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diagnosis treatment blood hlh lymphohistiocytosis product reactions syndrome cryofibrinogenemia essential et gout sapho sjogrens summary thrombocythemia transfusion transfusions
Hemophagocytic Lymphohistiocytosis (HLH) Pathophysiology: Triggering factor (infection,...)→Activation of CD8 T cells (IFN-y) → Excessive activation of macrophages
Hemophagocytic Lymphohistiocytosis ... Drugs, Unknown cause ... Nicolas Taar ... Lymphohistiocytosis #diagnosis #management ... treatment #summary #rheumatology
Hemophagocytic Lymphohistiocytosis (HLH) Treatment Primary HLH: - HLH-94 Protocol • Dexamethasone
Hemophagocytic Lymphohistiocytosis ... Options for severe cases ... EricsMedicalLectures/ #HLH #Hemophagocytic ... HLH #Treatment #management ... #hematology
Cryofibrinogenemia Summary Cryofibrinogenemia Epidemiology: • 40-70 years with a modest female predominance Cryofibrinogenemia: • The precipitation of a
levels >1g/L • Negative ... cryoglobulins • Negative ... causes of secondary ... Cryofibrinogenemia #diagnosis #rheumatology ... #hematology
SAPHO Syndrome Summary What? SAPHO syndrome is a rare chronic inflammatory disorder of bone, joint, and skin characterized
Rule out other causes ... affected areas and if negative ... SAPHO #Syndrome #Rheumatology ... #diagnosis #management ... #Dermatology
Blood Transfusion Reactions Immune Mediated: • Febrile Non-Hemolytic Transfusion Reaction (FNHTR): Most common immune reaction to transfusion.
Causes purpura & ... TRALI): Leading cause ... more likely to cause ... Transfusion #diagnosis #management ... #hematology #reactions
Blood Product Transfusions One Pager Summary Type and screen - determines blood type and detects in recipient
Causes purpura & ... TRALI): Leading cause ... more likely to cause ... 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
Hypocomplementemia • Can cause ... Nephrogenic DI Hematologic ... phenomenon (16%) MSK ... Sjogrens #Syndrome #Rheumatology ... #Diagnosis #Management
Gout - Diagnosis and Management Summary 3 Conditions for Gout to Manifest: 1. Hyperuricemia 2. Monosodium urate deposition in
Diagnosis and Management ... Needle-shaped, negatively ... Dual-energy CT → MSU ... 2 attacks in a year ... #treatment #rheumatology
Essential Thrombocythemia (ET) ET is a chronic myeloproliferative neoplasm. Most cases are related to mutations that affect
Most cases are related ... WBCs are usually near-normal ... Thrombocythemia #ET #hematology ... #diagnosis #management ... #hematology
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