Imerslund-Gr äsbeck Syndrome presenting with microangiopathic hemolytic anemia in a child.
We report a child presenting with TMA features with cobalamin deficiency. Because of her family history of vitamin B12 deficiency and proteinuria, the performed genetic analysis revealed that an Imerslund-Gräsbeck Syndrome with the detection of a homozygous mutation in AMN gene. PMID: 32045704 [PubMed - as supplied by publisher] (Source: European Journal of Medical Genetics)
Source: European Journal of Medical Genetics - February 8, 2020 Category: Genetics & Stem Cells Authors: Gurlek Gokcebay D, Akpinar Tekgunduz S, Cavdarli B Tags: Eur J Med Genet Source Type: research

Typical and Atypical Hemolytic Uremic Syndrome in the Critically Ill
Hemolytic uremic syndrome is characterized by microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury. Disseminated intravascular coagulation, thrombotic thrombocytopenic purpura, and hemolytic uremic syndrome have a similar clinical presentation. Diagnostic needs to be prompt to decrease mortality, because identifying the different disorders can help to tailor specific, effective therapies. However, diagnosis is challenging and morbidity and mortality remain high, especially in the critically ill population. Development of clinical prediction scores and rapid diagnostic tests for hemolytic uremic synd...
Source: Critical Care Clinics - January 31, 2020 Category: Intensive Care Authors: Carlos L. Manrique-Caballero, Sadudee Peerapornratana, Cassandra Formeck, Gaspar Del Rio-Pertuz, Hernando Gomez, John A. Kellum Source Type: research

Improved Outcomes of Transplant Associated Thrombotic Microangiopathy with Early Initiation of Eculizumab
Transplant-associated thrombotic microangiopathy (TA-TMA) is a rare, potentially lethal complication of allogeneic stem cell transplant. It is characterized by microangiopathic hemolytic anemia, thrombocytopenia, and renal dysfunction. Endothelial injury and terminal complement activation contribute to the pathogenesis of TA-TMA. Several risk factors have been identified including certain conditioning regimens, acute graft versus host disease (GvHD) and/or calcineurin inhibitors (CNI), and viral infections. (Source: Biology of Blood and Marrow Transplantation)
Source: Biology of Blood and Marrow Transplantation - January 24, 2020 Category: Hematology Authors: Muhammad Sardar, Emmanuel Katsanis, Keri Renee Maher Tags: 181 Source Type: research

Atypical hemolytic uremic syndrome: a case report
ConclusionThe atypical hemolytic uremic syndrome is a rare disease entity requiring a high index of suspicion to diagnose. It is a diagnosis of exclusion. Early diagnosis with prompt treatment will render a better outcome. The atypical hemolytic uremic syndrome needs to be considered in all patients with thrombotic microangiopathy. (Source: Journal of Medical Case Reports)
Source: Journal of Medical Case Reports - January 13, 2020 Category: General Medicine Source Type: research

Vitamin B12 Deficiency and Metabolism-Mediated Thrombotic Microangiopathy (MM-TMA)
Thrombotic microangiopathies (TMA) are characterized by microangiopathic hemolytic anemia, thrombocytopenia and organ damage resulting from mechanical factors, accumulation of the ultra-large von Willebrand factor multimers or complement-mediated abnormalities. Severe acquired vitamin B12 (Cobalamin - Cbl) deficiency or congenital defective Cbl metabolism could lead to a picture that mimics TMA. The later has been termed metabolism-mediated TMA (MM- TMA). This confusing picture is mediated partly by the large red cell fragmentation coupled with reduced platelet production in the absence of vitamin B12 and partly by the acc...
Source: Transfusion and Apheresis Science - December 31, 2019 Category: Hematology Authors: Waleed Sabry, Mohamed Elemary, Thierry Burnouf, Jerard Seghatchian, Hadi Goubran Tags: Review Source Type: research

Thrombotic Thrombocytopenic Purpura Associated with Pesticides: A Report of 4 Cases and Literature Review
Thrombotic thrombocytopenic purpura (TTP) is a disease characterized by the presence of microangiopathic hemolytic anemia (MAHA) and thrombocytopenia, caused by the congenital or acquired decrease of the enzyme activity which degrades unusual large vWF multimers. There is no identifiable cause in half of the acquired TTP cases. Herein, we report four possible pesticide-related cases with decreased ADAMTS13 enzyme activity, increased titer of ADAMTS13 inhibitor and typical clinical and laboratory presentation (Source: Transfusion and Apheresis Science)
Source: Transfusion and Apheresis Science - December 13, 2019 Category: Hematology Authors: Volkan Karaku ş, Egemen Kaya, Gizem Zorlu Görgülügil, Yelda Dere, Erdal Kurtoğlu Source Type: research

Congenital thrombotic thrombocytopenic purpura in a large cohort of patients carrying a novel mutation in ADAMTS13 gene
Congenital thrombotic thrombocytopenic purpura (cTTP; Upshaw-Schulman syndrome) is a life-threatening disorder caused by mutations in the ADAMTS-13 gene, that interfere with enzyme production or function and lead to increased amounts of highly active large VWF multimers, resulting in microangiopathic hemolytic anemia (MAHA), thrombocytopenia and disseminated thrombosis. (Source: Thrombosis Research)
Source: Thrombosis Research - December 5, 2019 Category: Hematology Authors: Oleg Pikovsky, Maram Arafat, Hilla Ovadia, Yoav Sharoni, Kayed Al-Athamen, Bibi Kanengisser-Pines, Anat Keren-Politansky, Itai Levi, Offer Erez, Ruti Parvari, Anat Rabinovich Tags: Letter to the Editors-in-Chief Source Type: research

Pregnancy Prolongation After Eculizumab Use in Early-Onset Preeclampsia.
CONCLUSION: Eculizumab may prolong pregnancy in early-onset preeclampsia. Additional research is needed to assess short-term and long-term maternal and newborn outcomes. PMID: 31764731 [PubMed - in process] (Source: Obstetrics and Gynecology)
Source: Obstetrics and Gynecology - November 27, 2019 Category: OBGYN Authors: Lu AB, Lazarus B, Rolnik DL, Palmer KR Tags: Obstet Gynecol Source Type: research

Therapeutic plasma exchange in thrombotic thrombocytopenic purpura.
po P Abstract Thrombotic thrombocytopenic purpura (TTP) is a life-threatening disease related to the formation of microvascular thrombosis and subsequent organ failure. The disease is accompanied with microangiopathic haemolytic anaemia, consumptive thrombocytopenia and lies on a severe deficiency in ADAMTS13, the von Willebrand factor-cleaving protease. In the acquired, immune-mediated form, this deficiency is due to the production of autoantibodies directed against the enzyme. Therapeutic plasma exchange has been used empirically for decades and still represents the cornerstone of TTP treatment. However, a bette...
Source: Presse Medicale - November 20, 2019 Category: General Medicine Authors: Picod A, Provôt F, Coppo P Tags: Presse Med Source Type: research

Vitamin B12 deficiency presenting as pseudo-thrombotic microangiopathy: a case report and literature review.
Authors: Fahmawi Y, Campos Y, Khushman M, Alkharabsheh O, Manne A, Zubair H, Haleema S, Polski J, Bessette S Abstract Pseudo-thrombotic microangiopathy (pseudo-TMA) is a recognized, yet uncommon, clinical presentation of vitamin B12 deficiency. Patients with pseudo-TMA present with microangiopathic hemolytic anemia (MAHA), thrombocytopenia and schistocytes. They are often misdiagnosed as thrombotic thrombocytopenia purpura (TTP) and receive unnecessary therapy. Here, we report a case of a 60-year-old male who presented with thrombocytopenia and normocytic normochromic anemia. Anemia work-up was remarkable for severe B12 deficiency (
Source: Clinical Pharmacology: Advances and Applications - November 8, 2019 Category: Allergy & Immunology Tags: Clin Pharmacol Source Type: research

Pregnancy Prolongation After Eculizumab Use in Early-Onset Preeclampsia.
CONCLUSION: Eculizumab may prolong pregnancy in early-onset preeclampsia. Additional research is needed to assess short-term and long-term maternal and newborn outcomes. PMID: 31698377 [PubMed - as supplied by publisher] (Source: Obstetrics and Gynecology)
Source: Obstetrics and Gynecology - November 4, 2019 Category: OBGYN Authors: Lu AB, Lazarus B, Rolnik DL, Palmer KR Tags: Obstet Gynecol Source Type: research

Extrarenal manifestations of the hemolytic uremic syndrome associated with Shiga toxin-producing Escherichia coli (STEC HUS)
AbstractHemolytic uremic syndrome is commonly caused by Shiga toxin-producingEscherichia coli (STEC). Up to 15% of individuals with STEC-associated hemorrhagic diarrhea develop hemolytic uremic syndrome (STEC HUS). Hemolytic uremic syndrome (HUS) is a disorder comprising of thrombocytopenia, microangiopathic hemolytic anemia, and acute kidney injury. The kidney is the most commonly affected organ and approximately half of the affected patients require dialysis. Other organ systems can also be affected including the central nervous system and the gastrointestinal, cardiac, and musculoskeletal systems. Neurological complicat...
Source: Pediatric Nephrology - October 29, 2019 Category: Urology & Nephrology Source Type: research

368PClinicopathological characteristics, survival and prognostic factors of breast cancer-related microangiopathic haemolytic anemia: A multicenter study
AbstractBackgroundCancer-related microangiopathic haemolytic anemia (MAHA) is a rare but life-threatening paraneoplastic syndrome. Only case reports or very small series (2 (OR  = 6.0, CI95% [1.8; 19.8]), one or more prior lines of treatment (OR = 2.9, [0.9; 8.7]), elevated bilirubin (OR = 5.5, [1.5; 20.6]), haemoglobin
Source: Annals of Oncology - October 1, 2019 Category: Cancer & Oncology Source Type: research

Anticomplement therapies in “secondary thrombotic microangiopathies”: ready for prime time?
The term thrombotic microangiopathy (TMA) refers to a syndrome consisting of  microangiopathic hemolytic anemia, thrombocytopenia, and organ dysfunction with normal coagulation parameters.1 The designations “primary” and “secondary” to TMA are arbitrary and reflect underlying mechanisms. The primary TMA syndromes include thrombotic thrombocytopenic purpura related t o a deficiency of the von Willebrand factor protease ADAMTS13, diarrhea-associated hemolytic uremic syndrome associated with infection with Shiga toxin producing enteric organisms, and complement-mediated TMA or atypical hemolytic u...
Source: Kidney International - September 19, 2019 Category: Urology & Nephrology Authors: Jai Radhakrishnan Tags: Controversies in Nephrology Source Type: research

Thrombotic microangiopathy: An under-recognised cause of snake-bite-related acute kidney injury
Conclusions: In conclusion, TMA was seen in 18.5% of patients with snake-bite-related AKI in our study and was associated with almost universal need for RRT, longer duration on RRT, and hospital stay compared with patients without TMA. (Source: Indian Journal of Nephrology)
Source: Indian Journal of Nephrology - September 11, 2019 Category: Urology & Nephrology Authors: Indu Ramachandra Rao Attur Ravindra Prabhu Shankar Prasad Nagaraju Dharshan Rangaswamy Source Type: research

A case of acute pancreatitis-induced microangiopathic hemolytic anemia with thrombocytopenia
We report a case of acute pancreatitis induced MAHA-T with end organ dysfunction and a normal ADAMTS13 level. Following three TPEs, the patient ’s clinical status, blood counts and hemolytic markers stabilized. Improvement occurred even after TPE was discontinued. The optimal management of AP-induced MAHA-T is poorly understood. Many centres are reporting good outcomes with the early initiation of TPE. MAHA-T associated with acute pancrea titis is often treated with early initiation of TPE. However, the value of TPE in altering the natural history of the condition is not well understood. Further study is required to ...
Source: Journal of Thrombosis and Thrombolysis - September 6, 2019 Category: Hematology Source Type: research

Distribution of transplantation-associated thrombotic microangiopathy (TA-TMA) and comparison between renal TA-TMA and intestinal TA-TMA: autopsy study
Thrombotic microangiopathy (TMA) is a syndrome caused by small vessel endothelial injury and clinically characterized by microangiopathic hemolytic anemia, elevated serum lactate dehydrogenase level, thrombocytopenia, and multiorgan injury [1]. Multiple factors can be the cause of a primary TMA [1]. ADAMTS13 deficiency-mediated TMA or Shiga toxin-mediated TMA are representative, and they have been familiarly called the thrombotic thrombocytopenic purpura (TTP) and Shiga toxin-related hemolytic uremic syndrome (ST-HUS), respectively [2,3]. (Source: Biology of Blood and Marrow Transplantation)
Source: Biology of Blood and Marrow Transplantation - September 3, 2019 Category: Hematology Authors: Rin Yamada, Tetsuo Nemoto, Kazuteru Ohashi, Akiko Tonooka, Shin-ichiro Horiguchi, Toru Motoi, Tsunekazu Hishima Source Type: research

Snakebite  associated thrombotic microangiopathy: a protocol for the systematic review of clinical features, outcomes, and role of interventions
DiscussionIt is anticipated that eligible studies will consist of small observational studies. Evidence gathered from this review will provide the first broader understanding of the clinical features, outcomes, prognosis, and management of snakebite-associated thrombotic microangiopathy.Systematic review registrationPROSPERO CRD42019121436 (Source: Systematic Reviews)
Source: Systematic Reviews - August 22, 2019 Category: International Medicine & Public Health Source Type: research

Patient Experience with Congenital (Hereditary) Thrombotic Thrombocytopenic Purpura: A Conceptual Framework of Symptoms and Impacts
ConclusionsThe final conceptual model, which includes salient symptoms and impacts of congenital thrombotic thrombocytopenic purpura and reflects the disease burden, was derived by integrating inputs from the literature review, expert opinion, and patient interviews, and will be used to develop a congenital thrombotic thrombocytopenic purpura –specific, patient-reported outcome instrument. (Source: The Patient - Patient-Centered Outcomes Research)
Source: The Patient - Patient-Centered Outcomes Research - July 30, 2019 Category: International Medicine & Public Health Source Type: research

TTP-like syndrome associated with Hemoglobin SC disease treated successfully with plasma and red cell exchange
ConclusionAlthough literature showed therapeutic plasma exchange alone can abrogate a similar clinical scenario, we did an in-depth review which concluded that in most of the TMA cases secondary to sickle cell disease, treatment with both with plasma exchange and red cell exchange transfusion are necessary. (Source: Leukemia Research Reports)
Source: Leukemia Research Reports - July 23, 2019 Category: Hematology Source Type: research

Caplacizumab to treat immune-mediated thrombotic thrombocytopenic purpura.
Authors: Poullin P, Bornet C, Veyradier A, Coppo P Abstract Immune-mediated thrombotic thrombocytopenic purpura (iTTP) is a rare and life-threatening disease characterized by microangiopathic hemolytic anemia, thrombocytopenia and multiorgan failure, resulting from autoantibody-mediated severe A disintegrin and metalloproteinase with thrombospondin motifs 13 (ADAMTS13) deficiency. In spite of treatment with plasma exchange and immunosuppression, patients remain at risk of exacerbations, refractoriness and death. Caplacizumab (Cablivi; Ablynx, a Sanofi company), a nanobody targeting von Willebrand factor (vWF), has ...
Source: Drugs of Today - June 30, 2019 Category: Drugs & Pharmacology Tags: Drugs Today (Barc) Source Type: research

Diclofenac-induced thrombotic thrombocytopenic purpura with concomitant complement dysregulation: a case report and review of the literature
ConclusionThrombotic thrombocytopenic purpura is a rare condition that can be acquired. Our case is rare because it represents the first report of diclofenac potassium-induced thrombotic thrombocytopenic purpura with subjacent complement activation and dysregulation. Early recognition and aggressive management led to a favorable outcome. (Source: Journal of Medical Case Reports)
Source: Journal of Medical Case Reports - June 23, 2019 Category: General Medicine Source Type: research

The Case | Acute kidney injury in an African woman with microangiopathic hemolytic anemia and HIV
A 59-year-old African woman was admitted with generalized weakness. The history consisted of hypertension, which was treated with lisinopril and nifedipine for many years. She denied any use of nonsteroidal anti-inflammatory drugs, herbal medications, or illicit drugs. Physical examination revealed normal vital signs, dry mucous membranes, and no evidence of lymphadenopathy, salivary gland enlargement, or rash. Laboratory evaluation revealed a serum creatinine level of 12.9 mg/dl and a blood urea nitrogen level of 149 mg/dl (baseline, 1.1 and 23 mg/dl, respectively, 3 months ago), a hemoglobin level of 8.2 g/dl, a platelet...
Source: Kidney International - June 19, 2019 Category: Urology & Nephrology Authors: Hatem Elabd, Tarek Rashid, Mennallah Elkholi, M. Barry Stokes Tags: Make Your Diagnosis Source Type: research

An update on pathogenesis and diagnosis of thrombotic thrombocytopenic purpura.
Authors: Joly BS, Coppo P, Veyradier A Abstract INTRODUCTION: Thrombotic thrombocytopenic purpura (TTP) is a rare and life-threatening thrombotic microangiopathy characterized by microangiopathic hemolytic anemia, consumption thrombocytopenia, and organ injury. TTP pathophysiology is based on a severe ADAMTS13 deficiency, the specific von Willebrand factor (VWF)-cleaving protease. Areas covered: VWF/ADAMTS13 interaction is specific, and ADAMTS13 conformation has been elucidated recently. ADAMTS13 deficiency is congenital or acquired. Several targets are used in TTP treatment. Expert opinion: Differential diagnosis ...
Source: Expert Review of Hematology - May 22, 2019 Category: Hematology Tags: Expert Rev Hematol Source Type: research

Deleterious Impact of a Novel CFH Splice Site Variant in Atypical Hemolytic Uremic Syndrome
In conclusion, genetic investigation for pathogenic variants and evaluation of their functional impact, in particular in the case of splice site variants, is clinically relevant and enables not only better molecular understanding but helps to guide therapy with complement inhibitors. (Source: Frontiers in Genetics)
Source: Frontiers in Genetics - May 15, 2019 Category: Genetics & Stem Cells Source Type: research

IJERPH, Vol. 16, Pages 1649: Management of STEC Gastroenteritis: Is There a Role for Probiotics?
Mauro Nicola Laforgia Shiga toxin-producing Escherichia Coli (STEC) infections routinely run as a common gastroenteritis, but in many cases they may evolve towards hemolytic uremic syndrome (HUS). HUS is a rare disease characterized by microangiopathic hemolytic anemia, thrombocytopenia, and acute renal failure. Gut microorganisms have a fundamental impact on human physiology, because they modulate normal intestinal functions and play a pivotal role in influencing the local and systemic immune responses. Despite surveillance established in many countries and major progresses in the understanding of STEC-HUS mechanism...
Source: International Journal of Environmental Research and Public Health - May 12, 2019 Category: Environmental Health Authors: Mario Giordano Maria Elisabetta Baldassarre Viviana Palmieri Diletta D. Torres Vincenza Carbone Luisa Santangelo Federico Gentile Raffaella Panza Federica Di Mauro Manuela Capozza Antonio Di Mauro Nicola Laforgia Tags: Review Source Type: research

Rare Functional Variants in Complement Genes and Anti-FH Autoantibodies-Associated aHUS
In conclusion, we have confirmed in our cohort of patients, the strong association between FHR1 deficiency and aHUS with anti-FH autoantibodies. Through an innovative approach based on the comparison with “supercontrols” carrying the homozygous CFHR1 deletion, identified by screening a large number of healthy adult subjects, we have documented that patients with anti-FHs aHUS are enriched in complement gene LPVs. This observation indicates that the pathogenesis of anti-FHs aHUS is complex and multiple “hits” are required for its clinical manifestation. We also document that the CFH H3 and the CD46GG...
Source: Frontiers in Immunology - May 1, 2019 Category: Allergy & Immunology Source Type: research

Thrombotic Microangiopathy in Cancer
Semin Thromb Hemost DOI: 10.1055/s-0039-1687893Thrombotic microangiopathy (TMA) is a rare but often devastating complication of cancer and cancer treatment. The syndrome is defined by thrombocytopenia (i.e., a platelet count of  30% decrease from baseline), microangiopathic hemolytic anemia, and some evidence of organ damage. Among the nine recognized groups of disorders causing TMA, the focus of this article will be on cancer and cancer treatment-related causes of TMA. This review will discuss the pathophysiology of TMA in cancer, chemotherapy-associated TMA, transplant-associated TMA, and newer therapeutic ...
Source: Seminars in Thrombosis and Hemostasis - April 30, 2019 Category: Hematology Authors: Weitz, Ilene Ceil Tags: Review Article Source Type: research

Clinical features and prognosis of patients with thrombotic thrombocytopenic purpura associated with systemic lupus erythematosus: a review of 25 cases
ConclusionPatients with TTP-SLE often had moderate to severe lupus disease activity. Testing of LDH level and blood smear should be performed when kidney and neurological symptoms arise in children with SLE. The use of combination therapy, glucocorticoids plus immunosuppressive agent, provided satisfactory clinical outcome. Patients with refractory TTP-SLE will also need plasma exchange therapy. (Source: Italian Journal of Pediatrics)
Source: Italian Journal of Pediatrics - April 29, 2019 Category: Pediatrics Source Type: research

Thrombotic microangiopathy in hematotoxic snakebites and its impact on the prognosis: an entity often overlooked
AbstractSnakebite associated thrombotic microangiopathy (TMA) is a spectrum of disorders characterized by microangiopathic hemolytic anemia, thrombocytopenia and acute kidney injury (AKI). We carried out this study to find out the prevalence of TMA in hematotoxic snake envenomation and to analyze its impact on the clinical outcome of patients. Retrospective data were collected from the medical records, hospital and lab information system after institutional ethics committee approval. Hematotoxic snake bite patients were categorized into Group 1 (with TMA) and Group 2 (without TMA). Chi square test, Mann –Whitney &lsq...
Source: Journal of Thrombosis and Thrombolysis - April 26, 2019 Category: Hematology Source Type: research

The Case | Microangiopathic hemolytic anemia in a young female
A 23-year-old female presented with headache, a blood pressure of 220/130 mm  Hg, and a creatinine level of 698 μmol/l, elevated from 87 μmol/l 6 months ago. She had microangiopathic hemolytic anemia, a platelet count of 75 × 109/l, a hemoglobin level of 95 g/l, and a lactate dehydrogenase level of 768 IU/l. Schistocytes were present on the peripheral blood smear, and the haptoglobin level was low (0.8 g/l). There was no history of diarrhea, viral illnesses, or new medications. Her urine dipstick demonstrated microscopic hematuria (degree of positivity [+]) and proteinuria (++; not formally quant...
Source: Kidney International - April 19, 2019 Category: Urology & Nephrology Authors: Haresh Selvaskandan, Minal Karavadra, Kethesparan Paramesparan, Michael Glasby, Osasuyi Iyasere Tags: Make Your Diagnosis Source Type: research

Circulating Heme Oxygenase-1 and Complement Activation in Transplant-Associated Thrombotic Microangiopathy
Transplant-associated thrombotic microangiopathy (TA-TMA) is a severe complication in patients following hematopoietic stem cell transplantation (HSCT). Previous investigations suggest that multiple factors resulting in endothelial cell injury lead to the occurrence of TA-TMA. Specifically, the abnormity of complement system contributes to the development of TA-TMA. TA-TMA shares a similar clinical manifestation with thrombotic thrombocytopenic purpura (TTP) and atypical hemolytic uremic syndrome (aHUS), which is characteristic with microangiopathic hemolytic anemia, platelet consumption, fibrin deposition in the microcirc...
Source: Biology of Blood and Marrow Transplantation - March 11, 2019 Category: Hematology Authors: Tingting Pan, Jiaqian Qi, Tao You, Shiyu Han, Liping Yang, Wenjing Miao, Depei Wu, Changgeng Ruan, Li Zhu, Yue Han Source Type: research

Seizure as the Presenting Symptom for Atypical Hemolytic Uremic Syndrome
Atypical hemolytic uremic syndrome (aHUS) is a complement-mediated disease manifesting in thrombocytopenia, microangiopathic hemolytic anemia, and acute kidney injury. It has a higher incidence of extrarenal manifestations, including central nervous system findings like seizure or stroke, pancreatitis, and cardiac manifestations. (Source: The Journal of Emergency Medicine)
Source: The Journal of Emergency Medicine - February 27, 2019 Category: Emergency Medicine Authors: Sandy Chan, Adam R. Weinstein Tags: Clinical Communications: Pediatric Source Type: research

Blood film says it all! Rare case of congenital TTP misdiagnosed as ITP
We report a case of a 16-year-old female who presented with bleeding diathesis. Peripheral blood film examination was consistent with microangiopathic hemolytic anemia with 7% fragmented red blood cells. The ADAMTS13 level was 40 ng/ml (reference range: 630–850 ng/ml). She responded to plasma exchange therapy and methylprednisolone and was discharged in a stable condition. (Source: International Journal of Pediatrics and Adolescent Medicine)
Source: International Journal of Pediatrics and Adolescent Medicine - February 21, 2019 Category: Pediatrics Source Type: research

An interesting case of catastrophic antiphospholipid syndrome
We present a rare case of catastrophic antiphospholipid syndrome which resulted in a rare complication of microangiopathic haemolytic anaemia requiring intensive care, prolonged inpatient stay and plasmophoresis. (Source: European Journal of Obstetrics, Gynecology, and Reproductive Biology)
Source: European Journal of Obstetrics, Gynecology, and Reproductive Biology - February 19, 2019 Category: OBGYN Authors: Tabassum Khan, Aisha Janjua, Shalini Patni, Bee Tan Tags: Movies Technique 3 and Extra Case Reports – Obstetrics Source Type: research

Catastrophic Antiphospholipid Syndrome Presenting as Congestive Heart Failure in a Patient with Thrombotic Microangiopathy.
We describe the case of a 46-year-old woman who presented with acute congestive heart failure as a manifestation of catastrophic antiphospholipid syndrome, which is characterized by rapidly progressing multiorgan involvement. Targeted therapy improved our patient's cardiomyopathy and saved her life. Increased recognition of thrombotic microangiopathy as an underlying pathophysiologic mechanism in heart failure and initiation of timely treatment may help to prevent death in patients with thrombotic microangiopathy. PMID: 30833839 [PubMed - in process] (Source: Texas Heart Institute Journal)
Source: Texas Heart Institute Journal - February 1, 2019 Category: Cardiology Authors: Tulai IM, Penciu OM, Raut R, Rudinskaya A Tags: Tex Heart Inst J Source Type: research

Thrombotic Microangiopathy after Pediatric Allogeneic Stem Cell Transplant: Potential Early Markers to Predict Individuals at High-Risk
Transplant associated thrombotic microangiopathy (TMA) is a severe complication of stem cell transplant (SCT), characterized by endothelial damage leading to microangiopathic hemolytic anemia, thrombocytopenia and organ damage. Mild cases of TMA do not require treatment, but severe cases are associated with organ damage and mortality. Soluble suppression of tumorigenicity-2 (ST2) is a member of the interleukin 1 receptor family and has been associated with cardiac as well as endothelial injury. Elevated soluble terminal complement complex (sC5b-9) has recently been shown to be associated with high-risk TMA. (Source: Biolog...
Source: Biology of Blood and Marrow Transplantation - January 31, 2019 Category: Hematology Authors: Christopher E Dandoy, Audrey Stegman, Abigail R Pate, Ava Stendahl, Priscila Badia Alonso, Sonata Jodele, Adam Lane, Stella M Davies Tags: 58 Source Type: research

Shiga toxin triggers endothelial and podocyte injury: the role of complement activation
AbstractShiga toxin (Stx)-producingEscherichia coli (STEC) is the offending agent in post-diarrhea-associated hemolytic uremic syndrome (HUS), a disorder characterized by thrombocytopenia, microangiopathic hemolytic anemia, and acute kidney failure, with thrombi occluding the renal microvasculature. Endothelial dysfunction has been recognized as the trigger event in the development of microangiopathic processes. Glomerular endothelial cells are susceptible to the toxic effects of Stxs that, via nuclear factor kappa B (NF- κB) activation, induce the expression of genes encoding for adhesion molecules and chemokines, c...
Source: Pediatric Nephrology - January 28, 2019 Category: Urology & Nephrology Source Type: research

Hemolytic Anemia of Malignancy: A Case Study Involving Signet Ring Cell Metastatic Breast Cancer with Severe Microangiopathic Hemolytic Anemia
Hemolytic anemia in the setting of malignancy is a rare manifestation of paraneoplastic syndrome with significant morbidity. Here we discuss a case involving metastatic breast cancer presenting with severe hemolytic anemia and renal failure secondary to thrombotic microangiopathy of malignancy. This case discusses the workup for secondary hemolytic anemia, a possible role for therapeutic plasma exchange in this setting, as well the current understanding of the management of microangiopathic hemolytic anemia of malignancy.Case Rep Oncol 2019;12:104 –108 (Source: Case Reports in Oncology)
Source: Case Reports in Oncology - January 27, 2019 Category: Cancer & Oncology Source Type: research

When the diagnosis is difficult to digest: severe vitamin B 12 deficiency secondary to pernicious anemia mimicking life-threatening thrombotic thrombocytopenic purpura
AbstractThrombotic thrombocytopenic purpura (TTP) is an uncommon yet life-threatening condition, usually marked by a classic pentad of microangiopathic hemolytic anemia (MAHA), thrombocytopenia, acute kidney injury (AKI), neurological disturbances, and fevers. Prompt recognition and treatment is essential to reduce morbidity and mortality. The rarity and variability of presentations, however, makes this a difficult diagnosis to make reliably, and in many cases, treatment is initiated empirically before the appropriate diagnostic testing is completed. After TTP and other common causes of thrombotic microangiopathy (TMA) hav...
Source: Journal of Hematopathology - January 11, 2019 Category: Pathology Source Type: research

Generation of a core set of items to develop classification criteria for Scleroderma renal crisis using consensus methodology
ConclusionsA core set of items that characterize SRC was identified using consensus methodology. This core set will be used in future data ‐driven phases of this project to develop classification criteria for SRC.This article is protected by copyright. All rights reserved. (Source: Arthritis and Rheumatology)
Source: Arthritis and Rheumatology - January 7, 2019 Category: Rheumatology Authors: Emily ‐Ann Butler, Murray Baron, Agnes B. Fogo, Tracy Frech, Cybele Ghossein, Eric Hachulla, Sabrina Hoa, Sindhu R. Johnson, Dinesh Khanna, Luc Mouthon, Mandana Nikpour, Susanna Proudman, Virginia Steen, Edward Stern, John Varga, Christop Tags: Brief Report Source Type: research

Treatment of Chemotherapy-Induced Thrombotic Microangiopathy with Eculizumab in a Patient with Metastatic Breast Cancer
We report the case of a 69-year-old woman with breast cancer who experienced a mitomycin-C-induced TMA manifestation. TMA did not respond to conservative therapy, plasmapheresis or rituximab and rapidly lead to dialysis dependency. Despite disease progression and metastases, eculizumab treatment was associated with recovered renal function and enabled the patient to avoid dialysis, improving her quality of life.Case Rep Oncol 2019;12:1 –6 (Source: Case Reports in Oncology)
Source: Case Reports in Oncology - January 3, 2019 Category: Cancer & Oncology Source Type: research

Acute Thrombotic Microangiopathy and Cortical Necrosis Following Administration of Alemtuzumab: A Case Report
We describe a 39-year-old woman with relapsing-remitting multiple sclerosis who developed acute kidney injury requiring renal replacement therapy after 1 dose of alemtuzumab, as well as microangiopathic hemolytic anemia and thrombocytopenia. Pathologic examination of a kidney biopsy specimen demonstrated extensive cortical necrosis and arteriolar fibrin thrombi with nonspecific immunofluorescence staining of immunoglobulin M and C3 and absence of immune deposits on electron microscopy. These findings were consistent with the diagnosis of acute thrombotic microangiopathy. She received dexamethasone and underwent plasmaphere...
Source: American Journal of Kidney Diseases - December 8, 2018 Category: Urology & Nephrology Source Type: research

Low titer A plasma in three AB patients for therapeutic plasma exchange
ConclusionsTPE was effectively performed without evidence of increased hemolysis using up to 47.8% of low titer A plasma. This approach can reduce strains on limited supplies of AB plasma whereas providing a vital treatment alternative for AB patients undergoing TPE with plasma replacement. (Source: Journal of Clinical Apheresis)
Source: Journal of Clinical Apheresis - November 23, 2018 Category: Hematology Authors: Alesia Kaplan, Joan Sevcik, Joseph E. Kiss Tags: RESEARCH ARTICLE Source Type: research

Variable Clinical Presentation of Congenital Thrombotic Thrombocytopenic Purpura in a Large Cohort of Patients Carrying a New Mutation in the CUB1 Domain of ADAMTS13 Gene
Conclusion:To the best of our knowledge, this is one of the largest congenital TTP cohorts described in the literature. This cohort is unique due to the fact that all members carry the same, previously unreported, ADAMTS13 gene mutation in the CUB1 domain of the gene. Our findings support an assumption that environmental and hereditary modifiers may influence disease course. Further research of the involved families may enable us to expand the understanding of the pathophysiology and develop better treatment options for this understudied rare condition.DisclosuresNo relevant conflicts of interest to declare. (Source: Blood)
Source: Blood - November 21, 2018 Category: Hematology Authors: Pikovsky, O., Arafat, M., Ovadia, H., Sharoni, Y., Al-Athamen, K., Kanengisser-Pines, B., Keren-Politansky, A., Levi, I., Parvari, R., Rabinovich, A. Tags: 311. Disorders of Platelet Number or Function: Poster I Source Type: research

Cell Derived Microparticles (MP) in Different Phases of Thrombotic Thrombocytopenic Purpura (TTP) and Effect of Exchange Plasmapheresis (EPP) on Their Profiles
In this study, we investigated MP profiles in active and remission phase of TTP and the effect of EPP on MP profiles. We also aimed to determine if MP profiles may be a useful measure for monitoring clinical course and tracking progress of therapy.METHODS: A retrospective study was conducted evaluating MP assays in patients with TTP. MP profiles were reviewed in acute and remission phases of TTP. Acute phase was defined as thrombocytopenia, clinical evidence of microangiopathy and hemolytic anemia and low ADAMTS13 activity. Remission was defined as sustained normalization of laboratory parameters and no further microangiop...
Source: Blood - November 21, 2018 Category: Hematology Authors: Ali, R. A., Paul, Y., McCauley, R. F., Yaniz, M., Gonzalez, M. Q., Horstman, L. L., Jy, W., Ahn, Y. S. Tags: 311. Disorders of Platelet Number or Function: Poster III Source Type: research

Thrombotic Microangiopathies Associated with Pregnancy: Diagnostic and Therapeutic Challenges
Several serious disorders may present during pregnancy or postpartum with thrombotic microangiopathy (p-TMA). Signs of microangiopathic hemolytic anemia and thrombocytopenia may arise due to pregnancy complications such as severe preeclampsia (sPE) and HELLP-syndrome (hemolysis, elevated liver enzymes, and low platelets) or severe independent diseases: atypical hemolytic uremic syndrome (aHUS), thrombotic thrombocytopenic purpura (TTP). Recent evidence and clinical similarities suggest a link sPE/HELLP to aHUS, a disease of excessive activation of the alternative complement pathway. Pregnancy-associated aHUS is a severe di...
Source: Blood - November 21, 2018 Category: Hematology Authors: Vinogradova, M., Kirsanova, T., Shmakov, R. Tags: 903. Outcomes Research-Non-Malignant Hematology: Poster III Source Type: research

Clinical Associations, Treatment, and Outcomes of Renal-Limited Thrombotic Microangiopathy
ConclusionRenal-limited TMA is a pathologic diagnosis that in our cohort was most likely to medications or underlying rheumatologic conditions. rTMA was managed by treating the underlying disorder or removing the causative medication. Though cause of rTMA was evident in the majority of patients, 17% had no identifiable precipitating factor predisposing to rTMA. Larger studies are required to delineate optimal management and surveillance strategies for these patients.DisclosuresNo relevant conflicts of interest to declare. (Source: Blood)
Source: Blood - November 21, 2018 Category: Hematology Authors: Saba, E. S., Cambron, J. C., Go, R. S., Leung, N., Sridharan, M. Tags: 311. Disorders of Platelet Number or Function Source Type: research

Quantification of Schistocytes from the Peripheral Blood Smear in Thrombotic Thrombocytopenic Purpura (TTP) Compared to Non-TTP Thrombocytopenic Hospitalized Patients
IntroductionThe diagnosis of TTP requires thrombocytopenia without an underlying cause and microangiopathic hemolytic anemia (MAHA)1. TTP is usually confirmed with a low ADAMTS level. However, the turnaround time for an ADAMTS level is several days and treatment for this rare disease cannot be delayed, since it is uniformly fatal if left untreated but highly responsive to plasma exchange therapy. A rapid diagnosis is required and still must be made on clinical findings and peripheral smear morphology. The degree of MAHA that is seen with TTP, though, has not been well defined, or correlated with ADAMTS results. Automated f...
Source: Blood - November 21, 2018 Category: Hematology Authors: O'Brien, T. E., Bowman, L., Hong, A., Goparaju, K. Tags: 311. Disorders of Platelet Number or Function Source Type: research

Evaluating and Correcting Iron Deficit in Atypical Hemolytic Uremic Syndrome (HUS)
Conclusions:While disease-directed therapy in atypical HUS (i.e. eculizumab) is required to effectively manage acute, complement-mediated hemolytic anemia and renal failure, correction of the calculated iron deficit in co-existing iron deficiency anemia, if present, can result in gradual but marked improvement in hemoglobin. Conversely, patients with atypical HUS who are actively hemolyzing with a normocytic anemia should still be evaluated for iron deficiency and undergo iron transfusions for any calculated deficit.DisclosuresNo relevant conflicts of interest to declare. (Source: Blood)
Source: Blood - November 21, 2018 Category: Hematology Authors: Reynolds, S. B., Tse, W. Tags: 311. Disorders of Platelet Number or Function Source Type: research