44 results
VEXAS (Vacuoles, E1 enzyme, X-linked, Autoinflammatory, Somatic) Syndrome
Clinical Syndrome:
 • Common Clinical Features: alveolitis, ear and
nose chondritis, skin ... vasculitis • Hematologic ... criteria for 1) hematologic ... blood myeloid cells ... Autoinflammatory #Somatic #rheumatology
Leukostasis vs Tumor Lysis Syndrome
Leukostasis:
 • Pathophysiology: Large, immature blasts and high WBC count cause hyperviscosity
Leukostasis: • Pathophysiology ... WBC >100k, + lab signs ... : • Pathophysiology ... Lysis of tumor cells ... diagnosis #management #hematology
Eosinophil Disorders Testing Algorithm
INDICATIONS FOR TESTING:
 • Peripheral blood eosinophilia/hypereosinophilia uncovered incidentally during medical evaluation or
Eosinophilia, 500-1,500 cells ... glucocorticoid treatment ... organ-specific signs ... symptoms (eg, skin ... Differential #diagnosis #hematology
Myelodysplastic Syndrome (MD) - Diagnosis and Management Summary
myelo (marrow) -dys- (abnormal) -plastic (shape) = funny-shaped cells
Myelodysplastic Syndrome ... = funny-shaped cells ... only curative treatment ... Myelodysplastic #Syndrome ... #hematology #oncology
CNS Manifestations of Systemic Lupus Erythematosus (SLE)

Clinical Manifestations: Demyelinating Syndrome, Headache, Movement disorders, Seizure disorders, Aseptic
Demyelinating Syndrome ... Disease, PRES Pathophysiology ... Puncture, EEG Treatment ... Erythematosus #SLE #CNS #neurology ... #rheumatology #
Acquired von Willebrand Syndrome - Diagnosis and Management
 • Autoantibodies to vWF: Lymphoproliferative disorders (e.g. lymphoma,
von Willebrand Syndrome ... adsorption onto cancer cells ... vonWillebrand #Syndrome ... Diagnosis #Management #treatment ... #hematology #differential
Behcet's Syndrome - Treatment
Ulcers:
 • Treatment: Topical steroids
 • Prevention: Colchicine
 • Azathioprine for refractory disease
Arthritis:
Behcet's Syndrome ... : • Treatment: ... refractory disease Skin ... Behcets #disease #Syndrome ... #pharmacology #rheumatology
Autoimmune Myositis
Necrotizing myopathy is associated with higher CK levels, greater level of muscle weakness, less systemic
heliotrope rash, Shaw sign ... , holster sign, ... anti-synthetase syndrome ... after 2 years of treatment ... Myositis #diagnosis #rheumatology
Systemic Lupus Erythematosus - Summary
Antinuclear (ANA) - 95% - Initial screening test
Anti-dsDNA - 50%
 • Associated
Associated with skin ... block • Sjogren syndrome ... polymyositis overlap syndrome ... severe disease Treatment ... Summary #diagnosis #rheumatology
Behçet's Syndrome
Systemic disease associated with inflammation of multiple organs, small-vessel vasculitis and large-vessel vasculopathy
Epidemiology:
 • Young
response to local skin ... injury) • Neurologic ... Arthritis, AS Treatment ... diagnosis #management #signs ... #symptoms #rheumatology