Combination regimens containing daratumumab for initial diagnosed acquired thrombotic thrombocytopenic purpura
AbstractThrombotic thrombocytopenic purpura (TTP) is a rare and life-threatening thrombotic microangiopathy characterized by microangiopathic hemolytic anemia, severe thrombocytopenia, and organ ischemia associated with disseminated microvascular platelet-rich thrombus. Before the introduction of plasma therapy, acute TTP was almost universally fatal, which improved survival from  <  10 to 80–90%. However, patients who survived an acute attack were at high risk for recurrence and long-term morbidity. It was reported that daratumumab can eradicate persistent ADAMTS13-inhibiting autoantibodies and restore ADAMTS13 a...
Source: Journal of Thrombosis and Thrombolysis - January 13, 2023 Category: Hematology Source Type: research

Thrombotic thrombocytopenic purpura developed after pegylated interferon treatment for hepatitis B infection
Thrombotic thrombocytopenic purpura (TTP) is a rare and life-threatening thrombotic microangiopathy characterized by microangiopathic hemolytic anemia, severe thrombocytopenia, and organ ischemia. It is relate... (Source: BMC Nephrology)
Source: BMC Nephrology - December 13, 2022 Category: Urology & Nephrology Authors: Shuqin Mei, Yun Feng, Linlin Cui, Jing Chen, Zhiguo Mao, Xuezhi Zhao, Changlin Mei and Yixin Qian Tags: Case report Source Type: research

The Challenging Management of Acute Thrombotic Microangiopathy in Pregnancy
We present a case of a 38-year-old woman at 40 weeks of second gestation, admitted with thrombotic microangiopathy being the final diagnosis not immediately clear.Acta Haematol (Source: Acta Haematologica)
Source: Acta Haematologica - December 8, 2022 Category: Hematology Source Type: research

A Case of Thrombotic Microangiopathy and Acute Sarcoidosis
We describe the case of a patient presenting with hypercalcemia, pancreatitis, and acute renal failure, followed by microangiopathic hemolytic anemia. Although there were no significant respiratory symptoms, thoracic radiology and mediastinal lymph node biopsy results were in keeping with sarcoidosis as the underlying cause of this multisystem presentation. Corticosteroids were commenced with clinical and biochemical improvement. This novel case highlights the need to consider sarcoidosis as part of the differential diagnosis for unusual multiorgan presentations and for early multidisciplinary involvement in such cases to ...
Source: Chest - November 7, 2022 Category: Respiratory Medicine Authors: Anthony W Martinelli William Dunn Mark E McClure Ieuan Walker Andrew Stewart Sumit Karia Stephen D Preston Sathia Thiru Nicholas Torpey Sanjay Ojha Emily Symington James A Nathan Source Type: research

A Case of Thrombotic Microangiopathy and Acute Sarcoidosis
We describe the case of a patient presenting with hypercalcemia, pancreatitis, and acute renal failure, followed by microangiopathic hemolytic anemia. Although there were no significant respiratory symptoms, thoracic radiology and mediastinal lymph node biopsy results were in keeping with sarcoidosis as the underlying cause of this multisystem presentation. Corticosteroids were commenced with clinical and biochemical improvement. This novel case highlights the need to consider sarcoidosis as part of the differential diagnosis for unusual multiorgan presentations and for early multidisciplinary involvement in such cases to ...
Source: Chest - November 7, 2022 Category: Respiratory Medicine Authors: Anthony W Martinelli William Dunn Mark E McClure Ieuan Walker Andrew Stewart Sumit Karia Stephen D Preston Sathia Thiru Nicholas Torpey Sanjay Ojha Emily Symington James A Nathan Source Type: research

Caplacizumab Use in a TTP Case Unresponsive to Conventional Therapy
Conclusion TTP cases unresponsive to conventional regimens can represent a challenging situation; however, poor outcomes could potentially be avoided with a novel therapy like Caplacizumab. In our patient, this medication was well tolerated, and platelet count normalized after two days of its introduction.PMID:36327969 (Source: Ir Med J)
Source: Ir Med J - November 3, 2022 Category: General Medicine Authors: J William C McCellistrim Y Nu ñez Source Type: research

Atypical hemolytic uremic syndrome: Consensus of diagnosis and treatment in taiwan
J Formos Med Assoc. 2022 Oct 30:S0929-6646(22)00390-4. doi: 10.1016/j.jfma.2022.10.006. Online ahead of print.ABSTRACTAtypical hemolytic uremic syndrome (aHUS), characterized by microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury, is a rare but life-threatening systemic disorder caused by the dysregulation of the complement pathway. Current advances in molecular analysis and pathogenesis have facilitated the establishment of diagnosis and development of effective complement blockade. Based on this recent consensus, we provide suggestions regarding the diagnosis and management of aHUS in Taiwan. The...
Source: J Formos Med Assoc - November 2, 2022 Category: General Medicine Authors: Min-Hua Tseng Shih-Hua Lin Jeng-Daw Tsai Mai-Szu Wu I-Jung Tsai Yeu-Chin Chen Min-Chih Chang Wen-Chien Chou Yee-Hsuan Chiou Chiu-Ching Huang Source Type: research

Atypical hemolytic uremic syndrome: Consensus of diagnosis and treatment in taiwan
J Formos Med Assoc. 2022 Oct 30:S0929-6646(22)00390-4. doi: 10.1016/j.jfma.2022.10.006. Online ahead of print.ABSTRACTAtypical hemolytic uremic syndrome (aHUS), characterized by microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury, is a rare but life-threatening systemic disorder caused by the dysregulation of the complement pathway. Current advances in molecular analysis and pathogenesis have facilitated the establishment of diagnosis and development of effective complement blockade. Based on this recent consensus, we provide suggestions regarding the diagnosis and management of aHUS in Taiwan. The...
Source: J Formos Med Assoc - November 2, 2022 Category: General Medicine Authors: Min-Hua Tseng Shih-Hua Lin Jeng-Daw Tsai Mai-Szu Wu I-Jung Tsai Yeu-Chin Chen Min-Chih Chang Wen-Chien Chou Yee-Hsuan Chiou Chiu-Ching Huang Source Type: research

Atypical hemolytic uremic syndrome: Consensus of diagnosis and treatment in taiwan
J Formos Med Assoc. 2022 Oct 30:S0929-6646(22)00390-4. doi: 10.1016/j.jfma.2022.10.006. Online ahead of print.ABSTRACTAtypical hemolytic uremic syndrome (aHUS), characterized by microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury, is a rare but life-threatening systemic disorder caused by the dysregulation of the complement pathway. Current advances in molecular analysis and pathogenesis have facilitated the establishment of diagnosis and development of effective complement blockade. Based on this recent consensus, we provide suggestions regarding the diagnosis and management of aHUS in Taiwan. The...
Source: J Formos Med Assoc - November 2, 2022 Category: General Medicine Authors: Min-Hua Tseng Shih-Hua Lin Jeng-Daw Tsai Mai-Szu Wu I-Jung Tsai Yeu-Chin Chen Min-Chih Chang Wen-Chien Chou Yee-Hsuan Chiou Chiu-Ching Huang Source Type: research

Atypical hemolytic uremic syndrome: Consensus of diagnosis and treatment in taiwan
J Formos Med Assoc. 2022 Oct 30:S0929-6646(22)00390-4. doi: 10.1016/j.jfma.2022.10.006. Online ahead of print.ABSTRACTAtypical hemolytic uremic syndrome (aHUS), characterized by microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury, is a rare but life-threatening systemic disorder caused by the dysregulation of the complement pathway. Current advances in molecular analysis and pathogenesis have facilitated the establishment of diagnosis and development of effective complement blockade. Based on this recent consensus, we provide suggestions regarding the diagnosis and management of aHUS in Taiwan. The...
Source: J Formos Med Assoc - November 2, 2022 Category: General Medicine Authors: Min-Hua Tseng Shih-Hua Lin Jeng-Daw Tsai Mai-Szu Wu I-Jung Tsai Yeu-Chin Chen Min-Chih Chang Wen-Chien Chou Yee-Hsuan Chiou Chiu-Ching Huang Source Type: research

Hemolytic-Uremic Syndrome in Children
Hemolytic uremic syndrome is characterized by a triad of microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney failure. Most cases are caused by Shiga-toxin-producing bacteria, especially Escherichia coli. Transmission occurs through ground beef and unpasteurized milk. STEC-HUS is the main cause of acute renal failure in children. Management remains supportive. Immediate outcome is most often. Atypical HUS represents about 5% of cases, has a relapsing course with more than half of the patients progressing to end-stage kidney failure. Most cases are due to variants in complement regulators of the alternative...
Source: Pediatric Clinics of North America - October 29, 2022 Category: Pediatrics Authors: Olivia Boyer, Patrick Niaudet Source Type: research

Three shades of black - secondary thrombotic microangiopathy
Indian J Pathol Microbiol. 2022 Oct-Dec;65(4):898-901. doi: 10.4103/ijpm.ijpm_1237_21.ABSTRACTThrombotic microangiopathy is a group of disorders characterized by microangiopathic hemolytic anemia, thrombocytopenia and organ damage. Wide age distribution and the heterogeneity in presentation demand a deeper understanding into the pathogenesis of TMA. Primary TMA is distinct from TMA associated with secondary causes and remains clinically occult till a precipitating factor aggravates it. The extent and severity of renal damage caused by each of them is also distinct. The first alerting signal could be the presence of schisti...
Source: Indian Journal of Pathology and Microbiology - October 29, 2022 Category: Pathology Authors: Rhituparna Das Saugat Dasgupta Moumita Sengupta Keya Basu Source Type: research

Three shades of black - secondary thrombotic microangiopathy
Indian J Pathol Microbiol. 2022 Oct-Dec;65(4):898-901. doi: 10.4103/ijpm.ijpm_1237_21.ABSTRACTThrombotic microangiopathy is a group of disorders characterized by microangiopathic hemolytic anemia, thrombocytopenia and organ damage. Wide age distribution and the heterogeneity in presentation demand a deeper understanding into the pathogenesis of TMA. Primary TMA is distinct from TMA associated with secondary causes and remains clinically occult till a precipitating factor aggravates it. The extent and severity of renal damage caused by each of them is also distinct. The first alerting signal could be the presence of schisti...
Source: Indian Journal of Pathology and Microbiology - October 29, 2022 Category: Pathology Authors: Rhituparna Das Saugat Dasgupta Moumita Sengupta Keya Basu Source Type: research

Three shades of black - secondary thrombotic microangiopathy
Indian J Pathol Microbiol. 2022 Oct-Dec;65(4):898-901. doi: 10.4103/ijpm.ijpm_1237_21.ABSTRACTThrombotic microangiopathy is a group of disorders characterized by microangiopathic hemolytic anemia, thrombocytopenia and organ damage. Wide age distribution and the heterogeneity in presentation demand a deeper understanding into the pathogenesis of TMA. Primary TMA is distinct from TMA associated with secondary causes and remains clinically occult till a precipitating factor aggravates it. The extent and severity of renal damage caused by each of them is also distinct. The first alerting signal could be the presence of schisti...
Source: Indian Journal of Pathology and Microbiology - October 29, 2022 Category: Pathology Authors: Rhituparna Das Saugat Dasgupta Moumita Sengupta Keya Basu Source Type: research