11 results
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
ANTI-MDA5 DERMATOMYOSITIS

Cutaneous manifestations:
• Periorbital heliotrope (blue-purple) rash with edema
• Erythematous rash on the face, or the
increases and ILD ... anti-NXP2 Abs Lung ... Phenotype 1 ... Dermatomyositis #rheumatology ... #dermatology #diagnosis
Common Variable Immunodeficiency (CVID)

What?
CVID: Immunodeficiency disorder with hypogammaglobulinemia -> increased infection risk secondary to impaired B-cell
• Affects 1:25,000 ... and 1:50,000 patients ... • Chronic lung ... hypogammaglobulinemia #immunology #hematology ... #diagnosis #management
SLE (Systemic Lupus Erythematosus)

Suspect:
Clinical evidence of (fatigue, rash, photosensitivity, inflammatory arthritis, weight loss, and fever) and
Non-autoimmune rheumatologic ... • Acute lupus pneumonitis ... • ANA titers > 1: ... Ro/La, anti-Jo-1, ... Erythematosus #Diagnosis #Rheumatology
SAPHO Syndrome Summary

What?
SAPHO syndrome is a rare chronic inflammatory disorder of bone, joint, and skin characterized
-1 inhibitors, IL ... inhibitors, and IL ... SAPHO #Syndrome #Rheumatology ... #diagnosis #management ... #Dermatology
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
Hemophilia A Prophylaxis (Factor Eight Deficiency)

Who Needs Prophylaxis?

Primary Prophylaxis:
 • Severe deficiency (activity level <1%) even
activity level <1% ... bleeding episodes • Mild-to-moderate ... FactorEight #Deficiency #hematology ... #management #ppx
Blood Transfusion Reactions

Immune Mediated:
 • Febrile Non-Hemolytic Transfusion Reaction (FNHTR): Most common immune reaction to transfusion.
Related Acute Lung ... 100,000,000, HCV 1 ... 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
Related Acute Lung ... 100,000,000, HCV 1 ... 2,000,000, HTLV 1 ... Transfusions #diagnosis #management ... #hematology #reactions
Anti-NXP-2 Dermatomyositis

What?
DM with generalized subcutaneous edema
Pts have more myalgias, more severe weakness, and an increased prevalence
Non-small-cell lung ... Prednisone at 1 ... Dermatomyositis #diagnosis #management ... #rheumatology # ... dermatology