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Sante Publique 2018 September October;30(5):671-677

Objective: In the obstetric medicine movement and in response to requests for appointments in internal medicine by obstetricians and midwives, we created an internal medicine consultation within the maternity ward of our General Hospital, and provide feedback after 1 year.

Methods: This retrospective descriptive study took place at the Robert Ballanger Intercommunal Hospital Center in Aulnay-sous-Bois in Seine-Saint-Denis (France) between 3rd March 2016 and 9th March 2017, the first year of the internal medicine consultation, one afternoon every 15 days, in the maternity level 2b.

Results: Out of 121 appointments, 93 consultations were conducted for 63 patients. Read More

BMJ Case Rep 2019 Jan 17;12(1). Epub 2019 Jan 17.

Medicine (Nephrology), Jacobi Medical Center / Albert Einstein College of Medicine, New York City, New York, USA.

Pregnancy-induced atypical haemolytic uremic syndrome (P-aHUS) is a rare condition characterised by microangiopathic haemolytic anaemia, thrombocytopenia and renal failure. It accounts for approximately 7% of total HUS cases. Here, we present a case of recurrent P-aHUS in a 25-year-old Hispanic woman. Read More

Horm Mol Biol Clin Investig 2019 Jan 17. Epub 2019 Jan 17.

Department of Obstetrics and Gynecology, Sibu Hospital, Ministry of Health Malaysia, 96000 Sibu, Sarawak, Malaysia.

Severe placental abruption is associated with high maternal and perinatal morbidity and mortality. Prompt delivery is usually mandatory in this situation. We report a case of a 33-year-old woman, gravida 5, para 3 + 1, at 26 weeks + 3 days' gestation who had severe placental abruption involving 40% of the placental surface complicated with maternal moderate anaemia and thrombocytopenia. Read More

Acta Med Indones 2018 Oct;50(4):343-345

Department of Internal Medicine, Faculty of Medicine, Padjadjaran University, Bandung, Indonesia.

A 38-year-old woman presented with general weakness and vaginal bleeding. One month prior, she had been diagnosed with Evans syndrome (haemolytic anemia with positive Coombs test and thrombocytopenia) and was given oral steroid as maintenance therapy. Her serology examination was negative for hepatitis B, hepatitis C, and human immunodeficiency virus (HIV). Read More

Mol Genet Metab Rep 2019 Mar 4;18:19-21. Epub 2019 Jan 4.

Mount Sinai Hospital, University of Toronto, Toronto, ON, Canada.

Gaucher disease (GD) is one of the commonest lysosomal storage diseases that is inherited in an autosomal recessive manner and affects 1 in 50,000 to 100,000 people in the general population. The frequency is much higher (1 in 500 to 1000) in people of Ashkenazi Jewish heritage due to a founder effect. GD is caused by decreased or absent activity of β-glucosidase with subsequent accumulation of the substrate glucosylceramide in macrophages due to genetic alterations in the gene. 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

Transfus Apher Sci 2018 Dec 16;57(6):790-792. Epub 2018 Nov 16.

Department of Medicine and Bioregulatory Science, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan; Department of Molecular Endocrinology and Metabolism, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University, Tokyo, Japan.

Upshaw-Schulman syndrome (USS) is an inherited type of thrombotic thrombocytopenic purpura (TTP) that is extremely rare, but often diagnosed during pregnancy. Reversible cerebral vasoconstriction syndrome (RCVS) is the transient stenosis of several cerebral arteries that is frequently diagnosed post-partum. We describe a 28-year-old woman with USS complicated by RCVS after delivery that was treated by plasma exchange with a good outcome. Read More

Eur J Paediatr Neurol 2018 Nov 11;22(6):989-1005. Epub 2018 Sep 11.

Departments of Child Health, Obstetrics and Gynaecology and Radiology, University Hospital Southampton, United Kingdom; Clinical and Experimental Sciences, University of Southampton, United Kingdom.

Fetal stroke is an important cause of cerebral palsy but is difficult to diagnose unless imaging is undertaken in pregnancies at risk because of known maternal or fetal disorders. Fetal ultrasound or magnetic resonance imaging may show haemorrhage or ischaemic lesions including multicystic encephalomalacia and focal porencephaly. Serial imaging has shown the development of malformations including schizencephaly and polymicrogyra after ischaemic and haemorrhagic stroke. Read More

Medicine (Baltimore) 2018 Oct;97(43):e12698

Department of Gynaecology.

Rationale: Placental site trophoblastic tumor (PSTT) is a rare form of gestational trophoblastic neoplasm (GTN).

Patient Concerns: A 28-year-old Chinese female with initial presentation of thrombotic microangiopathy (TMA) with symptoms of edema of the lower extremities and eyelid, thrombocytopenia and anemia.

Diagnoses: The primary diagnosis was chronic glomerulonephritis according to the related laboratory tests and clinical symptoms. Read More

J Atheroscler Thromb 2019 Feb 2;26(2):99-110. Epub 2018 Nov 2.

Division of Nephrology and Endocrinology, the University of Tokyo Hospital.

Atypical hemolytic uremic syndrome (aHUS) is a type of thrombotic microangiopathy (TMA) defined by thrombocytopenia, microangiopathic hemolytic anemia, and renal failure. aHUS is caused by uncontrolled complement activation in the alternative pathway (AP). A variety of genetic defects in complement-related factors or acquired autoantibodies to the complement regulators have been found in 50 to 60% of all cases. Read More

Microorganisms 2018 Oct 23;6(4). Epub 2018 Oct 23.

Laboratorio de Fisiopatogenia, Instituto de Fisiología y Biofísica Bernardo Houssay (IFIBIO Houssay-CONICET), Departamento de Fisiología, Facultad de Medicina, Universidad de Buenos Aires, Paraguay 2155, Buenos Aires 1121, Argentina.

Gastrointestinal infection with Shiga toxin-producing (STEC) causes diarrhea, hemorrhagic colitis, and hemolytic uremic syndrome (HUS), characterized by hemolytic anemia, thrombocytopenia and acute renal failure. The main virulence factor of STEC is Shiga toxin (Stx), which is responsible for HUS development. STEC can produce Stx type 1 and/or 2 (Stx1, Stx2) and their variants, Stx2 being more frequently associated with severe cases of HUS. Read More

Ther Apher Dial 2019 Feb 29;23(1):4-21. Epub 2018 Oct 29.

Division of Nephrology, Department of Pediatrics, All India Institute of Medical Sciences, New Delhi, India.

Atypical hemolytic uremic syndrome (aHUS), a rare variant of thrombotic microangiopathy, is characterized by microangiopathic hemolytic anemia, thrombocytopenia, and renal impairment. The condition is associated with poor clinical outcomes with high morbidity and mortality. Atypical HUS predominantly affects the kidneys but has the potential to cause multi-organ system dysfunction. Read More

BMC Res Notes 2018 Oct 5;11(1):704. Epub 2018 Oct 5.

Pharmacy Department, College of Health Sciences, Debre Markos University, P. O. Box, 269, Debre Markos, Ethiopia.

Objective: The aim of the study was to determine level of anemia and other hematological profiles in pregnant women attending antenatal care clinic in Debre Berhan Referral Hospital, Ethiopia.

Results: Prevalence of anemia was 2.8% and that of thrombocytopenia was 10. Read More

Rheum Dis Clin North Am 2018 11 7;44(4):635-649. Epub 2018 Sep 7.

Division of Renal Diseases and Hypertension, Department of Medicine, George Washington University, 2150 Pennsylvania Avenue, Washington, DC 20037, USA. Electronic address:

Thrombotic microangiopathies are heterogeneous disorders characterized by microangiopathic hemolytic anemia with thrombocytopenia and renal injury. There are a variety of causes, including metabolic disorders, infections, medications, complement disorders, pregnancy, malignancy, and autoimmune disorders. This review focuses on renal thrombotic microangiopathy in the setting of rheumatologic diseases. Read More

BMC Infect Dis 2018 Sep 27;18(1):485. Epub 2018 Sep 27.

Department of Obstetrics and Gynecology, School of Medical Sciences, University of Campinas, Campinas, Brazil.

Background: Antiretroviral therapy (ART) use in pregnancy presents unquestionable benefits in preventing mother-to-child transmission (MTCT) of HIV although it is associated with maternal adverse effects. The aim of this study was to evaluate the adverse effects of antiretroviral therapy in pregnant women infected with HIV.

Methods: Cohort study of pregnant women infected with HIV followed at the CAISM/UNICAMP Obstetric Clinic from 2000 to 2015. Read More

Acta Med Litu 2018 ;25(2):61-65

Department of Obstetrics and Gynaecology, Faculty of Medicine, Vilnius University, Vilnius, Lithuania.

Background: Uterine rupture at the site of a previous caesarean scar with abnormal placental penetration through the uterus wall with bladder invasion is a rare and serious pregnancy complication. Our aim was to report a case of uterine rupture with placenta percreta complicated by thrombotic microangiopathy.

Materials And Methods: We did a literature review and analysed medical documentation retrospectively. Read More

Vasa 2018 10 12;47(6):451-464. Epub 2018 Sep 12.

1 Division of Angiology, East Bavarian Center of Vascular Medicine, University Hospital Regensburg, Regensburg, Germany.

Antiphospholipid syndrome (APS) is an autoantibody-mediated acquired thrombophilia. It is characterized by the presence of antiphospholipid antibodies (APL) that are directed against phospholipid-binding plasma proteins, such as beta-2-glycoprotein I (b2GPI). Its main manifestations are recurrent vascular thromboses (so-called "thrombotic APS") and pregnancy complications ("obstetric APS"). Read More

Transfus Med Rev 2018 10 23;32(4):225-229. Epub 2018 Aug 23.

Department of Medicine: Hematology/Oncology, Massachusetts General Hospital, Boston, MA. Electronic address:

Thrombocytopenia is a common hematologic issue encountered by obstetricians and hematologists, detected in about 10% of all pregnancies. In the vast majority of cases, the thrombocytopenia will be attributed to gestational thrombocytopenia (GT), where the thrombocytopenia is mild, does not necessitate active management, and does not introduce maternal or fetal bleeding risk. Although GT is common, the specific mechanism responsible for it is not known with certainty, and therefore, differentiating it from other causes of thrombocytopenia can be challenging. Read More

Transfus Med Rev 2018 10 18;32(4):230-236. Epub 2018 Aug 18.

Department of Haematology, UCLH, Cardiometabolic Programme NIHR UCLH/UCL BRC, London, UK. Electronic address:

Thrombotic microangiopathies (TMAs) are associated with microangiopathic hemolytic anemia and thrombocytopenia, resulting in microvascular thrombosis and end-organ damage. In pregnancy, this may be the result of pregnancy-related TMAs such as preeclampsia; hemolysis, elevated liver enzymes, and low platelets; or pregnancy-associated TMAs, specifically thrombotic thrombocytopenic purpura (TTP) or complement-mediated hemolytic uremic syndrome (CM HUS). TTP and CM HUS are rare disorders, and their diagnosis may be missed, no less because features at presentation may be misdiagnosed as a pregnancy-related TMA, such as hypertension, proteinuria, fetal growth restriction, or in utero fetal death. Read More

Transfus Med Rev 2018 10 23;32(4):203-204. Epub 2018 Aug 23.

Massachusetts General Hospital, Boston, MA. Electronic address:

Rheumatology (Oxford) 2018 07;57(suppl_5):v18-v25

Division of Rheumatic Diseases, University of Texas Southwestern, Dallas, TX, USA.

This article describes three complicated cases in rheumatology and pregnancy. The first case elucidates the challenges in treating SLE in conjunction with pulmonary arterial hypertension, while the second case features an SLE-affected pregnancy with development of portal hypertension secondary to portal vein thrombosis related to APS. The third case is a pregnant woman with stable SLE who developed thrombotic microangiopathy caused by atypical haemolytic uraemic syndrome, and failed to improve despite multiple measures including biopsy and elective preterm delivery. Read More

Medicine (Baltimore) 2018 Aug;97(33):e11748

Department of Obstetrics and Gynecology, The First Affiliated Hospital of Anhui Medical University.

This study aims to investigate major complications or symptoms of pregnant women, causes of maternal near-miss, and issues that are relevant to severe maternal disease.A retrospective analysis was performed in the "maternal individual investigation form," which included all critical maternity patients admitted to the First Affiliated Hospital of Anhui Medical University from January 1, 2012 to September 31, 2015.A total of 14,014 pregnant patients who delivered at 28 to 42 weeks of gestation were included. Read More

Clin Med (Lond) 2018 Aug;18(4):335-339

Oxford University Hospitals NHS Foundation Trust, Oxford, UK

The autoimmune cytopenias are a group of disorders resulting primarily from autoantibody-mediated destruction of blood cells, with variable clinical sequelae depending on the severity and lineage affected. Disease presentation ranges from an asymptomatic finding on a routine full blood count to an acutely unwell patient suffering the clinical consequences of severe anaemia, neutropenia or thrombocytopenia. The cytopenia may be primary or secondary to underlying infectious, immune or malignant processes. Read More

Transfus Med Rev 2018 10 18;32(4):220-224. Epub 2018 Jun 18.

Department of Pathology and Laboratory Medicine, Emory University School of Medicine, Atlanta, GA.

The role of prophylactic transfusion therapy for the treatment of sickle cell disease during pregnancy is unclear. An analysis of the existing literature shows a limited number of publications that address this issue and specifically compare clinical outcomes in this population based on a treatment strategy of prophylactic transfusion versus transfusion only for clinical indications (on-demand transfusion). The existing studies show a wide variation in study design and outcomes measured. Read More

Am J Trop Med Hyg 2018 Sep 12;99(3):620-622. Epub 2018 Jul 12.

National Institute of Infectious Diseases IRCCS Lazzaro Spallanzani, Rome, Italy.

Congenital malaria (CM) is uncommon in both malaria-endemic and non-endemic countries. It may be caused by any spp., although and are the more frequent etiologic agents. Read More

BMC Hematol 2018 9;18:15. Epub 2018 Jul 9.

3School of Medical Laboratory Science, College of Health science, Addis Ababa University, Addis Ababa, Ethiopia.

Background: In pregnancy, hematological changes occur in order to meet the demands of the developing fetus and placenta, with major alterations in blood volume. Abnormal hematological profile affects pregnancy and its outcome. This study aimed to assess hematological profiles of pregnant women at a tertiary care teaching hospital. Read More

J Med Case Rep 2018 Jun 6;12(1):158. Epub 2018 Jun 6.

University Obstetrics Unit, De Soysa Hospital for Women, Colombo, Sri Lanka.

Background: Thrombotic thrombocytopenic purpura and peripartum cardiomyopathy are potentially lethal complications of pregnancy. We describe a case in which both of these developed in the same patient. The etiologies of both conditions remain uncertain, but they share immune hyperreactivity as a possible cause. Read More

J Med Case Rep 2018 Jun 1;12(1):147. Epub 2018 Jun 1.

Faculty of Medicine, University of Jordan and Jordan University Hospital, PO Box 2194, Amman, 11941, Jordan.

Background: Incidences of immune thrombocytopenic purpura occur in 1 in every 1000-10,000 pregnancies accounting for 3% of all thrombocytopenic pregnancies. A pre-existing immune thrombocytopenic purpura is known to be a risk factor for developing thrombocytopenia during pregnancy. We present here the treatment regime and management of a patient with known immune thrombocytopenic purpura who developed postpartum thrombotic thrombocytopenia with atypical response to traditional therapy. Read More

Reprod Health 2018 May 10;15(1):76. Epub 2018 May 10.

Department of Obstetrics and Gynecology, School of Medical Sciences, University of Campinas, Campinas, Brazil.

Background: Antiretroviral therapy (ART) in pregnancy was associated with a drastic reduction in HIV mother-to-child transmission (MTCT), although it was associated with neonatal adverse effects. The aim of this study was to evaluate the neonatal effects to maternal ART.

Methods: This study was a cohort of newborns from HIV pregnant women followed at the CAISM/UNICAMP Obstetric Clinic from 2000 to 2015. Read More

BMC Pregnancy Childbirth 2018 May 9;18(1):143. Epub 2018 May 9.

Clinical Trials Unit, London School of Hygiene and Tropical Medicine, Keppel Street, London, WC1E 7HT, UK.

Background: Early treatment with tranexamic acid reduces deaths due to bleeding after post-partum haemorrhage. We report the prevalence of haematological, coagulation and fibrinolytic abnormalities in Nigerian women with postpartum haemorrhage.

Methods: We performed a secondary analysis of the WOMAN trial to assess laboratory data and rotational thromboelastometry (ROTEM) parameters in 167 women with postpartum haemorrhage treated at University College Hospital, Ibadan, Nigeria. Read More

Medicine (Baltimore) 2018 Apr;97(17):e0401

Department of Obstetrics and Prenatal Medicine.

Rational: Lupus enteritis is a rare, severe complication of systemic lupus erythematosus (SLE). We report of a patient who presented with enteritis as manifestation of new-onset SLE during the first trimester of pregnancy.

Patients Concerns: The 23-year nulliparous patient was admitted to a district hospital with abdominal pain, nausea, vomiting and bloody diarrhea at a gestational age (GA) of 10 weeks. Read More

Pregnancy Hypertens 2018 Apr 16;12:29-34. Epub 2018 Feb 16.

Department of Obstetrics and Gynecology, Cedars-Sinai Medical Center, Los Angeles, CA, United States. Electronic address:

Thrombotic microangiopathy (TMA) disorders are characterized by microangiopathic hemolytic anemia, thrombocytopenia and end-organ injury. In pregnancy and postpartum, TMA is most commonly encountered with HELLP (hemolysis, elevated liver enzymes, low platelet count syndrome) or preeclampsia with severe features, but rarely TMA is due to thrombotic thrombocytopenic purpura (TTP) or atypical hemolytic uremic syndrome (aHUS). Due to overlapping clinical and laboratory features, TTP and aHUS are often mistaken for preeclampsia or HELLP. Read More

Arthritis Care Res (Hoboken) 2019 Jan;71(1):134-141

National and Kapodistrian University of Athens, Athens, Greece.

Objective: Although systemic lupus erythematosus (SLE) is the most common autoimmune disease associated with antiphospholipid antibodies (aPL), limited data exist regarding the impact of SLE on the clinical phenotype of aPL-positive patients. The primary objective of this study was to compare the clinical, laboratory, and treatment characteristics of aPL-positive patients with SLE with those of aPL-positive patients without SLE.

Methods: A secure web-based data capture system was used to store patient demographic characteristics and aPL-related clinical and laboratory characteristics. Read More

Transfusion 2018 07 17;58(7):1583-1587. Epub 2018 Apr 17.

Department of Obstetrics and Gynecology, Beth Israel Deaconess Medical Center, and Harvard Medical School, Boston, Massachusetts.

Background: Platelet (PLT) refractoriness presents a challenging problem for transfusion support, especially in the perioperative setting, where there is urgency for human leukocyte antigen (HLA)-compatible units, yet identification and provision of compatible PLT concentrates requires time.

Case Report: A 22-year-old G3P1 woman with thrombocytopenia due to aplastic anemia, likely autoimmune, presented in her third trimester for peripartum care and newly diagnosed HLA alloimmune PLT refractoriness. Despite early planning, the patient developed bleeding requiring urgent delivery. Read More

Blood Coagul Fibrinolysis 2018 Apr;29(3):330-337

Department of Medicine, University of California Irvine School of Medicine, Irvine, California, USA.

: 'Disseminated intravascular coagulation (DIC)' occurs commonly in critical illnesses such as sepsis, trauma, cancer, and complications of surgery and pregnancy. Mortality is very high. The pathogenesis has been ascribed to tissue factor-initiated coagulation disorder, resulting in disseminated microblood clots that are made of platelets, plasma factors, fibrins, and blood cells. Read More

Intern Med J 2018 06;48(6):624-636

Department of Medicine, University of Melbourne, Melbourne, Victoria, Australia.

Thrombotic microangiopathy (TMA) arises in a variety of clinical circumstances with the potential to cause significant dysfunction of the kidneys, brain, gastrointestinal tract and heart. TMA should be considered in all patients with thrombocytopenia and anaemia, with an immediate request to the haematology laboratory to look for red cell fragments on a blood film. Although TMA of any aetiology generally demands prompt treatment, this is especially so in thrombotic thrombocytopenic purpura (TTP) and atypical haemolytic uraemic syndrome (aHUS), where organ failure may be precipitous, irreversible and fatal. Read More

Lancet Glob Health 2018 05 20;6(5):e548-e554. Epub 2018 Mar 20.

Barts Research Centre for Women's Health, WHO Collaborating Centre, Queen Mary University of London, London, UK.

Background: Anaemia affects as many as half of all pregnant women in low-income and middle-income countries, but the burden of disease and associated maternal mortality are not robustly quantified. We aimed to assess the association between severe anaemia and maternal death with data from the WHO Multicountry Survey on maternal and newborn health.

Methods: We used multilevel and propensity score regression analyses to establish the relation between severe anaemia and maternal death in 359 health facilities in 29 countries across Latin America, Africa, the Western Pacific, eastern Mediterranean, and southeast Asia. Read More

Lakartidningen 2018 03 16;115. Epub 2018 Mar 16.

Hematologiskt Centrum, Karolinska Universitetssjukhuset - Stockholm, Sweden Hematologiskt Centrum, Karolinska Universitetssjukhuset - Stockholm, Sweden.

J Obstet Gynaecol 2019 Jan 14;39(1):118-119. Epub 2018 Mar 14.

a Department of Obstetrics , First Affiliated Hospital of China Medical University , Shenyang , China.

Vaccine 2018 03 23;36(13):1728-1735. Epub 2018 Feb 23.

Laboratorio de Fisiopatogenia, Departamento de Fisiología, Instituto de Fisiología y Biofísica Bernardo Houssay (IFIBIO Houssay-CONICET), Facultad de Medicina, Universidad de Buenos Aires, Paraguay 2155, 1121 Buenos Aires, Argentina. Electronic address:

E. coli O157:H7 is a foodborne pathogen responsible for bloody diarrhea, hemorrhagic colitis and hemolytic uremic syndrome (HUS). The objective of the present work was to evaluate the ability of colostral IgG obtained from Stx2-immunized cows to prevent against E. Read More

Eur J Haematol 2018 Jun 6;100(6):560-566. Epub 2018 Apr 6.

Department of Obstetrics and Gynaecology, University Hospital Waterford, Waterford, Ireland.

Thrombocytopenia, defined as a platelet count less than 150 000 per microlitre, occurs in 7%-12% of all pregnancies. Apart from anaemia, it is the most common haematological disorder in pregnancy. Despite its frequent occurrence, thrombocytopenia often leads to difficulties of diagnosis and management in pregnancy. Read More

J Obstet Gynaecol Can 2018 Feb;40(2):e134-e141

Toronto, ON.

Objectives: To review the principles of prenatal diagnosis of congenital cytomegalovirus (CMV) infection and to describe the outcomes of the affected pregnancies.

Outcomes: Effective management of fetal infection following primary and secondary maternal CMV infection during pregnancy. Neonatal signs include intrauterine growth restriction (IUGR), microcephaly, hepatosplenomegaly, petechiae, jaundice, chorioretinitis, thrombocytopenia and anemia, and long-term sequelae consist of sensorineural hearing loss, mental retardation, delay of psychomotor development, and visual impairment. Read More

Medicine (Baltimore) 2018 Feb;97(7):e9895

Lucian Blaga University of Sibiu, Faculty of Medicine Sibiu, Romania.

Rationale: Haemolytic-uremic syndrome is a rare but serious complication of bacterial and viral infections, which is characterized by the triad of: acute renal failure, microangiopathic haemolytic anemia and thrombocytopenia, sometimes severe, requiring peritoneal dialysis. In Europe, hemolytic-uremic syndrome (HUS) in paediatric pathology is primarily caused by Shiga toxin-producing Escherichia coli (STEC) O157, followed by O26. Beside these etiologies, there are other bacterial and viral infections, and also noninfectious ones that have been associated to lead to HUS as well: in the progression of neoplasia, medication-related, post-transplantation, during pregnancy or associated with the antiphospholipid syndrome, systemic lupus erythematosus or family causes with autosomal dominant or recessive inheritance. Read More

Nephrology (Carlton) 2018 Jun;23(6):507-517

Department of Medicine, University of Melbourne, Melbourne, Victoria, Australia.

Thrombotic microangiopathy (TMA) arises in a variety of clinical circumstances with the potential to cause significant dysfunction of the kidneys, brain, gastrointestinal tract and heart. TMA should be considered in all patients with thrombocytopenia and anaemia, with an immediate request to the haematology laboratory to look for red cell fragments on a blood film. While TMA of any aetiology generally demands prompt treatment, this is especially so in thrombotic thrombocytopenic purpura (TTP) and atypical haemolytic uraemic syndrome (aHUS), where organ failure may be precipitous, irreversible and fatal. Read More

J Med Case Rep 2018 Jan 22;12(1):15. Epub 2018 Jan 22.

Department of Internal Medicine, Federal University of Pernambuco, Haematology, Av. Prof. Moraes Rego 1235, 50670-90, Recife, Brazil.

Background: Thrombotic thrombocytopenic purpura is a very rare hereditary blood deficiency disorder of ADAMTS13 (von Willebrand factor-cleaving protease) and a life-threatening thrombotic microangiopathy characterized by thrombocytopenia and microangiopathic hemolytic anemia. The deficiency in ADAMTS13 metalloprotease, which cleaves the von Willebrand factor, may be congenital or acquired. The congenital form is caused by inherited mutations in the ADAMTS13 gene. Read More

BMJ Case Rep 2018 Jan 17;2018. Epub 2018 Jan 17.

Department of Internal Medicine, Michigan State University, Lansing, Michigan, USA.

Thrombotic thrombocytopenic purpura (TTP) is a potentially reversible, life-threatening medical emergency. We present a case of a 21-year-old female with evidence of haemolytic anaemia based on the presence of positive markers of haemolysis. Negative Coomb's test, thrombocytopenia and placental infarcts raised suspicion for a thrombotic microangiopathy. Read More