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Erdheim-Chester Disease: A Case Report Exploring Multisystemic Involvement.

Cureus

November 2024

Neuroradiology, Unidade Local de Saúde Vila Nova de Gaia | Espinho, Vila Nova de Gaia, PRT.

Erdheim-Chester disease (ECD) is a rare, multisystemic, non-Langerhans cell histiocytic neoplasm predominantly affecting middle-aged males in their fifth to seventh decades of life. It often presents with nonspecific symptoms, leading to a delay in its diagnosis. We report a case of an 85-year-old male with multisystemic manifestations, including retroperitoneal, skeletal, vascular, cardiac, orbital, and central nervous system (CNS) involvement.

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We found 18 patients with immunophenotype consistent with classic hairy cell leukemia (HCL) and BRAF mutations other than just V600E. Twelve had 1 non-V600E BRAF mutation and 6 had V600E with 1 (n=5) or 2 (n=1) non-V600E BRAF co-mutations.

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Long-term cultured calli may experience a biosynthetic shift due to the IAA-dependent expression of the rolA gene, which also affects ROS metabolism. The "hairy root" syndrome is caused by the root-inducing Ri-plasmid of Rhizobium rhizogenes, also known as Agrobacterium rhizogenes. The Ri-plasmid contains genes known as rol genes or root oncogenic loci, which promote root development.

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Hairy cell proliferations represent very different entities. They include hairy cell leukemia in its classic form (HCL), a well-defined entity, but also the variant form of HCL (LT-V ou HCL-V), whose presentation is far from HCL and whose prognosis is poorer. Other hairy cell proliferations include splenic red pulp lymphoma (SDRPL) and splenic marginal zone lymphomas (SMZL) with circulating villous cells.

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Pure Biclonal Hairy Cell Leukemia-Apt Diagnosis with Multicolor Flow Cytometry.

Int J Hematol Oncol Stem Cell Res

October 2024

Department of Pathology and Laboratory Medicine, Aga Khan University, Karachi, Pakistan.

Hairy cell leukemia (HCL) is a rare B-cell neoplasm that constitutes around 2 percent of all lymphoid leukemias and occurs more frequently in elderly males. The usual triad of HCL includes pancytopenia, splenomegaly, and hairy cells in the bone marrow. This is a case of an atypical presentation of biclonal HCL diagnosed on flow cytometry; the existence of biclonal HCL is extremely rare with very few case reports.

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