16 results
Pancytopenia - Differential Diagnosis

Congenital Diseases:
• GATA2 deficiency
• Fanconi’s anemia
• Wiskott-Aldrich syndrome
• Ribosomopathy or telomeropathy

Cancer:
• Leukemia
• Lymphoma

Inflammatory
Congenital Diseases ... Wiskott-Aldrich syndrome ... and Autoimmune Diseases ... Systemic lupus erythematosus ... • HLH • Paroxysmal
Macrophage Activation Syndrome (MAS)
Classified among the secondary or acquired forms of haemophagocytic lymphohistiocytosis (sHLH)

What?
A subset of
Systemic lupus erythematosus ... systemic lupus erythematosus ... • HLH Labs: ... #Diagnosis #Management ... #Hematology #Rheumatology
Systemic Lupus Erythematosus - Summary
Antinuclear (ANA) - 95% - Initial screening test
Anti-dsDNA - 50%
 • Associated
Systemic Lupus Erythematosus ... block • Sjogren syndrome ... polymyositis overlap syndrome ... Summary #diagnosis #rheumatology ... #management
Aortitis - Differential Diagnosis Framework
Non-Infectious Aortitis:
 • Vasculitides: Giant Cell Arteritis (GCA), Takayasu's arteritis, Cogan's Syndrome,
arteritis, Cogan's Syndrome ... : IgG4-Related Disease ... Systemic lupus erythematosus ... , Sarcoidosis, HLA-B27 ... Differential #Diagnosis #rheumatology
Hemophagocytic Lymphohistiocytosis (HLH)
Pathophysiology: Triggering factor (infection,...)→Activation of CD8 T cells (IFN-y) → Excessive activation of macrophages
• Autoimmune diseases ... Adult-onset Still disease ... Nicolas Taar ... Lymphohistiocytosis #diagnosis #management ... treatment #summary #rheumatology
Hemophagocytic Lymphohistiocytosis (HLH)
Definition: Overwhelming clinical syndrome associated with excessive macrophage activation and cytokine storm due to
Lymphohistiocytosis (HLH ... Overwhelming clinical syndrome ... age, however the disease ... Lymphohistiocytosis #diagnosis #management ... #treatment #hematology
CNS Manifestations of Systemic Lupus Erythematosus (SLE)

Clinical Manifestations: Demyelinating Syndrome, Headache, Movement disorders, Seizure disorders, Aseptic
Systemic Lupus Erythematosus ... Demyelinating Syndrome ... Cerebrovascular Disease ... CNS #neurology #rheumatology ... cerebritis #diagnosis #management
VEXAS (Vacuoles, E1 enzyme, X-linked, Autoinflammatory, Somatic) Syndrome
Clinical Syndrome:
 • Common Clinical Features: alveolitis, ear and
Clinical Syndrome ... Features: alveolitis, ear ... thromboembolic disease ... myeloid cells NEJM ... Autoinflammatory #Somatic #rheumatology
Behçet's Syndrome
Systemic disease associated with inflammation of multiple organs, small-vessel vasculitis and large-vessel vasculopathy
Epidemiology:
 • Young
Behçet's Syndrome ... common in Asia • HLA-B51 ... Giant retinal tears ... #diagnosis #management ... signs #symptoms #rheumatology
Susac Syndrome - Clinical Triad
 - Central nervous system dysfunction
 - Branch retinal artery occlusion
 -
Susac Syndrome - ... - Recurrent disease ... flares - 2-year ... Triad #Diagnosis #Management ... #Rheumatology