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Pancytopenia - Differential Diagnosis

Congenital Diseases:
• GATA2 deficiency
• Fanconi’s anemia
• Wiskott-Aldrich syndrome
• Ribosomopathy or telomeropathy

Cancer:
• Leukemia
• Lymphoma

Inflammatory
deficiency • Fanconi’s anemia ... Wiskott-Aldrich syndrome ... : • Systemic lupus ... Antibody-mediated hemolysis ... #Hematology
Clinical Features of Systemic Lupus (SLE)
General: Fever (50%), Depression, Fatigue (75%), Weight loss (50%)
Eye: Sjögrens (15%)
Skin
Demyelinating syndromes ... Endocarditis, Aortic ... ) Blood (75%): Anemia ... erythematosus #signs ... #symptoms #diagnosis
Classification of Vasculitis 
Vasculitis of large vessels 
 - Arteritis temporalis (giant-cell arteritis) 
 - Takayasu
- Inflammatory aortic ... central nervous system ... - systemic ... #Rheumatology # ... Diagnosis
Diagnostic Framework for Hemolysis - Intravascular vs Extravascular Causes
Intravascular Hemolysis:
 • Mechanical Trauma (Microangiopathic hemolytic anemia):
Diagnostic Framework ... Microangiopathic hemolytic anemia ... ) - HELLP syndrome ... Hemolytic uremic syndrome ... #hematology #anemia
Nail Disorders secondary to Systemic Diseases
Nail Fold Abnormality
 • SLE
 • Scleroderma
 • Dermatomyositis
Koilonychia - Spoon-shaped
Diseases Nail ... Iron deficiency anemia ... • Nephrotic syndrome ... #Secondary #dermatology ... #Differential #Diagnosis
Warm Antibody Autoimmune Hemolytic Anemia 
1) DIAGNOSE AIHA
 • Anemia: macrocytic > normocytic, ↑ reticulocytes
 •
1) DIAGNOSE AIHA ... reticulocytes • Hemolysis ... FOR a cause → Systematic ... #diagnosis #management ... #treatment #rheumatology
Hemolysis - Differential Diagnosis Framework

1) Environment
  - Fragmentation
  - MAHA: TTP, HUS, DIC, HELLP
Hemolysis - Differential ... Diagnosis Framework ... Drug-associated, PCH, Evans Syndrome ... Defects: Sickle cell anemia ... #hematology
Macrophage Activation Syndrome (MAS)
Classified among the secondary or acquired forms of haemophagocytic lymphohistiocytosis (sHLH)

What?
A subset of
Macrophage Activation Syndrome ... highly inflammatory disease ... with leukopenia, anemia ... #Diagnosis #Management ... #Hematology #Rheumatology
Approach to Thrombocytosis - Elevated Platelet Count - Differential Diagnosis Algorithm

Peripheral Smear concerning for malignancy?
- Differential Diagnosis ... blood loss, Acute hemolysis ... , deficiency anemia ... Vasculitides, IBD, Celiac disease ... Kawasaki, Nephrotic syndrome
Systemic Lupus Erythematosus - Summary
Antinuclear (ANA) - 95% - Initial screening test
Anti-dsDNA - 50%
 • Associated
block • Sjogren syndrome ... polymyositis overlap syndrome ... #SLE #Summary #diagnosis ... #rheumatology # ... management