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Blood-L
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
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
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
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
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
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
Acute Lymphoblastic Leukemia (ALL)
Ages < 15
May Present with Bone Marrow Involvement in Children
Neoplastic CD4+ Spread from Skin to Systemic Involvement
Sézary Cells With "Cerebriform Nuclei"
2 mins
Adult T Cell Lymphoma
Caused by HTLV 1
Japan, West Africa, Caribbean
Present with cutaneous lesions
Aggressive
1 min
Mantle Cell Lymphoma
Older Males
Activation of Cyclin D Gene
T 11;14
CD5
Poor Prognosis
55 secs
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
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
Disseminated Intravascular Coagulation (DIC)
Pathophysiology
Bleeding State
Activation of Clotting Factors
Deficiency of Clotting Factors
Causes
Sepsis
Trauma
Obstetric Complications
Acute Pancreatitis
Malignancy
Nephrotic Syndrome
Transfusion
2 mins
Thrombotic Thrombocytopenic Purpura (TTP)
Inhibition or deficiency of ADAMTS 13
vWF Multimers are Not Degraded
Microthrombi (and Emboli)
Symptoms
FAT RN Pentad
Fever
Anemia
Thrombocytopenia
Renal Dysfunction
Neurologic Abnormalities (Altered Mental status)
Treatment
Plasmapheresis
Corticosteroids
Splenectomy
2 mins
Acute Intermittent Porphyria (AIP)
Pathophysiology
Autosomal Dominant
Inhibited Porphobilinogen Deaminase
Increased Serum Porphobilinogen
Increased Serum Delta-ALA
Increased Urinary Porphyrin Precursors
Signs & Symptoms
Precipitated By P450 Inducers
Painful Abdomen
Polyneuropathy
Psychological Disturbances
Port Wine-Colored Urine
Treatment
Glucose and Hemin
Considerations
Avoid Triggers
3 mins
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