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Heme
Burkitt's Lymphoma
Adolescents or young adults
C-myc gene moves next to heavy chain Ig gene
T 8;14
Starry sky appearance
Sheets of lymphocytes with interspersed macrophages
Jaw lesion in endemic (African) form
Pelvis or abdomen in non endemic form (American form)
Associated with EBV
2 mins
Acute Myelogenous Leukemia (AML)
Myeloblasts Increase on Peripheral Smear
Median Onset 60 Years
T 15;17 M3 Subtype
Auer Rods
Disseminated Intravascular Coagulation (DIC) is a Common Presentation
M5 Gum Infiltration
Responds to Vitamin A
3 mins
Acute Lymphoblastic Leukemia (ALL)
Ages < 15
May Present with Bone Marrow Involvement in Children
Mediastinal Mass in Adolescents
TdT Positive
CALLA Positive
t(12;21) Better Prognosis
Spread to CNS and Testes
Most Responsive to Chemotherapy
2 mins
Chronic Lymphocytic Leukemia (CLL)
Signs and Symptoms
Age > 60 Years
B Cell Neoplasm
Smudge Cells
Often Asymptomatic
Cold IgM Autoimmune Hemolytic Anemia
Warm IgG Autoimmune Hemolytic Anemia
Richter's Transformation
3 mins
Hairy Cell Leukemia
Pathophysiology
Mature B Cell Tumor
Signs and Symptoms
Splenomegaly
Diagnosis
Flow Cytometry
Cells Have Filamentous Hair-Like Projections
Stains TRAP Positive
Dry Tap on Bone Marrow Aspiration
Treatment
Cladribine
Pentostatin
2 mins
Diffuse Large B Cell Lymphoma
Epidemiology
Most Common Non-Hodgkin’s Lymphoma
Usually in Older Adults
20% in Children
Features
May be Mature T Cell in Origin
Derives from Germinal Center
GI Tract and Brain
2 mins
Follicular Lymphoma
Derives from Germinal Center
Occurs in adults
T14;18
BCL 2 overexpression
BCL 2 inhibits apoptosis
Indolent
Generalized lymphadenopathy
Bone marrow involvement
2 mins
Multiple Myeloma Pathophysiology
Pathophysiology
Monoclonal Plasma Cell Cancer
Clock Face Chromatin
Diagnosis
M Spike on Protein Electrophoresis
Increased IgG and IgA
Fried Egg Appearance (BM Biopsy)
Rouleaux Formation
Stacked RBC's
Bence Jones Protein
2 mins
Hodgkin Lymphoma Pathophysiology
Pathophysiology
Reed-Sternberg Cells
Binucleate
Owl's Eyes Appearance
CD 15 and CD 30
B Cell Origin
Bimodal Age Distribution
2 mins
Chronic Myelogenous Leukemia (CML)
Pathophysiology
BCR-ABL
9;22 Translocation
Philadelphia Chromosome
Signs & Symptoms
Tyrosine Kinase
Low Alkaline Phosphatase
Splenomegaly
Considerations
Blast Crisis
Age 30 to 90 Years
Responds to Imatinib
3 mins
Polycythemia Vera Disease
Pathophysiology
JAK2 Mutation leading to increased red blood cells
Tyrosine Kinase
Symptoms
Erythromelalgia
Pruritus
Often After Hot Shower
Headache
Treatment
Phlebotomy
Aspirin
1 min
Primary Myelofibrosis
Characteristics
Bone Marrow Fibrosis
Extramedullary Hematopoiesis
JAK2 Kinase Mutation
Symptoms
Fatigue
Weight Loss
Splenomegaly
DIagnosis
Pancytopenia
Dacrocytes (Teardrop Cells)
Dry Tap on Bone Marrow Aspiration
Treatment
Stem Cell Transplantation
Transfusion
Ruxolitinib (JAK2 Inhibitor)
2 mins
Infectious Mononucleosis
Mechanism
Herpes-like Viral Infection
Signs and Symptoms
Fatigue
Fever
Sore Throat
Head and Body Aches
Hepatomegaly and Splenomegaly
Swollen Lymph Nodes in Neck and Armpits
Rash
Considerations
Suppressed Immune Response
Positive Monospot
2 mins
Bruton's Agammaglobulinemia
Pathophysiology
X-linked Recessive
Defective Bruton Tyrosine Kinase
Blocks B Cell Maturation
Signs & Symptoms
Decreased Immunoglobulins of All Classes
Bacterial Infections After Six Months
Maternal IgG Protects Before Six Months
2 mins
Selective IgA Deficiency
Pathophysiology
Defect in Class Switching
Decreased IgA
Signs and Symptoms
Often Asymptomatic
Increase in Atopic & Autoimmune Diseases
Sinopulmonary Infections
Increased Risk Giardia Lamblia Infections
Inflammatory Bowel Disease
Celiac Disease
Considerations
Anaphylaxis From IgA Containing Blood Products
2 mins
Thymic Aplasia (DiGeorge Syndrome)
Pathophysiology
DiGeorge Syndrome
22q11 Deletion
Failure to Develop Third and Fourth Pharyngeal Pouches
Increased risk of Cancer, Leukemias, and Lymphomas
Increased AFP levels
IgA deficiency
Repeated sinopulmonary infections
2 mins
Hyper IgM Syndrome
Pathophysiology
Deficient CD40 Ligand on Helper T-cells
Defective CD40 Receptor on B-cells
Inability to Class Switch
Signs and Symptoms
Pyogenic Abscess
Decreased IgG, IgA, IgE
4 mins
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