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395 Cards in this Set

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child has been anemic since birth. splenectomy would result in increasesd hematocrit in what dz?
spherocytosis
pt presents w/ fatigue, and blood tests show a macrocytic, megaloblastic anemia. what is the danger of givign folate alone
masks signs of neural damage with vit B12 deficiency
pt presents w/ anemia hypercalcemia, and bone pain on palpation; bone marrow biopsy shows a slide backed with cells that have a large, round, off center nucleus. What is the dx and what may be found on urinalysis
myltiple myeloma (plasma cell neoplasm); Bence Jones protein (Ig light chains)
AIDS pt has just been diagnosed with CA. What dneoplasms are associated w/AIDS?
B cell lympnoma, kaposi's sarcoma.
Pt w/ new CA dx and known hx of CHF is being evaluated for chemotherapy. What chemotherapeutic agent shoudl be avoided in this pt?
Doxorubicin (cardiotoxic)
chromosome analysis reveals the presence of the philadelphia chromosome, t(9;22). What is the latest targeted therapy for this dz and how does it work
Imatinib (Gleevec) is used to treate CML; a monoclonal Ab against the bcr-able tyrosine kinase
blood cell differentiation [p. 287]
--
these cells are anucleate, bioconcabe giving them a large surface area: volume ratio for easy gas exchange.
erythrocyte
eryth=_____; cyte=_____
red
cell
how do erythrocytes get their energy
mostly glucose (90%)
10% via HMP shunt
erythrocytes anarobically degrade glucose to _____
lactate
what is the survival time of erythrocytes
120 D
membrane of erythrocytes contain ______-_______ antiport which is important in the "physiologic chloride shifft," which allows the RBC to transport CO2 from the periphery to the lungs for elimination
cloride-bicarbinate antiport
erythrocytosis/polycythemia (def)
increased number of red cells
anisocytosis
RBCs of varying sizes
poikilocytosis
RBCs of varyng shapes
reticulocyte
immature erythrocyte
these blood cells are responsible for defence against infections.
leukocytes
give me leukocyte types (5)
granulocytes (basophils, eosinophils, neutrophils) and mononuclear cells (lymphocytes, monocytes
Normally leukocyte count
4000-10,000 per microliter
these blood cells mediate allergic reactions
basophils
basophils consist of ____ of all leukocytes
<1%
basophils have a _______ nucleus
bilobate
basophils have many granules that stain in this way ______
basophilic-stain redily wiht basic stains
give three contents of basophilic granules in basophils
heparin, histamine, leukotrienes (LTD-4)
these blood cells mediate type I hypersensitivity reactions.
mast cells
This drug prevents mast cell degranulation and is used to treat asthma
cromolyn sodium
Degranulation of mast cells involves release of these factors
histamine, heparin, eosonophil chemotactic factors
mast cells can bind to this Ab
IgE
Mast cells are very similar yet distinct from this cell type
basophils
Mast cells are found here
tissue
these cells defent against helminthic & protozoan infections and are hightly phagocytic for Ag-Ab complexes
eosinophil
eosinophils normally make up this percent of all leukocytes
1-6%
regarding appearance, eosinophils have a ____ nucleus & and are packed with large eosinophils granuls of uniform size
bilobate
eosinophils produce these 2 substances
histaminase and arylsulfatase
eosin=____
philic=_____
dye
loving
causes of eosinophilia
NAACP
neoplastic
asthma
allergic processes
collagen vascular dz
parasites
this blood cell is an acute inflammatory response cell
neutorphils
neutrophils make up _______ of WBCs
40-75%
neutrophils have a ______ nucleus
multilobed
hypersegmented polys are seen in __________
vit B12/folate deficiency
neutrophils have large, spherical, azurophilic primary granules that contain hydrolytic enzymes, lysosyme, myeloperoxidase, and lactoferrin--they are called this
lysosomes
these blood cells are large with a kidney-shaped nucleus. They have extensive "frosted glass" cytoplasm.
monocytes
mono=___
cyte=____
one
cell
moncytes cells differentiate into _____in tissues
macrophages
these blood cells are small and round with a densely staining nucleus & a small amount of pale cytoplasm
lymphocytes
___ lymphocytes produce Ab
B
___ lymphocytes manifest the cellular immune response as well as regulhumoralate B lymphocytes and macrophages
T
B lymphocytes are part of the _______ immune response
humoral
B cells arise from stem cells in the _______ where they also mature
bone marrow

mneu: B=bone marrow
After maturation B cells migrate to ______ (follicles of lymph nodes, white pulp of spleen, unencapsulated lymphoid tissue).
peripheral lymphoid tissue
When Ag is encountered, B cells differentiate into _____ and produce Ab.
plasma cells
Plasma cells recognize Ab because they have _______
memory
B lymphocytes can fx as antigen-presenting cell (APC) via _______
MHC II
these blood cells have an off-center nucleus, with clock face chromatin distribution, abundant RER and well developed Golgi apparatus
plasma cell
_________ is a plasma cell neoplasm
multiple myeloma
B cells differentiate into _______, which can produce large amounts of Ab specific to partifcular Ag
plasma cell
B lymphocytes have these cell markers
CD 19, CD20
this blood cells mediates cellular immune responses.
T lymphocyts
T cells originate from stem cells in the ________, but matures in the ________.
bone marrow
thymus

mneu: T is for Thymus
T cells differentiate into these three types of T cells
Cytotoxic T cells
Helper T cells
suppressor T cells
cytotoxic T cells have MHC ___ & CD____.
I,8

mneu: MHC x CD=8 (e.g., MHC 2 x CD4=8, & MHC 1 x CD8=8)
helper T cells have MHC ___ & CD____.
II, 4

mneu: MHC x CD=8 (e.g., MHC 2 x CD4=8, & MHC 1 x CD8=8)
CD stands for ______
cluster of differentiation
This cell has a long life in tissues. It Phagocytizes bacteria, cell debris, and senescent red cells & scavenges damaged cell and tissues.
macrophage
Macrophages differentiate from circulating blood _______
monocytes.
macrophages are activated by __________.
gamma-interferon
macrophages can fx as APC via _______.
MHC II
macro=______
phage=_______
large
eater
these cells are professional APCs. They are the main inducers of primary Ab response.
dendritic cells
Dendritic cells express these two things on their cell serfaces
MHC II & Fc receptors (FcR)
dendritic cells are called ________ cells on skin
Langerhan cells
plasma - clotting factors =(e.g., fibrinogen)
serum
blood composition [chart p. 290]
--
these cells are professional APCs. They are the main inducers of primary Ab response.
dendritic cells
Dendritic cells express these two things on their cell serfaces
MHC II & Fc receptors (FcR)
dendritic cells are called ________ cells on skin
Langerhan cells
plasma - clotting factors =(e.g., fibrinogen)
serum
blood composition [chart p. 290]
--
name 3 coagulation factor inhibitors involved in fibrinolysis
protein C & S
Antithrombin III
tPA
Protein C & protein S inactivate these two steps in the coagulation cascade
Va & VIIIa
Protein C & protein S are dependant on this vitamen
K
antithrombin III inactivates these four steps in the coagulation pathway
thrombin, IXa, Xa, & XIa
antithrombin III is activated by this anticoagulant
heparin
tPA generates _______, which cleaves fibrin
plasmin
convergence of coagulation, compliment and kinin pathways [image p. 291]
--
neoplasmic progression
normal cells->hyperplasia-> carcinoma in situ/preinvasive -> invasive carcinoma ->metastatic focus
hyperplasia is
increased cell number
this type of "plasia" shows an abnormal proliferation of cells w/ loss of size, shape and orientation
dysplasia
in carcinoma in situ neoplasmic cells have NOT invaded _________
basement membrane
carcinoma in situ neoplasmic cells have a _____ nuclear/cytoplasmic ratio and clumped cromatin
high
in invasive carcinoma cells have invaded the basement membrane using _______ & _______
collagenases and hydrolases
invasive carcinoma can metastasize if they reach _______ or ________ vessel
blood or lymphatic
metastasis is
spread to distant organ
in order for neoplasmic cells to metastasize they must survive the host _______
immune system
in the "seed and soil" theory of metastasis what is the seed and what is the soil.
seed=tumor embolus
soil=target organ-liver, lungs, bone, brain
this type of "plasia" is an increase in number of cells
hyperplasia
is hyperplasia reversible
yes
in this type of "plasia" 1 adult cell type is replaced by another
metaplasia
is metaplasia reversable
yes
metaplasia is often secondary to _______- as is the case w/ squamous metaplasia in trachea and bronchi of smokers
irritation
dysplasia
is abnormal growth with loss of cellular orietnation, shape, and size in comparison to normal tissue maturation
wat is concerning about dysplasia
it is often preneoplasmic
is dysplasia reversible
yes
In this type of "plasia" there are abnormal cells lacking differation.
anaplasia
in this type of cell "plasia" you see primitive cells of the same tissue, often equated with undifferentiated malignant neoplasms. You may see tumor giant cells.
anaplasia
this type of plasia describes a clonal proliferation of cells that is uncontrolled and excessive
neoplasia
the grade of a tumor referrs to the degree of __________ based on histologic appearance of tumor.
cellular differentiation
tumors are usually graded on a scale of ____ to _____
1-4
grade is often determined by the number of ______ per high power field
mitosis
_______ gives information about the character of the tumor itself versus _______ which tells us about the spread of the tumor in a specific pts
grade
stage

mneu: Stage=Spread
The ______ of a tumor tells us about the degree of localization/spread of a tumor based on the site and size of the primary lesion, spread to regional lymph nodes, presence of metastases
Stage
In the TNM staging system:
T=
N=
M=
T=size of tumor
N=Node involvement
M=Metasteses
Given the tumor name tell what the cell type is and whether it is benigh or malignant.
adenoma, papilloma
epithelium
benign
Given the tumor name tell what the cell type is and whether it is benign or malignant.
andinocarcinoma or papillary carcinoma
epithelium
malignant
Given the tumor name tell what the cell type is and whether it is benign or malignant.
leukemia, lymphoma
malignant tumor of the blood cells
Given the tumor name tell what the cell type is and whether it is benign or malignant.
hemangioma
benign tumor of the blood vessels
Given the tumor name tell what the cell type is and whether it is benign or malignant.
leiomyoma
benign tumor of the smooth mm
Given the tumor name tell what the cell type is and whether it is benign or malignant.
leiomyosarcoma
malignant tumor of smooth mm
Given the tumor name tell what the cell type is and whether it is benign or malignant.
rhabdomyoma
benign tumor of the skeletal mm
Given the tumor name tell what the cell type is and whether it is benign or malignant.
rhabdomyosarcoma
malignant tumor of the skeletal mm
Given the tumor name tell what the cell type is and whether it is benign or malignant.
osteosarcoma
malignant tumor of the bone
Given the tumor name tell what the cell type is and whether it is benign or malignant.
osteoma
benign tumor of the bone
Given the tumor name tell what the cell type is and whether it is benign or malignant.
lipoma
benign tumor of the fat cell
Given the tumor name tell what the cell type is and whether it is benign or malignant.
liposarcoma
malignant tumor of the fat cell
a maature teratoma is a _______ (benign/malignant) tumor of > 1 cell type
benign
a immature teratoma is a _______ (benign/malignant) tumor of > 1 cell type
malignant
the term carcinoma implies an _________ origin, whereas sarcoma denotes a ________ origin. both terms imply malignancy
epithelial
mesenchymal
give the neoplasm associated with the dz.
down syndrom
ALL, AML

mneu: we ALL fall DOWN
give the neoplasm associated with the dz.
xeroderma pigmentosum, albinism
melanoma and basal, squamous cell carcinoma of the skin
give the neoplasm associated with the dz.
chronic atrophic gastritis
gastric adenocarcinoma
give the neoplasm associated with the dz.
pernicious anemia
gastric adenocarcinoma
give the neoplasm associated with the dz.
postsurgical gastric remnance
gastric adenocarcinoma
give the neoplasm associated with the dz.
tuberous sclerosis (facial angiofibroma, seizures, mental retardation)
astrocytoma and cardiac rhabdomyoma
give the neoplasm associated with the dz.
actinic keratosis
squamous cell carcinoma of the skin
give the neoplasm associated with the dz.
barret's esophagus (chronic GI reflux)
esophageal adenocarcinoma
give the neoplasm associated with the dz.
Plummer-Vinson syndrome (atrophic glossitis, esophageal webs, anemia; all due to iron deficiency
squamous cell carcinoma of the esophagus
give the neoplasm associated with the dz.
cirrhosis (alcoholic, hep B or C)
hepatocellular carcinoma
give the neoplasm associated with the dz.
ulcerative colitis
colonic adenocarcinoma
give the neoplasm associated with the dz.
paget's dz of the bone
secondary osteosarcoma and fibrosarcoma
give the neoplasm associated with the dz.
immondeficiency states
malignant lymphomas
give the neoplasm associated with the dz.
AIDS
aggressive malignant lymphomas (non-Hodgkin's) and Kaposi's sarcoma
give the neoplasm associated with the dz.
Autoimmiune dz (e.g., Hashimoto's thyroiditis, myasthenia gravis)
benign and malignant thymomas
give the neoplasm associated with the dz.
Acanthosis nigricans (hyperpigmentation and epidermal thickening
visceral malignancy (stomach, lung, breast, uterus)
give the neoplasm associated with the dz.
dysplastic nevus
malignant melanoma
when oncogenes become functional, this results
Cancer
give the associated tumor for the oncogene:
abl
CML
give the associated tumor for the oncogene:
c-myc
burkitt's lymphmoma
give the associated tumor for the oncogene:
bcl-2
follicular and undifferentiated lympomas
this oncogene inhibits apoptosis
bcl-2
give the associated tumor for the oncogene:
erb-B2
breast ovarian, and gastric carcinomas
give the associated tumor for the oncogene:
ras
colon carcinoma
give the associated tumor for the oncogene:
L-myc
Lung tumor
give the associated tumor for the oncogene:
N-myc
Neuroblastoma
give the associated tumor for the oncogene:
ret
multiple endocrine neoplasia (MEN) types II & III
in this type of gene, loss of function of BOTH allels results in the expression of cancer
tumor suppressor gene
given the tumor suppressor gene and chromosome give the associated tumor:
Rb gene on Chromosome 13q
retinoblastoma, osteosarcoma
given the tumor suppressor gene and chromosome give the associated tumor:
BRCA1 & 2 on chromosome 17q & 13q
breast and ovarian CA
given the tumor suppressor gene and chromosome give the associated tumor:
p53 on chromosome 17p
most human CA, Li-Fraumeni syndrome
given the tumor suppressor gene and chromosome give the associated tumor:
p16 on 9p
Melanoma
given the tumor suppressor gene and chromosome give the associated tumor:
p16 on chromosome 9p
melanoma
given the tumor suppressor gene and chromosome give the associated tumor:
APC on chromosome 5q
colorectal CA
given the tumor suppressor gene and chromosome give the associated tumor:
WT1 on chromosome 11q
Wilms' tumor
given the tumor suppressor gene and chromosome give the associated tumor:
NF1 on chromosome 17q
Neurofibromatosis type 1
given the tumor suppressor gene and chromosome give the associated tumor:
NF2 on chromosome 22q
Neurofibromatosis type 2
given the tumor suppressor gene and chromosome give the associated tumor:
DPC on chromosome 18q
Pancreatic CA
given the tumor suppressor gene and chromosome give the associated tumor:
DCC on chromosome 18q
colon CA
tumor markers should be used as the primary tool for CA dz. T or F
F
Tumor markers can be used to confirm a diagnosis. T or F
T
Tumor markers can be used to monitor tumor for recurrencce. T or F
T
Tumor markers can be used to monitor response to therapy. T or F
T
this tumor marker is used for screening for prostate carcinoma
Prostate Specific Antigen (PSA)
this tumor marker is is very nonspecific but is produced by ~ 70% of colorectal and pancreatic CA. It is also produced by gastric and breast carcinomas
Carcinoembrionic Ag (CEA)
this tumor marker is normally made in the fetus it is seen in hepatocellular carcinomas and germ cell tumors of the testis (e.g., yolk sac tumor)
alpha-fetoprotien.
this tumor marker is seen in Hydatidiform moles, choriocarcinomas, and Gestational trophoblastic tumors
B-HCG
this tumor marker is seen in ovarian and malignant epithelial tumors
CA-125
this tumor marker is seen in melanoma, neural tumors, astrocytomas
S-100
this tumor marker is seen in bone metasteses, obstructive biliary dz, and Paget's dz of the bone
Alk phosphatase
this tumor marker is seen in neuroblastoma, lung and gastric CA
Bombesin
this tumor marker is seen in harry cell leukemia (B-cell neoplasm
Tartrate-resistant acid phosphatase (TRAP)
these are laminated, concentric, calcific spherules seen in several CAs.
psamoma bodies
what 4 CA are psammoma bodies seen in
1) Papillary adenocarcinoma of thyroid
2) Serious papillary cystadenocarcinoma of ovary
3) Meningioma
4) Malignant mesothelioma

mneu: PSaMMoma
Papillary (thyroid)
Serous (ovary)
Meningioma
Mesothelioma
Given the oncogenic virus, give the associated Cancer:
HTLV-1
adult T-cell leukemia
Given the oncogenic virus, give the associated Cancer:
HBV, HCV
hepatocellular carcinoma
Given the oncogenic virus, give the associated Cancer:
EBV
Burkitt's lymphoma, nasopharyngeal carcinoma
Given the oncogenic virus, give the associated Cancer:
HPV
Cervical carcinoma (16,18), penile/anal carcinoma
Given the oncogenic virus, give the associated Cancer:
HHV-8
Kaposi's sarcoma, body cavity fluid B cell lymphoma
given the chemical carcinogen, tell me the organ it acts on:
aflatoxins
liver (HCC)
given the chemical carcinogen, tell me the organ it acts on:
vinyl chloride
liver (angiosarcoma)
given the chemical carcinogen, tell me the organ it acts on:
CCl4
liver (centrilobular necrosis, fatty change)
given the chemical carcinogen, tell me the organ it acts on:
nitrosamines
esophagus, stomach
given the chemical carcinogen, tell me the organ it acts on:
cigarette smoke
larynx, lung
given the chemical carcinogen, tell me the organ it acts on:
Asbestos
Lung (mesothelioma & bronchogenic carcinoma)
given the chemical carcinogen, tell me the organ it acts on:
arsenic
skin (squamous cell carcinoma)
given the chemical carcinogen, tell me the organ it acts on:
Naphthalene (aniline) dies
Bladder (transitional cell carcinoma
given the chemical carcinogen, tell me the organ it acts on:
Alkylating agents
Blood (lleukemia
given the paraneoplastic effect, give the neoplasm: Cushing's syndrome due to ACTH
Small cell lung carcinoma
given the chemical carcinogen, tell me the organ it acts on:
SIADH
small cell lung carcinoma and intracranial neoplasms
given the chemical carcinogen, tell me the organ it acts on:
Hypercalcemia due to PTH, TGF alpha and beta, IL-1
squamous cell lung, renal cell, breast, multiple myeloma, bone mets
given the chemical carcinogen, tell me the organ it acts on: Polycytthemia due to increased erythropoietin
renal cell carcinoma or hemangioblastoma
given the chemical carcinogen, tell me the organ it acts on: Lambert-Eaton syndrome (mm weakness) due to Ab against presynaptic Ca++ channels at neuromuscular jx
thymoma, small cell lung Ca
given the chemical carcinogen, tell me the organ it acts on:
Gout, urate nephropathy due to hyperuicemia due to excess nucleic acid turnover (i.e., cytotoxic tx)
leukemia & lymphoma
what percentage of brain tumors are from metastesis
50%
what primaries commonly metastasize to brain (5)
Lung, Breast, Skin (melanoma), Kidney (renal cell carcinoma), GI

mneu: Lots of Bad Stuff Kills Glia
what are the most common sites of metastasis after regional lymph nodes
liver & lung
give the tumors that commonly metastasize to the liver in order of frequency
Colon>Stomach>Pancreas>Breast>Lung

mneu: Cancer Sometimes Penetrates Benight Liver
what is more common liver mets or liver primary
mets>> primary liver tumors
what are the primary tumors that metastasize to the bone
Prostate, thyroid, testes, breast lung, kidney

P.T. Barnum Loves Kids
What are the two most common tumors that metastasize to bone
breast & prostate
what is more common bone primaries or bone mets
BONE METS
incidence of prostate CA in males
32%
incidence of breast CA in females
32%
incidence of lung CA in males
16%
incidence of lung CA in females
13%
incidence of colon and rectal CA in males
12%
incidence of colon and rectal CA in females
13%
CA is the ___ leading cause of death in the US
2nd. Heart dz is 1st
mortality of lung CA in males
33%
mortality of lung CA in females
23%
mortality of Prostate CA in males
13%
mortality of Breast CA in females
18%
are lung CA deaths still rising in US despite nat'l efforts to prevent smoking
they've plateaud in males
still rising in females
normal shape of RBC
bioconcave
given the RBC form give the condition:
spherocytes
hereditary spherocytosis or autoimmune hemolysis
given the RBC form give the condition:
elliptocyte
hereditary eliptocytosis
given the RBC form give the condition:
macro-ovalocytes
megaloblastic anemia
marrow failure
in a blood smear of a pt w/ megaloblastic anemia there will be macro-ovalocytes and also this
hypersegmented PMNs
given the RBC form give the condition: helmet cell, shistocyte
DIC or traumatic hemolysis
given the RBC form give the condition:
Sickle cell
Sickle cell anemia
given the RBC form give the condition:
Teardrop cell
myeloid metaplasia w/ myelofibrosis
given the RBC form give the condition:
Acanthocytes
spiny appearance in abetalipoproteinemia
given the RBC form give the condition:
Target cells
HbC dz, Asplenia, Liver dz, Thalassemia

mneu: HALT
given the RBC form give the condition: Poikilocytes
Nonuniform shapes in TTP/HUS, microvascular damage, DIC
given the RBC form give the condition: Burr cell
TTP/HUS
MCV of microcytic hypocromic anemia
<80
MCV of macrocytic
>100
these vitamen deficiencies are associated with hypersegmented PMNs
vit B12 & folate
unlike folate deficiency, this vitamen deficiency is associated with neurological problems
vit B12
vit B12 deficiency is called
pernicous anemia
Decreased serum haptoglobin and increased serum LDH indicate this
RBC hemolysis
this test is used to distinguish between immune v. non-immune mediated RBC hemolysis
direct coombs test
iron deficiency, thalassemias, lead poisioning, and sideroblastic anemias cause this type of anemia
microcytic, hypochromic
pt labs show low serum iorn, increased TIBC, decreased ferritin. What is the dx.
iron deficiency (microcytic anemia)
pts blood smear shows target cells what is the dx?
thalassemia (microcytic anemia)
pt presents w/ vit B12/folate deficiency what type of anemia do you susbect
megaloblastic, macrocytic
drugs that block DNA synthesis (e.g., sulfa drugs, AZT) can cause this type of anemia
Macrocytic anemia
acute hemorrhage causes this type of anemia
normocytic, normochromic
normocytic normochromic anemia can be caused by enzyme defects such as (give 1)
G6PD deciciency or PK deficiency
normocytic normochromic anemia can be caused by RBC membrane defects such as _______
hereditary spherocytosis
normocytic normochromic anemia can be caused by bone marrow disorders such as
aplastic anemia, leukemeia
normocytic normochromic anemia can be caused by hemoglobinopathies such as
sickle cell dz
normocytic normochromic anemia can be caused by autoimmune _______ anemia
hemolytic
this type of anemia usually presents with the following labs: decreased TIBC, increased ferritin, increased storage of iron in bone marrow macrophages
anemia of chronic dz(ACD)
graphs [p. 298]
--
this is a pancytopenia characterized by severe anemia, neutorpenia, and thrombocytopenia that is caused by failure or destruction of multipotent myeloid stem cells, with inadequate production or release of differentiated cell lines
aplastic anemia
give 3 possible causes of aplastic anemia
radiation, benzene, chloramphenicol, alkylating agents, antimetabolites, viral agents (parovirus B19, EBV, HIV), Fanconi's anemia, idiopathic (immune mediated, primary stem-cell defect). May follow acute hepatitis.
pt presents with an infection. she reports malaise and fatigue. On physical exam she appears pale, with purpura, mucosal bleeding, and petechia. What are you concerned about. Blood smear shows pancytopenia w/ normal cell morphology.
aplastic anemia
how do you dx aplastic anemia.
bone marrow biopsy
What does bone marrow bipsy show.
hypocellular bone marrow with fatty infiltation
how do you tx aplastic anemia
withdraw the offending agent. allogenic bone marrow transplantation. rbc & platelet transfusion. G-GSF or GM-CSF.
this dz is caused by a HBS mutation which is a single amino acid replacement in B chain (substitution of normal glutamic acid w/ valine).
sickle cell anemia
what type of situation precipitates sickling
low O2 or dehydration (could be induced by exercise)
heterozygotes of sickle cell anemia is known as
sickle cell trait
heterozygotes of sickle cell trait are resistant to this dz
malaria (balanced polymorphism)
crescent shaped RBCs are known as ________
sickle cells
complications in homozygotes (sickle cell dz) can include aplastic crisis due to this viral infection
parovirus B19
complications in homozygotes (sickle cell dz) can include teh following (give 3)
autosplenectomy, increased risk of encapsulated organism infxn, salmonella osteomyelitis, painful crisis (vaso-occlusive), and splenic sequestration crisis.
new therapies for sickle cell anemia include
hydroxeourea (increased HgF) and bone marrow transplantation.
HbC or HbSC cell mutations cause this type of presentation
milder dz than HbSS
there are ___ alpha globin genes
4
in this disorder the alpha-golbin chain iis underproduce because of bad genes, 1-4. there is no compensatory increase in any other chains.
alpha thalassemia.
alpha thalassemia is present in these 2 continants
asia and africa
Beta thalassemia is present in these populations
mediterranean populations
this results in Beta 4 tetramers, lacks 3 alpha globin gnes
HbH
this dz involved gamma4 tetramers, and lacks all 4 alpha alpha globin genes and results in hydrops fetalis and intrauterine fetal dealth
Hb Barts
in this blood dyscrasia fetal hgb production is compensatorily increased but is inadequate.
B thalassemia
B thalassemia minor
Beta chain is underproduced (heterozygous)
Beta thalassemia major
Betea chain is absent
pt has severe anemia requiring blood transfuusion and skeletal deformities that arose as a result of marrow expansion. His frequent blood transfusions result in secondary hemochromatosis which eventually causes cardiac failure and death. what is his underlying condition.
Beta thalassemia
How severe is a heterozygote HbS/Beta thalassemia
mild to moderate dz
Beta thalassemia major
Betea chain is absent
pt has severe anemia requiring blood transfuusion and skeletal deformities that arose as a result of marrow expansion. His frequent blood transfusions result in secondary hemochromatosis which eventually causes cardiac failure and death. what is his underlying condition.
Beta thalassemia
How severe is a heterozygote HbS/Beta thalassemia
mild to moderate dz
pt ppresents with jaundice and increased serum bilirubin. Her retic count is also increased. What type of anemia do you suspect
hemolytic
why is retic count increased in hemolytic anemia
bone marrow compensating
what type of gallstones are commonly present in hemolytic anemias
pigment gallstones
this type of hemolytic anemia involves mostly extravascular hemolysis-often accelerated RBC destriction in liver Kupffer cells and spleen)
autoimmune anemia
what type of test do you do to test for hemolytic anemia
+ coombs test
this autoimmune hemolytic anemia is a chronic anemia commonly seen in pts with SLE, CLL, or with certain drugs (e.g., alpha-methyldopa)
Warm Agglutin
Warm agglutin involves this Ab
IgG

mneu: WARM weather is GGGreat
this autoimmune hemolytic anemia is an acute anemia triggered by cold. Often seeen during recovery from Mycoplasma pneumonia or infectious mononucleosis
Cold agglutin
Cold agglutinin involves this Ab
IgM

mneu: COLD ice cream. . .MMM
this type of autoimmune hemolytic anemia is seen in newborns due to Rh or other blood Ag incompatibility when the mother's Ab attack fetal RBCs
Erythroblast fetalis
In this test anti-Ig Ab is added to pts RBCs. There is agglutination if RBCs are coated with Ig
Direct Coombs test
In this test normal RBCs are added to pts serum. There is agglutination if serum has anti-RBC surface Ig
indirect Coombs
in this hemolytic anemia there is intrinsic, extravascular hemolysis due to spectrin or ankyrin defect. RBCs are small and round with no central pallor, meaning they have less menbrane, increased MCHC, and increased RDW.
hereditary spherocytosis
In hereditary spherocytosis pts are Cooombs ____, what test is then used to confirm
Coombs -
Osmotic fragility test
This hemolytic anemia involves intravascular hemolysis due to a membrane defect leading to increased sensitivity of RBCs to the lytic activity of complement
Paroxysmal nocturnal hemoglobinuria
what will you see in the urin of a pt siwth paroxysmal nocturnal hemoglobinuria
increased urine hemosiderin
this form of hemolytic anemia involves intravascular hemolysis seen in DIC, TTP/HUS, SLE, or malignant hypertension.
microangiopathic anemia
what is seen on a blood smear in a pt with microangiopathic anemia
shistocytes (helmet cells)
this condition involves an activation iof the coagulation cascade leading to microthrombi and global consumpton of platelets, fibrin, and coagulation factors
DIC
give the most common cause of DIC
obstrectic complications
what are some other causes of DIC (give 2)
gram negative sepsis, transfusion, trauma, malignancy, acute pancreatitis, nephrotic syndrome.
what are the lab findings of DIC:
PT __
PTT__
D-Dimer (fibrin split products)____
platelet count___
Blood smear ___________
PT ↑
PTT↑
D-Dimer (fibrin split products)↑
platelet count↓
Blood smear: helmet-shaped cellls and schistocytes
Give 3 examples of bleeding disorders resulting from platelet abnormalities:
1)idiopathic thrombocytopenic purpura (ITP)
2)Thrombotic thrombocytopenic purpura (TTP)
3)DIC
4) Aplastic anemia
5) caused by drugs
platelet abnormalities often result in this type of hemhorrage which involves mucus membrane bleeding, epistaxis, petechia, pupura, and increased bleeding time
microhemorrhage
pt presnts with microhemhorrage. Labs show antiplatelet antibodies and increased number of megakaryocytes. What platelet abnormality do you suspect
idiopathic thrombocytopenic purpura (ITP)
pt presnts with microhemhorrage and neurologic symptoms. Labs increased LDH. Blood smear shows schistocytes. What platelet abnormality do you suspect
Thrombotic thrombocytopenic purpura (TTP)
pt presnts with microhemhorrage. Labs increased D-Dimer (fibrin split products). Blood smear shows schistocytes. What platelet abnormality do you suspect
DIC
pt presnts with microhemhorrage and neurologic symptoms. Pt is a kidney transplant recepiant. What platelet abnormality do you suspect
platelet abnormality due to immunosuppressive drugs
Give 3 Coagulation factor defects that result in bleeding disorders
1) Hemophelia A
2) Hemophelia B
3) Von Willebrand's dz
Coagulation factor defects often involves this type of hemhorrage
Macrohemhorrage
macrohemorrhage often involves what lab changes in PT & PTT
increases
macrohemorrhage often involves easy bruising and hemarthroses. What is hemarthrosis?
bleeding into joints
Hemophilia A involves what factor deficiency
factor VIII
Hemophilia B involves what factor deficiency
factor IX
what is the most common coagulation factor defect
von willebrand'sdz
is Von Willebrand's dz mild or severe
mild
what is deficient in Von willebrands dz and what does this lead to
deficiency of von Willebrand factor resulting in defect of platelet adhesion
In von willebrand's dz there is a decrease in factor ___ survival
VIII
Different hemorrhagic disorders involve different lab values. Give the changes that exist in the following lab values with the d/o given:
DZ: QUALITATIVE PLATELET DEFECT
1)Platelet count
2) Bleeding time
3) PT
4) PTT
increased bleeding time
Different hemorrhagic disorders involve different lab values. Give the changes that exist in the following lab values with the d/o given:
DZ: THROMBOCYTOPENIA
1)Platelet count
2) Bleeding time
3) PT
4) PTT
decreased platelet count
increased bleeding time
Different hemorrhagic disorders involve different lab values. Give the changes that exist in the following lab values with the d/o given:
DZ: HEMOPHELIA A OR B
1)Platelet count
2) Bleeding time
3) PT
4) PTT
increased PTT
Different hemorrhagic disorders involve different lab values. Give the changes that exist in the following lab values with the d/o given:
DZ: VON WILLEBRANDS DZ
1)Platelet count
2) Bleeding time
3) PT
4) PTT
increased bleeding time
Different hemorrhagic disorders involve different lab values. Give the changes that exist in the following lab values with the d/o given:
DZ: DIC
1)Platelet count
2) Bleeding time
3) PT
4) PTT
decreased platelet count
increased bleeding time
increased PT & PTT
An example of a qualitative platelet defect would be bernard-soulier dz. What is deficient in this dz and what is the result
low GP Ib--resulting in a defect of platelet adhesion
An example of a qualitative platelet defect would be Glanzmann's thrombasthenia. What is deficient in this dz and what is the result?
low GP IIb-IIIa. resulting in a defect of platelet agGregation
PT involves this pathway including these 4 factors
extrinsic
factors II,V,VII, & X
PTT involves this pathway including these factors
intrinsic
all factors except VII
what is the most common type of Hodgkin's lymphoma
Nodular sclerosing (65-75%)
what is the prognosis of nodular sclerosing hodgkins lymphoma
excellent
doees NSH effect women or men more, how about age group
women more than men, primarily young adults
what is the perportion of reed sternburg (RS) cells to lymphocytes in nodular sclerosing Hodgkins lymphoma
3:1, Lymphos:RS cells
nodular sclerosing Hodgkins lymphoma has a special kind of RS cell what is it?
lacunar
this subtype of Hodgekin's lymphoma involves collagen banding
nodular sclerosing
this type of Hodgkins lymphoma has numerous lymphocytes and even more RS cells.
mixed cellularity (25%)
what is the prognosis for mixed cellularity hodgkins lymphoma
intermediate
this type of hodgkin's in more common in males under 35 y/o. It has far more lymphocytes than RS cells
lymphocyte predominant (6%)
what is the prognosis of lymphocyte predominant HL
excellent
this type of hodgekins lymphoma is rare. It effects older males with disseminated dz. the number of RS cells is high relative to the few lymphocytes. The prognosis of this HL is poor
lymphocyte depleted.
this is the distinctive tumor giant cell seen in hodgkins dz
reed sternberg cell
because teh RS cell is binucleate or bilobed w/ the 2 halves as mirror images it is sometimes called this type of cell. (think bird)
"owl eyes" cell
is the RS cell sufficient for dx of Hodgekins dz
no-neccessary but not sufficient
what varient of RS cells is found in nodular sclerosing hodgekins dz
lacunar cells
Non-Hodgkins lymphoma has several subtypes. This type presents in adulthood with a focal mass. It effects the B cells and is often low grade. It is similar to CLL
Small lymphocytic lymphoma
Non-Hodgkins lymphoma has several subtypes. This is the most common adult NHL. It involves B cells and is difficult to cure. There is an indolent cource.
Follicular lymphoma (small cleaved cell)
this dz is associated with t(14:18) and bcl-2 expression
Follicular lymphoma
what is bcl-2 responsible for
apoptosis
Non-Hodgkins lymphoma has several subtypes. This type usually effects older adults but 20% occurs in children. It is aggressive, but up to 50% are curable.
diffuse large cell
Does diffuse large cell NHL effect B or T cells?
80% B cells
20% mature T cells
Non-Hodgkins lymphoma has several subtypes. This type is most common in children. It commonly presents w/ ALL and a mediastinal mass. It is very aggressive
lymphoblastic lymphoma
what cells do lymphoblastic lymphoma attack
mature T cells
This type of lymphoma most often effects children. In africa it often presents with a jaw lesion. examination of bx under microscope will show "starry-sky" appearance (sheets of lymphocytes with interspersed macrophages.)
Burkitt's lymphoma
What virus is Burkitt's lymphoma ssociated with
EBV virus
what cells does Burkitt's lymphoma attack
B cells
This NHL is associated with t(8;14) c-myc gene moving next to heavy chain Ig gene (14)
Burkitt's
pt presents with infection and increased number of circulating leukocytes in the blood. Complications include anemia (low RBCs), hemorrhage (low platelets), bx of bone marrow, liver, spleen, & LN may show infiltrates of CA
leukemia
How do you leukemia dx?
usually bone marrow bx
this subtype of leukemia often effects children. bx shows lymphoblast. It is the most responsive to therapy and may spread to CNS & testes
ALL
this subtype of leukemia often effects adults. Bx shows myeloblasts & auer rods
AML
this subtype of leukemia often effects older adults. They often present with lymphadenopathy and hepatosplenomegly. There are few symptoms and often have an indulent course.
CLL
what would you see on peripheral blood smear of pt w/ CLL
increased smudge cells.
what type of autoimmune hemolytic anemia is CLL
warm antibody
what NHL is CLL very similar to.
SLL (small lymphocytic lymphoma.
this subtype of leukemia presents with splenomegly and labs show incerased neutrophils and metamyelocytes
AML
what chromosome and gene is CML associated with
Philadelphia chromosome (t[9;22],bcr-abl
what type of stem cell proliferation is involved in CML
myeloid
CML may accelerate to AML resulting in a _______
blast crisis
Leukocytes have very low levels of this in CML
alkaline phosphatase
chart [p.303]
--
these are peroxidase positive cytoplasmic inclusions in granulocytes and myeloblast.
auer bodies (rods)
Auer rods are primarily seen in thys type of leukemia
acute promyelocytic leukemia (M3).
treatment of AML M3 can release aurer rods resulting in this
DIC
leukemia classification[p.304]
--
chromosomal translocations can result in several disorders. Given the translocations give the associated disorder)
t(9;22)(philadelphia chromosome)-->bcr-abl hybrid
CML
chromosomal translocations can result in several disorders. Given the translocations give the associated disorder)
t(8:14)-->c-myc activation
Burkitt's lymphoma
chromosomal translocations can result in several disorders. Given the translocations give the associated disorder)
t(14;18)->bcl-2 activation
follicular lymphomas
chromosomal translocations can result in several disorders. Given the translocations give the associated disorder)
t(15;17)
M3 type of AML
M3 type of AML is responsive to this tx
trans retinoic acid
chromosomal translocations can result in several disorders. Given the translocations give the associated disorder)
t(11;22)
ewing's sarcoma
chromosomal translocations can result in several disorders. Given the translocations give the associated disorder)
t(11;14)
mantle cell lymphoma
This is the most common primary tumor arising within the bone in adults. It is a moloclonal plasma cell CA that arises in the marrow and produces large amounts of IgG or IgA
multiple myaloma
multiple myeloma plasma cells have this type of appearance (think breakfast foods)
fried egg
multiple myeloma produces IgG what percentage of the time and IgA what percentage of =the time
IgG=55%
IgA=25%
multiple myeloma can cause destructive bone lesions that can result in this abnormal lab finding
hypercalcemia
what are some other complications that can come with multiple myeloma (3)
renal insufficiency
susceptibility to infection
anemia
multiple myeloma is associated with this dz where abnormal protiens build up in the organs
amyloidosis
what might you see on x-ray of a pt with multiple myeloma
punched otu lytic bone lesions on x-ray
what might you see on serum protein electrophoresis of a pt with multiple myeloma
monoclonal immunoglobin spike (M protien)
what might you see in urine of pt with multiple myeloma
bence jones protien
what is bence jones protein
Ig light chains
what does the blood smear show in pts with multiple myeloma
rouleau formation
what is rouleau formation
RBCs stacked like poker chips
pt presents with an M spike indicating increased levels of IgM. They have hyperviscosity symptoms and no lytic bone lesions. Is it multiple myeloma? What else might you suspect.
No
Waldenstrom macroblobinemia