Pathology (Sections 11-15) Flashcards
Pattern of sarcomere formation associated with COVERLOAD?
Parallel
Pattern of sarcomere formation associated with VOLUME OVERLOAD?
Series
Heart Failure Cells
Hemosiderin-laden Macrophages
Nutmeg Liver
Centrilobular congestion and ischemia
Most common genetic cause of congenital heart defects
Trisomy 21
Marker of irreversibility in Congestive Heart Disease
Pulmonary Hypertension
Most common type of ASD
Secundum (90%)
Timetable for PFO Closure
Closed in 80% of individuals by 2 years
Most common Congenital Heart Disease
VSD
Most common type of VSD?
Perimembranous (90%)
Size of defect in non-restrictive VSD
> 10mm
Factors involved in PDA closure
Increased:
Oxygen Tension
Decreased:
Pulmonary Vascular Resistance
Prostaglandin E2
Components of ductus arteriosus in preterm infants that are ABSENT in term infants?
Mucoid Epithelium
Muscular Media
Most common cyanotic CHD
Tetralogy of Fallot
Primary defect in ToF
Anterosuperior displacement of infundibular septum
Mild pulmonary stenosis in ToF; clinically resembles isolated VSD
Pink ToF
Ventriculoarterial Discordance
CXR: Egg-on-the-side, Apple-on-a-stem
TGA
Absence of direct communication between RA and RV
Tricuspid Atresia
Coarctation found proximal to the PDA
Lower extremity cyanosis
RVH/Biventricular
Infantile Type
Coarctation distal to the arch vessels Upper extremity HPN Ribnotching Increased blood flow to intercostal vessels LVH
Adult Type
Vessels most commonly involved in MI
Left Anterior Descending (40-50%) Right Coronary (30-40%) Left Circumflex (15-20%)
Myocardial Infarction Progression:
Pale zone seen with triphenyltetrazolium chloride
2 to 3 hours
Myocardial Infarction Progression:
Conversion to a pale, soft, tan infarct
12 to 24 hours
Myocardial Infarction Progression:
Conversion to a fibrous scar
10 days to 2 weeks
Myocardial Infarction Progression:
Microscopically apparent coagulative necrosis
6 to 12 hours
Myocardial Infarction Progression:
Acute inflammation; neutrophilic predominance
1 to 3 days
Myocardial Infarction Progression:
Macrophagocytic predominance
3 to 7 days
Most sensitive and specific cardiac biomarkers for MI
Troponin T, Tropinin I
Biomarker used to assess possible rinfarction
CK-MB
returns to normal levels within 2 to 3 days
Pathophysiology: Dressler Syndrome
Fibrinous pericarditis in a post-MI setting
Also known as:
Postmyocardial infarct syndrome
Most common CAUSE of Sudden Cardiac Death
Coronary Artery Disease (80%)
Most common MECHANISM of Sudden Cardiac Death
Lethal Arrhythmia
Most common cause of arrhythmias
Ischemic injury
Earliest histologic change in hypertensive heart disease
Increased transverse diameter of cardiac myocytes
Most common valvular heart disease
Calcific Aortic Stenosis
Aschoff Bodies
Foci of T-Cells, Plasma Cells, and Anitschkow Cells
Pathognomonic of Rheumatic Fever
Anitschkow Cells
Enlarged macrophages found in Aschoff Bodies
Also called Caterpillar Cells
Fequency of involvement of valves in RF
Decreasing frequency:
Mitral
Aortic
Tricuspid
Pulmonic
MacCallum Plaques
Irregular subendocardial thickenings seen in regurgitant processes
Small cardiac vegetations seen along lines of closure, overlying fibrinoid necrosis
Verrucae
Deformity seen in calcification and fibrous bridging of valvular commisures
Fish Mouth / Button Hole
Major manifestations of RF
Mnemonic: J<3NES
Joints (Arthritis) Heart (Carditis) Nodules (Subcutaneous) Erythema Marginatum Sydenham Chorea
Hallmark of infective endocarditis
Vegetations
Most commonly affected valves in infective endocarditis
In general: Left-sided (Mitral, Aortic)
IV Drug Users: Right-sided (Pulmonic, Tricuspid)
Small-medium sized vegetations on BOTH sides of the valve leaflet
Libman-Sacks Endocarditis
Small, BLAND vegetations attached to the lines of closure
Nonbacterial Thrombotic Endocarditis
Previously known as Marantic Endocarditis
Flushing
Diarrhea
Dermatitis
Bronchoconstriction
Carcinoid Syndrome
Glistening white, intimal, plaque-like thickenings in endocardium
Usually affects right side of the heart
Associated with massive hepatic metastases
Carcinoid Heart Disease
Cardiomyopathy with systolic dysfunction
(EF < 40%)
Titin defect (in 20% of cases)
Dilated Cardiomyopathy
Massive, assymetric myocyte hypertrophy with myofiber disarray and interstitial fibrosis
Associated with defective Beta Myosin Heavy Chain
Diastolic dysfunction; EF may be preserved
Hypertrophc Cardiomyopathy
Cardiomyopathy secondary to excess catecholamines from psychologic stress
Tako-tsubo Cardiomyopathy
Pathophysiology: Arrhythmogenic RV Cardiomyopathy
Defective desmosomes
Severely thinned RV myocardium with fatty infiltrates and fibrosis
Pathophysiology: Naxos Syndrome
Plakoglobin Mutation
SSx of ARVC + Hyperkeratosis of plantar and palmar skin surfaces
Most common infectious cause of myocarditis
Viral (Coxsackie A and B, Enterovorus)
Atrial involvement (LA > RA) Ball-valve Obstruction Solitary, sessile/pedunculated mass mottled with hemorrhage
Most comkon primary cardiac tumor in adults
Myxoma
Ventricular involvement
Multiple gray-white masses
Spider cells
Most common primary cardiac tumor in children
Rhabdomyoma
Beck Triad
- Increased JVP
- Arterial hypotension
- Muffled Heart Sounds
Systolic retraction of the rib cage and diaphragm and pulsus paradoxus in the setting of CHRONIC pericarditis
Adhesive mediastinopericarditis
Single most important long-term limitation of cardiac transplantation
Allograft arteriopathy (Diffuse intimal proliferation of coronary arteries leading to ischemia)
Clinically significant neutropenia (< 500/mm^3)
Most common cause: Drug Toxicity
Agranulocytosis
Lymphadenitis histologically composed of:
- Light zone of Centrocytes
- Dark zone of Centroblasts
- Tingible Body Macrophages
Follicular Hyperplasia (B-Cell)
Lymphadenitis histologically composed of:
- Immunoblasts
- Hypertrophic sinusoidal and vascular endothelial cells
Parafollicular Hyperplasia (T-Cells)
Lymphadenitis histologically composed of an increased number of macrophages and cells lining lymphatic sinusoids
Sinus Histiocytosis / Reticular Hyperplasia
Congenital form of Neutropenia
Korstmann Syndrome
Most common cancer in children
Acute Lymphocytic blastic Leukemia
Most common leukemia of adults in the western world
Chonic Lymphocytic Leukemia,
Small Lymphocytic Lymphoma
Most commonly n ndolent lymphoma in adults
Follicular Lymphoma
Most common lymphoma of adults
Diffuse Large B-Cell Lymphoma
Most common form of Non-Hodgkin Lymphoma
Diffuse Large B-Cell Lymphoma
Early childhood Presents as BM Failure Hypercellular marrow with lymphoblasts Immunostain: TdT (95%) Metastases: Meninges, Testes (sanctuary site)
B-Cell Acute lymphoblastic leukemia
Adolescent males Presents as thymic masses NOTCH-1 Mutation (70%) Hypercellular marrow with lymphoblasts Immunostain: TdT (95%) Metastases: Meningeal, Testes (sanctuary site)
T-Cell Acute lymphoblastic leukemia
Poor prognostic factors in ALL
- Age less than 2 and greater than 10 years
2. WBC > 100,000
Favorable prognostic factors in ALL
- Age 2-10
- WBC < 100,000
- Hyperdiploidy
- Trisomy 4, 7, and 10
- T(12;21) mutation
60 years, M > F
Hypogammaglobulinemia
PBS: Small lymphocytes, Smudge Cells
Peripheral lymphocytosis: > 5,000/mm^3
Chronic lymphocytic leukemia
Difference between Chronic Lymphocytic Leukemia (CLL) and Small Lymphocytic Lymphoma (SLL)
Peripheral Blood Lymphocytosis
CLL: > 5,000 / mm^3
SLL: < 5,000 / mm^3
Pathophysiology: Richter Syndrome
Transformation of CLL into more aggressive lymphomas, usually Diffuse Large B-Cell Lymphoma
Middle Age
Painless, generalized lymphadenopathy
BCL2/t(14;18) Mutation in 90%
BMA: Paratrabecular Lymphoid Aggregates (85%)
Histologically similar to B-Cell Hyperplasia WITHOUT Tingible Body Macrophages
Incurable
Follicular Lymphoma
60 years, M > F
Rapidly enlarging nodal or extranodal mass
BCL6, BCL2 Mutation (10-20%)
Aggressive, rapidly fatal
Diffuse Large B-Cell Lymphoma
Immunostians used in DLBCL
- CD45 (lymphoid)
- CK (Epithelial)
- Vimentin (mesenchymal)
Mandibular mass Latent EBV Infection in ALL cases MYC t(8:14) Mutation "Starry Sky" Pattern Aggressive but treatable
Endemic Burkitt Lymphoma
Ileocecal or peritoneal mass Latent EBV Infection in 10-20% MYC t(8:14) Mutation "Starry Sky" Pattern Aggressive but treatable
Sporadic Burkitt Lymphoma
"Punched-out" bony lesions Pain, Hypercalcemia, Recurrent Infections Cyclin overexpression Histology: Russel Bodies, Dutcher Bodies Bence-Jones Proteinuria (Ig light-chain) M Proteins (monoclonal antibodies)
Multiple Myeloma
Indicator of poor prognosis in Multiple Myeloma
Elevated IL-6
“Solitary MM”
Most progress to multiple myeloma within 7-10 years
Solitary Plasmacytoma
Same presentation as CLL/SLL
Plasma Cell Differentiation
Hyperviscosity Syndrome
No bone manifestations
Lymphoplasmacytic Lymphoma
Waldenstrom Macroglobulnemia
Features: Hyperviscosity Syndrome
- Visual Changes
- Neurologic Deficits
- Bleeding
- Cryoglobulinemia
Painless lymphadenopathy
Naive B-Cell origin
No prolymphocytes, centroblasts
Poor prognosis
Mantle Cell Lymphoma
Seen in tissues with CHRONIC inflammation
Memory B-Cell origin
Regresses when inciting agent is removed
Marginal Zone Lymphoma
Splenomegaly B-Cell Origin Associated with atypical mycobacteria "Dry Tap" Excellent prognosis
Hairy Cell Leukemia
T-Cell Origin
Hallmark: Large anaplastc cells with horseshoe-shaped nuclei
(+) CD30, ALK
Anaplastic Large Cell Lymphoma
CD4+ T-Cell Origin
Cloverleaf Cells (multilobulated nuclei)
HTLV-1 (retrovirus) infection
Poor prognosis
Adult T-Cell Leukemia/Lymphoma
CD4+ Helper T-Cell Origin in skin
Pautrier Microabscesses
Sezary Syndrome
Mycosis Fungoides
Also known as Cutaneous T-Cell Lymphoma
Pautrier Microabscess
Infiltration of epidermis and upper dermis by Sezary Cells
Seen in Cutaneous T-Cell Lymphoma
Pathophysiology: Sezary Syndrome
Generalized exfoliative erythroderma with leukemia of Sezary Cells
T-Cells with cerebriform nuclei
Seen in Cutaneous T-Cell Lymphoma
Sezary Cells
Reed-Sternberg Cells
“Owl-eye Nuclei”
Diagnostic of Hodgkin Lymphoma
Lymphoma more often localized to single axial group of nodes
Contiguous Spread
Rare extranodal presentation
Hodgkin Lymphoma
Sexual predisposition of HL
M > F
except Nodular Sclerosis-type; M=F
Hodgkin Lymphoma most commonly associated with HIV
Lymphocyte Depleted HL
FAB M3 Mutation
Faggot Cells
Associated with DIC
Treatable: ATRA
Acute Promyelocytic Leukemia