17 results
Hemophagocytic Lymphohistiocytosis (HLH)
Pathophysiology: Triggering factor (infection,...)→Activation of CD8 T cells (IFN-y) → Excessive activation of macrophages
accumulation of clinical ... Assessment • Clinical ... • Autoimmune diseases ... Treatment: • ... #summary #rheumatology
Hemophagocytic Lymphohistiocytosis (HLH)
Definition: Overwhelming clinical syndrome associated with excessive macrophage activation and cytokine storm due to
: Overwhelming clinical ... age, however the disease ... • Bicytopenia Treatment ... diagnosis #management #treatment ... #hematology
Hemophagocytic Lymphohistiocytosis (HLH) Treatment
Primary HLH:
 - HLH-94 Protocol
    • Dexamethasone
) Treatment Primary ... or autoimmune disease ... , treatment of underlying ... for refractory disease ... #hematology
Macrophage Activation Syndrome (MAS)
Classified among the secondary or acquired forms of haemophagocytic lymphohistiocytosis (sHLH)

What?
A subset of
fever syndromes Clinical ... Fever is the main clinical ... Treatment: • Corticosteroids ... Diagnosis #Management #Hematology ... #Rheumatology
Behçet's Syndrome
Systemic disease associated with inflammation of multiple organs, small-vessel vasculitis and large-vessel vasculopathy
Epidemiology:
 • Young
association • M > F Clinical ... skin injury) • Neurologic ... and diplopia) • Vascular ... Arthritis, AS Treatment ... signs #symptoms #rheumatology
CNS Manifestations of Systemic Lupus Erythematosus (SLE)

Clinical Manifestations: Demyelinating Syndrome, Headache, Movement disorders, Seizure disorders, Aseptic
Erythematosus (SLE) Clinical ... Cerebrovascular Disease ... autoantibodies that will cause ... Erythematosus #SLE #CNS #neurology ... #rheumatology #
Cryofibrinogenemia Summary

Cryofibrinogenemia Epidemiology:
 • 40-70 years with a modest female predominance

Cryofibrinogenemia:
 • The precipitation of a
Hyperviscosity, - Vascular ... Diagnosis: • + Clinical ... cryoglobulins • Negative causes ... Cryofibrinogenemia #diagnosis #rheumatology ... #hematology
Hodgkin's Lymphoma Overview

Hodgkin's lymphoma (HL) is an uncommon hematological malignancy arising from mature B cells. It
of about three cases ... Hodgkin Lymphoma - Clinical ... number of sites of disease ... Hodgkin Lymphoma Treatment ... classification #hematology
Clarkson’s Disease - Capillary leak syndrome
Epidemiology: Roughly 150 published cases, Median age 50 years, No sex
150 published cases ... Relapsing-remitting course +++ Clinical ... IVIg) • Limit vascular ... spacing of IVIg cures ... #rheumatology
Disseminated Intravascular Coagulation (DIC) Overview

Increased Clotting and Consumption of Clotting Factors

Findings:
 • Bleeding
 • Recent history
organ damage Clinical ... • Severe Liver Disease ... Treat primary cause ... #treatment #management ... #hematology