Survey of Disease - Exam 2

hematologic system

study of the form and structure of blood and blood forming tissues

parts of blood

plasma and formed elements (erythrocytes, leukocytes, thrombocytes)

signs and symptoms of hematologic disorders

edema
congestion
infarction
thrombosis
embolism
lymphadenopathy
bleeding and bruising
splenomegaly
shock

shock symptoms

rapid, weak pulse
hypotension
cool, moist skin
pallor
weak/absent peripheral pulses

hereditary hemochromatosis

autosomal recessive hereditary disorder
- excessive iron absorption by the small intestine

clinical manifestations of hereditary hemochromatosis

weakness
chronic fatigue
myalgias
joint pain
elevated hemoglobin and liver enzymes
hepatomegaly
tissue damage

disorders of leukocytes

reaction of the body to infection, inflammation, tissue damage, or degeneration

three main groups of leukocytes

granulocytes, monocytes, lymphocytes

types of granulocytes

neutrophils (defense against invading pathogens), eosinophils, basophils (allergic reactions, infections)

monocytes

immature cell until leaves the blood and travels to the tissues

lymphocytes

B and T cells

leukocytosis

increase in the number of leukocytes in the blood, also called neutrophilia

leukocytosis develops within...

1-2 hours after onset of acute hemorrhage

clinical manifestations of leukocytosis

fever, headache, SOB, localized or systemic inflammation or infection

leukopenia

reduction of number of leukocytes, never beneficial
- HIV
- alcohol
- nutritional deficiencies
- drug induced conditions
- connective tissue disorders
- leukemia, lymphoma, myeloma, Hodgkin's lymphoma (chemo)

leukemia

malignant tumor of the blood forming cells that replaces normal bone marrow with a malignant clone or lymphocytic or myelogenous cells

leukemia classified according to morphology:
lympho-
myelo-
blastic-
cytic-

lympho- lymphoid or lymphatic system
myelo- myeloid or bone marrow origin
blastic- large, immature (functionless) cells
cytic - mature, smaller cells

leukemia inhibits...

normal cell production

acute leukemia (rapid clinical course)

accumulation of immature lymphoid or myeloid cells in the bone marrow and peripheral blood

chronic leukemia (prolonged course)

accumulation of mature lymphoid or myeloid cells

3 main symptoms of leukemia

anemia
infection
bleeding tendencies (thrombocytopenia)

anemia

abnormalities in the quantity or quality of erythrocytes, classified according to etiologic factors or morphology

etiologic factors of anemia

excessive blood loss
increased destruction of erythrocytes
decreased production of erythrocytes

disease related anemia categories

· Iron deficiency associated with chronic GI blood loss
· Chronic or inflammatory diseases
· Nutritional conditions
· Infectious diseases
· Neoplastic diseases

WHO level of hemoglobin

- 14 g/100 ml for men
- 12 g/100 ml for women

anemia clinical manifestations

· Fatigue
· Weakness
· Pallor
· Tachycardia
· Angina
· Koilonychia (spoon shaped nails)
· Neuropsychiatric
· Heart failure and hypoxic damage to liver and kidney

thrombocytopenia causes

- Inadequate platelet production from the bone marrow
- Increased platelet destruction outside the bone marrow
- Splenic sequestration

symptoms of thrombocytopenia

· Mucosal bleeding
· Epistaxis
· Petechiae/purpura
· Hematuria
· Easy bruising

acute myeloid leukemia (AML) prevalence

uncommon but mostly in adults > 45 years old
diagnosed more frequently in children

risk factors of AML

unknown
exposure to radiation and virus
genetic disorders - Down syndrome

AML

abnormal cell growth of T and B cells

clinical manifestations of AML

fever, frequent infections, CNS, hepatosplenomegaly, lymphadenopathy, superior vena cava syndrome

chronic myeloid leukemia

neoplasm of hematopoietic stem cell

genetic abnormalities created in the stem cell

Philadelphia chromosome
overproduction of myeloid cells
Abnormal cell in peripheral blood and hyperplasia in the bone marrow

clinical manifestations of chronic myeloid leukemia

early: asymptomatic, fatigue, enlarged lymph nodes
later: lymphadenopathy, splenomegaly, hepatomegaly, weight loss, bone pain, bone marrow infiltration

common type of adult leukemia

chronic myeloid leukemia

Hodgkin's lymphoma

lymphoid neoplasm with histologic finding of giant Reed Sternberg cells in the lymph nodes
transformation from a normal B cell to a malignant cell

peak ages of Hodgkin's lymphoma

25-30, after age of 55

clinical manifestations of Hodgkin's

- Cervical, axillary, and para-aortic lymph nodes
- B symptoms (weight loss, fever, significant night sweats)
- Tracheal and bronchial compression
- Obstruction of GI tract
- Invade adjacent tissue
- Effusions
- Pruritus
- Pain

Non-Hodgkin's Lymphoma

large group of lymphoid malignancies that present as solid tumors, involvement of lymph nodes and extra nodal lymphoid tissues
- malignant transformation of a single lymphocyte

three subtypes of non-Hodgkin's

Burkitt's lymphoma
Follicular lymphoma
Mantle Cell lymphoma

risk factors of non-Hodgkin's

HIV, environmental, bacterial, EBV

clinical manifestations of non-Hodgkin's

- Lymphadenopathy
- Extranodal (Nasopharynx, GI tract, Bone)
- Abdominal lymphoma
- Compression of trachea or bronchus
- Superior vena cava syndrome
- Polyarthritis
- Fever, night sweats, pallor, fatigue, weight loss
- CNS

multiple myeloma

primary malignant neoplasm of plasma cells arising in the bone marrow
- affects bones and bone marrow of the vertebrae, ribs, skull, pelvis, femur

risk factors for multiple myeloma

unknown
occupational
blacks more than whites, men more than women

multiple myeloma progression

· Immature B lymphocyte > centroblasts > centrocytes > plasmablasts > adhesion molecules > plasmacytosis > bind osteoclasts

clinical manifestations of multiple myeloma

infections
musculoskeletal (hypercalcemia, muscle wasting, cancer related fatigue)
renal
neurologic

Von Willebrand's Disease

most common inherited bleeding disorder caused by lack or dysfunction of vWF

type 1 vWF

mild to moderate symptoms
inherited autosomal dominant

type 2 vWF

dysfunction in vWF
inherited autosomal dominant

type 3 vWF

decreased plasma levels of vWF
low levels of clotting factor VIII
more severe
autosomal recessive

clinical manifestations of Type 1 vWF

mucosal and skin bleeding
prolonged oozing after surgery
petechiae

clinical manifestations of type 2 vWF

excessive menstruation

clinical manifestations of type 3 vWF

severe mucosal or skin bleeding
hemarthrosis
muscular hematomas

hemophilia

bleeding disorder inherited as a sex linked autosomal recessive trait

hemophilia A (most common)

lack of clotting factor VIII

hemophilia B

deficiency of factor IX

level of severity of hemophilia depends

defect in the clotting factor gene

clinical manifestations of hemophilia

· Excessive bruising from minor trauma
· Persistent bleeding
· Hemarthrosis
· Recurrent bleeding into muscles
· Intracranial bleeding
· Problems with coagulation after trauma or surgery
· Excessive uterine bleeding

important part of hemophilia

regular exercise - promotes wellness, protects joints and enhances function, decrease frequency of bleeds
- isometric muscle exercise to prevent muscle atrophy

sickle cell disease

- autosomal recessive disorder characterized by the presence of an abnormal form of Hb within the erythrocytes
- RBCs change from biconcave disk to sickle once oxygen is released

how is sickle cell disease inherited?

two sickle cell genes are inherited or one sickle cell gene and another Hb is inherited

thalassemia

inherited defect in ability to produce hemoglobin
- sickle cell trait and b-thalassemic disorder = sickle b thalassemia syndrome

risk factors of sickle cell

both parents must have sickle Hb gene

sickle cell trait and one has HbC trait =

HbSC disease

symptoms of sickle cell are induced by

stress, dehydration, hypoxia, fatigue

sickle cell pathogenesis

-polymerization (Deoxygenated Hb, lower blood pH)
- sickling eventually become irreversible (10-20 days)
- hemolyses
- deprive tissue from receiving an adequate blood supply
- lack of nitric oxide production

clinical manifestations of sickle cell disease

pain
bone and joint episodes
vascular complications
pulmonary episodes
neurologic manifestations
hand foot syndrome (fever and pain)
splenic
renal complications

endocrine system

- synthesize and release hormones which are transported by the bloodstream to cells and organs for specific regulatory effect
- integration between nervous system and endocrine system

endocrine functions

- differentiation of the reproductive and central nervous system
- stimulation of sequential growth and development during childhood and adolescence
- coordination of the male and female reproductive systems
- maintenance of optimal internal environment
- initiation of corrective and adaptive responses when emergency demands occur

endocrine glands

secrete, target, action

hypothalamic pituitary interface

neural stimulation to posterior pituitary
- ADH
- oxytocin

hormonal control of the anterior pituitary

simulating hormones (ACTH, TSH, gonadotropic)
effector hormones (growth hormone, prolactin)

negative feedback system

promoting or inhibiting secretion

positive feedback system

increasing hormone secretion
- surge of LH to promote ovulation

pathology occurs when...

result of dysfunction of releasing tropic or effector hormones or when defects occur in the target function

ectopic hormone production

production of hormone or hormone like substance from another source

small cell carcinoma can cause...

Cushing's syndrome

neuroendocrine response to stress

sympathetic nervous system is aroused during stress
medulla of the edrenal gland releases catecholamines into blood stream (epinephrine, norepinephrine, dopamine, released due to exercise, thermal changes, stress)

cortisol regulates...

metabolism of proteins, carbs, lipids to cause an elevation in blood glucose levels
- source of energy and protein synthesis

cortisol dampens..

inflammatory response and inhibits fibroblast proliferation

cortisol modulates

perceptual and emotion functioning

endorphins

opiate like peptides produced at neural synapses in the CNS
- modulate transmission of pain perception
- producing sedation and euphoria

musculoskeletal signs and symptoms of endocrine disease

RA
proximal muscle weakness
tender points/trigger points
carpal tunnel syndrome
periarthritis and calcific tendinitis
chonedrocalcinosis
spondyloarthropathy

carpal tunnel syndrome

- usually bilateral
- tenosynovitis (hand stiffness)
- ischemia related in DM
- pregnancy

periarthritis and calcific tendinitis occurs in

shoulders

chonedrocalcinosis

pseudo gout-calcium salts in joint cartilage
- hypothyroidism, hyperthyroidism, acromegaly
- shoulder impingement (fibromyalgia and hypothyroidism)

spondyloarthropathy

hemochromatosis, ochronosis, acromegaly, DM

Cushing's Disease

caused by tumor on pituitary gland that causes the gland to produce too much ACTH

Cushing's Syndrome

due to causes outside the body that increase the levels of cortisol, such as taking medications containing cortisol, such as corticosteroids

types of pituitary gland disorders (anterior lobe)

- hyperpituitarism
- GH secreting adenomas
- gigantism
- acromegaly
- hypopituitarism

what are pituitary gland disorders (anterior lobe) caused by?

tumors, pituitary infarction, genetic disorders, trauma

hyperpituitarism (Cushing's Disease)

oversecretion of ACTH by a pituitary tumor which results in overproduction of adrenocortical hormones

systemic effects of hyperpituitarism

- abnormal growth patterns (caused by overproduction of GH)
- amenorrhea, galactorrhea, gynecomastia

local effects of hyperpituitarism

visual field abnormalities, headaches, sleepiness

GH secreting adenomas produce

cartilaginous and connective tissue overgrowth

gigantism

overgrowth of long bones
- develops abruptly in children before the epiphyses of the bones close

acromegaly

increased bone thickening and hypertrophy of the soft tissues, developing slowly after the closure of the epiphyses

acromegaly affects...

face, jaw, hands, feet

local manifestations of acromegaly

headache, diplopia (double vision), blindness, lethargy, induced myopathy with muscle weakness, influences joint mobility