9 In '09---T

Front 1

Mutation leading to precipitation of Hbg subject to hypoxia and this causes a sickle-shape instead of the biconcave disk

Back 1

Sickle cell disease

Front 2

an autosomal dominant disorder characterized by the production of
rbcs that are sphere-shaped rather than biconcave shape (they lack central pallor).

Back 2

Hereditary Spherocytosis

Front 3

A mutation in ankyrin is the most common defect; mutations in spectrin, band 2, or band 3 account for other defects.

Back 3

Hereditary Spherocytosis

Front 4

Membrane transport activities of the rbc are also affected; rbcs are osmotically fragile; splenomegaly a result

Back 4

Hereditary Spherocytosis

Front 5

Erythrocytes are more rigid and more fragile and they are destroyed easier. They adhere more readily to endothelium and may pile up in capillaries.

Back 5

Sickle cell disease

Front 6

Persistent neutrophilia due to a redistribution of neutrophils

Back 6

Leukemoid Reaction

Front 7

Do you see splenomegaly in leukemoid reaxn?

Back 7

NO

Front 8

Myelocytes, metamyelocytes, band forms, and neutrophils appear in peripheral blood

Back 8

(4) Chronic granulocytic leukemia

Front 9

Most frequent causes of ______________ are inflammation, infection, and malignancy. Eos and Bas increase is rare

Back 9

Leukemoid Reaction

Front 10

________ commonly presents with increased Eos and Bas.

Back 10

Chronic granulocytic leukemia

Front 11

Phospholipase B crystals that are found in diseases associated
with eosinophilia and are seen in secretions or excretions (sputum, feces) or CT.
Found in sputum of asthmatics.

Back 11

(5) Charcot Leyden Crystals

Front 12

______________is characterisitic of bone
marrow affected by tumors of hematopoietic cells.

Back 12

Hypercellular marrow

Front 13

is a platelet count < 150,000/ul

Back 13

Thrombocytopenia

Front 14

The leading cause of
decreased platelet production is _________which impairs the maturation of ___________

Back 14

Alcohol

megakaryocytes

Front 15

An X-linked disorder characterized by deficient
NADPH oxidase in the cell membranes of neutrophils and monocytes

Back 15

(8) Chronic Granulomatous disease (CGD

Front 16

2 tests for ________ are the nitroblue tetrazolium test (NBT) where neutrophils change to a blue color if the RBP is intact. The new, more sensitive test involves oxidation of dihydrorhodamine to fluorescent rhodamine.

Back 16

(8) Chronic Granulomatous disease (CGD

Front 17

reduced production of O2 means the respiratory burst (RBP) is ________

Back 17

Absent

Front 18

Monocyte count greater than 10% of total wbc count or greater than 800/µl; Many or just about
Anything causes it- bacterial, viral, and protozoan infections;

Back 18

monocytosis

Front 19

List 2 malignancies with associated monocytosis.

Back 19

Hodgkin lymphoma and chronic myelogenous leukemia

Front 20

depletion of CD4+ T helper cells due to HIV

Back 20

AIDS

Front 21

swelling occuring most often in arms or legsassociated with lymph

Back 21

lymphedema

Front 22

lymphatic vessel tumor

Back 22

lymphangioma

Front 23

cyst resulting from blockage of lymphatic vessel

Back 23

cystic hygroma

Front 24

inflammation of tonsils

Back 24

tonsillitis

Front 25

_______ are palpable and diagnostic of upper respiratory infection.

Back 25

enlarged lymph nodes

Front 26

loss of immunological tolerance against self. Exs: rheumatoid arthritis, Graves’ disease

Back 26

Autoimmune disease

Front 27

congenital absence of thymus, associated with abnormal cell-mediated immunity. Death usually results from recurrent infections

Back 27

DiGeorge syndrome

Front 28

neoplastic transformation of lymphocytes

Back 28

Hodgkin's disease

Front 29

What type of virus mediates AIDS?

Back 29

RNA retrovirus (HIV)

Front 30

HIV utilizes a glycoprotein (gp120) on its surface to bind to ______ protein on the surface of T helper cells

Back 30

CD4

Front 31

What enzyme allows the viral genome to be incorporated into the t-cell DNA?

Back 31

reverse transcriptase

Front 32

What does HIV inject into the t-cell?

Back 32

reverse transcriptase and RNA encoded viral genome

Front 33

HIV replication occurs intracellularly/extracellularly??

Back 33

intracellularly

Front 34

Which cell type decreases as a direct result of HIV/AIDS?

Back 34

CD4 T-Helper cells

Front 35

_________ pathogens are the major threat to individuals diagnosed with AIDS.

Back 35

Opportunistic

Front 36

What is the immune response to AIDS?

Back 36

increase in CD8+ cytotoxic t-cells and Ab

Front 37

List 2 opportunistic pathogens and 2 malignancies associated with AIDS.

Back 37

Pneumonia/Toxoplasmosis

Kaposi Sarcoma/non-Hodgkins

Front 38

What are the methods used to diagnose AIDS?

Back 38

PCR

CD4+ count

Front 39

What is the mech of action of AZT?

Back 39

inhibits reverse transcriptase

Front 40

rare, inherited disorders causing improper development of lymphatic vessels

Back 40

primary lymphedema

Front 41

begins in infancy; malformation of lymph nodes  lymphedema

Back 41

Milroy's disease

Front 42

lymphedema occurs in childhood or at puberty; lack of valves in lymphatic vessels  backward flow  lymphedema

Back 42

Meige disease

Front 43

milroy's and meige disease are both classified as _________ lymphedemas.

Back 43

primary

Front 44

causes include surgery (removal of lymph nodes and/or vessels – breast cancer); radiation treatment for cancer (scarring and damage to lymph nodes and/or vessels); obstruction by cancerous mass; infection

Back 44

secondary lymphedema

Front 45

arise due to incomplete development & obstruction; failure to connect with venous system  obstruction of lymph flow  formation of cysts

Back 45

lymphangioma

Front 46

What drug is used to treat lymphangiomas? What does it do?

Back 46

experimental drug OK-432 (Grp A strep + pencillin  stimulates inflammatory response  neutrophils, macrophages, & NK cells  endothelium becomes permeable  lymphatic fluid drains

Front 47

symmetrical accumulation of fluid around neck and head region; ~ 5% of stillbirths (Wisconsin);

Back 47

Cystic Hygromas

Front 48

often associated with chromosomal abnormalities (Turner, Trisomys 21, 18 and 13); continued swelling of lymphatic vessels   in intravascular volume  cardiac failure

Back 48

Cystic Hygromas

Front 49

Usually caused by Group A streptococcal bacteria
4.Treatment – antibiotics

Back 49

Tonsilitis

Front 50

Infection  activation & migration of B cells to nodules in the cortex of lymph nodes

Back 50

enlarged lymph nodes

Front 51

________ cell proliferation is to blame for the enlargment of lymph nodes.

Back 51

B

Front 52

Malfunction of immune system  no longer distinguish self from non-self

Back 52

Autoimmune disease

Front 53

List 3 examples of autoimmune diseases.

Back 53

Graves, Lupus, and RA...

Front 54

What is the common tx for autoimmune diseases?

Back 54

Corticosteroids/immunosuppresive drugs

Front 55

Congenital absence of thymus & parathyroid glands  abnormal development of 3rd and 4th pharyngeal pouches.

Back 55

DiGeorge Syndrome

Front 56

Associated w/ abnormal cell-mediated immunity but normal humoral immunity

Back 56

DiGeorge Syndrome

Front 57

What type of immunity is abnormal in DiGeorge syndrome?

Back 57

Cell mediated immunity

Front 58

Pt's with DiGeorge usually die from _______ or _________.

Back 58

Tetany

Uncontrollable infection

Front 59

Characterized by Reed-Sternberg cells (most likely B-cell derived)

Back 59

Hodgkin's Lymphoma

Front 60

What virus is commonly associated with Hodgkin's Lymphoma?

Back 60

Epstein-Barr

Front 61

Symptoms include: painless enlargement of lymph nodes; splenomegaly; heptamegaly; petechia due to decrease in platelet number; weight loss; fatigue

Back 61

Hodgkin's Lymphoma

Front 62

How is Hodgkin's Lymphoma Dx?

Back 62

Biopsy

Front 63

What is the typical tx for Hodgkin's Lymphoma?

Back 63

chemotherapy, bone marrow &/or peripheral blood transplants, immunotherapy (mAbs) &/or radiation therapy

Front 64

An absence or decrease in what hormone is evident in Diabetes Insipidus?

Back 64

ADH Anti-Diuretic Hormone

Front 65

A lesion in the ______ or _________ is associated with Diabetes Insipidus.

Back 65

Hypothalmus

Posterior Pituitary

Front 66

What happens to the urine of pt's with Diabetes Insipidus?

Back 66

becomes dilute due to lack of ADH; Large volume of urine

Front 67

DX: very thirsty pt, with massive quantities of dilute urine?

Back 67

Diabetes Insipidus

Front 68

What is the tx for DI?

Back 68

synthetic ADH

Front 69

Tumors of the anterior pituitary usually composed of secretory cells

Back 69

pituitary adenomas

Front 70

What is the tx for pituitary adenomas?

Back 70

Surgery

Front 71

What is the effect of pituitary adenomas?

Back 71

supression or excess increase in secretions of the pars distalis

Front 72

____ and _____cross placental barrier; critical in early stages of brain development  deficiency during fetal development results in irreversible damage to CNS including  numbers of neurons, defective myelination, and mental retardation.

Back 72

T3 and T4

Front 73

T3 and T4 also stimulate gene expresson for _______

Back 73

Growth hormone; somatotropin

Front 74

_______________(cretinism) combination of CNS damage and stunted growth

Back 74

Congenital hypothyroidism

Front 75

Diffuse enlargement of thyroid gland and protrusion of eyeballs (exophthalmic goiter

Back 75

Grave's disease

Front 76

What happens histologically to follicle cells when pt has Grave's disease?

Back 76

become columnar; increase in thyroid hormones

Front 77

Caused by binding of autoimmune IgG Abs to TSH receptors  stimulation of thyroid follicular cells &  thyroid hormone secretion

Back 77

Grave's disease

Front 78

What is the tx for Grave's disease?

Back 78

surgical removal of thyroid gland or radiotherapy (labeled iodine) to destroy most active follicular cells; immunosuppression

Front 79

What is the most common symptom of thyroid disease?

Back 79

Simple Goiter

Front 80

Goiter is nlargement of thyroid gland due to excessive release of ______ from anterior pituitary

Back 80

TSH

Front 81

What causes Goiter?

Back 81

deficiency of Iodine in diet

autoimmune – Hashimoto’s
thyroiditis

Front 82

Over-activity of parathyroid glands  excess secretion of PTH & subsequent bone resorption.

Back 82

Hyperparathyroidism

Front 83

Associated with high blood Ca2+ levels,  may lead to deposition of Ca2+ salts in kidneys (kidney stones – leading to UTI) and walls of blood vessels

Back 83

Hyperparathyroidism

Front 84

a chronic, blistering skin disorder caused by the action of IgG antibodies to surface antigens (desmoglein & plakin family) on keratinocytes, an autoimmune disorder;

Back 84

Pemphigus Vulgaris

Front 85

excessive production of collagen in the dermis; systemic sclerosis can occur in other organs (kidney, GI tract, lungs) but when it occurs in the skin it is called scleroderma;

Back 85

scleroderma

Front 86

characterized by tightening of skin (skin becomes parchment like), radial furrowing around lips; Raynaud’s phenomena is a clinical finding

Back 86

Scleroderma

Front 87

__ and ____ collagen are extensively deposited in papillary layer and then eventually the reticular layer in scleroderma.

Back 87

Type 1

Type 2

Front 88

What do we see histologically with scleroderma?

Back 88

a lot of enlarged collagen bundles, occluded or disappearance of vasculature/appendages in dermis. T cells accumulate at some point, secrete cytokines and this results in increased collagen synthesis—this points to an autoimmune disorder

Front 89

Albinism - typically categorized as _________positive or negative

Back 89

tyrosinase

Front 90

Albinism can be a defficiencin what enzyme?

Back 90

tyrosinase

Front 91

What cell is involved in albinism?

Back 91

Melanocytes

Front 92

-- A noncontagious skin disorder that most commonly appears as inflamed, edematous skin lesions covered with a silvery white scale

Back 92

Psoriasis

Front 93

The most common type of psoriasis is ___________and it is most common on the extensor surfaces of the knees, elbows, scalp, and trunk

Back 93

plaque psoriasis

Front 94

__________appears as erythematous raised plaques with silvery white scales on the scalp

Back 94

Scalp Psoriasis

Front 95

associated with partial albinism of hair and skin, results from mutation in myosin Va gene.

Back 95

Griscelli Syndrome

Front 96

_________is a molecular motor protein that binds to actin and the melanosome when it is released from the microtubule in dendrite

Back 96

Myosin Va

Front 97

plugging of the outlet of a hair follicle by keratin debris; androgen receptors on sebaceous gland cells stimulate division/maturation of cells and production of sebum

Back 97

Acne Vulgaris

Front 98

______ = sebum outflow to the skin surface is blocked and follicles are distended

Back 98

A comedo

Front 99

What are the 3 causes of epidermolysis bullosa (EB)?

Back 99

- defects in keratin within the basal layer,

-defect in laminin,

-defect in Type VII collagen-anchoring fibrils

Front 100

Elevated levels of ___ and ___ in sweat is an indicator for Cystic Fibrosis.

Back 100

Sodium

Chloride

Front 101

In Uremia, when the body is unable to get rid of excess Urea, there are increased levels of ______ in sweat.

Back 101

UREA

Front 102

Individuals with UREMIA often have high concentrations of urea in their sweat. When this sweat evaporates urea crystals are evident on the skin. This crystalling is called _______.

Back 102

Urea frost

Front 103

Epidermal repair is usually effected by __________ proliferation.

Back 103

Basal cell

Front 104

With extensive trauma ________ and ________ effect epidermal repair.

Back 104

Hair follicle epithelia

sweat gland epithelia

Front 105

The repair of an incision or laceration requires stimulation of growth of both the ______ and _____________.

Back 105

Dermis

Epidermis

Front 106

1. Decrease in or total absence of plasma insulin; elevated blood glucose levels and the loss of glucose in urine.

Back 106

Diabetes Mellitus

Front 107

Which type of DM results from low blood insulin levels?

Back 107

Type 1 DM

Front 108

Characterized by insatiable hunger, unquenchable thirst, and excessive urination

Back 108

Type 1 DM

Front 109

Which type of DM shows up before age 20? Juvenile DM

Back 109

Type 1 DM

Front 110

What pancreatic cells are damaged with Type 1 DM?

Back 110

Islet beta cells---secrete Insulin

Front 111

Which type of DM is "insulin-resistant"?

Back 111

Type 2 DM

Front 112

Why is Type 2 DM called insulin-resistant?

Back 112

decreased binding of insulin to its receptor and defects in induction of signal transduction pathway

Front 113

Characterized by secretion of inadequate amounts of adrenocortical hormones

Back 113

Addison's disease

Front 114

Usually due to destruction of adrenal cortex

Back 114

Addison's disease

Front 115

Defined in patients who have 2 or more tumors of endocrine glands

Back 115

Multiple endocrine neoplasia MEN

Front 116

Most often caused by autoimmune disease or tuberculosis
Symptoms include: weight loss, muscle weakness, fatigue, low BP, skin hyperpigmentation
Life-threatening & requires steroid treatment

Back 116

Addison's disease

Front 117

: prevalence 1:35,000 associated w/ pituitary adenoma, parathyroid hyperplasia, pancreatic tumors

Back 117

MEN 1

Front 118

hyperplasia, medullary pheochromocytoma, and thyroid carcinoma

Back 118

MEN 2

Front 119

What did Abe Lincoln have: pathology?

Back 119

MEN 2b