown. Read More

Int J Mycobacteriol 2019 Jan-Mar;8(1):107-109

Department of Internal Medicine, Sri Balaji Action Medical Institute, New Delhi, India.

Pulmonary tuberculosis can have a wide variety of presentations including hematological manifestations. We report a case of a young male patient who presented with complaints of generalized petechiae, gum bleeding, systemic lymphadenopathy, and severe thrombocytopenia. His bone marrow revealed normal megakaryocytes, and in the absence of hepatosplenomegaly, a diagnosis of immune thrombocytopenic purpura (ITP) was made. Read More

Front Immunol 2019 25;10:240. Epub 2019 Feb 25.

Research Laboratory, MTA-SE Research Group of Immunology and Hematology, 3rd Department of Internal Medicine, Hungarian Academy of Sciences and Semmelweis University, Budapest, Hungary.

Pentraxin-3 (PTX3) and C-reactive protein (CRP) have been shown to regulate complement activation , but their role has not been investigated in complement consumption . Thrombotic microangiopathies (TMA) are often accompanied by complement overactivation and consumption, therefore we analyzed the relation of the systemic pentraxin levels to the complement profile, laboratory parameters and clinical outcome of TMA patients. We determined the PTX3 and CRP levels, complement factor and activation product concentrations in blood samples of 171 subjects with the diagnosis of typical hemolytic uremic syndrome (STEC-HUS) ( = 34), atypical HUS (aHUS) ( = 44), secondary TMA ( = 63), thrombotic thrombocytopenic purpura (TTP) ( = 30) and 69 age-matched healthy individuals. Read More

Haematologica 2019 Mar 7. Epub 2019 Mar 7.

Fondazione IRCCS Ca Granda Ospedale Maggiore Policlinico di Milano, UOC Ematologia, Milano, Italy

Haematologica 2019 Mar 7. Epub 2019 Mar 7.

Amgen Inc., Thousand Oaks, CA, USA.

Children with immune thrombocytopenia ≥6 months completing a romiplostim study received weekly subcutaneous romiplostim (1-10 mg/kg targeting platelet counts of 50-200x10/L) in this extension to examine romiplostim's long-term safety and efficacy. Sixty-five children received romiplostim for a median (range) of 2.6 (0. Read More

Biomed Res Int 2019 30;2019:1756109. Epub 2019 Jan 30.

Department of Blood Transfusion, Fujian Medical University Union Hospital, Gulou District, Fuzhou City, Fujian Province 350001, China.

Purpose: To evaluate the impact of a combination of fresh frozen plasma (FFP) and cryosupernatant plasma (CP) as a replacement fluid in therapeutic plasma exchange (TPE) on early therapeutic response and long-term survival of patients with thrombotic thrombocytopenic purpura (TTP).

Materials And Methods: A total of 44 patients with suspected TTP were screened by Bentley and PLASMIC scores. Twenty-seven patients treated with TPE using the FFP and CP combination as the replacement fluid were enrolled and divided into two groups: 11 patients who received TPE with CP-dominant replacement fluid (FFP/CP<1) and 16 patients who received TPE with FFP-dominant replacement fluid (FFP/CP>1). Read More

Zhonghua Xue Ye Xue Za Zhi 2019 Feb;40(2):141-143

Jiangsu Institute of Hematology, Key Laboratory of Thrombosis and Hemostasis of Ministry of Health, The First Affiliated Hospital of Soochow University, Collaborative Innovation Center of Hematology, Suzhou, 215006, China.

Acta Med Okayama 2019 Feb;73(1):91

Erratum to "High-dose Dexamethasone Therapy as the Initial Treatment for Idiopathic Thrombocytopenic Purpura: Protocol for a Multicenter, Open-label, Single Arm Trial" Vol.72, No.2, pp197-201. Read More

Haematologica 2019 Feb 28. Epub 2019 Feb 28.

Hôpital Lariboisière, APHP, France;

Oncologist 2019 Feb 28. Epub 2019 Feb 28.

Division of Hematology/Oncology, Vanderbilt University Medical Center, Nashville, Tennessee, USA.

Immune checkpoint inhibitors have improved outcomes for patients with numerous hematological and solid cancers. Hematologic toxicities have been described, but the spectrum, timing, and clinical presentation of these complications are not well understood. We used the World Health Organization's pharmacovigilance database of individual-case-safety-reports (ICSRs) of adverse drug reactions, VigiBase, to identify cases of hematologic toxicities complicating immune checkpoint inhibitor therapy. Read More

Intern Med J 2019 Feb 28. Epub 2019 Feb 28.

Department of Internal Medicine, Reference center of autoimmune cytopenias, University Hospital Henri-Mondor, APHP, UPEC, Créteil, France.

Background: increased risk of thrombosis has been reported in immune thrombocytopenic purpura (ITP), but the characteristics, risk factors of occurrence, recurrence and management of venous thromboembolic events (VTEs) have been poorly investigated.

Aims: to describe VTE and ITP characteristics, distribution of VTE risk factors and their impact on VTE features and recurrence.

Methods: retrospective study of patients with ITP and VTE registered in databases of 3 reference French centers of ITP RESULTS: Among 49 patients, 66 VTEs were recorded. Read More

Med Arch 2018 Dec;72(6):453-455

Clinic of Hemodialysis, Clinical Center University of Sarajevo, Bosnia and Herzegovina.

Introduction: Plasmapheresis is often used as a therapy in the treatment of thrombotic thrombocytopenic purpura (TTP). TTP is manifested in thrombotic microangiopathy, consumed thrombocytopenia, hemolytic anemia and acute kidney injury with HUS development, neurologic dysfunction, and fever.

Case Report: we will present a case of a patient with acute kidney injury and refractory TTP at the beginning of hospitalization, subsequently manifested in secondary nephrotic syndrome. Read More

Platelets 2019 Feb 27:1-8. Epub 2019 Feb 27.

a Department of Pediatrics , Fujian Medical University Union Hospital , Fuzhou , Fujian Province , China.

Immune thrombocytopenic purpura (ITP) is an autoimmune disorder presenting with low platelet count <100 × 10/L. The condition affects both adults and children. Thrombopoietin receptor agonists (TPO-RAs) are second-line of therapy that includes Romiplostim and Eltrombopag, which stimulate the production of normally functioning platelets. Read More

Clin Appl Thromb Hemost 2019 Jan-Dec;25:1076029618825309

1 Division of Hematology, Department of Medicine, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand.

The current systematic review and meta-analysis aimed to summarize the results of all available studies to compare the efficacies of rituximab and conventional treatment for acquired thrombotic thrombocytopenic purpura (TTP). Three investigators independently searched studies in the MEDLINE and EMBASE databases published before December 11, 2018. To be included in the meta-analysis, studies needed to be randomized-controlled or cohort studies comparing the efficacies of rituximab and conventional therapy for TTP treatment. Read More

Nephrol Dial Transplant 2019 Feb 25. Epub 2019 Feb 25.

Sorbonne Université, Urgences Néphrologiques et Transplantation Rénale, Assistance Publique-Hôpital de Paris (APHP), Hôpital Tenon, Paris, France.

Background: Among the severe complications of preeclampsia (PE), acute kidney injury (AKI) is problematic if features of thrombotic microangiopathy (TMA) are present. Although a haemolysis enzyme liver low-platelets syndrome is considerably more frequent, it is vital to rule out a flare of atypical haemolytic and uraemic syndrome (aHUS). Our objective was to improve differential diagnosis procedures in post-partum AKI. Read More

Haematologica 2019 Feb 21. Epub 2019 Feb 21.

Inselspital, Bern University Hospital;

Congenital thrombotic thrombocytopenic purpura is an autosomal recessive inherited disease with a clinically heterogeneous course and an incompletely understood genotype-phenotype correlation. In 2006, the Hereditary TTP Registry started recruitment with the purpose of improving the understanding of this ultra-rare disease. The objective of this study is to present characteristics of the cohort until the end of 2017 and to explore the relationship between overt disease onset and ADAMTS13 activity with emphasis on the recurring ADAMTS13 c. Read More

Cureus 2018 Dec 8;10(12):e3706. Epub 2018 Dec 8.

Hematology, Thomas Jefferson University Hospital, Philadelphia, USA.

Spontaneous tumor lysis syndrome is an exceedingly rare manifestation of metastatic prostate cancer. It can masquerade as thrombotic thrombocytopenic purpura (TTP) or complement-mediated hemolytic uremic syndrome (HUS). These entities present with microangiopathic hemolytic anemia, thrombocytopenia, and renal failure, and improve with the initiation of plasma exchange and steroids. Read More

J Community Hosp Intern Med Perspect 2019 11;9(1):59-61. Epub 2019 Feb 11.

Department of Medicine, Unity Hospital, Rochester Regional Health, Rochester, NY, USA.

Immune thrombocytopenic purpura (ITP) is a bleeding disorder characterized by isolated thrombocytopenia (platelet count <150,000 u/L), which is not associated with a systemic illness. ITP is reported in approximately 2 per 100,000 adults. The mean age of diagnosis is 50 years. Read More

BMC Musculoskelet Disord 2019 Feb 19;20(1):88. Epub 2019 Feb 19.

Department of Orthopaedic Surgery, Faculty of Medicine and Graduate School of Medicine, Hokkaido University, Kita-15 Nishi-7, Kita-ku, Sapporo, 060-8638, Japan.

Background: The incidence of bilateral corticosteroid-induced osteonecrosis of the femoral head (ONFH) is high. Although the precise mechanism of corticosteroid-induced ONFH development is unclear, hepatic enzyme abnormalities such as low activity of hepatic cytochrome P450 3A could be one cause. Herein, we report the case of a patient who developed ONFH in the contralateral hip after the dose of corticosteroids for idiopathic thrombocytopenic purpura was increased. Read More

J Clin Apher 2019 Feb 18. Epub 2019 Feb 18.

Department of Medicine, West Virginia University Hospital, Morgantown, West Virginia.

Thrombotic microangiopathies are rare diseases associated with significant morbidity and mortality. The treatment of thrombotic thrombocytopenic purpura (TTP) and drug-induced thrombotic microangiopathy (DITMA) remains a diagnostic dilemma as they present similarly but respond differently to standard treatment with plasma exchange. TTP is a deficiency of a disintegrin and metalloprotease with thrombospondin type 1 motif 13 resulting in von Willebrand factor aggregates. Read More

Int J Prev Med 2019 15;10. Epub 2019 Jan 15.

Department of Anesthesiology and Intensive Care Medicine, Tabriz University of Medical Sciences, Tabriz, Iran.

Introduction: Abu Bakr Mohammad Ibn Zakariya Al-Razi (865-925 CE), who was known as "Rhazes" in the west, was a famous scientist of medieval ages. He has more than 200 books and treatises. His masterpiece on medicine "" contains around 900 case reports. Read More

Autoimmun Rev 2019 Feb 14. Epub 2019 Feb 14.

Center for Autoimmune Diseases Research (CREA), School of Medicine and Health Sciences, Universidad del Rosario, Bogota, Colombia.. Electronic address:

Overt polyautoimmunity (PolyA) corresponds to the presence of more than one well-defined autoimmune disease (AD) manifested clinically in a single patient. The current study aimed to describe the main characteristics of juvenile PolyA in a pediatric rheumatology setting and analyze the chronological aspects, index cases, familial autoimmunity, and clustering pattern. This was a cross-sectional and multicenter study in which 313 children with overt PolyA were included. Read More

Blood 2019 Feb 15. Epub 2019 Feb 15.

Department of Haematology, University College London Hospital, London, United Kingdom.

Congenital Thrombotic Thrombocytopenic Purpura (cTTP) is an ultra rare thrombomicroangiopathy caused by an inherited deficiency of ADAMTS13. There is limited data on the genotype-phenotype correlation and no consensus on treatment. We reviewed the largest cohort of cTTP cases, diagnosed in the UK, over the past 15 years. Read More

Case Rep Hematol 2019 14;2019:7425320. Epub 2019 Jan 14.

Division of Hematology, Department of Internal Medicine, McGovern Medical School, The University of Texas Health Science Center at Houston, 6411 Fannin Street, Houston 77030, USA.

Here we report a case of atypical thrombotic thrombocytopenic purpura that presented as an ischemic cerebrovascular accident. A 56-year-old man with multiple cardiovascular risk factors presented with sudden left-sided weakness, slurred speech, and left facial droop. He showed mild improvement when he was treated with thrombolytic therapy according to the hospital stroke protocol. Read More

J Thromb Haemost 2019 Feb 14. Epub 2019 Feb 14.

Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Angelo Bianchi Bonomi Hemophilia and Thrombosis Center and Fondazione Luigi Villa, Milan, Italy.

Essentials Congenital thrombotic thrombocytopenic purpura (cTTP) is a very rare thrombotic microangiopathy. Its rarity and great phenotype heterogeneity may account for misdiagnosis. We report the history of a middle-aged woman with cTTP, misdiagnosed until adulthood. Read More

Eur J Haematol 2019 Feb 13. Epub 2019 Feb 13.

Barts and The London School of Medicine and Dentistry, London, UK.

Background: Romiplostim is a thrombopoietin-mimetic peptibody for adult refractory chronic immune thrombocytopenia (ITP). We aimed to describe ITP patients receiving romiplostim, platelet counts and romiplostim usage in UK clinical practice.

Methods: This was a retrospective cohort study of patients in the UKITP Registry who received romiplostim between October 2009 and January 2015, including data up to 6 months before romiplostim initiation through follow-up. Read More

Curr Opin Rheumatol 2019 Mar 1. Epub 2019 Mar 1.

Division of Rheumatology, Department of Internal Medicine, University of Michigan.

Purpose Of Review: Although antiphospholipid syndrome (APS) is best known for conveying increased risk of thrombotic events and pregnancy morbidity, thrombocytopenia is also recognized as a common association. In this review, we will explore the relationship between thrombocytopenia and APS, highlighting our evolving understanding - and persistent knowledge gaps - through clinically oriented questions and answers.

Recent Findings: A history of thrombocytopenia likely portends a more severe APS phenotype (including increased risk of thrombosis). Read More

Case Rep Womens Health 2019 Jan 23;21:e00097. Epub 2019 Jan 23.

University of South Alabama, Children's and Women's Hospital, Department of Obstetrics and Gynecology, 251 Cox Street, Mobile, AL 36604, United States.

Cerebrovascular disease is not uncommon during pregnancy as a result of either venous or arterial occlusion, or a hemorrhagic event, resulting in ischemia. Pregnancy may alter the prognosis of these neurologic disorders, with increased risks of morbidity and mortality for the mother and the developing fetus. Etiologies of stroke during pregnancy and the postpartum period include preeclampsia, eclampsia, HELLP syndrome, posterior reversible encephalopathy syndrome (PRES), amniotic fluid embolism, postpartum angiopathy, postpartum cardiomyopathy, thrombotic thrombocytopenic purpura (TTP), hemolytic uremic syndrome (HUS), cerebral venous thrombosis, CNS infections, and maternal thrombophilia. Read More

Case Rep Obstet Gynecol 2019 14;2019:2093612. Epub 2019 Jan 14.

Department of Obstetrics and Gynecology, Toyooka Public Hospital, 1094, Tobera, Toyooka City, Hyogo 6680065, Japan.

The simultaneous or sequential development of autoimmune hemolytic anemia (AIHA) and idiopathic thrombocytopenic purpura (ITP) is known as Evans syndrome. We experienced a case of Evans syndrome that developed AIHA during pregnancy and ITP long after delivery. The patient was a 35-year-old pregnant woman (gravida 2, para 1). Read More

Lab Med 2019 Feb 6. Epub 2019 Feb 6.

Department of Laboratory Medicine, Hallym University Sacred Heart Hospital, Hallym University College of Medicine, Anyang, South Korea.

Objective: To examine the kinetic characteristics of platelet (PLT) destruction and thrombopoiesis by using mean platelet volume (MPV) and platelet distribution width (PDW).

Methods: Using the ADVIA2120i instrument, we measured PLT counts, MPV, and PDW in 153 healthy individuals, 35 patients with immune thrombocytopenic purpura (ITP), and 48 patients with essential thrombocytopenia (ET).

Results: In the ITP group, the MPV and PDW were higher than those values in healthy individuals. Read More

Rinsho Ketsueki 2019 ;60(1):28-32

Department of Hematology, Faculty of Medicine, University of Tsukuba.

A 66-year-old male undergoing maintenance hemodialysis presented with mild thrombocytopenia. He also had aortic valve stenosis and required aortic valve replacement. In addition, he required anticoagulation therapy with warfarin because of chronic subclavian artery occlusion. Read More

Folia Biol (Praha) 2018 ;64(4):113-124

Institute of Pathological Physiology, First Faculty of Medicine, Charles University, Prague, Czech Republic.

Human body reacts to physical, chemical and biological insults with a complex inflammatory reaction. Crucial components and executors of this response are endothelial cells, platelets, white blood cells, plasmatic coagulation system, and complement. Endothelial injury and inflammation are associated with elevated blood levels of cell membrane-derived microvesicles. Read More

Front Pediatr 2018 22;6:413. Epub 2019 Jan 22.

Department of Pediatrics, University Hospital Würzburg, Würzburg, Germany.

Henoch-Schönlein Purpura (HSP) or IgA vasculitis is the most common systemic vasculitis of childhood and may affect skin, joints, gastrointestinal tract, and kidneys. Skin manifestations of HSP are characteristic and include a non-thrombocytopenic palpable purpura of the lower extremities and buttocks. Rarely, HSP may initially present as or evolve into hemorrhagic vesicles and bullae. Read More

Front Immunol 2018 22;9:3135. Epub 2019 Jan 22.

Department of Immunology, Second Faculty of Medicine, Charles University and Motol University Hospital, Prague, Czechia.

Common variable immunodeficiency disorder (CVID) is one of the most frequent inborn errors of immunity, increased occurrence of malignancies, particularly lymphomas, and gastric cancers, has long been noted among CVID patients. Multifactorial etiology, including immune dysregulation, infections, chronic inflammation, or genetic background, is suggested to contribute to tumor development. Here, we present the results of the first Czech nationwide study focused on epidemiology, immunology and genetic background in a cohort of CVID patients who also developed tumors The cohort consisted of 295 CVID patients followed for 3,070 patient/years. Read More

Wilderness Environ Med 2019 Mar 31;30(1):66-78. Epub 2019 Jan 31.

Faculty of Medicine, University of Peradeniya, Peredeniya, Sri Lanka.

Thrombotic microangiopathy (TMA), which includes the spectrum of hemolytic uremic syndrome and thrombotic thrombocytopenic purpura, is an uncommon complication of hump-nosed pit viper envenomation. We describe 4 cases of TMA following hump-nosed pit viper (Hypnale spp) bites in Sri Lanka. The first case is a typical TMA that spontaneously resolved with supportive treatments. Read More

Biol Blood Marrow Transplant 2019 Jan 30. Epub 2019 Jan 30.

Blood and Marrow Transplant Center, Florida Hospital Cancer Institute, Orlando, Florida. Electronic address:

Thrombocytopenia after allogeneic hematopoietic stem cell transplantation (allo-SCT) can pose significant problems in management of patients. Eltrombopag is a small-molecule thrombopoietin receptor agonist that has been approved for use in immune thrombocytopenic purpura and aplastic anemia; but its use after allo-SCT is limited. Between 2014 and 2017, we treated 13 patients with eltrombopag for poor platelet engraftment without evidence of relapse at the time of initiation, including 6 patients with primary platelet engraftment failure and 7 with secondary platelet engraftment failure. Read More

BMJ Case Rep 2019 Jan 31;12(1). Epub 2019 Jan 31.

Division of Respirology, Department of Internal Medicine, The Jikei University Hospital, Tokyo, Japan.

It is unknown whether tyrosine kinase inhibitors targeting epidermal growth factor receptor (EGFR) can be discontinued in patients in whom -mutated lung cancer has well stabilised. We present a case of a 73-year-old Japanese woman with no history of smoking. Right pulmonary lower lobectomy, lymph node dissection and segmental resection of the right middle lobe were performed. Read More

Am J Case Rep 2019 Jan 31;20:131-133. Epub 2019 Jan 31.

Department of Nephrology, University Hospital in Split, Split, Croatia.

BACKGROUND Thrombotic thrombocytopenic purpura (TTP) in children is a rare life-threatening syndrome, characterized by microangiopathic hemolytic anemia, thrombocytopenia with renal dysfunction, neurologic symptoms, and fever. TTP is usually caused by deficient activity of von Willebrand factor cleaving protease (ADAMTS13), due to either gene mutations or acquired via anti-ADAMTS13 autoantibodies. It can be triggered by bone marrow or solid organ transplantation, cardiothoracic-, abdominal-, and orthopedic surgeries, infections including very rarely Helicobacter pylori infection. Read More

Curr Health Sci J 2018 Apr-Jun;44(2):186-191. Epub 2018 Mar 27.

University of Oradea, Faculty of Medicine and Pharmacy.

Helicobacter Pylori (HP) persistently colonizes the stomach in about 50% of the globe population and it is the main risk factor for peptic ulcer, as well as for gastric adenocarcinoma and MALT gastric lymphoma. The treatment for HP revolutionized the management of the peptic ulcer disease, providing permanent healing in many cases. Preventing colonization of HP would be the primary prevention of gastric malignancy and peptic ulceration. Read More

J Biol Eng 2019 22;13. Epub 2019 Jan 22.

Institute of Biomechanics, School of Biosciences and Bioengineering, South China University of Technology, Guangzhou, 510006 China.

Background: ADAMTS13 (A disintegrin and metalloprotease with a thrombospondin type 1 motif 13) cleaves Von Willebrand factor (VWF) to regulate its size, thereby preventing aberrant platelet aggregation and thrombus. Deficiency of ADAMTS13 caused by either genetic mutations or by inhibitory autoantibodies against ADAMTS13 leads to thrombotic thrombocytopenic purpura (TTP). Recently, ADAMTS13 was reported to adopt a "closed" conformation with lower activity and an "open" one resulting from the engagements of VWF D4-CK domains or antibodies to the distal domains of ADAMTS13, or mutations in its spacer domain. Read More

Haematologica 2019 Jan 24. Epub 2019 Jan 24.

Peking University People's Hospital, Peking University Institute of Hematology;

Peripheral enhanced complement activation has long been considered as one of the major pathogenesis of immune thrombocytopenia. Impaired bone marrow microenvironment, especially the dysfunction of mesenchymal stem cells, has been observed in patients with immune thrombocytopenia. However, the potential role of the complement system involved in impaired bone marrow microenvironment remains poorly understood. Read More

Dermatol Online J 2018 Sep 15;24(9). Epub 2018 Sep 15.

School of Medicine, University of California San Diego, La Jolla, California.

Scabies surrepticius is a unifying term that represents non-classical presentations of scabies mite infestation. A patient with scabies surrepticius is described: a man with scabies masquerading as prurigo nodularis. The 91-year-old man had metastatic prostate cancer and presented with diffuse pruritic nodules. Read More

JCI Insight 2019 Jan 24;4(2). Epub 2019 Jan 24.

Department of Otorhinolaryngology-Head and Neck Surgery, University of Maryland School of Medicine, Baltimore, Maryland, USA.

The antiinflammatory effects of i.v. Ig (IVIG) in the treatment of autoimmune disease are due, in part, to the Fc fragments of Ig aggregates. Read More

J Clin Immunol 2019 Jan 22. Epub 2019 Jan 22.

Centre de Référence National des Cytopénies Autoimmunes de l'Enfant (CEREVANCE), University Hospital of Bordeaux, Bordeaux, France.

Purpose: Immune thrombocytopenic purpura (ITP) and autoimmune hemolytic anemia (AIHA) are associated in the definition of Evans syndrome (ES). The occurrence of neurological involvement in this population is poorly described and suggests an underlying primary immunodeficiency (PID). We aimed to describe the clinical manifestations, evolution, and PID profiles of these patients. Read More

Case Rep Hematol 2018 20;2018:2464619. Epub 2018 Dec 20.

The T.W. Lewis Melanoma Center of Excellence, Banner MD Anderson Cancer Center, Gilbert, AZ, USA.

Ipilimumab is a monoclonal antibody that enhances the efficacy of the immune system by targeting a cytotoxic T-lymphocyte-associated protein 4 (CTLA-4), which is a protein receptor that downregulates the immune system. Nivolumab is also a humanized monoclonal antibody that targets another protein receptor that prevents activated T cells from attacking the cancer; this receptor is called programmed cell death 1 (PD-1). The FDA approved ipilimumab combined with nivolumab as a frontline therapy for patients with metastatic melanoma or renal cell carcinoma and as a second-line therapy for patients with microsatellite instability-high (MSI-H) metastatic colon cancer. Read More