Dabrafenib and trametinib in Langerhans cell histiocytosis and other histiocytic disorders
Haematologica. 2023 Sep 21. doi: 10.3324/haematol.2023.283295. Online ahead of print.ABSTRACTStandard treatment for Langerhans Cell Histiocytosis (LCH) is chemotherapy, with high failure rates. Since MAP-kinase activating mutations are found in most cases, BRAF- and MEKinhibitors have been used successfully to treat patients with refractory or relapsed disease. However, data on long-term responses in children are limited and no data exist on their use as first-line therapy. We treated thirty-four patients (26 LCH, 2 Juvenile Xanthogranuloma, 2 Rosai-Dorfman Disease, 4 presumed single site-CNS histiocytosis) with either dab...
Source: Haematologica - September 21, 2023 Category: Hematology Authors: Eily Cournoyer Justin Ferrell Susan Sharp Anish Ray Michael Jordan Christopher Dandoy Michael Grimley Somak Roy Robert Lorsbach Arnold C Merrow Adam Nelson Allison Bartlett Jennifer Picarsic Ashish Kumar Source Type: research
Dabrafenib and trametinib in Langerhans cell histiocytosis and other histiocytic disorders
Haematologica. 2023 Sep 21. doi: 10.3324/haematol.2023.283295. Online ahead of print.ABSTRACTStandard treatment for Langerhans Cell Histiocytosis (LCH) is chemotherapy, with high failure rates. Since MAP-kinase activating mutations are found in most cases, BRAF- and MEKinhibitors have been used successfully to treat patients with refractory or relapsed disease. However, data on long-term responses in children are limited and no data exist on their use as first-line therapy. We treated thirty-four patients (26 LCH, 2 Juvenile Xanthogranuloma, 2 Rosai-Dorfman Disease, 4 presumed single site-CNS histiocytosis) with either dab...
Source: Haematologica - September 21, 2023 Category: Hematology Authors: Eily Cournoyer Justin Ferrell Susan Sharp Anish Ray Michael Jordan Christopher Dandoy Michael Grimley Somak Roy Robert Lorsbach Arnold C Merrow Adam Nelson Allison Bartlett Jennifer Picarsic Ashish Kumar Source Type: research
Clinical phenotypes, molecular analysis, and outcomes of patients with Rosai-Dorfman disease
Leukemia, Published online: 20 September 2023; doi:10.1038/s41375-023-02032-6Clinical phenotypes, molecular analysis, and outcomes of patients with Rosai-Dorfman disease (Source: Leukemia)
Source: Leukemia - September 20, 2023 Category: Hematology Authors: Long Chang Bing Qiao Hao Cai He Lin Ming-hui Duan Jian Li Dao-bin Zhou Gaurav Goyal Chun-yan Sun Xin-xin Cao Source Type: research
Liquid biopsy of cerebrospinal fluid enabling the detection and therapeutic targeting of the BRAFV600E mutation in a patient with overlapping Erdheim-Chester/Rosai-Dorfman disease
(Source: Journal of Neurology)
Source: Journal of Neurology - September 19, 2023 Category: Neurology Source Type: research
Differential diagnosis of stage 2, 3 and 4 pressure injuries of the pelvis and lower extremity: a case series
J Wound Care. 2023 Sep 1;32(Sup9):S22-S36. doi: 10.12968/jowc.2023.32.Sup9.S22.ABSTRACTConstant, unrelieved pressure of local tissue, particularly over bony prominences, may provoke damage that progresses to necrosis and pressure injury (PI). Differentiating PIs from conditions of similar appearance is imperative to minimising complications and implementing prompt treatment. This case series describes several conditions that may be mistaken for a PI. Outlined are the key differences in patient history, presentation and clinical cues that assist in correctly identifying the true pathology behind these conditions. Conditions...
Source: Herpes - September 8, 2023 Category: Infectious Diseases Authors: Jennifer Caudill Tim Chrisman Joanna Chen Kelsee Zajac Richard Simman Source Type: research
Differential diagnosis of stage 2, 3 and 4 pressure injuries of the pelvis and lower extremity: a case series
J Wound Care. 2023 Sep 1;32(Sup9):S22-S36. doi: 10.12968/jowc.2023.32.Sup9.S22.ABSTRACTConstant, unrelieved pressure of local tissue, particularly over bony prominences, may provoke damage that progresses to necrosis and pressure injury (PI). Differentiating PIs from conditions of similar appearance is imperative to minimising complications and implementing prompt treatment. This case series describes several conditions that may be mistaken for a PI. Outlined are the key differences in patient history, presentation and clinical cues that assist in correctly identifying the true pathology behind these conditions. Conditions...
Source: Herpes - September 8, 2023 Category: Infectious Diseases Authors: Jennifer Caudill Tim Chrisman Joanna Chen Kelsee Zajac Richard Simman Source Type: research
Successful anesthesia management with inhalation anesthesia in Rosai-Dorfman disease: a case report
We report a case of a patient in his 20s who had recurrent RDD and had general anesthesia with perioperative management. He was obese and hepatic insufficiency. This case report adds to the literature regarding the perioperative anesthetic management of RDD with central nervous system involvement.PMID:37647362 | DOI:10.1177/03000605231195161 (Source: Cell Research)
Source: Cell Research - August 30, 2023 Category: Cytology Authors: Xinlei Zhang Xiangjun Zhou Hanjie Liu Dezhi Li Song Liu Yu Lu Source Type: research
A Rare Case of Pseudo-Malignant Paranasal Extranodal Rosai-Dorfman Disease
We report a rare pseudo-malignant paranasal extranodal Rosai-Dorfman disease. A 69-year-old-man presented nasal bleeding and nasal obstruction. Paranasal mass was detected in the left nasal cavity and computed tomography (CT) findings are the sphenoid sinus, maxillary sinus, and ethmoid sinus were involved with inconstant bone thickening, however, no bone destruction was detected. Magnetic resonance imaging scans show iso-intensity signal in T1-weighed image and T2-weighed image. Positron emission tomography/CT fluorodeoxyglucose (FDG) uptake in posterior ethmoid sinus and sphenoid sinus, bilateral cervical lymph node, cla...
Source: Ear, Nose and Throat Journal - August 26, 2023 Category: ENT & OMF Authors: Koichi Tamura Takeshi Tsuda Kazuya Takeda Sho Obata Masako Kurashige Eiichi Morii Hidenori Inohara Source Type: research
Unifocal Type-3 Mixed Histiocytosis in a 10-Years Old Child
We describe a 10 year-old boy with unifocal (type-3) mixed histiocytosis - Langerhans cell histiocytosis combined with a lesion with features intermediate between Rosai-Dorfman disease and reticulohistiocytosis. Sixty months after excision, the child is disease free.DISCUSSION: Cutaneous type-3 mixed histiocytosis (Langerhans cell histiocytosis with Rosai-Dorfman disease/reticulohistiocytosis) may occur in older childhood, be unifocal, and be cured by surgical excision.PMID:37606204 | DOI:10.1080/15513815.2023.2245489 (Source: Fetal and Pediatric Pathology)
Source: Fetal and Pediatric Pathology - August 22, 2023 Category: Pathology Authors: Arturo Bonometti Source Type: research
