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diagnosis hematology blood product reactions syndrome activation antinxp2 antinxp2dm dermatomyositis erythematosus factoreight hemophilia hemophiliaa lupus macrophage mas ppx prophylaxis sjogrens
Hemophilia A Prophylaxis (Factor Eight Deficiency) Who Needs Prophylaxis? Primary Prophylaxis: • Severe deficiency (activity level <1%) even
(Factor Eight Deficiency ... activity level <1% ... bleeding episodes • Mild-to-moderate ... #FactorEight #Deficiency ... #hematology #management
Macrophage Activation Syndrome (MAS) Classified among the secondary or acquired forms of haemophagocytic lymphohistiocytosis (sHLH) What? A subset of
cytokines, such as IL ... -1 and IL-6, leading ... rash) • Heart, lung ... Syndrome #Diagnosis #Management ... #Hematology #Rheumatology
SLE (Systemic Lupus Erythematosus) Suspect: Clinical evidence of (fatigue, rash, photosensitivity, inflammatory arthritis, weight loss, and fever) and
Non-autoimmune rheumatologic ... disease • Prolidase deficiency ... • Acute lupus pneumonitis ... • ANA titers > 1: ... Ro/La, anti-Jo-1,
Blood Transfusion Reactions Immune Mediated: • Febrile Non-Hemolytic Transfusion Reaction (FNHTR): Most common immune reaction to transfusion.
recipients w/ IgA deficiency ... Related Acute Lung ... 2,000,000, HTLV 1 ... Transfusion #diagnosis #management ... #hematology #reactions
Blood Product Transfusions One Pager Summary Type and screen - determines blood type and detects in recipient
recipients w/ IgA deficiency ... Related Acute Lung ... 2,000,000, HTLV 1 ... Transfusions #diagnosis #management ... #hematology #reactions
Sjogren's Syndrome Overview Epidemiology: • F > M: 9:1 • 5-6th Decades (can be any age) Autoimmune exocrinopathy multisystemic disease
• Interstitial Pneumonitis ... phenomenon (16%) MSK ... MALT Lymphoma • Lung ... Sjogrens #Syndrome #Rheumatology ... #Diagnosis #Management
Anti-NXP-2 Dermatomyositis What? DM with generalized subcutaneous edema Pts have more myalgias, more severe weakness, and an increased prevalence
Dermatomyositis: • ILD ... Prednisone at 1 ... Dermatomyositis #diagnosis #management ... #rheumatology # ... dermatology
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