Article
Immunology
Yanming Cui, Jianbo Wei, Xiang Peng
Summary: Vaccination in patients with autoimmune diseases is controversial due to the possibility of thrombotic thrombocytopenic purpura. This case report describes a patient with systemic lupus erythematosus who developed thrombotic thrombocytopenic purpura after rabies vaccination. Prompt identification and treatment saved the patient from danger. This highlights the potential unexpected adverse effects of attenuated vaccines in patients on long-term immunosuppressive drugs and with autoimmune diseases.
FRONTIERS IN IMMUNOLOGY
(2022)
Article
Medicine, General & Internal
Ju-Yang Jung, Ji-Won Kim, Chang-Hee Suh, Hyoun-Ah Kim
Summary: This study describes a 16-year-old girl with SLE and immune thrombocytopenia who was diagnosed with TTP. She was successfully treated with plasmapheresis and rituximab therapy, and her relapse after 18 months was controlled using the same management.
Article
Pediatrics
Yoko Takagi, Yasuko Kobayashi, Ayako Hirakata, Mariko Takei, Satoshi Ogasawara, Chikage Yajima, Yuka Ikeuchi, Akira Matsumoto, Yoshiyuki Ogawa, Hiroshi Handa, Masanori Matsumoto, Hirokazu Arakawa, Takumi Takizawa
Summary: In this case report, an 11-year-old girl was diagnosed with acquired TTP caused by SLE. The patient showed good response to treatment and did not experience recurrence of TTP. Measurement of ADAMTS13 activity and anti-ADAMTS13 antibody titer were necessary for diagnosis, prognosis, and predicting recurrence in SLE-related TMA.
FRONTIERS IN PEDIATRICS
(2022)
Review
Rheumatology
Tomoyuki Mutoh, Keiichi Ohashi, Taichi Nagai, Akira Sugiura, Masataka Kudo, Hiroshi Fujii
Summary: This case report and literature review demonstrate that early intervention with RTX may improve treatment response and prognosis in SLE-associated TTP, regardless of the degree of ADAMTS13 activity.
RHEUMATOLOGY INTERNATIONAL
(2023)
Review
Rheumatology
Shen-Ju Liang, Quan-You Zheng, Meng-Shan Li, Ming-Ye Lv, Wen-Ting Chen, Yi Yang
Summary: This article describes a case study of a patient with SLE-TTP who showed good efficacy and outcomes after treatment with Belimumab. Additionally, a focused review of the pathogenesis, diagnosis, and therapeutic strategies for SLE-TTP is provided.
CLINICAL RHEUMATOLOGY
(2022)
Review
Genetics & Heredity
Sanober Nusrat, Kisha Beg, Osman Khan, Arpan Sinha, James George
Summary: Hereditary thrombotic thrombocytopenic purpura (hTTP), also known as Upshaw-Schulman syndrome, is a rare genetic disorder caused by mutations in the ADAMTS13 gene that leads to decreased or absent production of the plasma von Willebrand factor (VWF)-cleaving metalloprotease ADAMTS13. The disease can cause thrombocytopenia, impairment of blood circulation, and organ damage. Prophylaxis with plasma infusions or plasma-derived factor VIII products is the primary treatment for preventing acute episodes.
Article
Rheumatology
Jian Zhang
Summary: This study utilized bioinformatics approaches to uncover the molecular mechanisms underlying the association between systemic lupus erythematosus and acquired ADAMTS13-deficient thrombotic thrombocytopenic purpura. The findings identified three core genes associated with the pathogenesis of both diseases and suggested two potential drugs for treatment.
Article
Medicine, General & Internal
Lars M. M. Asmis, Andreas Serra, Alexander Krafft, Abraham Licht, Elke Leisinger, Jana Henschkowski-Serra, Michael T. T. Ganter, Steffen Hauptmann, Marianne Tinguely, Johanna A. Kremer A. Hovinga
Summary: A diagnosis of hereditary thrombotic thrombocytopenic purpura (TTP) was made in a pregnant woman at 30 weeks' gestation. Her condition did not respond to plasma exchange, but recombinant ADAMTS13 normalized her platelet count.
NEW ENGLAND JOURNAL OF MEDICINE
(2022)
Article
Cell Biology
Jon Shaffer, Angela Grove
Summary: This case report demonstrates the efficacy of a second course of caplacizumab in a patient with relapsed TTP, showing significant improvement in platelet count. The patient was able to complete the treatment successfully without complications or further relapses.
Editorial Material
Medicine, General & Internal
Pooja Deepak, Roha Saeed Memon, Fizza Tariq, Hassan Ahmed, Shaheen Bhatti
Summary: This study reports a case of a 58-year-old male presenting with parkinsonism and gastrointestinal symptoms, ultimately diagnosed with SLE with an overlapping picture of TTP. Challenges and delays were encountered in the diagnosis and management of the patient in a resource-constrained setting. The patient's age, presence of overlapping conditions, and multi-organ involvement were factors contributing to the treatment failure.
JOURNAL OF THE PAKISTAN MEDICAL ASSOCIATION
(2021)
Article
Hematology
George Goshua, Pranay Sinha, Jeanne E. Hendrickson, Christopher Tormey, Pavan K. Bendapudi, Alfred Ian Lee
Summary: The addition of caplacizumab to standard of care in acquired TTP treatment is not cost-effective due to the high cost of the medication and lack of improvement in relapse rates. Further study on the long-term impact of caplacizumab on health system cost is warranted.
Article
Urology & Nephrology
Momin H. Alkhatib, Sam Kant, Steven Menez, Laila Lakhani, Christopher John Sperati, Derek M. Fine, Lois J. Arend, Mohamed G. Atta
Summary: This study compared the clinical and prognostic characteristics of thrombotic microangiopathy (TMA) with class IV lupus nephritis in SLE patients. TMA patients showed significant abnormalities in certain laboratory markers and had a worse prognosis.
JOURNAL OF NEPHROLOGY
(2021)
Article
Immunology
Liping Xu, Xinwei Song, Qiaoding Dai, Tianrong Guan, Yan Zhang, Na Lin, Ji-an Wang
Summary: This case report highlights the efficacy of Belimumab in treating refractory Lupus Cystitis, with significant improvements in symptoms, urinary markers, and protein levels observed in the patient. No relapse of lupus cystitis or SLE activity occurred during the 10-month follow-up period, demonstrating positive outcomes with Belimumab treatment.
INTERNATIONAL IMMUNOPHARMACOLOGY
(2021)
Review
Hematology
Rebecca J. Shaw, Tina Dutt
Summary: Thrombotic thrombocytopenic purpura is a rare and potentially fatal condition with neurological complications that have a significant impact on patient prognosis. Neuroimaging may play a crucial role in guiding treatment and preventing vascular diseases as our understanding of the acute and chronic stages of TTP deepens. Reduced ADAMTS13 activity levels are associated with increased thrombotic risk, and novel therapies may offer neuroprotective effects. Screening and timely intervention for TTP patients are of great importance.
BRITISH JOURNAL OF HAEMATOLOGY
(2022)
Review
Medicine, General & Internal
Senthil Sukumar, Bernhard Lammle, Spero R. Cataland
Summary: TTP is a rare but dangerous disorder characterized by hemolysis, thrombocytopenia, and ischemic damage, often caused by ADAMTS13 deficiency. Front-line treatment typically involves plasma exchange and immunosuppression, as well as drugs like rituximab and caplacizumab. Refractory cases may require alternative therapies.
JOURNAL OF CLINICAL MEDICINE
(2021)
Article
Hematology
E. O. Artemenko, S. I. Obydennyi, K. S. Troyanova, G. A. Novichkova, D. Y. Nechipurenko, M. A. Panteleev
Summary: In this study, we investigated the adhesive properties of circulating plasma-derived and platelet-derived microvesicles (MVs) and their potential role in thrombus growth. The results showed that both types of MVs weakly bound fibrinogen but did not stably adhere to immobilized fibrinogen. Moreover, the presence of these MVs did not significantly affect thrombus formation. Therefore, MVs present in the plasma of healthy individuals are unlikely to directly contribute to thrombus formation under arterial flow conditions.
THROMBOSIS RESEARCH
(2024)
Letter
Hematology
Giuseppe Lippi, Camilla Mattiuzzi, Emmanuel J. Favaloro
THROMBOSIS RESEARCH
(2024)
Article
Hematology
Marium Malik, Maha Al-Ghafry, Abraham Haimed, Julia Su, Maribel Lema, Linda Shore-Lessersson, Suchitra S. Acharya
Summary: This study analyzed and evaluated leukemia-associated coagulopathy in newly diagnosed pediatric leukemia patients using CCTs and ROTEM. The results showed that fibrinogen and platelets played a significant role in causing coagulopathies in this population. This suggests the utility of using CCTs and ROTEM in the pediatric population to evaluate hemostatic function and guide blood product replacement.
THROMBOSIS RESEARCH
(2024)
Review
Hematology
Mohammad M. Elahi, Alexandra N. Witt, Edward L. G. Pryzdial, Paul B. McBeth
Summary: Thrombotic disease may be underdiagnosed in microgravity conditions, and the underlying factors are still poorly understood. A case of internal jugular vein thrombosis was recently diagnosed in a low-risk female astronaut, and six out of ten additional crew members showed risk factors for jugular venous flow. Observations in space and studies on Earth suggest that microgravity affects blood flow, coagulation, and vascular function.
THROMBOSIS RESEARCH
(2024)
Review
Hematology
Stian Ingemann-Molden, Christina Krogner Caspersen, Nanna Rolving, Anette Arbjerg Hojen, Frederikus A. Klok, Erik L. Grove, Barbara Cristina Brocki, Jane Andreasen
Summary: This study found that no single PROM covered all aspects considered important by PE patients. PROMs recommended in the ICHOM core set covered most aspects, but worrisome thoughts, hypervigilance around symptoms, and uncertainty of illness were not covered.
THROMBOSIS RESEARCH
(2024)
Article
Hematology
Haomin Yan, Tsutomu Sasaki, Yasufumi Gon, Kumiko Nishiyama, Hideaki Kanki, Hideki Mochizuki
Summary: The incidence of cancer-associated stroke has increased with the prolonged survival times of cancer patients. In this study, the researchers focused on the driver gene KRAS and found that KRAS mutations exacerbated neurological deficits after stroke and promoted tumor growth. They also identified the upregulation of pro-inflammatory responses and the promotion of STAT3 phosphorylation in mice with KRAS mutations. Additionally, the researchers found that colorectal cancer patients with stroke onset within 3 months after cancer diagnosis had a worse prognosis.
THROMBOSIS RESEARCH
(2024)
Letter
Hematology
Tomoki Togashi, Satomi Nagaya, Makiko Meguro-Horike, Haruto Matsumoto, Yuta Imai, Koichi Yamaguchi, Yoshinari Fujii, Haruka Moriya, Yuika Kikuchi, Ibuki Yasuda, Shin-ichi Horike, Eriko Morishita
THROMBOSIS RESEARCH
(2024)
Article
Hematology
Marta Najarro, Winnifer Briceno, Carmen Rodriguez, Alfonso Muriel, Sara Gonzalez, Ana Castillo, Ignacio Jara, Parth Rali, Catalin Toma, Behnood Bikdeli, David Jimenez
Summary: The CPES score is a useful tool in identifying the risk of hemodynamic deterioration in normotensive patients with acute PE, with acceptable C-statistic, excellent specificity, and low positive predictive value.
THROMBOSIS RESEARCH
(2024)
Article
Hematology
Lei Liu, Huifang Xu, Jian Wang, Haiyan Wang, Saisai Ren, Qian Huang, Mingyan Zhang, Hui Zhou, Chunyan Yang, Lu Jia, Yu Huang, Hao Zhang, Yanling Tao, Ying Li, Yanan Min
Summary: This study investigated the gut microbiota metabolites and cytokines, chemokines, and growth factors in patients with ITP and found their association with treatment responsiveness. The findings suggest that imbalanced secretion of cyto(chemo)kins/growth factors and inadequate levels of TMAO are characteristic of patients with ITP.
THROMBOSIS RESEARCH
(2024)
Article
Hematology
Roxanne Mcknight, Mohammad Salameh, Lee Ann Jones, Greta Anton, Lindsay Carter
Summary: This study evaluated the safety of fixed-dose heparin titration boluses in underweight patients with VTE. The results showed that although underweight patients required more frequent holding of heparin, there was no significant increase in bleeding risk.
THROMBOSIS RESEARCH
(2024)