15 results
Macrophage Activation Syndrome (MAS)
Classified among the secondary or acquired forms of haemophagocytic lymphohistiocytosis (sHLH)

What?
A subset of
A subset of hemophagocytic ... CD163 staining of bone ... is found in the bone ... Syndrome #Diagnosis #Management ... #Hematology #Rheumatology
Clinical Classification of Pulmonary Hypertension

1. Pulmonary arterial hypertension from pulmonary vasculopathy
Idiopathic pulmonary arterial hypertension
Heritable gene mutations
Clinical Classification ... mutations - BMPR2 (bone ... pulmonary disease ... multifactorial mechanisms Hematologic ... Hypertension #PHTN #Classification
Hemophagocytic Lymphohistiocytosis (HLH)
Pathophysiology: Triggering factor (infection,...)→Activation of CD8 T cells (IFN-y) → Excessive activation of macrophages
Hemophagocytic Lymphohistiocytosis ... , Biological + Bone ... /ml, ↑ LDH • Bone ... Lymphohistiocytosis #diagnosis #management ... treatment #summary #rheumatology
Hemophagocytic Lymphohistiocytosis (HLH)
Definition: Overwhelming clinical syndrome associated with excessive macrophage activation and cytokine storm due to
Hemophagocytic Lymphohistiocytosis ... age, however the disease ... Hemophagocytosis in bone ... Lymphohistiocytosis #diagnosis #management ... #treatment #hematology
Adult-Onset Still's Disease
Clinical triad: Fever, Arthralgia/arthritis, Skin rash ± odynophagia
+ Biological inflammation: Increased neutrophils, Hyperferritinemia, decreased
Adult-Onset Still's Disease ... autoinflammatory diseases ... hepatitis • Pulmonary ... #diagnosis #management ... #treatment #rheumatology
SAPHO Syndrome Summary

What?
SAPHO syndrome is a rare chronic inflammatory disorder of bone, joint, and skin characterized
inflammatory disorder of bone ... osteoarticular and skin disease ... SAPHO #Syndrome #Rheumatology ... #diagnosis #management ... #Dermatology
Hemophagocytic Lymphohistiocytosis (HLH) Treatment
Primary HLH:
 - HLH-94 Protocol
    • Dexamethasone
Hemophagocytic Lymphohistiocytosis ... or autoimmune disease ... for refractory disease ... HLH #Treatment #management ... #hematology
Nontuberculous Mycobacteria (NTM) Infections 

Mycobacterial species other than those belonging to the Mycobacterium tuberculosis complex and
Illnesses: • Pulmonary ... Growers (Runyon Classification ... Growers (Runyon Classification ... Hematologic malignancy ... ) - Bone Marrow
Vasculitis and Vasculitides - Differential Diagnosis Framework

When to Consider Vasculitis:
 • Purpura, ischemic skin lesions
 •
progressing GN • New pulmonary ... glomerulonephritis and/or pulmonary ... arthritis, obstructive pulmonary ... differential #diagnosis #rheumatology ... #classification
Polycythemia Vera (PV) - Diagnosis and Management Summary
Diagnostic Criteria:
 • Elevated hemoglobin and/or hematocrit AND
 •
Diagnosis and Management ... Hypercellular bone ... von Willebrand disease ... PV #Diagnosis #Management ... Summary #treatment #hematology