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diagnosis rheumatology hemophagocytic hlh lymphohistiocytosis treatment antinxp2 antinxp2dm calcium cppd crisis deposition dermatology disease erythematosus hemeonc lupus macrophage mas nephrology
Macrophage Activation Syndrome (MAS) Classified among the secondary or acquired forms of haemophagocytic lymphohistiocytosis (sHLH) What? A subset of
Macrophage Activation ... Petechial or purpuric rash ... with leukopenia, anemia ... 5000 to 10,000 ng/mL ... #Management #Hematology
SLE (Systemic Lupus Erythematosus) Suspect: Clinical evidence of (fatigue, rash, photosensitivity, inflammatory arthritis, weight loss, and fever) and
Still disease, dermatomyositis ... Malignancy (e.g. hematologic ... lymphopenia, low PLT • Anemia ... La, anti-Jo-1, SCL ... Chronic B-cell activation
Hemophagocytic Lymphohistiocytosis (HLH) Pathophysiology: Triggering factor (infection,...)→Activation of CD8 T cells (IFN-y) → Excessive activation of macrophages
Lymphohistiocytosis (HLH) Pathophysiology ... →Activation of CD8 ... IFN-y) → Excessive activation ... Lymphohistiocytosis #diagnosis #management ... treatment #summary #rheumatology
Hemophagocytic Lymphohistiocytosis (HLH) Definition: Overwhelming clinical syndrome associated with excessive macrophage activation and cytokine storm due to
Overwhelming clinical syndrome ... excessive macrophage activation ... Ferritin >500 ng/mL ... Lymphohistiocytosis #diagnosis #management ... #treatment #hematology
Stiff Person Syndrome (SPS) - Diagnosis and Management Summary Epidemiology: • Prevalence: 1-2 cases per million -
Diagnosis and Management ... and pernicious anemia ... Pathophysiology ... Anti-GAD Ab (>1000u/ml ... SPS #Diagnosis #Management
Anti-NXP-2 Dermatomyositis What? DM with generalized subcutaneous edema Pts have more myalgias, more severe weakness, and an increased prevalence
(blue-purple) rash ... • Erythematous rash ... dorsal part of the MCP ... #diagnosis #management ... dermatology
Scleroderma Renal Crisis (SRC) Clinical Presentation of Scleroderma Renal Crisis: 1) Acute kidney injury 2) Abrupt onset of hypertension 3)
microangiopathic hemolytic anemia ... (MAHA) Pathophysiology ... endothelial cell activation ... of arteries Management ... Renal #Crisis #rheumatology
Hemophagocytic Lymphohistiocytosis (HLH) High mortality without prompt recognition and management. HLH is a critical diagnostic consideration in
recognition and management ... Syndrome) 3. ... - Cytopenias (anemia ... Pathophysiology ... #Hematology #HemeOnc
TAFRO TAFRO syndrome was first described in 2010, standing for: - Thrombocytopenia - Anasarca - Fever - Reticulin fibrosis - Organomegaly TAFRO syndrome
of idiopathic MCD ... cell lymphoma Pathophysiology ... mimics that of MCD ... TAFRO #diagnosis #management ... #rheumatology #
Pseudogout Summary - Calcium Pyrophosphate Deposition Disease (CPPD) Pathophysiology: Pyrophosphate produced by chondrocytes likely precipitates with calcium to
Disease (CPPD) Pathophysiology ... locations: Wrist, MCP ... Crowned Dens Syndrome ... Deposition #Disease #Rheumatology ... #diagnosis #management
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