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diagnosis rheumatology differential oncology clarksons classification common cryofibrinogenemia cryoglobulins dermatologic gammopathy gpa granulomatosis hypogammaglobulinemia immunodeficiency immunology leak mgus paraproteinemias polyangiitis
Monoclonal Gammopathy of Clinical Significance (MGUS) - Differential Diagnosis Neurologic-Centered: (+) Systemic Features: POEMS, AL amyloid, Cryoglobulinemia
of Clinical Significance ... : • Systemic capillary ... leak syndrome ( ... Ig deposition disease ... Differential #Diagnosis #hematology
Cryoglobulinemia Summary Cryoglobulins: • Cryoglobulins are Igs in the serum that precipitate in vitro at temperatures below
• They can cause ... Cryoglobulins Cause ... Syndrome: • Clinical ... • Autoimmune diseases ... - Composed of monoclonal
Paraproteinemias Entities That Can Feature A Monoclonal Protein/M Component: • MM • WM • MGUS • MGCS • MGRS • Splenic Marginal Zone
• Heavy Chain disease ... Clarkson/Systemic capillary ... (Cryoglobulinemic ... Sneddon-Wilkinson disease ... Paraproteinemias #Hematology
Dermatologic Manifestations of Granulomatosis with Polyangiitis (GPA) Skin involvement in granulomatosis with polyangiitis (GPA) is common and
GPA causes disease ... Rarely dominate the clinical ... purpura • Mixed cryoglobulinemia ... • Goodpasture syndrome ... Polyangiitis #GPA #dermatology
Common Variable Immunodeficiency (CVID) What? CVID: Immunodeficiency disorder with hypogammaglobulinemia -> increased infection risk secondary to impaired B-cell
to 8 years Clinical ... DDX - Other causes ... : • Nephrotic syndrome ... (Epstein-Barr syndrome ... hypogammaglobulinemia #immunology #hematology
Cryofibrinogenemia Summary Cryofibrinogenemia Epidemiology: • 40-70 years with a modest female predominance Cryofibrinogenemia: • The precipitation of a
malignancies, vasculitis ... 2019 (COVID-19) ... cryoglobulins • Negative causes ... Antiphospholipid syndrome ... #hematology
Clarkson’s Disease - Capillary leak syndrome Epidemiology: Roughly 150 published cases, Median age 50 years, No sex
- Capillary leak ... Relapsing-remitting course +++ Clinical ... Monoclonal gammopathy ... #Capillary #leak ... #management #timeline
Sjogren's Syndrome Overview Epidemiology: • F > M: 9:1 • 5-6th Decades (can be any age) Autoimmune exocrinopathy multisystemic disease
salivary ducts Clinical ... ) • Cutaneous vasculitis ... Nephrogenic DI Hematologic ... : • 7%, Cryoglobulinemia ... Diagnosis #Management
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