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BMJ Case Rep 2019 Jan 14;12(1). Epub 2019 Jan 14.

Department of Hematology-Oncology, Louisville School of Medicine, Louisville, Kentucky, USA.

A 61-year-old woman with chronic lymphocytic leukaemia, with Richter's transformation to a diffuse, large, B-cell lymphoma, treated with six cycles of rituximab, cyclophosphamide, doxorubicin, vincristine and prednisone and in complete remission, presented to the hospital after her platelets were found to be 2×10³/µL in outpatient laboratory studies. She initially underwent a platelet transfusion without improvement. This was followed by 4 days of high-dose dexamethasone and intravenous immunoglobulin, which again yielded no meaningful effect. Read More

Case Reports Hepatol 2018 9;2018:5305691. Epub 2018 Dec 9.

Department of Gastroenterology and Medicine, Fukuoka University Faculty of Medicine, 7-45-1 Nanakuma, Jonan, Fukuoka 814-0180, Japan.

Patients with autoimmune hepatitis (AIH) may sometimes have concomitant idiopathic thrombocytopenic purpura (ITP). Severe thrombocytopenia in ITP interferes with percutaneous liver biopsy for pathological diagnosis of AIH. Here, we report a case of AIH with ITP in a 63-year-old woman. Read More

Acta Haematol 2019 Jan 10;141(2):79-83. Epub 2019 Jan 10.

Autoimmune diseases, including autoimmune hemolytic anemia and immune thrombocytopenic purpura, have been described in patients with non-Hodgkin lymphoma (NHL) after immunochemotherapy. However, the underlying pathogenesis remains unclear. We examined NHL patients with autoimmune cytopenia and all patients were treated with rituximab-containing therapy. Read More

Rinsho Ketsueki 2018 ;59(12):2574-2577

Third Department of Internal Medicine, Yamaguchi University School of Medicine.

A 30-year-old primigravid woman without a history of thrombocytopenia was referred to our hospital because of severe thrombocytopenia (<1,000 thrombocytes/µl) at 16 weeks of gestation and diagnosed with idiopathic thrombocytopenic purpura (ITP). There was no improvement in the platelet count after treatment with 0.5-1. Read More

N Engl J Med 2019 Jan 9. Epub 2019 Jan 9.

From the Department of Haematology, University College London Hospitals, Cardiometabolic Program, National Institute for Health Research UCLH-UCL Biomedical Research Center, London (M.S.); the Division of Hematology, Department of Internal Medicine, Ohio State University, Columbus (S.R.C.); Fondazione Istituti di Ricovero e Cura a Carattere Scientifico Ca' Granda Ospedale Maggiore Policlinico, Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, and the Department of Pathophysiology and Transplantation, University of Milan, Milan (F.P.); the Department of Hematology, Saint-Antoine University Hospital, Paris (P.C.); the Department of Medicine 1, Division of Hematology and Hemostasis, Medical University of Vienna, Vienna (P.K.); the Department of Hematology and Central Hematology Laboratory, Inselspital, Bern University Hospital, University of Bern, Bern, Switzerland (J.A.K.H.); the Division of Hematology, Duke University School of Medicine, Durham, NC (A.M.); the Hematology Department, Universidad Católica de Valencia Hospital Dr. Peset, Valencia, Spain (J.R.); the Departments of Medicine and Laboratory Medicine, St. Michael's Hospital and University of Toronto, Toronto (K.P.); and Clinical Development, Ablynx, Zwijnaarde, Belgium (F.C., D.B., H.D.W., R.K.Z.).

Background: In acquired thrombotic thrombocytopenic purpura (TTP), an immune-mediated deficiency of the von Willebrand factor-cleaving protease ADAMTS13 allows unrestrained adhesion of von Willebrand factor multimers to platelets and microthrombosis, which result in thrombocytopenia, hemolytic anemia, and tissue ischemia. Caplacizumab, an anti-von Willebrand factor humanized, bivalent variable-domain-only immunoglobulin fragment, inhibits interaction between von Willebrand factor multimers and platelets.

Methods: In this double-blind, controlled trial, we randomly assigned 145 patients with TTP to receive caplacizumab (10-mg intravenous loading bolus, followed by 10 mg daily subcutaneously) or placebo during plasma exchange and for 30 days thereafter. Read More

Acta Chir Belg 2019 Jan 9:1-8. Epub 2019 Jan 9.

a 2nd Department of General Surgery , Jagiellonian University Medical College , Kraków , Poland.

Background: We aimed to evaluate the outcomes of laparoscopic splenectomy (LS) in patients with immune thrombocytopenic purpura (ITP) compared with operated for other indications.

Methods: Retrospective cohort study of patients who underwent LS in 1998-2017. Group 1 consisted of 256 patients operated for ITP, and Group 2 of 231 operated for other indications. Read More

Health Sci Rep 2018 May 2;1(5):e50. Epub 2018 May 2.

Department of Central Clinical Laboratory Osaka Medical College Hospital Takatsuki Osaka Japan.

Background: Hematologic disorders, including myelodysplastic syndrome (MDS), are difficult to identify in routine hematologic examinations using automated hematology analyzers. However, the practical uses of mean platelet component and mean platelet volume (MPV) measured by these analyzers as screening markers for MDS, remain unclear.

Methods: Mean platelet component and MPV values were measured in the peripheral blood of patients with MDS, aplastic anemia, idiopathic thrombocytopenic purpura, myeloproliferative neoplasms, and in healthy controls using an automated hematologic analyzer. Read More

Platelets 2019 Jan 7:1-5. Epub 2019 Jan 7.

a National Medical Research Center for Cardiology , Ministry of Health , Moscow , Russian Federation.

Newborns from mothers with immune thrombocytopenic purpura (ITP) have a risk of thrombocytopenia due to passage of maternal antiplatelet antibodies into fetal/neonatal circulation. We looked for predictors of neonatal thrombocytopenia (nTP) in pregnant women with ITP. One hundred pregnant women with platelet count <100 × 10/l, no non-immune causes of thrombocytopenia and increased platelet associated IgG (PA-IgG) were included in the study. Read More

Cir Cir 2019 ;87(1):23-27

Servicio de Cirugía General, Hospital General de Zona #32 Dr. Mario Madrazo Navarro, Instituto Mexicano del Seguro Social, Ciudad de México, México.

Introduction: Laparoscopic splenectomy was reserved for selected cases, however and because of the improvement in surgical technique and laparoscopic instruments, nowadays, there are few contraindications.

Method: Retrospective and observational study of patients surgically treated of laparoscopic splenectomy secondary to hematologic diseases in a period span of 6 years. We analyzed demographic, preoperative, transoperative and postoperative variables. Read More

Pediatr Dev Pathol 2019 Jan 2:1093526618822108. Epub 2019 Jan 2.

9 Division of Hematology Oncology, Boston Children's Hospital, Boston, Massachusetts.

Germline mutations in RUNX1 result in autosomal dominant familial platelet disorder with associated myeloid malignancy (FPDMM). To characterize the hematopathologic features associated with a germline RUNX1 mutation, we reviewed a total of 42 bone marrow aspirates from 14 FPDMM patients, including 24 cases with no cytogenetic clonal abnormalities, and 18 with clonal karyotypes or leukemia. We found that all aspirate smears had ≥10% atypical megakaryocytes, predominantly characterized by small forms with hypolobated and eccentric nuclei, and forms with high nuclear-to-cytoplasmic ratios. Read More

Maxillofac Plast Reconstr Surg 2018 Dec 10;40(1):39. Epub 2018 Dec 10.

Department of Oral and Maxillofacial Surgery, School of Dentistry, Kyungpook National University, 2177 Dalgubeol-daero, Jung-gu, Daegu, 41940 Republic of Korea.

Background: Patients with chronic ITP (idiopathic thrombocytopenia) frequently do not require comprehensive medication for daily life. Usually, it had been regarded that postoperative bleeding after a simple or surgical extraction is easily controlled by simple local measures even in patients with ITP. This lack of regular medication usage can sometimes lead practitioners or patients to underestimate the potential life-threatening risk of ITP. Read More

CEN Case Rep 2018 Dec 18. Epub 2018 Dec 18.

Department of Obstetrics and Gynecology, Jichi Medical University School of Medicine, 3311-1 Yakushiji, Shimotsuke, 329-0498, Japan.

Measurement of the soluble fms-like tyrosine kinase 1 (sFlt-1)/placental growth factor (PlGF) ratio may be clinically useful to discriminate systemic lupus erythematosus (SLE) from preeclampsia. Here, we present a pregnant woman with new-onset SLE with hypertension, with the measurement of the sFlt-1/PlGF ratio during pregnancy. A 31-year-old Japanese nulliparous woman, who had been diagnosed with idiopathic thrombocytopenic purpura at 10 years, had a systolic blood pressure of 120 mmHg and was negative for proteinuria at 12 weeks. Read More

J Clin Med 2018 Dec 14;7(12). Epub 2018 Dec 14.

2nd Department of General Surgery, Jagiellonian University Medical College, 31-501 Kraków, Poland.

Prediction of intraoperative difficulties may be helpful in planning surgery; however, few studies explored this issue in laparoscopic splenectomy (LS). We performed retrospective analysis of consecutive 468 patients undergoing LS from 1998 to 2017 (295 women; median age 47 years). The patients were divided into difficult LS and control groups. Read More

Eur Rev Med Pharmacol Sci 2018 Dec;22(23):8415-8422

Department of Hematology and Rheumatology, The First Affiliated Hospital of Fujian Medical University, Fuzhou, China.

Objective: To explore the effects of microRNA-212-5p (miR-212-5p) on biological functions of acute myeloid leukemia (AML) and to find the potential molecular mechanism.

Patients And Methods: We measured the expression level of miR-212-5p in 35 AML patients and 20 patients with idiopathic thrombocytopenic purpura (ITP) as control cases. Besides, the miR-212-5p expression at cellular level was checked as well. Read More

J Stroke Cerebrovasc Dis 2018 Dec 12. Epub 2018 Dec 12.

Department of Neurology, Yokohama Rosai Hospital, Yokohama, Kanagawa, Japan.

Background: Although it was suggested that idiopathic thromobocytopenic purpura (ITP) can be a paradoxical cause of cerebral infarction, previous reports indicate that cerebral infarction associated with ITP occurs when thrombocytopenia is already evident at the onset of cerebral infarction.

Case Report: We report a case of multiple cerebral infarction that preceded acute exacerbation of ITP. An 80-year-old woman with a history of ITP presented with tetraplegia, and brain magnetic resonance imaging revealed multiple infarction in bilateral cerebral and cerebellar hemispheres. Read More

Mult Scler Relat Disord 2018 Dec 3;28:31-33. Epub 2018 Dec 3.

Department of Neurology, St Vincent's Hospital, 390 Victoria St, Darlinghurst, Sydney, NSW 2010, Australia.

Secondary autoimmune disorders are a recognised complication of alemtuzumab treatment for multiple sclerosis. We report a case of autoimmune encephalitis manifesting as a polymorphic epilepsia partialis continua / status epilepticus seven months after the second course of alemtuzumab in a patient with previous autoimmune hypothyroidism and immune thrombocytopenic purpura. An MRI revealed multifocal cortical abnormalities and neuronal loss was evident on biopsy. Read More

Clin Lab 2018 Oct;64(11)

Background: X-linked thrombocytopenia (XLT) is a milder form of Wiskott-Aldrich syndrome (WAS), characterized predominantly by thrombocytopenia with small-sized platelets. Mutations in the WAS gene are responsible for the disease. We herein detected a new mutation in the WAS gene responsible for XLT in a 3-generation Chinese pedigree. Read More

Clin Adv Hematol Oncol 2018 Oct;16(10):670-676

Mayo Clinic, Rochester, Minnesota.

Autoimmune hemolytic anemia (AIHA) and immune thrombocytopenia purpura (ITP) have been associated with B-cell lymphoproliferative disorders. Here, we review the epidemiology, pathogenesis, diagnosis, and treatment of these autoimmune disorders, specifically in the setting of B-cell malignancies. AIHA and ITP are classically associated with chronic lymphocytic leukemia (CLL) but have also been reported in plasmacytic and lymphoproliferative disorders. Read More

J Pediatr Hematol Oncol 2019 Jan 4. Epub 2019 Jan 4.

Thalassemia and Hemoglobinopathy Research Center, Research Institute of Health, Ahvaz Jundishapur University of Medical Sciences, Ahvaz, Iran.

Background: Autoimmune thrombocytopenia in immune thrombocytopenic purpura (ITP), thrombotic thrombocytopenic purpura (TTP), and heparin-induced thrombocytopenia (HIT) is associated with immunologic degradation of platelets and reduced platelet counts in patients, leading to bleeding risk in patients. Considering the role of human leukocyte antigens (HLA) in the development of immune response, in this review, we examine the relationship between HLA and pathogenesis of the above-mentioned diseases.

Methods: Relevant English-language literature was searched and retrieved from Google Scholar search engine and PubMed database (1979 to 2018). Read More

Haematologica 2018 Dec 6. Epub 2018 Dec 6.

Laboratory for Thrombosis Research, IRF Life Sciences, KU Leuven Campus Kulak Kortrijk;

In autoantibody-mediated autoimmune diseases, autoantibody profiling allows to stratify patients and link autoantibodies with disease severity and outcome. However, in immune-mediated thrombotic thrombocytopenic purpura patients, stratification according to antibody profiles and their clinical relevance has not been fully explored. We aimed at developing a new type of autoantibody profiling assay for immune-mediated thrombotic thrombocytopenic purpura based on the use of anti-idiotypic antibodies. Read More

Rev Bras Ginecol Obstet 2018 Dec 7;40(12):803-807. Epub 2018 Dec 7.

Department of Anaesthesiology, Centro Hospitalar Lisboa Norte, Lisboa, Portugal.

Thrombocytopenia is the most common hemostatic change in pregnancy, but severe thrombocytopenia is rare. One of the causes, immune thrombocytopenic purpura (ITP), is characterized by increased platelet destruction by immunoglobulin G (IgG) antibodies, presenting a high risk of hemorrhage for the patient, but also for the fetus, since antibodies may cross the placenta. We present the case of a 23-year-old pregnant woman with a history of Langerhans cell histiocytosis of the mandible submitted to surgery and chemotherapy when she was 10 years old, with diagnosis of ITP since then. Read More

J Addict Med 2018 Nov 29. Epub 2018 Nov 29.

Department of Internal Medicine (CC, KR, YG, ZF, MM); Department of Hematology (AP-P); Division of Academic Affairs and Research, Orlando Regional Healthcare, Orlando, FL (SJC).

: Idiopathic thrombocytopenic purpura (ITP) is an autoimmune disorder defined as low platelet count with normal bone marrow in the absence of other causes of thrombocytopenia. It is caused by autoantibodies binding to several platelet surface antigens which lead to premature destruction of the platelet by the reticuloendothelial system. ITP can be primary or secondary, and it is known to be associated with various infections and drugs. Read More

Hematology Am Soc Hematol Educ Program 2018 11;2018(1):568-575

Imperial College Health Care NHS Trust, Hammersmith Hospital, London, UK.

In 1996 and 2011, the American Society of Hematology (ASH) supported efforts to create guidelines for the diagnosis and management of patients with immune thrombocytopenia (ITP). These guidelines used different approaches to arrive at recommendations for testing and treatment. Despite differences in methodology, in both cases there was a paucity of randomized trials to inform recommendations. Read More

Hematology Am Soc Hematol Educ Program 2018 11;2018(1):561-567

Michael G. DeGroote School of Medicine, Department of Medicine and Department of Pathology and Molecular Medicine, McMaster University, Hamilton, ON, Canada.

In this report, we will review the various clinical and laboratory approaches to diagnosing immune thrombocytopenia (ITP), with a focus on its laboratory diagnosis. We will also summarize the results from a number of laboratories that have applied techniques to detect anti-platelet autoantibodies as diagnostic tests for ITP. Although there is considerable variability in methods among laboratories, there is general agreement that platelet autoantibody testing has a high specificity but low sensitivity. Read More

Hematology Am Soc Hematol Educ Program 2018 11;2018(1):539-547

Division of Hematology, Department of Medicine, Johns Hopkins University School of Medicine, Baltimore, MD.

The advent of plasma exchange has dramatically changed the prognosis of acute thrombotic thrombocytopenic purpura (TTP). Recent insights into TTP pathogenesis have led to the development of novel therapies targeting pathogenic anti-ADAMTS13 antibody production, von Willebrand factor (VWF)-platelet interactions, and ADAMTS13 replacement. Retrospective and prospective studies have established the efficacy of rituximab as an adjunct to plasma exchange for patients with acute TTP, either upfront or for refractory disease. Read More

Hematology Am Soc Hematol Educ Program 2018 11;2018(1):530-538

Department of Medicine and Department of Pathology and Laboratory Medicine, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA.

Thrombotic thrombocytopenia purpura (TTP) is a rare, life-threatening disease with an incidence of approximately 2 persons per million per year. It is characterized by severe deficiency of the von Willebrand cleaving protease, ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13), leading to formation of platelet-rich thrombi in the microvasculature. Prompt initiation of appropriate therapy, particularly plasma exchange, may be life-saving. Read More

Hematology Am Soc Hematol Educ Program 2018 11;2018(1):405-411

Department of Pediatrics, Hematology/Oncology Section, Baylor College of Medicine, Houston, TX.

Immune thrombocytopenia (ITP) has historically been thought to occur in 2 distinct forms: childhood ITP and adult ITP. This division is based largely on the presumption that childhood ITP is often benign and self-limited, whereas ITP in adults tends to be more chronic and difficult to treat. Although data exist to justify a different approach to the diagnosis and treatment in young children and the elderly, ITP in older children, adolescents, and younger adults is likely to share more similar pathology. Read More

Vaccine 2019 Jan 30;37(2):366-371. Epub 2018 Nov 30.

Kitasato Institute for Life Sciences, Laboratory of Viral Infection, 5-9-１ Shirokane Minato-ku, Tokyo 108-8641, Japan. Electronic address:

Vaccine adverse events and controversial safety issues have occurred in recent decades in Japan: aseptic meningitis following the measles-mumps-rubella combined vaccine (MMR), anaphylaxis after immunization with live virus vaccines and inactivated split influenza vaccine, an increased incidence of febrile illness following the simultaneous administration of inactivated vaccines, and chronic pain with neurological illness after immunization with the human papilloma virus vaccine (HPV). Vaccine adverse events are a matter of concern for the public as well as general practitioners; some are within the range of assumptions that adverse reactions after live attenuated vaccines are related to the nature of their parental wild-type viruses. Vaccines stimulate the innate immunity of host immunological defense mechanisms and induce the development of specific acquired immunity. Read More

Zhongguo Shi Yan Xue Ye Xue Za Zhi 2018 Dec;26(6):1746-1751

Department of Microbiological immunoplay，Zhanjiang 524023，Guangdong Province, China.

Objective: To detect the expression of JAK2/STAT3 mRNA in peripheral blood T cells from the patients with chronic idiopathic thrombocytopenic purpura（CITP）, and to explore the relationship between JAK2/STAT3 mRNA and CITP.

Methods: CITP group and healthy control group were set in this study, The JAK2/STAT3 mRNA expression level in peropheral blood T cells of 2 groups was detected with the RT-PCR and agarose gel electrophoresis.

Results: JAK2 mRNA expression level in CITP group was significantly higher than that in control group（P<0. Read More

Haematologica 2018 Dec 4. Epub 2018 Dec 4.

Institute of Medical Sciences, Ashgrove Road West, University of Aberdeen, UK

Platelet destruction in immune thrombocytopenia is caused by autoreactive antibody and T cell responses, most commonly directed against platelet glycoprotein IIb/IIIa. Loss of self-tolerance in the disease is also associated with deficient activity of regulatory T cells. Having previously mapped seven major epitopes on platelet glycoprotein IIIa that are recognised by helper T cells from patients with immune thrombocytopenia, the aim was to test whether peptide therapy with any of these sequences, alone or in combination, could inhibit responses to the antigen in humanized mice expressing HLA-DR15. Read More

Med Sci Monit 2018 Dec 4;24:8767-8772. Epub 2018 Dec 4.

Department of Clinical Laboratory, Qilu Hospital, Shandong University, Jinan, Shandong, China (mainland).

BACKGROUND The disequilibrium of T helper (Th) cells play an important role in the occurrence and development of immune thrombocytopenic purpura (ITP). Th22 cells, as a newly discovered subset of T lymphocytes, plays an important role in autoimmune disorders and inflammatory diseases. MATERIAL AND METHODS This study explored the role of different lymphocyte subsets in chronic ITP. Read More

Immunotherapy 2018 Nov 30. Epub 2018 Nov 30.

Grifols Bioscience Research Group, Grifols, Barcelona, Spain.

Aim: To evaluate the safety and efficacy of 10% intravenous immunoglobulin (IVIG; Flebogamma 10% DIF) in individuals with chronic immune thrombocytopenic purpura (ITP).

Patients & Methods: Patients aged 3-70 years, diagnosed with chronic ITP, received 1 g/kg IVIG over two consecutive days.

Results:  64 evaluable patients (51 adults, 13 children) with chronic ITP received IVIG. Read More

BMC Surg 2018 Nov 26;18(1):108. Epub 2018 Nov 26.

Department of Hepatobiliary Surgery, The First Affiliated Hospital of Chongqing Medical University, No.1 Youyi Road, Chongqing, 400016, China.

Background: Laparoscopic splenectomy (LS) is regarded as a second-line treatment for medically refractory idiopathic thrombocytopenic purpura (ITP), but the predictive factors for the long-term postoperative responses to ITP are still a matter of debate. We aimed to investigate the factors that can predict the long-term response after LS for Chinese patients with medically refractory ITP.

Methods: From January 2011 to September 2016, 78 Chinese patients with ITP who underwent LS were retrospectively analyzed. Read More

Transfus Apher Sci 2018 Dec 30;57(6):800-803. Epub 2018 Oct 30.

International Consultancy in Blood Components Quality/Safety, Audit/Injection and DDR Strategies, London, UK. Electronic address:

Although Immune thrombocytopenic purpura is a common disorder that family physicians, internists and hematologists face in their everyday practice, its diagnosis rests only on "exclusion" and its therapy is based on algorithms where "trial and error" is the rule. Flow cytometry, if simplified and standardized, could provide a quicker and better diagnostic accuracy. Studies of the lymphocyte subset using flow cytometry and more elaborate immune studies are paving the way for a better understanding of the disease and in identification of prognostic markers. Read More

Nefrologia 2018 Nov 21. Epub 2018 Nov 21.

Enfermedades Renales Hereditarias, Servicio de Nefrología, Fundació Puigvert, Barcelona, España; Instituto de Investigaciones Biomédicas Sant Pau (IIB-Sant Pau), Barcelona, España; Universidad Autònoma de Barcelona, REDinREN, Instituto de Investigación Carlos III, Barcelona, España. Electronic address:

MYH9 related diseases are caused by mutations in the MYH9 gene and constitute a rare group of genetic entities. Its inheritance follows an autosomal dominant pattern. The MYH9 gene, encodes the nonmuscle myosin heavy chain IIA, expressed in different tissues and especially in podocytes and mesangial cells. Read More

Pediatr Neonatol 2018 Nov 3. Epub 2018 Nov 3.

University of Health Sciences, Ankara Child Health and Diseases Hematology Oncology Training and Research Hospital, Department of Hematology, Ankara, Turkey.

Objective: Immune thrombocytopenic purpura (ITP) is the most common cause of acquired thrombocytopenia children. The aim of this retrospective study is to describe presenting features and clinical characteristics of ITP and evaluate clinical course, treatment modalities, and complications and determine the effects of preceding infection history, age, gender, treatment modality, and admission platelet count on chronicity.

Method: Two hundred and eleven patients who were diagnosed ITP and followed-up in Department of Pediatric Hematology, Ankara Children Hematology Oncology Education and Research Hospital between January 2008 and September 2012 were included. Read More

Therapie 2018 Oct 26. Epub 2018 Oct 26.

London School of Hygiene and Tropical Medicine, LA Risk Research, London EC1R 5BD, United Kingdom.

The systematic case-referent method is a special case-referent design originally developed for pharmacoepidemiologic research purposes. It consists in the systematic collection of series of incident cases of various disorders and the assembling of a general reference pool, from which "controls" are secondarily selected to be matched to specific cases. Both series are collected independently from each other and with no a priori hypothesis to be investigated. Read More

Nat Prod Res 2018 Nov 16:1-5. Epub 2018 Nov 16.

a State Key Laboratory of Critical Technology in Innovative Chinese Medicine, TCM Research Center , Tasly Academy , Tianjin , China.

Qi Bai Granule (QBG), a traditional Chinese medicine formula for the treatment of idiopathic thrombocytopenic purpura, is composed of seven herbs. It is necessary to learn its chemical composition for quality control. In this study, a method for rapid separation and structural identification of the constituents in QBG was established by UPLC-ESI-Q-TOF-MS in negative and positive ion mode. Read More

Case Rep Nephrol 2018 23;2018:6051083. Epub 2018 Oct 23.

Medical student, Javeriana University, Bogota, Colombia.

We had the challenged to treat a 40-year-old female with Systemic Scleroderma who was showing unspecific symptoms. During her time at the hospital she rapidly develops renal dysfunction, associated with hypertension. She required renal replacement therapy initiation and we observed a decline in hemoglobin and platelets numbers. Read More

Front Immunol 2018 25;9:2468. Epub 2018 Oct 25.

Children's Hospital, University of Helsinki and Helsinki University Hospital, Helsinki, Finland.

Mutations in , encoding a non-coding RNA molecule, underlie cartilage-hair hypoplasia (CHH), a syndromic immunodeficiency with multiple pathogenetic mechanisms and variable phenotype. Allergy and asthma have been reported in the CHH population and some patients suffer from autoimmune (AI) diseases. We explored AI and allergic manifestations in a large cohort of Finnish patients with CHH and correlated clinical features with laboratory parameters and autoantibodies. Read More

Gastroenterol Res Pract 2018 9;2018:6090878. Epub 2018 Oct 9.

Division of Hemato-Oncology, Department of Internal Medicine, Kangnam Sacred-Heart Hospital, Hallym University Medical Center, Hallym University College of Medicine, Seoul, Republic of Korea.

Objective: Several recent reviews of published studies have shown that the eradication of infection in patients with ITP improved thrombocytopenia in about half of the cases. However, most included studies were observational case series. We performed the first meta-analysis of randomized trials to gain a better insight into the effect of eradication in ITP patients. Read More

Thromb Res 2018 12 24;172:80-85. Epub 2018 Oct 24.

Center for Research on Health Care Data Center, University of Pittsburgh, Pittsburgh, PA, United States of America; Department of Medicine, University of Pittsburgh, Pittsburgh, PA, United States of America; Division of General Internal Medicine, University of Pittsburgh, Pittsburgh, PA, United States of America; Department of Biostatistics, University of Pittsburgh, Pittsburgh, PA, United States of America.

Background: Immune thrombocytopenia (ITP) is increasingly recognized as a thrombophilic disorder. However, no further investigation of risk factors has been conducted to date. This study evaluated classic and disease-specific correlates of thrombosis among ITP patients. Read More

Medicine (Baltimore) 2018 Nov;97(44):e13025

Hematology Unit, Medical Specialty Center, Bangkok Hospital Hat Yai, Bangkok Dusit Medical Services, Hat Yai, Songkhla, Thailand.

Rationale: Thrombotic thrombocytopenic purpura (TTP) and hemophagocytic lymphohistiocytosis (HLH) are rare hematologic conditions and have high mortality. Both TTP and HLH result from deregulation of the immune system. There are no published reports of coexisting TTP and HLH in elderly patients. Read More

Indian J Hematol Blood Transfus 2018 Oct 2;34(4):711-718. Epub 2018 Apr 2.

1Division of Hematology, The Second Affiliated Hospital of Shantou University Medical College, Dongxia Road North, Shantou, 515041 Guangdong Province China.

To observe the differences in proteins between adult patients with chronic immune thrombocytopenic purpura (ITP) and healthy adults. 30 patients with chronic ITP and 30 healthy controls were enrolled into the study. The platelet total protein was extracted from peripheral venous blood of 10 chronic ITP patients and 10 healthy controls respectively, and subjected to two-dimensional electrophoresis (2-DE) to find the differential protein spot between chronic ITP patients and healthy controls, then the differential protein spots were identified by mass spectrometry. Read More

Indian J Hematol Blood Transfus 2018 Oct 5;34(4):707-710. Epub 2018 Feb 5.

Adana Numune Research and Education Hospital, Pediatric Hematology-Oncology, Adana, Turkey.

Child patients of chronic thrombocytopenic purpura with severe and resistant thrombocytopenia were evaluated to observe whether their clinical or laboratory states improve by one of the thrombomimetic therapeutic agent called Eltrombopag as in adults in a single center of different country from previous studies. Nineteen patients with chronic immune thrombocytopenia were treated with Eltrombopag to dose in international guidelines. Approximately half (11/19:58%) of the patients benefitted from the treatment with Eltrombopag either by an increase of platelet levels at safe levels with a decrease in the frequency of bleedings which needed rescue treatment. Read More

Hematology 2019 Dec 25;24(1):134-138. Epub 2018 Oct 25.

a Department of Medicine , Padova University School of Medicine , Padova , Italy.

Objectives: The relevance of detecting antibodies against anticardiolipin, β2-glycoprotein I (β2gpI) or lupus anticoagulant (LA), collectively called antiphospholipid autoantibodies (APA), in subjects with immune thrombocytopenia (ITP) is still a debated issue. In particular, whether APA profile may affect the clinical course of ITP is unknown.

Methods: In this study, we report our experience in a cohort of ITP patients with APA with specific interest to the relevance of different antiphospholipid antibody profiles in clinical outcome and response to treatment. Read More

Arterioscler Thromb Vasc Biol 2018 Nov;38(11):2731-2743

From the Division of Laboratory Medicine, Department of Pathology, The University of Alabama at Birmingham.

Objective- ADAMTS13 (a disintegrin and metalloprotease with thrombospondin type 1 repeats-13) cleaves VWF (von Willebrand factor). This process is essential for hemostasis. Severe deficiency of plasma ADAMTS13 activity, most commonly resulting from autoantibodies against ADAMTS13, causes thrombotic thrombocytopenic purpura. Read More

Hum Vaccin Immunother 2018 Oct 23:1-10. Epub 2018 Oct 23.

a Department of Clinical Immunology and Immunotherapy , Medical University of Lublin , Lublin , Poland.

Splenectomy is a surgery indicated in case of splenic rupture after injury, when there are tumors in the spleen, or as a treatment for certain diseases, such as idiopathic thrombocytopenic purpura and spherocytosis. The aims of the study were to assess the immunological response to the Haemophilus influenzae type b (Hib) vaccine and the post-vaccination changes in lymphocyte subsets and cell activation markers in splenectomized patients and healthy volunteers. Blood samples were collected from 25 patients that had undergone splenectomy and from 15 healthy, non-splenectomized volunteers. Read More

Res Pract Thromb Haemost 2018 Oct 8;2(4):640-652. Epub 2018 Oct 8.

Institute of Cardiovascular Sciences College of Medical and Dental Sciences University of Birmingham Birmingham UK.

Background: Inherited thrombocytopenias (ITs) are a heterogeneous group of disorders characterized by low platelet counts and often disproportionate bleeding with over 30 genes currently implicated. Previously the UK-GAPP study using whole exome sequencing (WES) identified a pathogenic variant in 19 of 47 (40%) patients of which 71% had variants in genes known to cause IT.

Aims: To employ a targeted next-generation sequencing platform to improve efficiency of diagnostic testing and reduce overall costs. Read More

J Blood Med 2018 10;9:171-184. Epub 2018 Oct 10.

Department of Hematology, Internal Medicine Division, Dr José E González University Hospital, School of Medicine of the Universidad Autónoma de Nuevo León, Monterrey, Nuevo León, México,

Evans syndrome (ES) is a rare and chronic autoimmune disease characterized by autoimmune hemolytic anemia and immune thrombocytopenic purpura with a positive direct anti-human globulin test. It is classified as primary and secondary, with the frequency in patients with autoimmune hemolytic anemia being 37%-73%. It predominates in children, mainly due to primary immunodeficiencies or autoimmune lymphoproliferative syndrome. Read More