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Thrombotic Microangiopathies In Systemic Lupus Erythematosus

Dr. Laurent ARNAUD @Lupusreference

#TMAs #Thrombotic #Microangiopathies #Systemic #Lupus #Erythematosus #SLE #hematology
Microangiopathies In Systemic Lupus ... Microangiopathies #Systemic #Lupus ... Erythematosus #SLE ... #hematology #rheumatology ... differential #diagnosis #causes
Purpura - Differential Diagnosis Framework

Non-blanchable, dark red to purple, hemorrhagic skin lesions that result from leakage
: • Vascular occlusion ... : - Thrombocytopenia ... Drug induced - Bone ... Differential #Diagnosis #hematology ... #nonpalpable #dermatology
Causes of Thrombocytopenia and Platelet Disorders - Differential Diagnosis and Workup
History:
 - Prior platelet count, family
Causes of Thrombocytopenia ... test, ANA (e.g., SLE ... candidate) - Bone ... or if a primary hematologic ... #Workup #hematology
EULAR/ACR Classification Criteria for Systemic Lupus Erythematosus
Clinical Domains:
 • Constitutional domain: Fever
 • Cutaneous domain: Non-scarring
EULAR/ACR Classification ... pericarditis • Hematologic ... domain: Leukopenia, Thrombocytopenia ... #EULARACR #Classification ... #Criteria #SLE
Causes of Thrombocytopenia - Differential Diagnosis Framework
Decreased Production:
 - Drugs (chemo, yang, sulfa, acetaminophen, NSAIDs, quinine,
Causes of Thrombocytopenia ... folate def - Bone ... - Autoimmune: SLE ... Medicine @SCVMCMed #Thrombocytopenia ... Diagnosis #Framework #hematology
Causes of Thrombocytopenia - Differential Diagnosis Algorithm
 
Decreased Megakaryopoiesis
 • Aplastic Anemia
 • Toxic Damage (e.g.
Causes of Thrombocytopenia ... Autoimmune • ITP • SLE ... Heart Valve) #Thrombocytopenia ... Diagnosis #Algorithm #Causes ... #Hematology
Causes of Bone/Joint Pain in Sickle Cell Disease
Vaso-occlusive pain:
 • Mechanism: ischemic pain
 • Can present
Causes of Bone/Joint ... Disease Vaso-occlusive ... dz difficult to see ... differential #diagnosis #causes ... #hematology
Macrophage Activation Syndrome (MAS)
Classified among the secondary or acquired forms of haemophagocytic lymphohistiocytosis (sHLH)

What?
A subset of
disease • Systemic lupus ... SJIA], systemic lupus ... erythematosus [SLE ... leukopenia, anemia, and thrombocytopenia ... Diagnosis #Management #Hematology
Diagnostic Framework for Hemolysis - Intravascular vs Extravascular Causes
Intravascular Hemolysis:
 • Mechanical Trauma (Microangiopathic hemolytic anemia):
Extravascular Causes ... microangiopathy: Thrombotic thrombocytopenic ... usually IgG) (e.g. lupus ... Extravascular #Causes ... differential #diagnosis #hematology
Thrombotic Microangiopathy - TMAs
1. Not all MAHAs are due to TMAs but (almost) all TMAs cause
almost) all TMAs cause ... triad of MAHA + thrombocytopenia ... catastrophic APS, SRC, SLE ... Microangiopathy #TMAs #hematology