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Dorela Lame, Michelangelo Pianelli, Shahram Kordasti, Erika Morsia, Attilio Olivieri, Antonella Poloni
Sarcoidosis, a multi-organ system disease, often presents insidiously. Thrombocytopenia in sarcoidosis is frequent because of hypersplenism, granulomas infiltrating the bone marrow, or immune thrombocytopenia (ITP). The diagnosis of ITP relies on exclusionary criteria, given the absence of a definitive laboratory diagnostic feature. In the era prior to modern ITP management, sarcoidosis-associated ITP was known to manifest severely, often showing resistance to treatment and an increased risk of mortality. In this case, we present a young male who was admitted to a district hospital's emergency room, displaying symptoms of hematuria, gingival bleeding, and a petechial rash...
March 4, 2024: Hematology Reports
#2
Omar Hariri, Omar Al Laham, Lina Haffar, Mohammad Ghannam, Ammar Mohammad
INTRODUCTION AND IMPORTANCE: Intimomedial mucoid degeneration is a rare vascular disorder first depicted in 1977. It involves mucin deposition in arterial layers. This will cause elastic tissue degeneration and aneurysm formation. This pathology predominantly affects the aorta. However, it could involve other smaller vessels. Surgical treatment could become complicated by a bleeding diathesis. Therefore, a precise surgical technique is necessary to avoid the ensuing complications. CASE PRESENTATION: We present the case of a previously healthy 27-year-old Middle Eastern male who presented to our surgical clinic following the incidental discovery of an infrarenal abdominal aortic aneurysm following a blunt trauma to the left flank incurred during a fall...
March 2024: Annals of Medicine and Surgery
#3
Yoshimi Shinohara, Ryo Miyaoka, Junkoh Yamamoto
BACKGROUND: Accidental hypothermia poses a significant threat to the elderly, and its prevalence might increase due to aging and increasing isolation of individuals in Japan. Here, a series of four consecutive cases of accidental hypothermia in elderly patients with intracranial hemorrhage who underwent surgical treatment at our institution is presented. CASE DESCRIPTION: All patients were admitted to the emergency department with a diagnosis of intracranial hemorrhage...
2024: Surgical Neurology International
#4
JOURNAL ARTICLE
Kalpana Rautela, Ashish Kumar, Suresh K Rana, Arun Jugran, Indra D Bhatt
The genus Malaxis (family Orchidaceae), comprises nearly 183 species available across the globe. The plants of this genus have long been employed in traditional medical practices because of their numerous biological properties, like the treatment of infertility, hemostasis, burning sensation, bleeding diathesis, fever, diarrhea, dysentery, febrifuge, tuberculosis, etc. Various reports highlight their phytochemical composition and biological activities. However, there is a lack of systematic review on the distribution, phytochemistry, and biological properties of this genus...
January 30, 2024: Chemistry & Biodiversity
#5
Filagot Mikru, Ayenew Gucho, Jiksa Muleta, Ermias Tilahun, Kalkidan Haile, Eden Belayneh
INTRODUCTION: Small bowel bleeding can be overt or occult. Despite advances in imaging and endoscopy, the diagnosis and treatment of small bowel bleeding remain challenging due to its length and location. Diagnostic procedures such as push enteroscopy, capsule endoscopy and intraoperative enteroscopy are recommended to identify the source of bleeding. CASE PRESENTATION: A 33-year-old female with no prior history of bleeding diathesis presented with massive lower GI bleeding...
February 2024: International Journal of Surgery Case Reports
#6
JOURNAL ARTICLE
Steven P Treon, Shayna Sarosiek, Jorge J Castillo
Mutations in MYD88 (95-97%) and CXCR4 (30-40%) are common in Waldenstrom macroglobulinemia (WM). TP53 is also altered in 20-30% of WM patients, particularly those previously treated. Mutated MYD88 upregulates and activates HCK that drives BTK pro-survival signaling. Both nonsense and frameshift CXCR4 mutations occur in WM. Nonsense variants such as CXCR4S338X show greater resistance to BTK-inhibitors. Covalent BTK-inhibitors (cBTK-i) produce major responses in 70-80% of WM patients. MYD88 and CXCR4 mutation status can impact time to major response, depth of response and/or progression-free survival (PFS) in WM patients treated with cBTK-i...
January 11, 2024: Blood
#7
Asmae Ettamri Ouaaba, Raquel Behar Lagares
Acquired von Willebrand syndrome (AvWS) is a rare bleeding disorder caused by dysfunction of the von Willebrand factor (vWF), leading to bleeding manifestations. It usually occurs due to an underlying disorder in patients with no family or personal history of bleeding diathesis. The exact mechanism causing this syndrome is not fully understood, but it involves a complex interplay of factors. Specifically, vWF deficiency or reduced activity can occur due to antibodies, adsorption of vWF onto tumor cells, shear stress, or increased proteolysis...
July 2023: Curēus
#8
P Rossignon, F Grandjean, A Claessens, N Weynants
Acquired coagulopathies resulting from factor X deficiency are rare and typically associated with amyloidosis or plasma cell dyscrasia. Factor X plays a pivotal role in the coagulation cascade, converting prothrombin into thrombin and facilitating the formation of fibrinogen and thrombus. While its occurrence following common infections is extremely rare, isolated cases have been documented. We present a rare case of bleeding diathesis in a patient with community-acquired pneumonia, where prolonged activated partial thromboplastin time (aPTT) and prothrombin time (PT) led to the diagnosis of an infectious-triggered acquired circulating inhibitor targeting factor X...
2023: Case Reports in Hematology
#9
Waheed Ul Hassan, Muhammad Ahmad, Basel Abdelazeem, Ujwala Koduru, Arvind Kunadi
Factor VIII deficiency is mostly seen in hemophilia A, an X-linked recessive disorder. Patients would have a past medical history of bleeding diathesis or a family history of bleeding disorder. Acquired deficiencies of factor VIII are rare; some cases have been reported in solid malignancies. We present this case of acquired factor VIII deficiency in chronic myeloid leukemia (CML). A 72-year-old man was incidentally found to have leukocytosis at 31,000 and a platelet count of 3.2 million on a routine complete blood count (CBC)...
November 2023: Curēus
#10
JOURNAL ARTICLE
Balrik Singh Kailey, Michael Koa-Wing, Nilesh Sutaria, Tom Mott, Afzal Sohaib, Norman Qureshi, Christine Shi, Pritpal Padam, James Howard, Prapa Kanagaratnam
BACKGROUND: Patients with atrial fibrillation (AF) and likelihood of bleeding can undergo left atrial appendage occlusion (LAAO) as an alternative method of stroke prophylaxis. Short-term anti-thrombotic drugs are used postprocedure to offset the risk of device-related thrombus, evidence for this practice is limited. OBJECTIVES: To investigate optimal postimplant antithrombotic strategy in high bleeding-risk patients. METHODS: Patients with AF and high-risk for both stroke and bleeding undergoing LAAO were advised their perioperative drug therapy by a multidisciplinary physician panel...
November 14, 2023: Journal of Cardiovascular Electrophysiology
#11
REVIEW
Renato Marino
Acquired Hemophilia A (AHA) is a rare autoimmune disorder characterized by the onset of a sudden and unexpected bleeding episode in a patient with no personal or family history of bleeding diathesis, and with a typical laboratory feature, i.e., a prolonged activated partial thromboplastin time that is not otherwise explained. This bleeding disorder is caused by autoantibodies directed against the coagulation factor VIII (FVIII). AHA is idiopathic in 50% of cases and is secondary to well-defined diseases in the remaining 50%...
September 28, 2023: Medicina
#12
JOURNAL ARTICLE
Joana Infante, Graça Esteves, João Raposo, João Forjaz de Lacerda
Early death (ED) is still the major obstacle to cure in acute promyelocytic leukemia (APL). Most studies focus on 30-day ED; however, little is known on predictors of death before starting APL treatment (very early death - VED) and on predictors of 7-day ED, the period with most deaths due to thrombohemorrhagic diathesis. We hypothesized whether the severity of the coagulopathy of APL could predict VED and 7-day ED. We also aimed to evaluate other characteristics associated with these outcomes. We undertook a retrospective, single-center observational study including newly diagnosed APL patients admitted to our institution between January 2000 and November 2022...
August 31, 2023: Annals of Hematology
#13
JOURNAL ARTICLE
Dashini Pillay, Nadine Rapiti
INTRODUCTION: In acquired hemophilia A (AHA), the body produces auto-antibodies against Factor VIII. Although AHA is rare, with an incidence of 1.5 patients/1 million population/year, there is a strong association with human immunodeficiency virus (HIV) infection. The accurate interpretation of screening coagulation tests is critical to identify patients with AHA, as the mortality rate secondary to bleeding is high. METHODS: This was a retrospective case series which included all newly diagnosed AHA patients that were referred to Hemophilia care unit at King Edward VIII Hospital, Durban, South Africa from January 2011 to December 2021...
2023: Journal of Primary Care & Community Health
#14
REVIEW
Alaire Buchholz, Keith Hansen, Rachel Rodel
Postpartum hemorrhage (PPH) continues to be one of the leading causes of maternal morbidity and mortality worldwide. The four main causes of PPH are uterine atony, lacerations, retained placenta, and bleeding diathesis. In the patient with PPH, immediate evaluation is needed to diagnose and treat the underlying cause of hemorrhage. Uterotonic agents such as oxytocin remain first line for prevention and treatment of uterine atony. Studies have evaluated the antifibrinolytic tranexamic acid (TXA) as an adjunctive therapy in the prevention and treatment of PPH...
April 2023: South Dakota Medicine: the Journal of the South Dakota State Medical Association
#15
Laurent Sattler, Jordan Wimmer, Agathe Herb, Anne-Cécile Gerout, Olivier Feugeas, Dominique Desprez
BACKGROUND: Glanzmann thrombasthenia (GT) is a rare congenital platelet function disorder associated with a severe bleeding diathesis. Thrombotic manifestations remain a rare condition. We report here the first case of recurrent venous thromboembolism (VTE) successfully treated with apixaban in a patient with GT. Our patient's morbid obesity was an additional challenge. KEY CLINICAL QUESTION: The Key Clinical Question was to determine if direct oral anticoagulants are suitable for patients with both obesity and GT...
May 2023: Research and Practice in Thrombosis and Haemostasis
#16
Sanya Thomas, Geoffrey Guenther, Jared H Rowe, Craig D Platt, Akiko Shimamura, Ofer Levy, Lakshmi Ganapathi
Severe congenital neutropenia caused by jagunal homolog 1 ( JAGN1 ) mutation is a rare condition resulting from maturation arrest secondary to endoplasmic reticulum stress response from impaired neutrophil protein glycosylation. Here, we report a case of a 4-year-old boy who presented with a history of recurrent infections and manifestations, including recurrent intracranial hemorrhage. A review of similar cases reported in the literature indicates that a bleeding diathesis has not been previously described in these patients...
2023: Frontiers in Pediatrics
#17
JOURNAL ARTICLE
Yan-Hong Sun, Wen-Jian Nie, Dan-Dan Tian, Qing Ye
OBJECTIVE: Children with lupus anticoagulant hypoprothrombinemia syndrome (LAHPS) are characterized by prolonged activated partial thromboplastin time (APTT) and prothrombin time (PT), lupus anticoagulant positivity and low prothrombin (factor II, FII) levels. Bleeding or thrombosis tendencies related to LAHPS in children can occur due to the development of anti-prothrombin antibodies that are usually linked to autoimmune or infectious diseases. METHODS: We report three pediatric cases of LAHPS and describe details on their clinical symptoms, laboratory characteristics, treatment...
September 2023: Lupus
#18
JOURNAL ARTICLE
Roxane Labrosse, Julia Chu, Myriam Armant, John K Everett, Danilo Pellin, Niharika Kareddy, Andrew L Frelinger, Lauren A Henderson, Amy E O'Connell, Amlan Biswas, Jet Coenen-van der Spek, Alexandra Marina Miggelbrink, Claudia Fiorini, Hriju Adhikari, Charles C Berry, Vito Adrian Cantu, Johnson Fong, Jason Roy Jaroslavsky, Derin F Karadeniz, Quan-Zhen Li, Shantan Reddy, Aoife M Roche, Chengsong Zhu, Jennifer S Whangbo, Colleen Dansereau, Brenda L Mackinnon, Emily Morris, Stephanie M Koo, Wendy B London, Safa Baris, Ahmet Ozen, Elif Karakoc-Aydiner, Jenny McDade Despotovic, Lisa R Forbes Satter, Akihiko Saitoh, Yuta Aizawa, Alejandra King, Mai Anh T Nguyen, Vy Do Uyen Vu, Scott B Snapper, Anne Galy, Luigi D Notarangelo, Frederic D Bushman, David A Williams, Sung-Yun Pai
Wiskott-Aldrich syndrome (WAS) is a rare X-linked disorder characterized by combined immunodeficiency, eczema, microthrombocytopenia, autoimmunity, and lymphoid malignancies. Gene therapy (GT) to modify autologous CD34+ cells is an emerging alternative treatment with advantages over standard allogeneic hematopoietic stem cell transplant for patients who lack well-matched donors, avoiding graft-versus-host-disease. We report the outcomes of a phase I/II clinical trial in which 5 patients with severe WAS underwent GT using a self-inactivating lentiviral (SIN-LV) vector expressing the human WAS cDNA under the control of a 1...
July 21, 2023: Blood
#19
JOURNAL ARTICLE
Weronika Lebowa, Joanna Zdziarska, Tomasz Sacha
We present a case of a 30-year-old man suffering from chronic refractory immune thrombocytopenia (ITP) from early childhood. The patient was treated with all the therapeutic methods available in Poland, without platelet response: corticosteroids, intravenous immunoglobulins, splenectomy, cyclophosphamide, vinblastine, azathioprine, mycophenolate mofetil, rituximab, ciclosporin A, romiplostim, and eltrombopag. He continued to function persistently with deep thrombocytopenia, symptoms of hemorrhagic diathesis, and one episode of spontaneous subarachnoid bleeding...
July 1, 2023: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
#20
JOURNAL ARTICLE
S Garry, J Wauchope, J M Hintze, E J Ryan, E O'Cathain, C B Heffernan
OBJECTIVE: To assess patient factors to predict treatment success of Naseptin for recurrent paediatric epistaxis. METHODS: This prospective cohort study of paediatric patients referred to a tertiary paediatric otolaryngology clinic with recurrent epistaxis treated with Naseptin cream and education. Patients with red flag symptoms and bleeding diathesis were omitted, along with patients with concurrent otolaryngology complaints. Statistical analysis included logistic regression analysis to assess for predictive factors contributing to treatment success...
August 2023: International Journal of Pediatric Otorhinolaryngology
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