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Achievement of Clinical Remission in Life-Threatening Rosai-Dorfman Disease With Cladribine.
Cureus
November 2024
Hematology and Medical Oncology, Kettering Health, Kettering, USA.
Rosai-Dorfman disease (RDD) is a rare proliferative histiocytic disorder characterized by sinus histiocytosis with massive lymphadenopathy, rarely presenting with severe and life-threatening extra-nodal features. The rarity of RDD, clinically variant phenotype, limited data, and lack of a current standardized management approach make treatment decisions difficult. Herein, we present a case of life-threatening, disseminated RDD with rare clinical features of recurrent pericardial effusion, bilateral pleural effusions, and abdominal tissue fibrosis successfully treated with six cycles of cladribine, achieving clinical remission.
View Article and Find Full Text PDF[Hemophagocytic Lymphohistiocytosis: Clinical Characteristics and Diagnostic Prediction Model].
Zhongguo Shi Yan Xue Ye Xue Za Zhi
October 2024
Department of Medical Intensive Care Unit, The Third Affiliated Hospital of Sun Yat-Sen University, Guangzhou 510630, Guangdong Province, China.
Objective: To investigate the clinical characteristics of patients with hemophagocytic lymphohistiocytosis (HLH) and quantify the diagnostic value of various indexes in patients with elevated soluble interleukin-2 receptor (sCD25), so as to construct a diagnostic prediction model of HLH.
Methods: The clinical characteristics of 121 patients with elevated sCD25 (≥2 400 U/ml) in the Third Affiliated Hospital of Sun Yat-Sen University were analyzed retrospectively. The patients were divided into HLH group and non-HLH group according to the diagnostic criteria of HLH.
[Clinical Crrelation and Prognostic Analysis of ALBI Score in Secondary Hemophagocytic Syndrome in Children].
Zhongguo Shi Yan Xue Ye Xue Za Zhi
October 2024
Department of Pediatrics, The Affiliated Hospital of Zunyi Medical University； Department of Pediatrics, Guizhou Children's Hospital, Collaborative Innovation Center for Tissue Injury Repair and Regenerative Medicine of Zunyi Medical University, Zunyi 563000，Guizhou Province, China.
Objective: To explore the clinical correlation and prognostic value of the Albumin-Bilirubin (ALBI) score in children with secondary hemophagocytic syndrome(sHLH).
Methods: A retrospective analysis was conducted on the data of children's sHLH cases clearly diagnosed in the Affiliated Hospital of Zunyi Medical University from January 2012 to March 2023. Survival analysis was conducted according to the ALBI classification.
Prominent tissue hemophagocytic lymphohistiocytosis obscuring primary cutaneous gamma/delta (γδ) T-cell lymphoma.
J Cutan Pathol
December 2024
Department of Pathology and Area Laboratory Services, Brooke Army Medical Center, JBSA-Fort Sam Houston, Texas, USA.
Primary cutaneous gamma/delta (γδ) T-cell lymphoma (PCGDTCL) is a rare, aggressive malignant neoplasm of γδ T lymphocytes arising within the skin and subcutis. We present a challenging case of PCGDTCL diagnosed in a 35-year-old male soldier who presented with constitutional symptoms, pancytopenia, hemophagocytic lymphohistiocytosis (HLH), disseminated lymphadenopathy, and cutaneous lesions on his extremities and back following a deployment to Iraq and Syria. Histopathologic evaluation of an excisional biopsy showed that PCGDTCL can be focal, localized to the subcutaneous adipose tissue, and obscured by predominant HLH in the surrounding tissues.
View Article and Find Full Text PDFHaemophagocytic lymphohistiocytosis secondary to disseminated histoplasmosis in a patient with leprosy.
BMJ Case Rep
September 2024
Medical Center Health Bowling Green, Bowling Green, Kentucky, USA.
Multidrug therapy has significantly reduced the global burden of Hansen's disease; however, complications from long-term treatment persist. A male resident of southern Kentucky, in his 30s and of Micronesian descent, presented with worsening abdominal pain associated with anorexia, fatigue, functional decline and occasional haemoptysis. He was compliant with multidrug therapy for leprosy.
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