Heme Onc USMLE Flashcards by Mani Yavi

child has been anemic since birth. splenectomy would result in increasesd hematocrit in what dz?

spherocytosis

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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

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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)

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AIDS pt has just been diagnosed with CA. What dneoplasms are associated w/AIDS?

B cell lympnoma, kaposi’s sarcoma.

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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)

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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

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these cells are anucleate, bioconcabe giving them a large surface area: volume ratio for easy gas exchange.

erythrocyte

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eryth=_____; cyte=_____

red

cell

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how do erythrocytes get their energy

mostly glucose (90%)
10% via HMP shunt

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erythrocytes anarobically degrade glucose to _____

lactate

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what is the survival time of erythrocytes

120 D

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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

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erythrocytosis/polycythemia (def)

increased number of red cells

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anisocytosis

RBCs of varying sizes

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poikilocytosis

RBCs of varyng shapes

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reticulocyte

immature erythrocyte

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these blood cells are responsible for defence against infections.

leukocytes

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give me leukocyte types (5)

granulocytes (basophils, eosinophils, neutrophils) and mononuclear cells (lymphocytes, monocytes

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Normally leukocyte count

4000-10,000 per microliter

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these blood cells mediate allergic reactions

basophils

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basophils consist of ____ of all leukocytes

<1%

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basophils have a _______ nucleus

bilobate

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basophils have many granules that stain in this way ______

basophilic-stain redily wiht basic stains

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give three contents of basophilic granules in basophils

heparin, histamine, leukotrienes (LTD-4)

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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

___ lymphocytes manifest the cellular immune response as well as regulhumoralate B lymphocytes and macrophages

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

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

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

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

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

Tumor markers can be used to confirm a diagnosis. T or F

Tumor markers can be used to monitor tumor for recurrencce. T or F

Tumor markers can be used to monitor response to therapy. T or F

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)

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

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

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

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

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