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diagnosis agglutinin anemia autoinflammatory cold e1 gammopathy gap globulin hemolytic manifestations mds mgus myelodysplasia paraproteinemias plasmacytoma protein proteingap significance skin
VEXAS (Vacuoles, E1 enzyme, X-linked, Autoinflammatory, Somatic) Syndrome Clinical Syndrome: • Common Clinical Features: alveolitis, ear and
Clinical Syndrome ... thromboembolic disease ... arteritis (4%) ... cells NEJM Review ... Autoinflammatory #Somatic #rheumatology
Cutaneous manifestations associated with myelodysplastic syndrome • Myeloid hemopathy with the most frequent skin lesions (20%)
syndrome • Myeloid ... appearing during the disease ... #syndrome #differential ... #diagnosis #dermatology ... #oncology #clinical
Monoclonal Gammopathy of Clinical Significance (MGUS) - Differential Diagnosis Neurologic-Centered: (+) Systemic Features: POEMS, AL amyloid, Cryoglobulinemia
(MGUS) - Differential ... nemaline myopathy Cutaneous-centered ... Ig deposition disease ... #Diagnosis #hematology ... #oncology
Myelodysplastic Syndromes (MDS) What? Myelodysplastic syndromes are a group of disorders caused by blood cells that are poorly
formed or don’t work ... , or all three myeloid ... Myeloproliferative Disease ... blood count with differential ... #hematology #oncology
Cold Agglutinin Disease 3 Types of Cold Sensitive Antibodies: 1. Cold Agglutinins (CAD) 2. Donath-Landsteiner
Cold Agglutinin Syndrome ... Viral Infection Clinical ... presentation) • Cutaneous ... , Myeloma • Waldenström ... hemolytic #anemia #hematology
Paraproteinemias Entities That Can Feature A Monoclonal Protein/M Component: • MM • WM • MGUS • MGCS • MGRS • Splenic Marginal Zone
Lymphoma (HCV) • CLL ... gangrenosum • Cutaneous ... Paraproteinemias #Hematology ... #Monoclonal #Differential ... #Diagnosis #Oncology
Elevated Globulin - Protein Gap (Total Protein - Albumin > 4) Work-up of an Elevated Globulin Gap: •
Albumin > 4) Work-up ... changes (POEMS syndrome ... (RA, Temporal arteritis ... gastric tumors • Hematology ... #diagnosis #hematology
Solitary Cell Plasmacytoma What? • Plasma cell neoplasms can present as a single lesion (solitary plasmacytoma) • In 3%
plasmacytoma) Clinical ... IFE, FLC assay Pathology ... #Plasmacytoma #Oncology ... #Diagnosis #Workup ... #Hematology
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