Renal crystal-storing histiocytosis successfully treated with bortezomib-based regimen
SummaryCrystal-storing histiocytosis (CSH) represents a rare disease seen in patients with monoclonal gammopathy of unknown significance, multiple myeloma, lymphoplasmocytic lymphoma, and other hematological disorders. In the kidneys, it commonly involves the interstitium but can also involve the glomeruli or the vasculature. Herein we present a case of CSH involving the renal interstitium in a patient who presented with rapidly progressing renal failure. He was treated with cyclophosphamide, dexamethasone and bortezomib-based chemotherapy and afterwards his renal function improved back to baseline. CSH is a rare mono...
Source: Memo - Magazine of European Medical Oncology - November 7, 2023 Category: Cancer & Oncology Source Type: research
The clinical spectrum and prognostic factors of Erdheim-Chester disease and mixed Langerhans cell histiocytosis and Erdheim-Chester disease
AbstractErdheim-Chester disease (ECD) is a rare and probably fatal multisystemic non-Langerhans cell histiocytosis (LCH). To comprehensively investigate the clinical features, genomic analysis, treatments, and prognostic factors of ECD, we retrospectively analyzed the clinical data of 75 ECD patients and 10 mixed LCH and ECD patients in our center. The median age at diagnosis was 46 years (range, 5–70). ECD patients were older at diagnosis (p = 0.006) and had more cardiac involvement (p = 0.011) as well as vascular (p = 0.031) involvement compared to mixed LCH and ECD patients. 64.8% of ECD patients and 87....
Source: Annals of Hematology - November 3, 2023 Category: Hematology Source Type: research
Single-system pulmonary langerhans cell histiocytosis with only tracheobronchial involvement: a case report
Pulmonary Langerhans cell histiocytosis (PLCH) only with airway involvement manifested as diffuse thickening of the tracheobronchial walls is rare. (Source: BMC Pulmonary Medicine)
Source: BMC Pulmonary Medicine - October 28, 2023 Category: Respiratory Medicine Authors: Xin Peng, Hui Liu, Xinyu Zhang, Huaibi Huo and Ting Liu Tags: Case Report Source Type: research
Is it possible to predict multisystem Langerhans cell histiocytosis with lung involvement based on radiological findings detected by computed tomography?
Conclusion:The high intensity of cigarette smoking and low severity of cysts lesions can be a predictor of isolated PLCH. (Source: European Respiratory Journal)
Source: European Respiratory Journal - October 27, 2023 Category: Respiratory Medicine Authors: Radzikowska, E., Jeskiewicz, M., Blasinska, K., Wakulinski, J., Sobiecka, M., Wiatr, E., Załeska, M., Langfort, R., Bestry, I. Tags: Rare ILD/DPLD Source Type: research
Clinical characteristics of diffuse cystic lung diseases at a national referral centre
Conclusions: Our data indicates that despite their rarity, DCLDs share common features, yet differ in presentation and clinical course. With increased risk of malignancy and possible progressive disease, it is increasingly important to screen for these conditions. (Source: European Respiratory Journal)
Source: European Respiratory Journal - October 27, 2023 Category: Respiratory Medicine Authors: O'Brien, H., Gill, C., O'Callaghan, M., Lynn, E., Hickey, F., Khan, J., Franciosi, A., Keane, M. P., Mc Carthy, C. Tags: Rare ILD/DPLD Source Type: research
Lipid metabolism in interstitial lung diseases: serum amyloid A a new potential biomarker in idiopathic pulmonary fibrosis
Conclusions: SAA could be a potential biomarker of IPF that can predict clinical course, prognosis and survival of IPF patients. Monitoring SAA serum levels could help to identify rapidly progressive phenotype of IPF or at risk of acute exacerbation.References[1] Vietri, L. et al. Respiratory Investigation 2019; 57(5):430-434. (Source: European Respiratory Journal)
Source: European Respiratory Journal - October 27, 2023 Category: Respiratory Medicine Authors: Vietri, L., D'Alessandro, M., Bergantini, L., Cameli, P., Bargagli, E. Tags: Idiopathic interstitial pneumonias Source Type: research
Mutant PIK3CA is a targetable driver alteration in histiocytic neoplasms
Blood Adv. 2023 Oct 23:bloodadvances.2022009349. doi: 10.1182/bloodadvances.2022009349. Online ahead of print.ABSTRACTLangerhans cell histiocytosis (LCH) is an inflammatory myeloid neoplasm characterized by the accumulation of clonal mononuclear phagocyte system cells expressing CD1a and CD207. In the past decade, molecular profiling of LCH, as well as other histiocytic neoplasms demonstrated that these diseases are driven by MAP kinase (MAPK) activating alterations, with somatic BRAFV600E mutations in >50% of LCH patients, and clinical inhibition of MAPK signaling has demonstrated remarkable clinical efficacy. At the s...
Source: Adv Data - October 24, 2023 Category: Epidemiology Authors: Benjamin H Durham Oshrat Hershkovitz-Rokah Omar I Abdel-Wahab Mariko Yabe Young Rock Chung Gilad Itchaki Maayan Ben-Sasson Vered A Asher-Guz David Groshar Seyram A Doe-Tetteh Tina Alano David B Solit Ofer Shpilberg Eli L Diamond Roei D Mazor Source Type: research
Mutant PIK3CA is a targetable driver alteration in histiocytic neoplasms
Blood Adv. 2023 Oct 23:bloodadvances.2022009349. doi: 10.1182/bloodadvances.2022009349. Online ahead of print.ABSTRACTLangerhans cell histiocytosis (LCH) is an inflammatory myeloid neoplasm characterized by the accumulation of clonal mononuclear phagocyte system cells expressing CD1a and CD207. In the past decade, molecular profiling of LCH, as well as other histiocytic neoplasms demonstrated that these diseases are driven by MAP kinase (MAPK) activating alterations, with somatic BRAFV600E mutations in >50% of LCH patients, and clinical inhibition of MAPK signaling has demonstrated remarkable clinical efficacy. At the s...
Source: Adv Data - October 24, 2023 Category: Epidemiology Authors: Benjamin H Durham Oshrat Hershkovitz-Rokah Omar I Abdel-Wahab Mariko Yabe Young Rock Chung Gilad Itchaki Maayan Ben-Sasson Vered A Asher-Guz David Groshar Seyram A Doe-Tetteh Tina Alano David B Solit Ofer Shpilberg Eli L Diamond Roei D Mazor Source Type: research
Neurological syndromes and potential triggers associated with antibodies to neuronal surface antigens
CONCLUSION: We identified seasonal variability associated with neuronal surface antibodies suggesting environmental triggers. Also, we described the coexistence of NMDAr-IgG encephalitis with histiocytosis. In our series, most patients received second-line immunotherapy. We observed neurologic improvement after treatment even in cases of delayed diagnosis. Increasing the recognition and availability of tests and treatments for these conditions is of paramount importance in low- and middle-income countries.PMID:37864878 | DOI:10.1016/j.msard.2023.105022 (Source: Herpes)
Source: Herpes - October 21, 2023 Category: Infectious Diseases Authors: Bruna Klein da Costa Paula de Oliveira Pinto Lia Staub Gisele Hansel Guilherme Vanik Pinto Lucas Porcello Schilling Giordani Rodrigues Dos Passos William Alves Martins Jefferson Becker Raphael Machado Castilhos Andr é Palmini Douglas Kazutoshi Sato Source Type: research
Sea-blue histiocytosis
Ann Pathol. 2023 Oct 19:S0242-6498(23)00202-X. doi: 10.1016/j.annpat.2023.10.001. Online ahead of print.ABSTRACTA 78-year-old woman with hypertrophic cardiomyopathy underwent a septal myomectomy and valve replacement. In the immediate postoperative period she developed shock of mixed etiology and died. At autopsy, hepatomegaly and splenomegaly were identified, with PAS and Giemsa positive intracellular ceroid granular deposits. Sea-blue histiocytosis is an extremely rare, chronic and benign deposit disease. It is characterized by hepatosplenomegaly, thrombocytopenia and lymphadenopathy. The presence of ceroid substance in ...
Source: Annales de Pathologie - October 21, 2023 Category: Pathology Authors: M ónica Bronte Anaut Javier Arredondo Montero Source Type: research
Neurological syndromes and potential triggers associated with antibodies to neuronal surface antigens
CONCLUSION: We identified seasonal variability associated with neuronal surface antibodies suggesting environmental triggers. Also, we described the coexistence of NMDAr-IgG encephalitis with histiocytosis. In our series, most patients received second-line immunotherapy. We observed neurologic improvement after treatment even in cases of delayed diagnosis. Increasing the recognition and availability of tests and treatments for these conditions is of paramount importance in low- and middle-income countries.PMID:37864878 | DOI:10.1016/j.msard.2023.105022 (Source: Herpes)
Source: Herpes - October 21, 2023 Category: Infectious Diseases Authors: Bruna Klein da Costa Paula de Oliveira Pinto Lia Staub Gisele Hansel Guilherme Vanik Pinto Lucas Porcello Schilling Giordani Rodrigues Dos Passos William Alves Martins Jefferson Becker Raphael Machado Castilhos Andr é Palmini Douglas Kazutoshi Sato Source Type: research
Sea-blue histiocytosis
Ann Pathol. 2023 Oct 19:S0242-6498(23)00202-X. doi: 10.1016/j.annpat.2023.10.001. Online ahead of print.ABSTRACTA 78-year-old woman with hypertrophic cardiomyopathy underwent a septal myomectomy and valve replacement. In the immediate postoperative period she developed shock of mixed etiology and died. At autopsy, hepatomegaly and splenomegaly were identified, with PAS and Giemsa positive intracellular ceroid granular deposits. Sea-blue histiocytosis is an extremely rare, chronic and benign deposit disease. It is characterized by hepatosplenomegaly, thrombocytopenia and lymphadenopathy. The presence of ceroid substance in ...
Source: Annales de Pathologie - October 21, 2023 Category: Pathology Authors: M ónica Bronte Anaut Javier Arredondo Montero Source Type: research
Sea-blue histiocytosis
Ann Pathol. 2023 Oct 19:S0242-6498(23)00202-X. doi: 10.1016/j.annpat.2023.10.001. Online ahead of print.ABSTRACTA 78-year-old woman with hypertrophic cardiomyopathy underwent a septal myomectomy and valve replacement. In the immediate postoperative period she developed shock of mixed etiology and died. At autopsy, hepatomegaly and splenomegaly were identified, with PAS and Giemsa positive intracellular ceroid granular deposits. Sea-blue histiocytosis is an extremely rare, chronic and benign deposit disease. It is characterized by hepatosplenomegaly, thrombocytopenia and lymphadenopathy. The presence of ceroid substance in ...
Source: Annales de Pathologie - October 21, 2023 Category: Pathology Authors: M ónica Bronte Anaut Javier Arredondo Montero Source Type: research
Sea-blue histiocytosis
Ann Pathol. 2023 Oct 19:S0242-6498(23)00202-X. doi: 10.1016/j.annpat.2023.10.001. Online ahead of print.ABSTRACTA 78-year-old woman with hypertrophic cardiomyopathy underwent a septal myomectomy and valve replacement. In the immediate postoperative period she developed shock of mixed etiology and died. At autopsy, hepatomegaly and splenomegaly were identified, with PAS and Giemsa positive intracellular ceroid granular deposits. Sea-blue histiocytosis is an extremely rare, chronic and benign deposit disease. It is characterized by hepatosplenomegaly, thrombocytopenia and lymphadenopathy. The presence of ceroid substance in ...
Source: Annales de Pathologie - October 21, 2023 Category: Pathology Authors: M ónica Bronte Anaut Javier Arredondo Montero Source Type: research
