Inflammatory diseases in hematology: a review
Am J Physiol Cell Physiol. 2022 Aug 8. doi: 10.1152/ajpcell.00356.2021. Online ahead of print.ABSTRACTHematopoietic cells are instrumental in generating and propagating protective inflammatory responses to infection or injury. However, excessive inflammation contributes to many diseases of the blood, bone marrow, and lymphatic system. We review three clinical categories of hematological inflammatory diseases in which recent clinical and translational advances have been made. The first category are monogenic inflammatory diseases. Genotype-driven research has revealed that previously mysterious diseases with protean manifes...
Source: Am J Physiol Cell Ph... - August 8, 2022 Category: Cytology Authors: Ryan Henrie Hannah Cherniawsky Krista Marcon Eric J Zhao Angelina Marinkovic Persia Pourshahnazari Stephen Parkin Luke Y C Chen Source Type: research
Inflammatory diseases in hematology: a review
Am J Physiol Cell Physiol. 2022 Aug 8. doi: 10.1152/ajpcell.00356.2021. Online ahead of print.ABSTRACTHematopoietic cells are instrumental in generating and propagating protective inflammatory responses to infection or injury. However, excessive inflammation contributes to many diseases of the blood, bone marrow, and lymphatic system. We review three clinical categories of hematological inflammatory diseases in which recent clinical and translational advances have been made. The first category are monogenic inflammatory diseases. Genotype-driven research has revealed that previously mysterious diseases with protean manifes...
Source: American Journal of Physiology. Cell Physiology - August 8, 2022 Category: Cytology Authors: Ryan Henrie Hannah Cherniawsky Krista Marcon Eric J Zhao Angelina Marinkovic Persia Pourshahnazari Stephen Parkin Luke Y C Chen Source Type: research
Inflammatory diseases in hematology: a review
Am J Physiol Cell Physiol. 2022 Aug 8. doi: 10.1152/ajpcell.00356.2021. Online ahead of print.ABSTRACTHematopoietic cells are instrumental in generating and propagating protective inflammatory responses to infection or injury. However, excessive inflammation contributes to many diseases of the blood, bone marrow, and lymphatic system. We review three clinical categories of hematological inflammatory diseases in which recent clinical and translational advances have been made. The first category are monogenic inflammatory diseases. Genotype-driven research has revealed that previously mysterious diseases with protean manifes...
Source: Am J Physiol Cell Ph... - August 8, 2022 Category: Cytology Authors: Ryan Henrie Hannah Cherniawsky Krista Marcon Eric J Zhao Angelina Marinkovic Persia Pourshahnazari Stephen Parkin Luke Y C Chen Source Type: research
Inflammatory diseases in hematology: a review
Am J Physiol Cell Physiol. 2022 Aug 8. doi: 10.1152/ajpcell.00356.2021. Online ahead of print.ABSTRACTHematopoietic cells are instrumental in generating and propagating protective inflammatory responses to infection or injury. However, excessive inflammation contributes to many diseases of the blood, bone marrow, and lymphatic system. We review three clinical categories of hematological inflammatory diseases in which recent clinical and translational advances have been made. The first category are monogenic inflammatory diseases. Genotype-driven research has revealed that previously mysterious diseases with protean manifes...
Source: American Journal of Physiology. Cell Physiology - August 8, 2022 Category: Cytology Authors: Ryan Henrie Hannah Cherniawsky Krista Marcon Eric J Zhao Angelina Marinkovic Persia Pourshahnazari Stephen Parkin Luke Y C Chen Source Type: research
122 Profiling immune network in a case of regressing Rosai-Dorfman disease, a rare histiocytic dermatosis
Rosai-Dorfman disease (RDD) is a rare and heterogeneous histiocytic disorder characterized by myeloid cell proliferation, accompanying infiltrates of mixed inflammatory cells. The pathophysiology of this chronic inflammatory disease remains poorly understood presenting therapeutic challenges. Here, we report the immune profile of a case of a 47-year-old female patient previously diagnosed as RDD using single cell RNA sequencing(scRNA-seq). The patient first presented with disfiguring nodular skin lesions with leonine face. (Source: Journal of Investigative Dermatology)
Source: Journal of Investigative Dermatology - July 20, 2022 Category: Dermatology Authors: K. Chung, J. Hwang, D. KIm Source Type: research
Extranodal Rosai-Dorfman Disease Involving the Pancreas: A Case Report and Literature Review
No abstract available (Source: Pancreas)
Source: Pancreas - July 1, 2022 Category: Gastroenterology Tags: Letters to the Editor Source Type: research
Rosai-dorfman-destombes disease: a rare cause of obstructive jaundice
Daud Akhtar: NO financial relationship with a commercial interest | Mira Donaldson: NO financial relationship with a commercial interest | Nabeel Akhtar: NO financial relationship with a commercial interest | S. Ian Gan: YES financial relationship with a commercial interest;Romark:Stock Shareholder (Source: Gastrointestinal Endoscopy)
Source: Gastrointestinal Endoscopy - June 1, 2022 Category: Gastroenterology Authors: Daud Akhtar, Mira A. Donaldson, Nabeel H. Akhtar, S. Ian Gan Tags: Tuesday, May 24, 2022 2:00 PM 3:30 PM Source Type: research
217 Histiocytes and the brain: a rare case of Rosai-Dorfman disease responsive to trametinib
We report the case of a 70-year-old female who presented with pan-hypopituitarism, and was found to have leptomeningeal enhancement and an infundibular mass lesion on MRI. Brain biopsy confirmed a histological diagnosis of RDD, and initially she was managed conservatively.
Four years later her cognition, gait and level of functioning deteriorated. Serial MRIs showed slow progres- sion of the lesions. Treatment with pulsed methylprednisolone followed by an oral taper proved ineffec- tive. A multi-disciplinary team decision was made to start treatment with Trametinib (1mg once daily).
A repeat MRI after 6 months showed a red...
Source: Journal of Neurology, Neurosurgery and Psychiatry - May 27, 2022 Category: Neurosurgery Authors: Hawken, J., Hu, M., Johnston, A., Edwards, M. Tags: Poster Presentations Source Type: research
Successful treatment of multisystemic Rosai-Dorfman disease with lenalidomide and dexamethasone: a case report
(Source: Annals of Hematology)
Source: Annals of Hematology - May 23, 2022 Category: Hematology Source Type: research
Primary cutaneous Rosai-Dorfman-Destombes disease with features mimicking IgG4-related disease: A challenging case report and literature review
We report a case of a 57-year-old patient, who presented with an indolent skin nodule in the left sub-nipple area. Surgical excision was performed. Histological examination concluded to the diagnosis of cutaneous RDD with histological features mimicking IgG4-related disease. The diagnosis of systemic RDD with cutaneous involvement was ruled out after exclusion of extracutaneous involvement. No recurrence or systemic progression was observed during follow-up. The diagnosis of primary cutaneous RDD is very uncommon and hence is challenging for pathologist and dermatologist especially with features of IgG4-related disease. Ca...
Source: The Australasian Journal of Dermatology - May 18, 2022 Category: Dermatology Authors: Mouna Zghal Saadia Makni Ines Saguem Amir Karra Soumaya Graja Naourez Gouiaa Tahya Sellami Manel Mellouli Source Type: research
Primary cutaneous Rosai-Dorfman-Destombes disease with features mimicking IgG4-related disease: A challenging case report and literature review
We report a case of a 57-year-old patient, who presented with an indolent skin nodule in the left sub-nipple area. Surgical excision was performed. Histological examination concluded to the diagnosis of cutaneous RDD with histological features mimicking IgG4-related disease. The diagnosis of systemic RDD with cutaneous involvement was ruled out after exclusion of extracutaneous involvement. No recurrence or systemic progression was observed during follow-up. The diagnosis of primary cutaneous RDD is very uncommon and hence is challenging for pathologist and dermatologist especially with features of IgG4-related disease. Ca...
Source: The Australasian Journal of Dermatology - May 18, 2022 Category: Dermatology Authors: Mouna Zghal Saadia Makni Ines Saguem Amir Karra Soumaya Graja Naourez Gouiaa Tahya Sellami Manel Mellouli Source Type: research
Primary cutaneous Rosai-Dorfman-Destombes disease with features mimicking IgG4-related disease: A challenging case report and literature review
We report a case of a 57-year-old patient, who presented with an indolent skin nodule in the left sub-nipple area. Surgical excision was performed. Histological examination concluded to the diagnosis of cutaneous RDD with histological features mimicking IgG4-related disease. The diagnosis of systemic RDD with cutaneous involvement was ruled out after exclusion of extracutaneous involvement. No recurrence or systemic progression was observed during follow-up. The diagnosis of primary cutaneous RDD is very uncommon and hence is challenging for pathologist and dermatologist especially with features of IgG4-related disease. Ca...
Source: The Australasian Journal of Dermatology - May 18, 2022 Category: Dermatology Authors: Mouna Zghal Saadia Makni Ines Saguem Amir Karra Soumaya Graja Naourez Gouiaa Tahya Sellami Manel Mellouli Source Type: research
Primary cutaneous Rosai-Dorfman-Destombes disease with features mimicking IgG4-related disease: A challenging case report and literature review
We report a case of a 57-year-old patient, who presented with an indolent skin nodule in the left sub-nipple area. Surgical excision was performed. Histological examination concluded to the diagnosis of cutaneous RDD with histological features mimicking IgG4-related disease. The diagnosis of systemic RDD with cutaneous involvement was ruled out after exclusion of extracutaneous involvement. No recurrence or systemic progression was observed during follow-up. The diagnosis of primary cutaneous RDD is very uncommon and hence is challenging for pathologist and dermatologist especially with features of IgG4-related disease. Ca...
Source: The Australasian Journal of Dermatology - May 18, 2022 Category: Dermatology Authors: Mouna Zghal Saadia Makni Ines Saguem Amir Karra Soumaya Graja Naourez Gouiaa Tahya Sellami Manel Mellouli Source Type: research
Neurology and the histiocytoses: a case of Rosai-Dorfman-Destombes disease
We present a challenging diagnostic and management case of Rosai-Dorfman-Destombes disease in a 48-year-old woman with a relapsing, partially steroid-responsive syndrome comprising patchy, non-length-dependent radiculoneuropathy with diffuse pachymeningitis and widespread systemic disease, and recent dramatic response to novel mitogen-activated kinase pathway inhibition. We discuss the clinical characteristics, diagnosis, recent breakthroughs in pathogenesis and emerging treatment options for Rosai-Dorfman disease and for the histiocytoses with neurological sequelae, including Langerhans cell histiocytosis and Erdheim-Ches...
Source: Practical Neurology - May 9, 2022 Category: Neurology Authors: Antonia S Carroll Carolynne M Doherty Julian Blake Stephen J Hunt Chandrashekar Hoskote Feargal McNicholl Rahul Phadke Oonagh Sheehy Fion D Bremner Shirley D'Sa Christopher McNamara Mary M Reilly Source Type: research
Neurology and the histiocytoses: a case of Rosai-Dorfman-Destombes disease
We present a challenging diagnostic and management case of Rosai-Dorfman-Destombes disease in a 48-year-old woman with a relapsing, partially steroid-responsive syndrome comprising patchy, non-length-dependent radiculoneuropathy with diffuse pachymeningitis and widespread systemic disease, and recent dramatic response to novel mitogen-activated kinase pathway inhibition. We discuss the clinical characteristics, diagnosis, recent breakthroughs in pathogenesis and emerging treatment options for Rosai-Dorfman disease and for the histiocytoses with neurological sequelae, including Langerhans cell histiocytosis and Erdheim-Ches...
Source: Practical Neurology - May 9, 2022 Category: Neurology Authors: Antonia S Carroll Carolynne M Doherty Julian Blake Stephen J Hunt Chandrashekar Hoskote Feargal McNicholl Rahul Phadke Oonagh Sheehy Fion D Bremner Shirley D'Sa Christopher McNamara Mary M Reilly Source Type: research
