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Cureus 2018 Nov 4;10(11):e3541. Epub 2018 Nov 4.

Hematology / Oncology, The Brooklyn Hospital Center, Brooklyn, USA.

Breast tumors have a predilection for metastasizing to the bone leading to cells being displaced by the cancer cells subsequently producing immature leukocytes and erythrocytes in the peripheral blood. We present a case of a 57-year-old female who was found to have myelophthisic anemia secondary to stage four lobular breast carcinoma metastasized to the bone marrow after being misdiagnosed as having thrombotic thrombocytopenia purpura. Diagnosis of myelophthisic anemia requires a thorough workup and treatment is based upon secondary management of the malignancy. Read More

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

Zhonghua Er Ke Za Zhi 2019 Jan;57(1):50-54

Hematology Oncology Center, Beijing Children's Hospital, Capital Medical University, Beijing 100045, China.

To explore the clinical features and points of diagnosis and treatment for congenital thrombotic thrombocytopenic purpura (TTP) in children. The clinical manifestations, laboratory tests, genetic analysis and treatments of 5 children with congenital TTP hospitalized in Beijing Children's Hospital, Capital Medical University from February 2015 to July 2017 were analyzed retrospectively. Among the 5 children with congenital TTP diagnosed by genetic monitoring and enzymology, there were 1 male and 4 females, 3 cases had suspicious positive family history, the age of onset was several hours after birth (range several hours after birth to 28 months). 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

J Thromb Haemost 2019 Jan 10. Epub 2019 Jan 10.

Department of Pathophysiology and Transplantation, Università degli Studi di Milano, Milan, Italy.

Background: Severe ADAMTS13 deficiency has been recognized as the main risk factor for recurrence of thrombotic thrombocytopenic purpura (TTP). Several conditions including surgery may influence the levels of ultra large Von Willebrand factor and ADAMTS13, acting as a trigger for an acute TTP event.

Objectives: To report our experience for management of six patients affected with acquired TTP who underwent elective surgery after a prophylactic treatment to restore ADAMTS13 activity levels. Read More

Cardiol Res 2018 Dec 7;9(6):392-394. Epub 2018 Dec 7.

Oxford Heart Centre, Oxford University Hospitals NHS Foundation Trust, Oxford, UK.

Thrombocytopenia is a recognized complication following aortic valve replacement (AVR). While post-operative thrombotic thrombocytopenic purpura (TTP) is less common than heparin-induced thrombocytopenia (HIT), it is associated with high mortality and morbidity and prompt diagnosis and treatment is vital. In this case report, we describe the first reported case of TTP after AVR using the trifecta bio-prosthesis. 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

World J Cardiol 2018 Dec;10(12):254-266

Division of Cardiology, Lang Research Center, New York Presbyterian Medical Group - Queens Hospital, Flushing, NY 11355, United States.

Thrombotic thrombocytopenic purpura (TTP) is a multisystem disorder that essentially can affect any organ in the human body. The hallmark of the pathogenesis in TTP is the large von Willebrand factor multimers on platelet-mediated micro-thrombi formation, leading to microvascular thrombosis. Autopsy studies showed that cardiac arrest and myocardial infarction are the most common immediate causes of death in these patients. 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

J Thromb Haemost 2018 Dec 29. Epub 2018 Dec 29.

Hemostasis Branch, Division of Plasma Protein Therapeutics, Office of Tissues and Advanced Therapies, Center for Biologics Evaluation and Research, US FDA, Silver Spring, MD, 20993.

Von Willebrand Factor (VWF) and its cleaving protease ADAMTS-13 (A Disintegrin and Metalloproteinase with Thrombospondin type 1 motif, member 13) are essential components to hemostasis. These plasma proteins have also been implicated in number of disease states, including those affecting children. The best described abnormality is the congenital form of Thrombotic Thrombocytopenic Purpura (TTP) resulting from germline mutations in the ADAMTS-13 gene. Read More

Medicine (Baltimore) 2018 Dec;97(52):e13737

Department of Molecular and Biomedical Sciences, Jožef Stefan Institute, Ljubljana, Slovenia.

Rationale: Two clinical cases are reported of envenomation by the nose-horned viper (Vipera ammodytes ammodytes) venom of a 9-year-old boy and of an 84-year-old woman.

Patient Concerns: Both patients had been bitten on their extremities by such a snake in August near Split, a town in southern Croatia.

Diagnoses: Clinical manifestation of envenomation was severe in the case of the boy, being characterized by a severe coagulopathy. 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

Arterioscler Thromb Vasc Biol 2019 Jan;39(1):e29

Nursing 2019 Jan;49(1):29-30

BMC Infect Dis 2018 Dec 22;18(1):691. Epub 2018 Dec 22.

National Hospital of Sri Lanka, Colombo 10, Sri Lanka.

Background: Leptospirosis is a zoonosis commonly prevalent in tropical countries. Clinical course of leptospirosis varies from mild to severe disease. Here we present a case of leptospirosis complicated with Guillain-Barre Syndrome (GBS), papillitis, and Thrombotic Thrombocytopenic Purpura(TTP). Read More

Pediatr Nephrol 2018 Dec 19. Epub 2018 Dec 19.

Division of Nephrology, Department of Pediatrics, Faculty of Medicine, Erciyes University, Kayseri, Turkey.

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

Thromb J 2018 13;16:35. Epub 2018 Dec 13.

Department of Molecular Medicine and Haematology, Faculty of Health Sciences, University of Witwatersrand and National Health Laboratory Service, Office 3B20, 7 York Road, Parktown, Johannesburg, 2191 South Africa.

Background: The thrombotic microangiopathies (TMAs) is a heterogeneous group of relatively uncommon but serious disorders presenting with thrombocytopenia and microangiopathic haemolysis. Thrombotic thrombocytopenic purpura (TTP) is one of these microangiopathic processes. HIV infection is an acquired cause of TTP but the pathogenesis is poorly understood. 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

Transfus Apher Sci 2018 Dec 5. Epub 2018 Dec 5.

Department of Haematology and Cell Biology, Faculty of Health Sciences, University of the Free State, Bloemfontein, South Africa; National Health Laboratory Service (NHLS), Universitas Hospital Haematology Academic Laboratories, Bloemfontein, South Africa.

Objective: Thrombotic thrombocytopenic purpura (TTP) results from a deficiency in the Von Willebrand factor (VWF) cleaving protease, ADAMTS13. Treatment involves plasma exchange (PEX) therapy with either fresh frozen plasma (FFP), cryosupernatant (CSP) or solvent/detergent-treated plasma (SDP), available in South Africa as Bioplasma FDP. The aim of the study was to generate in vitro data on these products, and to explore possible differences between the products that may offer treatment advantages. 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

Transfusion 2018 Dec 12. Epub 2018 Dec 12.

Department of Internal Medicine, College of Medicine, Department of Biostatistics & Epidemiology, College of Public Health, University of Oklahoma Health Sciences Center, Oklahoma City, Oklahoma.

Background: Appropriate management to prevent relapses of acquired, autoimmune thrombotic thrombocytopenic purpura (TTP) is not clear. Rituximab (375 mg/m /week × 4) is effective treatment for acute episodes but it is not consistently effective for prevention of relapses. Maintenance rituximab, 375 mg/m /3 months for 2 years, is commonly used to prevent progression of follicular lymphoma, but the outcome of maintenance rituximab to prevent TTP relapses has been rarely reported. Read More

Trans R Soc Trop Med Hyg 2018 Dec 13. Epub 2018 Dec 13.

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

Background: Hump-nosed pit vipers (Hypnale hypnale) are highly venomous snakes found in Sri Lanka and the Western Ghats region of India. They are the most common cause of venomous snakebites in Sri Lanka, mainly causing local envenoming leading to pain, swelling and necrosis. Systemic envenoming causing acute kidney injury and coagulopathy are more commonly recognized following their bites. 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

J Clin Apher 2019 Feb 8;34(1):44-50. Epub 2018 Dec 8.

Department of Molecular Medicine and Haematology, Faculty of Health Sciences, National Health Laboratory Service, University of the Witwatersrand, Johannesburg, South Africa.

Introduction: Thrombotic thrombocytopenic purpura (TTP) is associated with high mortality if not managed timeously with therapeutic plasma exchange (TPE). TTP secondary to human immunodeficiency virus (HIV) infection is unique to sub-Saharan Africa. The management and outcome of TTP in the era of improved access to therapy has not been described. 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

Nursing 2019 Jan;49(1):22-29

Vincent M. Vacca, Jr., is an adjunct faculty member at Massachusetts College of Pharmacy and Health Sciences in Boston, Mass.

Acquired autoimmune thrombotic thrombocytopenic purpura (TTP)-the most common form of TTP-is a life-threatening hematologic disease characterized by hemolytic anemia and thrombocytopenia. Acquired autoimmune TTP can cause signs and symptoms of neurologic and other organ involvement, with mortality approaching 90% if the disease is not promptly recognized and treated. Since the introduction of plasma exchange in 1991, the acquired autoimmune TTP survival rate has increased to 78%. 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):548-552

Hematology-Oncology Section, Department of Medicine, College of Medicine, University of Oklahoma Health Sciences Center, Oklahoma City, OK.

Although risk for relapse may be the greatest concern following recovery from acquired, autoimmune thrombotic thrombocytopenic purpura (TTP), there are multiple other major health issues that must be recognized and appropriately addressed. Depression may be the most common disorder following recovery from TTP and may be the most important issue for the patient's quality of life. Severe or moderate depression has occurred in 44% of Oklahoma Registry patients. 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

Postgrad Med 2018 Dec 4. Epub 2018 Dec 4.

a Department of Pediatric Rheumatology , Cukurova University Faculty of Medicine , Adana , Turkey.

Objectives: Henoch Schönlein Purpura (HSP) is characterized by non-thrombocytopenic palpable purpura, abdominal pain and arthralgia/arthritis. We aimed to describe the clinical presentations of children with HSP in a single center and compare the prevalence of each manifestations between patients with MEFV variants, particularly in exon 10 and those without.

Methods: This cohort retrospectively included 144 HSP (59 females, 85 males) patients without FMF symptoms and followed for at least 6 months. Read More

Cureus 2018 Sep 25;10(9):e3358. Epub 2018 Sep 25.

Internal Medicine, Brookdale University Hospital, New York, USA.

Atypical hemolytic uremic syndrome (aHUS) is a kidney disorder that is frequently unrecognized during its progression, and misdiagnosed with more common etiologies of microangiopathic hemolytic anemia (MAHA): hemolytic uremic syndrome, disseminated intravascular coagulation, and thrombotic thrombocytopenic purpura (TTP). During pregnancy, the diagnosis of aHUS is furthermore challenging. The clinical presentation of aHUS may mimic pre-eclampsia as it occurred to the patient described in the case report. Read More