23 results
Dermatologic Manifestations of Granulomatosis with Polyangiitis (GPA)

Skin involvement in granulomatosis with polyangiitis (GPA) is common and
Dermatologic Manifestations ... of Granulomatosis ... vasculitis #Dermatologic ... Polyangiitis #GPA #dermatology ... rash #diagnosis #rheumatology
Proposed Treatment Algorithm for MAS / HLH

#MAS #HLH #Hemophagocytic #Lymphohistiocytosis #Management #Treatment #Algorithm #Hematology
Hemophagocytic #Lymphohistiocytosis ... #Management #Treatment ... #Algorithm #Hematology
Von Willebrand Disease - Clinical Presentation, Diagnosis and Management

- Dr. Eric Strong @DrEricStrong - Strong Medicine
Von Willebrand Disease ... Diagnosis and Management ... VonWillebrand #Disease ... #Diagnosis #Management ... #hematology #treatment
Common Variable Immunodeficiency (CVID)

What?
CVID: Immunodeficiency disorder with hypogammaglobulinemia -> increased infection risk secondary to impaired B-cell
Malabsorption • Granulomatous ... breast cancer Granulomatous ... and M panel Management ... hypogammaglobulinemia #immunology #hematology ... #diagnosis #management
Macrophage Activation Syndrome (MAS)
Classified among the secondary or acquired forms of haemophagocytic lymphohistiocytosis (sHLH)

What?
A subset of
haemophagocytic lymphohistiocytosis ... hemophagocytic lymphohistiocytosis ... highly inflammatory disease ... Syndrome #Diagnosis #Management ... #Hematology #Rheumatology
Hemophagocytic Lymphohistiocytosis (HLH)
Definition: Overwhelming clinical syndrome associated with excessive macrophage activation and cytokine storm due to
Hemophagocytic Lymphohistiocytosis ... age, however the disease ... Hemophagocytic #Lymphohistiocytosis ... #diagnosis #management ... #treatment #hematology
Hemophagocytic Lymphohistiocytosis (HLH)
Pathophysiology: Triggering factor (infection,...)→Activation of CD8 T cells (IFN-y) → Excessive activation of macrophages
Hemophagocytic Lymphohistiocytosis ... • Autoimmune diseases ... Adult-onset Still disease ... #diagnosis #management ... treatment #summary #rheumatology
Hemophagocytic Lymphohistiocytosis (HLH) Treatment
Primary HLH:
 - HLH-94 Protocol
    • Dexamethasone
Hemophagocytic Lymphohistiocytosis ... or autoimmune disease ... for refractory disease ... HLH #Treatment #management ... #hematology
Acquired von Willebrand Syndrome - Diagnosis and Management
 • Autoantibodies to vWF: Lymphoproliferative disorders (e.g. lymphoma,
Diagnosis and Management ... thrombocythemia), Autoimmune disease ... Syndrome #Diagnosis #Management ... #treatment #hematology
Hemophilia - Diagnosis and Management
Diagnosis:
 • Consistent bleeding history (unless screening for disease in family members
Diagnosis and Management ... screening for disease ... with established disease ... Chronic Management ... #treatment #hematology