Episode 24: Doctor Dan gives part 2 of his Cancer lecture series available in full at www.Medical-Mastermind-Community.com.
Xeroderma pigmentosa – sun exposed areas, auto recessive, can cause all skin cancers (BCC, SCC, and melanomas), and the defect is in DNA repair enzymes. Other DNA repair defects are associated [...]
General Information
group of conditions associated with blister formation
Blister = fluid-filled raised area
Ø Vesicle = < 5 mm in diameter
Ø Bulla = > 5 mm in diameter
Ø Pustule = blister filled with pus
Acantholysis = loss of the cohesion between the keratinocytes due to disruption of intercellular bridges
Ballooning degeneration
Dyskeratosis
Spongiosis [...]
Blood Collection
Only 8 out of 100 million eligible blood donors actually donate
We use a VOLUNTARY donor system
Ø paid donors associated with higher rick of passing on transmissible diseases
Blood is a “drug”Þ regulated by the FDA
How do we select blood donors?
Ø there is a uniform donor “screening” process
Ø process ensures [...]
Introduction to Soft Tissue Tumors
soft tissue = complex of non-epithelial extraskeletal structures of the body
soft tissue includes: fibrous (Connective) tissue, adipose tissue, skeletal muscle, blood and lymph vessels, and peripheral nerves
Derivation of most of the soft tissue - mesoderm
Exception - neuroectodermal for peripheral nerves
Age has a bearing on type [...]
Epidermal Tumors - Benign
Seborrheic Keratosis
“stuck-on” flat topped papules or plaques
exceedingly common on the trunks of individuals over the age of 40
keratosis = presence of hyperkeratosis (thickened stratum corneum)
Seborrheic = lesions more common in areas with numerous sebaceous glands
Cream to brown colored (more white on distal extremities)
MICRO: linear [...]
General Information on Papulosquamous Diseases
the papulosquamous diseases are generally scaly, like the eczematous diseases
difference from the eczemas
1) epidermis is generall thicker
2) more clinically elevated scaly condition
3) better described as a plaque than a patch
4) eventually develop acanthosis and hyperkeratosis to a greater degree
5) more sharply defined
some diseases may be both papulosquamous or [...]
Definition - group of diseases having as a common denominator the proliferation of one, or usually more than one, of the bone marrow-derived cell lines. The proliferation may be medullary or extramedullary
Classification of Diseases
1) Chronic myelogenous leukemia - CML
2) Agnogenic myeloid metaplasia (myelofibrosis) - AMM
3) Polycythemia rubra vera - PRV
4) Primary Thrombocytopenia - [...]
Definition
malignant disorders of the immune system involving the uncontrolled proliferation of lymphocytes
most patients present with lymphadenopathy, but sometimes they present with symptoms related to the growth of an extranodal lymphoma.
Etiology
Unknown
Proposed - clonal expansion of lymphoid cells that have undergone a maturation arrest at some point in their ontologic development.
[...]
Definitions
1) Leukemia - malignant neoplasm of hematopoietic stem cells
2) Lymphoma - malignant neoplasm of lymphocytes which usually arises in lymph nodes but can arise from lymphoid tissue anywhere in the body
3) Leukocytosis - increased numbers of WBCs in the peripheral blood
4) Granulocytopenia - absolute decrease in the number of neutrophils in the peripheral blood
5) Thrombocytopenia [...]
Review of Normal Structure
Joints are Classified as:
1) Solid (non-synovial)
these joints provide the structural rigitidy
minimal movement
no joint space
grouped according to the type of connective tissue that connects the ends of the bones
Ø Examples
1. fibrous synarthroses - cranial sutures
2. cartilagenous synarthroses - [...]
RBCs are incapable of surviving the normal 120-day life span
Can be due to 1)intrinsic defects in RBC structure/function or 2) a hostile environment
Definitions
1) Hemolysis - any cond. char. by a sig. decreased RBC life span
2) Compensated Hemolytic state - the resulting increased RBC production is able to keep up with the destruction
3) [...]
Definition
1) Hemoglobinopahty - genetic defect that results in an abnormal structure of one of the globin chains of the hemoglobin molecule
2) Thalassemia - a genetic defect that results in production of an abnormally low quantity of a given hemoglobin chain or chains; the result is an imbalance in production of globin chains and [...]
Basic Information on Eczematous Diseases
basic pathologic change common to most eczemas = Spongiosis
Ø inter-cellular edema between keratinocytes
exocytosis - inflammatory cells migrate into the epidermis
Acanthosis - thickened Malphigian (spinous) layer in the epidermis
Hyperkeratosis - thickened stratum corneum
Lichenified - chronically thickened skin
Lichen Simplex Chronicus - when the skin gets very [...]
I. Basic Histo:
v “Barry Sanders Goes Long Constantly”
v Skin = Mostly Keratinocytes
II. Path Findings Not Unique to the Skin
A. H & E: Tumors = Blue (basophilic nuclear staining),
W More malig = more blue
W Pink = usually benign
B. Granulomas
W Enlarged Macs -> Epithelioid Cells -> Fuse -> Multinucl Giant Cells
W DDx:
1. Infectious: mycobacterial[caseating] (leprosy [...]
INTRODUCTION
Normal Skin Histology
Epidermis
Keratinocytes make up most of each layer of the epidermis
1) Cornified Layer - stratum corneum
Ø anucleated
2) Granular Layer - stratum granulosum
Ø flattened cells with tiny basophilic keratohyaline granules
Ø 1-2 cell layers thick
3) Spinous Layer - stratum spinosum
Ø usu the largest region
4) Basal Layer - stratum basalis
Ø REGENERATIVE LAYER
Ø May have [...]
Introduction
Sites of 1o tumors that most commonly metastasize to bone
Ø Breast, Lung, Prostate, Thyroid, Kidney
Some common bone tumors and tumor-like lesions seen in general practice
Ø osteochondroma, enchondroma, osteoid osteoma, osteomyelitis, metaphyseal fibrous cortical defect, fibrous dysplasia, metastases
At this time, all Orthopaedic organizations recommend that bone tumors be managed only by Orthopaedic Surgeons [...]
Review of Normal Structure:
Mature/Secondary/Lamellar Bone
slowly produced & highly organized
Remember - anything other than lamellar bone in the adult skeleton is abnormal
HISTO
Ø few osteocytes, parallel arrangement of type I collagen fibers, uniform osteocytes
Immature/Primary/Woven Bone
rapidly produced & ill-arranged
low tensile strength
HISTO
Ø irregular arrangement of type I collagen fibers, numerous [...]
Anemia - Pathophysiologic Consequences and Clinical Investigation
Definition of Anemia
any condition resulting from a significant decrease in the total body erythrocyte mass
working definition - hemoglobin < 12 g/dL or hematocrit < 37 cL/L
usually results from the body’s compensation to maintain blood volume in the response to rbc loss.
Physiologic Compensation for the [...]
Normal Anatomy
two lobes, one isthmus
follicular cells - produce thyroid hormones
C cells - produce calcitonin (parafollicular)
Colloid - stores thyroid hormone extracellularly
Abnormalities of Development
agenesis
Maldescent
Ø can result in a pyramidal lobe
Ø ectopic thyroid tissue
Ø pathway of descent - foramen cecum to thyroid and cricoid cartilage
Ø can be found in the [...]
Definitions
teratology - “the study of monsters”, the science of congenital malformations
Teratogenesis - 1) the path. Process resulting in a malformation 2) cell injury, mitotic arrest, or impeded gene-enzyme activation resulting in structural or functional malformation
Congenital Malformation - a structural defect present at birth with or without a genetic defect
Congenital [...]
Acute Gastritis
Pathology
congested, edematous gastric mucosa
coagulation or hemorrhage of gastric mucosa
can have many acute erosions into the gastric mucosa
Clinical Presentation
Epigastric pain
Tender, nausea, vomiting
Bleeding
Ranges from occult (hidden) to major hemorrhage
Most common causes
Aspirin, NSAIDS, EtOH
Usually self-limited
Chronic Gastritis
General
Remember H.pylori and NSAIDS are the most common [...]
Even though the Small Intestine is the longest part of the GI tract, it has less than 5% of all the neoplasms of the GI tract!!
Benign tumors of the Small Intestine
Brunner’s Gland Adenoma
can be both focal or diffuse
usually found on the posterior wall of the duodenum at the junction between the [...]
Herpesvirus Infections - HSV1 & HSV2
History
replicate in the skin, mucous membranes, oropharynx, and genital tract
the lesions are vesicular
can reactivate repeatedly
Diseases
adults - fever blisters, genital lesions
neonates - stomatitis, conjunctivitis, encephalitis
immunosuppressed - disseminated disease
Pathology
Intranuclear inclusions (Cowdry A bodies - acidophilic, homogenous with clear halo surrounding)
Ø Replicate in nucleus
[...]
Introduction
Two functional components
1) anterior lobe - adenohypophysis
Ø derived from Rathke’s pouch
Ø Secretory portion of the pituitary
Ø Three morphological cell types (acidophils, basophils, chromophobes); Chromophobes make up the majority of the anterior lobe cells
Ø Five functional cell types produce 6 hormones (PATFGL) - (Point after try, FGL = field goal)
1) Prolactin - mammotrophs
2) Adrenocorticotrophic hormone [...]
Development Malformation in the Lower Urinary Tract
Duplication of the renal pelvis and ureter
common
80% are unilateral
May be asymptomatic
May result in (if there is obstruction to the lumen of the duplication)
1) hydroureter
2) hydronephrosis
3) susceptibility to infection (pyelonephritis)
Ureteral Valves
unknown developmental basis
could represent defective innervation with local inability to maintain ureteral peristalsis
[...]
Incidence
Cancer is 2nd most common cause of death in children aged 1-14 - 10%. (Accidents #1 - 42%)
Leukemia accounted for ~33% of all cancers in children in the US in 1985.
CNS tumors was second with ~20%.
They are followed by: lymphomas, neuroblastoma, Wilms tumor, Bone cancer, rhabdomyosarcoma
Overall survival [...]
Normal Development
4th week of gestation - lung bud appears
5th-20th week - lung buds continue to branch
Pseudoglandular period (Weeks 6-16) - continuing bronchial branching; differentiation of certain cells
Canalicular Period (Weeks 16-28) - period of acinar development, differentiation of [...]
Normal Anatomy
Embryology
1. Upper parathyroids derived from brachial pouch 4; found on posterior Superior aspect of the thyroid.
2. lower derived from B.P. 3; can be found from inferior pole of the thyroid to the thymus. May be intrathyroidal or intrathymic
Gross Anatomy
1. in infancy - composed mainly of sheets of chief cells
2. at [...]
Carbohydrate Disorders
Galactosemia
Biochemical Pathway
Galactose + ATP, 1 Galactose-1-P 2 UDP Galactose + Glucose 1-P 3 UDP Glucose + Galactose-1-P 4 Glucose-1-P
Involved enzymes and their consequences if deficient or absent
1- galactokinase - some problems, not as fatal as 2
2- Galactose-1-P uridyl transferase - Fatal
3- UDP Galactose-4-epimerase - rare; no consequences
4- Hexose-1-P uridyl transferase
[...]
Mechanism of Injury to Fetus and Newborn
1) anoxia, hypoperfusion, and ischemia
2) drugs and metabolites
3) Infection
cause cell detah, malformation, Growth retardation, and inflammation
Anoxia, Hypoperfusion and Ischemia
Susceptibility
early embryo (< 14 days) - resists most noxious stimuli
later embryonic (14-56 days) - more susceptible; can lead to malformation or death
The fetus has a greater [...]
Remember, that most maternal disease do not affect the fetus.
Ways the maternal disease affects the fetus
1) transplacental disease - ex. Rubella
2) premature labor induction
3) altered fetal physiology - ex. Maternal Diabetes Mellitus
4) interference with fetal resp. or nutrition - Maternal HTN
5) Nutritional Status of the momma
Infection Effects on fetus Incidence
Rubella Abortion; Malformations high during 1st [...]
Acute Pancreatitis
common disease
both sexes; 5th and 6th decades
Classification
acute interstitial pancreatitis
Acute hemorrhagic pancreatitis
Pathogenesis
Caused by pancreatic enzyme release that results in enzymatic autodigestion
Theory
Ø functional or structural duct obstruction coincides with active pancreatic secretion
Ø small ducts dilate
Ø disruption of small intralobular ducts
Ø enzymatic auto digestion
Necrosis occurs as a result [...]
Penis
Normal Anatomy of the Penis
paired corpora cavernosa
single corpus spongiosum
Tyson’s glands produce smegma
Glans - distal extension of corpus spongiosum - covered by prepuce
Corpus spongiosum envelopes the penile urethra
Normal anatomical position at erection
Pathology of the Penis
Congenital Abnormalities
Ø Malformations of the urethral groove and canal
¨ Hypospadia - opening of the [...]
Normal Anatomy
falciform ligament divides into two lobes (right lobe is larger)
dual afferent blood supply - hepatic artery and portal vein
60-70% of blood supply comes from portal vein
single venous drainage system
Histology and Metabolism
Classic lobules - central vein surrounded by hepatocytes with portal tracts (hepatic artery, portal vein, and bile [...]
Physiologic Jaundice of Infancy
In term infants
1) rises <5mg/24 hr
2) peaks at 2-4 days
3) levels at 5-6 mg/dl
Premies
1) variable level and rise
2) peaks at about 4-7 days
Work up further if:
1) jaundice w/in the first 24 hours
2) serum bili rises >5mg/dl/24hrs
3) Serum bilirubin > 12 mg/dl (term) or 14 mg/dl (premies)
4) Duration longer than [...]
3rd among reported diseases in incidence (STDs and chicken pox - #1 & #2)
1-2 million deaths worldwide
1.2 million Hep B carriers in the U.S.
300 million carriers worldwide
Acute Hepatitis
Asymptomatic Hepatitis
inapparent disease unless the patient somehow gets liver function tests
HAV is especially at risk for being asymptomatic
Symptomatic Hepatitis
Icteric
[...]
Hemochromatosis = a disorder of iron metabolism with excess deposition of iron in the tissues, bronze skin pigmentation, hepatic cirrhosis, and diabetes mellitus.
IRON OVERLOAD SYNDROMES
No physiological mech. for iron excretionÞ iron will accumulate in the body either from 1) increased intestinal absorption or from 2) parenteral administration
Normal total body iron stores [...]
Introduction
infectious diseases are important to hematologists because:
1) they may simulate malignancies (lymphoma and leukemia)
2) may directly or indirectly cause anemia or abnormal peripheral blood counts
3) may involve direct infection of hematopoietic or lymphoid tissues
Infections related to Lymphoid Tissues
characteristics
1) lymph node enlargement (deuh!)
2) pain
Ø if pain is present generally it characterizes infectious [...]
Normal Morphogenesis
Embryonic period = conception to 8 weeks gestation
Ø 8 week division between the embryonic period and fetal period is one of the few definites as far as time goes on development
Ø the embryo measures less that 3 cm (30 mm) in CR length.
Fetal period = 8 weeks gestation to birth
Ø [...]
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