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

What?
A subset of
• Systemic lupus ... manifestations: from easy bruising ... purpura to mucosal bleeding ... with leukopenia, anemia ... #Rheumatology
Diagnostic Framework for Hemolysis - Intravascular vs Extravascular Causes
Intravascular Hemolysis:
 • Mechanical Trauma (Microangiopathic hemolytic anemia):
) - HELLP syndrome ... Hemolytic uremic syndrome ... usually IgG) (e.g. lupus ... • Infections: Malaria ... #anemia
Bleeding and Bruising - Disorders of Hemostasis - Differential Diagnosis Framework

== Quantitative or Qualitative defect in
Bleeding and Bruising ... inhibitor and lupus ... von Willebrand syndrome ... • Lupus anticoagulant ... of factor VII should
SLE (Systemic Lupus Erythematosus)

Suspect:
Clinical evidence of (fatigue, rash, photosensitivity, inflammatory arthritis, weight loss, and fever) and
Non-autoimmune rheumatologic ... Shrinking lung syndrome ... Photosensitivity • Malar ... lymphopenia, low PLT • Anemia ... Erythematosus #Diagnosis #Rheumatology
Myelodysplastic Syndromes (MDS)

What?
Myelodysplastic syndromes are a group of disorders caused by blood cells that are poorly
formed or don’t work ... Easy or unusual bruising ... Myeloproliferative Disease ... Workup: • Complete ... #diagnosis #hematology
Leukemias Overview: ALL, CML, AML, APML, CLL

Acute leukemias > 20% blasts in the peripheral blood smear
extramedullary disease ... PLT: Petechiae, bruising ... develop leukostasis syndrome ... - infiltrates (anemia ... • Tumor lysis syndrome
Causes of Purpura or Easy Bruising in Children

Platelet count reduced, i.e. thrombocytopenia
 - Increased platelet destruction
Purpura or Easy Bruising ... SLE (systemic lupus ... Congenital heart disease ... - Bernard—Soulier ... Scurvy #Purpura #Bruising
Rheumatology Workup - Laboratories Studies in Rheumatic Diseases

• Septic arthritis - Gram stain and culture of
Rheumatology Workup ... joint • Systemic lupus ... ANAs RF test should ... the presence of anemia ... Arthritis #laboratory #workup
Elevated Globulin - Protein Gap (Total Protein - Albumin > 4)

Work-up of an Elevated Globulin Gap:
•
Albumin > 4) Work-up ... gap (>4 g/dL) should ... • Heavy chain disease ... changes (POEMS syndrome ... Thalassemia, Sickle cell anemia