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742 Cards in this Set

  • Front
  • Back

Product of cycloocygenase pathway

Prostaglandins

Prostaglandin causing fever

PGE2 (fEEEEver)

Product of 5-lipooxygenase pathway

Leukotrienes

Leukotreine attracting neutrophils

LTB4

Leukotreine mediating vasoconstriction, bronchospasm, and inc permeability (3)

LTC4, LTD4, LTE4

What cells causes inc vasc permeability

Pericytes contract causing inc permeability

3 ways mast cells are activated

Tissue trauma,


complement C3a, C5a,


crosslinking of cell surface IgE by antigen

Action of histamine

Vasodilation and inc vasc permeability

Second phase of mast cell response

Production of leukotrienes

How to activate hageman factor

Subendothelial or tissue collagen

Mediates pain (2)

PGE2 and Bradykinin

Mediates fever (2)

IL1, TNF

Action of IL1 and TNF

activates perivascular cells in hypothalamus -> increase COX activity -> increase PGE2 -> raises temp

Steps of neutrophil activation

Margination -> Rolling -> Adhesion -> Transmigration and Chemotaxis

P selectin came from

Weibel-Palade bodies

"Speed bumps"

Selectins

Proteins in Weibel Palade bodies (2)

VWF and P selectins

E selectins is induced by (2)

TNF , IL1

Selectins bind to ___ on leukocytes

Sialyl lewis X

Cellular adhesion molecules are upregulated by (2)

TNF, IL1

Cellular adhesion molecules will bind to

Integrins

Integrins are upregulated by (2)

C5a, LTB4

AR defect of integrins (CD18 subunit)

Leukocyte adhesion deficiency

Clinical findings of leukocyte adhesion deficiency

Delayed separation of umbilical cord


Inc circulating neutrophil


Recurrent bacte infections, lack pus formation

4 chemotaxis attractants

IL8, C5a, LTB4, bacterial products

AR protein trafficking defect, microtobule defect, impaired phagolysosome formation

Chediak higashi syndrome

Increased pyogenic infxn, neutropenia, giant granules in leukocutes, defective hemostasis, albinism, peripheral neuropathy

Chediak higashi syndrome

Most effective mechanis of killing microbes

Oxygen dependent killing

Enzyme converting O2 to superoxide

NADPH oxidase

Enzyme converting superoxide to H2O2

Superoxide dismutase

Enzyme converting H2O2 to HOCl

Myeloperoxidase

NADPH Oxidase defect, Xlinked or AR, poor O2 dependent killing

Chronic granulomatous disease

Organisms prone to cause infection in CGD



Name the 5 organisms

Catalase positive organisms (catalase destroys H2O2 needed for O2 dependent killing)



S aureus, B cepacia, S marcescens, Nocardia, Aspergillus

Used to screen CGD

Nitroblue tetrazolium test


(+) colorless - CGD


tests O2 -> O2-

Defective conversion of H202 to HOCl

MPO deficiency

Infection prone in MPO deficiency

Candida infection

Mediators of resolution and healing by macrophage (2)

IL10, TGF-B

B7 on antigen presenting cell binds to __ on CD4 cells

CD28 (28 ÷ 7 = 4)

TH subset helping CD8 T cells

TH1 subset

TH1 generate (2) mediators

IL2 (Tcell growth factor, CD8 T cell activator)


IFN Y (macrophage activator)

TH subset helping B cells

TH2

TH2 generate (3) mediators

IL4 (class switching to IgG and IgE)


IL5 (eosinophil chemotaxis and activation, maturation of B cells to plasma cells, class switching to IgA)


IL10 (inhibits TH1 phenotype)

Secreted by TH1 subset, a T cell growth factor, CD8 T cell activator

IL2

Secreted by TH1 subset, macrophage activator

IFN Y

Secreted by TH2 subset, causes class switching to IgG and IgE

IL4

Secreted by TH2 subset, causes eosinophil chemotaxis and activation, maturation of B cells to plasma cells, class switching to IgA

IL5

Secreted by TH2 subset, inhibits TH1 phenotype

IL10

Mediator of apoptosis

Activate caspase

2 mechanisms of cytotoxic killing

Secretion of perforins and granzymes -> apoptosis


Expression of FasL, binds to Fas on target cell -> apoptosis

3 ways to activate the caspases

Intrinsic mitochondrial pathway (cytochrome C)


Extrinsic receptor pathway (FasL and TNF binding)


Granzymes


2 signals for B cell activation

MHC II- CD4


CD40 - CD40 Ligand

IL4 and IL5 from TH cells acts on B cells by

Mediating Bcell isotype switching, hypermutation, maturation to plasma cells

Key cell that defines a granuloma

Epitheloid histiocytes (macrophages with abundant pink cytoplasm)

Granuloma type which lack central necrosis

Non caseating

(5) Examples of non caseating granuloma

Reaction to foriegn material


Sarcoidosis


Beryllium exposure


Chron disease


Cat scratch disease

Key differentials for caseating granulomas

TB and fungal infections

Converts macrophages to epitheloid histiocytes and giant cells

IFN Y

Step of granuloma formation

Macrophage MHC II-CD4 Tcells -> Macrophage secrete IL12-> IL12 induce CD4 differentiation to TH1 -> TH1 secrete IFN Y -> converts macrophages to epitheloid histiocytes and giant cells

Developmental failure of 3rd and 4th pharyngeal pouch -> lack of thymus (T cell def), lack of parathyroids (hypocalcemia), abnormalities of heart, great vessels

DiGeorge syndrome

Disease due to 22q11 microdeletion

DiGeorge syndrome

Adenosine deaminase deficiency, cytokine recpetor defects, MHC II defienciency

SCID severe combined immunodeficiency

Lack of immunoglobulin, disordered B cell maturation, naive B cells cannot mature to plasma cells

X linked agammaglobulinemia

Mutation in bruton tyrosine kinase

X linked agammaglobulinemia


Signalling molecule for B cells to become plasma cells

Infections prone in x linked agammaglobulinemia

Bacterial infections, enterovirus, Giardia infections

Low Ig due to B cell or T helper cell defects, increased risk for autoimmune dse and lymphoma

Common variable immunodeficiency

Most common Ig deficiency

IgA deficiency

High propensity for IgA deficiency

Celiac disease

Mutation in CD40 or CD40 ligand, cytokines necessary for Ig class switching is not produced, Low IgA, IgG, IgE results in recurrent pyogenic infections

Hyper IgM syndrome

Thrombocytopenia, Eczema, Recurrent infections, x linked

Wiskott Aldrich syndrome

C5-C9 deficiency

Risk for Neisseria infections

C1 inhibitor deficiency

Hereditary angioedema

Most common renal damage in SLE

Diffuse proliferative glomerulonephritis

Vegetations in both sides if the valve in SLE

Leibman-Sacks endicarditis

Anti-histone antibody

Drug induced SLE

3 drug inducing SLE

Hydralazine, procainamide, isoniazid

Antibody against proteins bound to phospholipids

APAS

2 Antibodies in APAS

Anticardiolipin and lupus anticoagulant

False positive labs caused by Lupus anticoagulant

False positive syphilis and false increase PTT

Anti ribonucleoprotein antibodies

Sjogren syndrome

Autoimmune disorder most commonly associated with Sjogren syndrome

Rheumatoid arthritis

Complication of Sjogren syndrome, unilateral enlargement of parotid

Increased risk for B cell lymphoma

Autoimmune tissue damage with activation of fibroblasts and deposition of collagen

Scleroderma

Anti DNA topoisomerase 1 antibody

Diffuse type scleroderma

Most common organ associated with diffuse type scleroderma

Esophagus, disordered motility, dysphagia for solids and liquids

Localized type scleroderma

CREST



Calcinosis / anti Centromere antibodies


Raynaud phenomenon


Esophageal dysmotility


Sclerodactyly


Telangiectasia of skin

U1 ribonucleoprotein antibodies

Mixed connective tissue disease

SLE, systemic sclerosis, polymyositis

Mixed connective tissue disease

Marker for hematopoietic cells

CD34

Type 3 collagen

Blood vessels, granulation tissue, embryonic tissue

Converts Type 3 to Type 1 collagen in granulation tissue

Collagenase

What is the cofactor needed for collagenase converts Type 3 to Type 1 collagen in granulation tissue

Zinc

Repair growth factors which is an epithelial and fibroblast growth factor

TGF A

Repair growth factors which is an important fibroblast growth factor, and inhibits inflammation

TGF B

Repair growth factors which is an endothelium, smooth muscle and fibroblast growth factor

Platelet derived GF

Repair growth factors which is for angiogenesis and skeletal development

Fibroblast GF

Repair growth factors which is for angiogenesis

Vascular endothelial GF

Cofactor of lysyl oxidase

Copper

Collagen type of hypertrophic scar

Type 1

Collagen type excess in keloid

Type 3

Aflatoxin

Hepatocellular carcinoma

Alkylating agents

Leukemia / lymphoma

Alcohol

Squamous cell CA, pancreatic CA, hepatocellular CA

Alcohol

Chronic pancreatitis -> pancreatic CA

Arsenic

Squamous cell CA of skin, lung CA

Asbestosis

Lung CA, mesothelioma

Nitrosamines

Stomach CA (smoked foods)

Naphthylamine

Urothelial CA of bladder

Vinyl chloride

Angiosarcoma of liver

EBV

Nasopharyngeal CA

EBV

Burkitt lymphoma

Ionizing radiation

AML, CML, Papillary CA of thyroid

Non ionizing radiation

Basal cell CA, Squamous cell CA, Melanoma

PDGF B over expression

Astrocytoma

Epidermal growth factor receptor (HER2/neu) amplification

Breast CA

Point mutation RET (neural growth factor receptor)

MEN 2A, 2B, sporadic medullary CA of thyroid

Pount mutation KIT (Stem cell growth factor receptor)

GIST gastrointestinal stromal tumor

Point mutation RAS gene (GTP binding protein)

Carcinomas, melanoma, lymphoma

Overactivation of ABL (tyrosine kinase) with BCR, t(9:22)

CML

c-MYC, t(8:14)

Burkitt lymphoma

N-MYC amplification

Neuroblastoma

L-MYC amplification

Lung CA (small cell)

"Starry sky appearance"

Burkitt lymphoma

Cyclin D, t(11:14)

Mantle cell lymphoma

IH stain Keratin (+)

Epithelium

IH stain Vimentin (+)

Mesenchyme

IH stain Desmin (+)

Muscle

IH stain GFAP (+)

Neuroglia

IH stain Neurofilament (+)

Neurons

IH stain Chromogranin (+)

Neuroendocrine cells (small cell, carcinoid)

IH stain S-100 (+)

Melanoma

Germline mutation of p53

Li- Fraumeni syndrome

Releases E2F and causes progression from G1 to S

Rb

Sporadic mutation of Rb

Unilateral Retinoblastoma

Germline mutation of Rb

Bilateral retinobalstoma, osteosarcoma

Stabilizes the mitochondrial membrane, blocking release of cytochrome c

Bcl2

Overexpression of Bcl2

Follicular lymphoma, t(14:18)

Process of metastasis

Down regulate e-cadherin


Cells attach to laminin


Destroy the BM using collagenase type 4


Attach to fibronectin in ECM and spread locally


Enter the BV or lymphatics

Carcinomas that spread hematogenously (4)

Renal cell CA


Hepatocellular CA


Follicular CA of thyroid


ChorioCA

"Omental Caking"

Ovarian carcinoma seeding in body cavities

Most common cause of thrombocytopenia in children and adults

ITP

Source of the antibodies in ITP

Spleen

Decrease in ADAMTS13 enzyme

TTP (cannot degrade large multimer of vWF -> abnormal plt adhesion -> micro thrombi)

2 disease which causes microangioapathic hemolytic anemia

TTP, HUS

Bacteria causing HUS

Ecoli O157:H7 (E coli verotoxin -> damages endothelial cells -> plt microthrombi)

Most common inherited coagulation disorder

von Willebrand disease

vWF helps stabilize what coagulation factor

Factor 8

Treatment of vWD

Desmopressin

Increases vWF release from Weibel Palade bodies

Desmopressin

Necessary for gamma carboxylation of factors 2, 7, 9, 10 C and S

Vitamin K

Activates vitamin K in the liver

Epoxide reductase

Heparin binds to what molecule on the surface of platelets

Platelet factor 4

Increase fibrin split products and D-dimers

DIC

Increase fibrinogen split products but not D-dimers

Disorder of fibrinolysis (overactivation of plasmin)

Treatment of overactivity of plasmin

Aminocaproic acid (blocks activation of plasminogen)

Protein C and S inactivates factors (2)?

Factors 5 and 8

Increase risk for warfarin skin necrosis

Protein C or S deficiency

Most common inherited cause of hypercoagulable state?

Factor V Leiden

Why oral contraceptives cause hypercoagulable state?

Estrogen induces production of coagulation factors

Chronic form of decompression sickness causing multifocal ischemic necrosis of bone

Caisson disease

Embolus characterized by squamous cells and keratin debris

Amniotic fluid embolus

Absorption of iron occurs in

Duodenum

Channel that absorbs Iron in enterocytes to the blood

Ferroportin

Form of Iron that is easily absorbed

Ferrous (maintained in ferrous state by stomach acid)

IDA, esophageal web, atrophic glossitis

Plummer-Vinson syndrome


Presents with anemia, dysphagia and beefy red tongue

Acute phase reactant that sequesters Iron in storage sites

Hepcidin

Suppresses EPO production in Anemia of chronic disease

Hepcidin

Inc Ferritin, dec TIBC, dec Iron, dec saturation, inc Free protoporhyrin

Anemia of chronic disease

Dec Ferritin, inc TIBC, dec Iron, dec saturation, inc Free protoporhyrin

Iron deficiency anemia

Anemia due to defect in protoporphyrin synthesis

Sideroblastic anemia

Rate limiting step in the production if protoporohyrin

ALAS amino levolinic acid synthase


Succinyl CoA to ALA

Cofactor of ALAS

Vit B6

Enzyme converting ALA to Porphobilinogen

ALA dehydrogenase ALAD

Enzyme converting protoporphyrin and Iron to heme

Ferrochelatase


Occur in mitochondria

Cause of ringed sideroblast

Iron accumulate in mitochondria due to defective protoporphyrin synthesis

Most common cause of acquired sideroblastic anemia

Alcoholism - mitochondrial poison

Lead inhibits 2 enzymes in heme synthesis

ALAD and ferrocheletase

Inc ferritin, dec TIBC, inc serum iron, inc saturation

Sideroblastic anemia


(Iron overloaded state like hemochromatosis)

Inc ferritin, dec TIBC, inc serum iron, inc saturation

Sideroblastic anemia


(Iron overloaded state like hemochromatosis)

Tetramers of Beta chains

Hgb H, 3 alpha chains deleted

Tetramers of Gamma chains

Hgb Barts, 4 alpha chains deleted

Aplastic crisis with parvovirus B19 occurs with

Beta thalassemia major

Folate is absorbed in

Jejunum

Vit B12 is absorbed in

Ileum

What binds to Vit B12 to be absorbed in the ileum

Intinsic factor

Converts methylmalonic acid to succinyl CoA

Vit B12


Subacute combined degeneration of the spinal cord due to accumulation of methylmalonic acid

Accumulation of methylmalonic acid in spinal cord due to Vit B12 def

Subacute combined degeneration of the spinal cord

3 tethering proteins involved in hereditary spherocytosis

Spectrin, ankyrin, band 3.1

Factors which increase risk of sickling

Hypoxemia


Dehydration


Acidosis

Drug increases levels of Hgb F

Hydroxyurea

What is the target of Parvovirus B19

Erythroid precursors

Increased risk of infection with encapsulated organisms due to autosplenectomy occurs in

Sickle cell anemia

Bacteria which cause of osteomyelitis in sickle cell anemia

Salmonella paratyphi

Over all common cause of osteomyelitis

S aureus

Most common cause of death of Sickle cell anemia in adults

Acute chest syndrome (pneumonia)

How to screen for sickle cell disease

Metabisulfite screen


Positive in both disease and trait

Test which confirms HbS

Hb electrophoresis

Acquired defect in myeloid stem cells resulting in absent GPI

PNH


GPI attaches decay accelerating factor DAF and MIRL in cells which prevents degradation by complement

Screening and confirmatory test for PNH

Screening - Sucrose test


Confirm - acidifying serum test

Marker tested in flow cytometry for PNH

Detect lack of CD55 (DAF)

Main cause of death in PNH

Thrombosis of hepatic, portal and cerebral veins due to destruction of platelets

Infectious agents causing IgM mediated hemolysis (2)

Mycoplasma pneumoniae


Infectious mononucleosis

Cortisol can cause lymphopenia die to

It causes apoptosis of lymphocytes

Cell type that is earliest to be damaged due radiation

Lymphocytes

Immature neutrophils have decreased levels of this marker

CD16

Cortisol causes neutrophilia by?

Increasing the shift of neutrophils from marginated pool to the circulating pool

Marker increased in hodgkin lymphoma causing eosinophilia

IL5

Basophilia is associated with what leukemia

CML

Bacterial infxn causing lymphocytic leukocytosis

Bordatella pertussis

Substance that increases lymphocytes in bordatella pertussis infxn

Lymphocytosis promoting factor

Tcell type increased in Infectious mononucleosis

CD8 Tcells

Which part of the lymph node os affected in infectious mononucleosis

Paracortex

Part of the spleen affected in infectious mononucleosis

Periarterial lymphatic sheath (PALS) in the white pulp due to Tcells

Atypical lymphocytes is associated with

Infectious mononucleosis (CD8 Tcells)

Screening test which detects IgM heterophile antibodies in IM

Monospot test

Definitive diagnistic test for IM

EBV viral capsid antigen test

Infectious mononucleos may become dormant in which cells

B cells

Rash may be associated in IM if administered with this drug

Penicillin

Marker to detect lymphoblasts

TDT + (DNA polymerase)

Marker for myelobalsts

MPO +

Classic surface markers of Bcell ALL

CD 10, CD19, CD20

Most common type of ALL

Bcell ALL

Bcell ALL that is more commonly seen in kids and has good prognosis

t(12:21)

Bcell ALL that is more commonly seen in adults and has poor prognosis

t(9:22) also seen in CML (Ph+ ALL)

Classic surface markers of Tcell ALL

CD2, 3, 4, 5, 6, 7, 8

ALL type most common in teenagers

Tcell ALL (T for Teenager)

"Acute lymphoblastic lymphoma"

T cell ALL (because classically forming a mass in mediastinum (Thymus))

t(15:17) type of leukemia

APL

t(15:17) encodes for what receptor

RAR receptor is disrupted (promyelocytes accumulate)

Auer rodes in APL can cause what complication

DIC

Leukemia with numerous Auer rods

APL

When given to APL can bind to RAR receptor, cause maturation of blasts

ATRA

Monoblasts preferentially infiltrate which part of the body

Gums

Myelocytic leukemia that lacks MPO

Acute Megakaryocytic leukemia

Cytopenia with hypercellular bone marrow

Myelodisplastic syndrome

Blast percentage in MDS

<20%

CLL is a chronic proliferation of ___ cells

Naive B cells

CLL express what markers

CD5, CD20

Important complication of CLL that could become a more aggressive tumor

Diffuse large B cell lymphoma

Hairy cell leukemia is a neoplastic proliferation of what cell type

Mature B cells

TRAP positive leukemia

Hairy cell

Part of the spleen that is enlarged in Hairy cell leukemia

Red pulp (normally white pulp is where lymphocytes are present, but not in this case)

Drug with good response in hairy cell leukemia

2CDA (Adenosine deaminase inhibitor) adenosine accumulates in t cells causing damage

Neoplastic proliferation of mature CD4 Tcells

Mycosis fungoides

Mycosis fungoides is caused by neoplastic proloferation of what cell

CD4 Tcells

CD4 preferentially infiltrate which part of the body

Skin (rash, plaques, nodules) aggregates of neoplastic T cell in epidermis

Aggregates of neoplastic T cells in epidermis in Mycosis Fungoides

Pautrier microabscesses

If mycosis fungoides spread to the blood, it is called

Sezary syndrome

Classic appearance of lymphocytes with ceribriform nuclei

Sezary cells

First line treatment for CML which blocks tyrosine kinase activity

Imatinib

Clinical finding asso with accelerated phase of CML

Enlarging spleen

LAP result in CML

LAP negative (LAP positive in leukemoid reaction)

Mutation in Polycythemia vera

JAK2 kinase mutation

Secondline treatment in PV

Hydroxyurea

EPO levels in PV

Decreased due to negative feedback

Mutation in essential thrombocythemia

JAK2 kinase

Excessively produced by megakaryocytes causing marrow fibrosis

PDGF

Painless lymphadenopathy causes

Chronic infection, metastatic carcinoma, lymphoma

Part of the lymph node enlarged in viral infections

Paracortex

Part of the lymph node enlarged in viral rheumatoid arthritis and early HIV

Follicles

Classic finding in lymphnodes which are draining tissue with cancer

Sinus histiocytes

Part of the lymph node where the T cells live

Paracortex

Part of the lymph node where the B cells live

Cortex ("B and C" close together)

Lmyphoma of neoplastic small B cells

Follicular lymphoma

Follicular lymphoma is a neoplastic proliferation of what cells?

small B cells (CD20+) that make follicle loke nodules

Over expression of BCL2 causes

Inhibition of apoptosis ( inc cells in follicular lymphoma due to defective apoptosis)

Anti CD20 antibody drug for follicular lymphoma

Rituximab

Follicular lymphoma may progress to

Diffuse large B cell lymphoma

Lymphoma by t(11:14) mutation

Mantle cell lymphoma

Gene drive production of mantle cell lymphoma

Cyclin D1 overexpression (promotes G1 to S)

Lymphoma associated with chronic inflammatory states

Marginal zone lymphoma

Gastric MALToma is associated with what bacteria

H pylori

3 types of small B cell lymphoma

Follicular, mantle cell, marginal zone

Neoplastic proliferation of intermediate sized B cells

Burkitt lymphoma

t(8:14) is a translocation of

C-myc of Burkitt's lymphoma (oncogene that promotes cell growth)

Lymphoma asso woth high mitotic growth and "starry sky appearance"

Burkitt lymphoma

Most common form of Non HL

Diffuse large B cell lymphoma

Reed sternberg cell

Hodgkin's lymphoma

Surface markers in Hodgkins lymphoma

CD15, CD30

Large B cell with multilobed nuclei and prominent nucleoli

Reed-Sternberg cell (owls eye appearance)

B symptoms

Fever, chills, night sweats

Most common for of HL

Nodular sclerosis

Classic presentation of enlarging cervical or mediastinal LN in young adult female

Nodular Sclerosis HL

Special type of Reed sternberg cell found in nodular sclerosis

Lacunar cells (lake like cells of RS cells)

HL subtype with best prognosis

Lymphocyte rich subtype

HL subtype with worst prognosis

Lymphocyte poor subtype

HL subtype with abundnat eosinophils

Mixed cellularity

Marker elevated in multiple myeloma

IL6 (plasma cells)

Neoplastic plasma cells activate __ receptors on osteoclasts in MM

RANK receptor (osteoclast activating factor)

M spike on protein electriphoresis

Multiple myeloma (monoclonal IgG or IgA)

Increase in serum free light chains and deposit in tissues

Primary amyloidosis in MM

Free light chains excreted in urine

Bence Jones protein

M spike in serum electrophoresis comprised of IgM

Waldenstroms macroglobulinemia

Birbeck or tennis racket granules

Langerhans histiocytosis

Markers of Langerhans cell histiocytosis

CD1a, S100

Malignant proliferation of Langerhans cells in <2y/o

Letterer - Siwe disease

Malignant proliferation of Langerhans cells in >3y/o

Hand-Schuller-Christian disease

Most common form of vaculitis in older adults (>50y/o)

Temporal (Giant cell) arteritis

Most common form of vaculitis in older adults (<50y/o)

Takayasu arteritis

Temporal (Giant cell) arteritis promarily affects the branches of what major artery?

Carotid

Takayasu arteritis promarily affects the branches of what major artery?

Aortic arch at branch points

Arteritis that is also called "pulseless disease"

Takayasu arteritis

Arteritis that affects muscular arteries

Medium vessel arteritis

Necrotizing vasculitis involvong most organs EXCEPT the lung in young adults

Polyarteritis nodosa

Transmural inflammation asso with fibrinoid necrosis

Polyarteritis nodosa

'String of pearl' appearance on imaging

Polyarteritis nodosa

Preferrential artery involved in kawasaki disease

Coronary artery

Treatment of Kawasaki disease

Aspirin and IVIg

Necrotizing vasculitis involving digits, (ulceration, gangrene, autoamputation) associated with smoking

Buerger disease

Necrotizing granulomatous vasculitis involving nasopharynx, lungs, kidneys, middle aged males,

Wegener granulomatosis

Serum C-ANCA (cytaplosmic)

Wegener granulomatosis

Treatment for Wegener granulomatosis

Cyclophosphamide and corticosteroids

Serum P-ANCA

Microscopic polyangitis and Churg Strauss

Same as Wegener granulomatosis w/o nasopharyngeal involvement and granulomas

Microscopic polyangitis

Necrotizing granulomatous vasculitis involving eosinophils, multiple organs esp heart and lungs, with asthma and peripheral eosinophilia

Churg Strauss syndrome

Vasculitis due to IgA immune complexes

Henoch Schonlein Purpura

Most common vasculitis in children

Henoch Schonlein Purpura

Vasculitis asso with PALPABLE purpura on buttocks and legs, GI pain and bleed, hematuria, occurs following URTI

Henoch Schonlein Purpura

Most common site of abdominal aortic aneurysm

Below the renal arteries above the bifurcation

Tree bark appearance of thoracic aorta

Thoracic aortic aneurysm

Triad of hypotension, pulsatile abdominal mass, flank pain

Abdominal aortic aneurysm rupture

Hallmark of subendocardial ischemia

ST segment depression

% of blockage that causes chest pain in stable angina

<70%

Chest pain at rest

Unstable angina

Due to rupture of atherosclerotic plaque with thrombosis and INCOMPLETE occlusion of a coronary artery

Unstable angina

Vasospasm of the coronary artery

Prinzmetal angina

ECG finding in prinzmetal angina

Transmural ischemia causing ST segment elevation

Due to rupture of atherosclerotic plaque with thrombosis and COMPLETE occlusion of a coronary artery

Myocardial infarction

Cardiac marker useful for detection of reinfarction days after MI

CK MB since peak is 24hrs and normal by 3 days

CK-MB rises ___ hrs

4-6hrs

CK-MB peaks ___ hrs

24 hrs

CK-MB returns to normal ___ hrs

72 hrs / 3 days

Troponin rises ___ hrs

2-4 hrs

Troponin peaks ___ hrs

24 hrs

Troponin returns to normal ___ days

7-10 days

1 day, 1 week, 1 month

< 1 day - coagulative necrosis


1 day to 1 week - inflammatory phase


1 week to 1 month - granulation tissue


After 1 month - scar

Papillary muscle is supplied by what coronary artery

Right coronary artery (infarction would cause mitral insufficiency)

Autoimmune destruction if pericardium after MI

Dressler syndrome

Septal defect asso with fetal alcohol syndrome

VSD

Most common ASD

Ostium secundum

ASD asso with Down syndrome

Ostium primum

Congenital heart defect asso with maternal diabetes

TGA

Congenital heart defect asso with Turner syndrome

Coarctation of the aorta ("coarcturner of the aorta")

Nothcing of the ribs

Coarctation of the aorta adult form

Aschoff bodies

ARF Myocarditis

Bounding pulses, head bobbing, pulsating nailbed

Aortic regurgutation

Mid systolic click

MVP

Opening snap followed by diastolic rumble

Mitral stenosis

Most common cause of IE in IV drug users

S aureus

Valve involved in IE by S aureus

Tricuspid valve

Most common cause of IE in prosthetic valves

S epidermidis

Most common cause of IE in colorectal CA

S bovis

Most common cause of IE with negative culture

HACEK organisms

Erythematous nontender lesions on the palms nd soles

Janeway lesions

Painful lesions on fingers or toes

Osler nodes (ouch -osler)

Sterile vegetations on both sides of the mitral valve asso with SLE

Libman-Sacks endocarditis

Valve defect in Libman-Sacks endocarditis

Mitral regurgitation

Heart defect due to Autosomal dominant (AD) genetic mutations in sarcomere proteins

Hypertrophic cardiomyopathy

Most common site of myxoma

Left atrium

Benign hamartoma of cardiac muscle, most common primary cardiac tumor in children

Rhabdomyoma

Benign hamartoma of cardiac muscle, most commonly associated with tuberous sclerosis

Rhabdomyoma

Part of the heart most commonly involved in metastatic tumors of the heart

Pericardium causing pericairdial effusion

Benign tumor of tge nasal mucosa compised of large BV and fibrous tissue, seen in adolescent males with profuse epistaxis

Angiofibroma

Virus asso with nasopharyngeal carcinoma

EBV

Most common cause of acute epiglottitis, esp in non immunized children

H influenza type b

Most common cause of laryngotracheobronchitis

Parainfluenza virus

Asso with barking cough and inspiratory stridor

Croup

Most common cause of secondary pneumonia

S aureus

Most common cause of pneumonia superimposed on COPD

H influenzae, moraxella catarrhalis

Intracellular bacteria visualized by silver stain

Legionella pneumophila

Most common cause of atypical pneumonia usually in young adults

Mycoplasma pneumoniae

Most common cause of atypical pneumonia in infants

RSV

Anaerobic bacteria in oropharynx

Bacteroides, Fusobacterium, Peptococcus

Reid index in chronic bronchitis

>50% (normally <40% )

Most common cause of centriacinar emphysema

Smoking

Most common cause of panacinar emphysema

A1 antitrypsin deficiency

Early phase of asthma

IgE mediated activation of mast cells (IgE induced by IL4)

Late phase of asthma

Inflammation (secretion of major basic protein that perpetuates bronchoconstriction)

Crystalline aggregates of eosinophil major basic protein

Charcor leyden crystals

FEV1: FVC ratio of restrictive lung disease

Increase >80% (more decreased FVC: decreased FEV1)

FEV1: FVC ratio of obstructive lung disease

Decrease <80% (decreased FVC: more decreased FEV1)

Pathogenesis of idiopathoc pulmo fibrosis

Injured pneumocytes secrete TGF B which induces fibrosis

Pneumoconiosis that increases risk for TB

Silicosis

Ferroginous bodies

Asbestosis

Asteroid body

Sarcoidosis

Why noncaseating granulomas produce hypercalcemia

Secretes 1 alpha hydroxylase activity (converts vitamin D)

Normal mean pulmonary arterial pressure

10mmHg

mean pulmonary arterial pressure in pulmonary hypertension

>25mmHg

Mutation asso with primary pulmonary hypertension

BMPR2 (which leads to proliferation of vascular smooth muscle)

Hallmark of ARDS

formation of hyaline membrane inside the alveoli

Pathogenesis of ARDS

Activation of neutrophils induces protease mediated and free radical damage of type I and II pneumocytes

Major component of lung surfactant

Phosphatyl choline

Normal LS ratio

>2

Insulin is a lung surfactant (stimulant or inhibitor?)

Inhibitor (that is why maternal diabetes cause neonatal RDS)

Asso with keratin pearls or intercellular bridges

Squamous cell lung CA

Asso with Eaton- Lambert syndrome

Small cell lung CA

Bronchioloalveolar CA arises from what cells

Clara cells

Asso with Clara cells

Bronchioloalveolar CA

Asso with chromogranin + lung tumor

Carcinoid tumor (well differentiated neuroendocrine cells)

Most common source of metastatic cancer to the lung

Breast and colon CA

Multiple cannon ball nodules of the lung

Metastatic tumor of the lung

Compression of the sympathetic chain causes ptosis, pinpoint pupils and anhidrosis

Pancoast tumor

Recurrent apthous ulcer, genital ulcer and uveitis

Behcet syndrome

Hairy leukoplakia is asso with what virus

EBV

Most common tumor of the salivary gland

Pleomorphic adenoma

Most common site of Pleomorphic adenoma

Parotid gland

2nd most common tumor of the salivary gland

Warthin tumor

Benign cystic tumor of the salivary gland with abundant lymphocytes and germinal centers

Warthin tumor

Painful hematemesis

Mallory weiss syndrome

Painless hematemesis

Esophageal varices

Esophageal rupture

Boerhave syndrome

Tumor of the upper 1/3 of esophagus metastasize to what lymph node

Cervical nodes

Tumor of the middle 1/3 of esophagus metastasize to what lymph node

Mediastinal or tracheobronchial nodes

Tumor of the lower 1/3 of esophagus metastasize to what lymph node

Celiac and gastric nodes

Persistent herniation of the bowel into the umbilical cord, abdominal contents covered by peritoneum and amnion of umbilical cord

Omphalocele

Congenital malformation of the abdominal wall

Gastroschisis

Gastritis caused by severe burn

Curling ulcer

Gastritis caused by increased intracranial pressure

Cushing ulcer

Pathogenesis of Cushing ulcer

Inc ICP -> inc vagal stimulation -> -inc Ach > Ach bind to parietal cell -> inc acid production

Most common site fof H pylori gastritis

Antrum

3 receptors in parietal cells that help regulate the amount of acid produced

Ach receptor


Gastrin receptor


Histamine receptor

Part of the stomach where parietal cells are located

Body and fundus

Part of the stomach where parietal cells are located

Body and fundus

Most common site of peptic ulcer disease

Proximal duodenum

Site of peptic ulcer that usually leads to rupture of gastroduodenal artery or acute pancreatitis

Posterior ulcer

Epigastric pain that worsens with meals is what type of ulcer

Gastric ulcer (duodenal ulcer - improves with meals)

Gastric ulcer is usually located on the

lesser curvature of the antrum

Rupture of gastric ulcer carries risk of bleeding from what artery

Left gastric artery (because runs along lesser curvature of the stomach)

Characteristics of bening ulcer

Small


Punched out hole


Margins is normal, no piling up of mucosa at the edge of ulcer

Type of gastric carcinoma that usually presents as large irregular ulcer with heaped up margins

Intestinal type

Type of gastric carcinoma that most commonly involves the lesser curvature of the stomach

Intestinal type

Type of gastric carcinoma that is asso with signet ring cells tagt diffusely infiltrate the gastric wall

Diffuse type

Desmoplasia results in thickening of the stomach wall called

Linitis plastica

Presence of seborrheic keratosis in the skin asso with gastric carcinoma

Leser trelat sign

Type of gastric carcinoma that metastasizes to the periumbilical region?

Sister mary joseph nodule in intestinal type

Type of gastric carcinoma that metastasizes to the bilateral ovaries and it is also called

Krukenberg tumor seen in diffuse type

Duodenal atresia is asso with what syndrome

Down syndrome ("Duodenal - Down")

Double bubble sign

Duodenal atresia ("DUOdenal - DOUble bubble")

True diverticulum

Meckel diverticulum

Outpouching of all three layers of the bowel wall

Meckel diverticulum

Arises due failure of vitelline duct to involute

Meckel diverticulum

Most common congenital anomaly of the GI tract

Meckel diverticulum

Meckel diverticulum is usually located within __ feet from the ileocecal valve

2 feet

Most common location of volvulus in elderly

Sigmoid colon

Most common location of volvulus in yoing adults

Cecum

Most common cause of intussiception in children

Lymphoid hyperplasia (in terminal ileum to cecum)

Most common cause of intussiception in adults

Tumor

Transmural infarction of the intestine is usually due thrombosis or embolus to what 2 structures

SMA and mesenteric vein

Immune mediated damage of the small bowel villi due to gluten exposure

Celiac disease

HLA asso with celiac disease

HLA DQ2, DQ8

Most pathogenic component of gluten

Gliadin

Pathogenesis of Celiac disease

Gluten has gliadin -> gliadin deaminated by tissue transglutaminase -> deaminated gliadin presented by APCs via MHC2 -> helper Tcells mediate tissue damage

Asso with IgA deposition at the tips od dermal papillae in Celiac disease

Dermatitis herpetiformis (resolves with gluten free diet)

Asso with flattening of the villi, hyperplasia of intestinal crypts and increased intraepithelial lymphocytes

Celiac disease

Celiac disease most commonly involves damage in what part of GIT

Duodenum

Same as celiac disease except it occurs in tropical regions and arises after infectious diarrhea and responds to antibiotic

Tropical sprue

Trooical sprue the damage is more commonly located in whar part of GIT

Jejunum and ileum (compared to duodenum in celiac disease)

Systemic tissue damage characterized by fat malabsorption and steatorrhea

Whipples disease

Classic site of involvement in whipples disease

Lamina propria of small bowel

Deficiency of Apo B48 and B100

Abetalipoprotenemia

Deficiency of Apo B48 and B100

Abetalipoprotenemia

Carcinoid tumor secretes what hormone

Serotonin

Bronchospasm, diarrhea, flushing of the skin

Carcinoid syndrome

Carcinoid tumor that metastasize to the liver results in

Carcinoid syndrome (increase serotonin in the circulation causing bronchospasm, (carcinoid synd), diarrhea, flushing ("BCDF")

If serotonin secreted by carcinoid tumor of liver passes the right atrium, what heart defects will it cause

Tricuspid regurg, pulmonary stenosis (right sided valvular fibrosis)

Key histologic hallmark of ulcerative colitis

Crypt abscesses with neutrophils

Ulcerative colitis UC or chrons disease CD: mucosal and submucosal ulcers

UC

Ulcerative colitis UC or chrons disease CD: full thickness inflammation

CD

Ulcerative colitis UC or chrons disease CD: begins in rectum and extend only up to cecum (small bowel unaffected)

UC

Ulcerative colitis UC or chrons disease CD: "continuous involvement of the bowel"

UC

Ulcerative colitis UC or chrons disease CD: crypt abscess

UC

Ulcerative colitis UC or chrons disease CD: loss of haustra "lead pipe" sign on imaging

UC

Ulcerative colitis UC or chrons disease CD: presence of pseudopolyps

UC

Ulcerative colitis UC or chrons disease CD: complications could lead to toxic mega colon

UC

Ulcerative colitis UC or chrons disease CD: associated with primary sclerosing cholangitis

UC

Ulcerative colitis UC or chrons disease CD: associated with p-ANCA

UC

Associated with p-ANCA (3)

Churgs strauss, ulcerative colitis and microscopic polyangitis

Ulcerative colitis UC or chrons disease CD: smoking is protective

UC

Ulcerative colitis UC or chrons disease CD: skipped lesions

CD

Ulcerative colitis UC or chrons disease CD: anywhere from mouth to anus

CD

Most common site of chrons disease in the intestine

Terminal ileum

Ulcerative colitis UC or chrons disease CD: left lower quadrant pain (rectum) with bloody diarrhea

UC

Ulcerative colitis UC or chrons disease CD: right lower quadrant pain (ileum) with non bloody diarrhea

CD

Ulcerative colitis UC or chrons disease CD: hsitologic hallmark is lymphoid aggregates with granulomas

CD

hsitologic hallmark of chrons disease

lymphoid aggregates with granulomas

Ulcerative colitis UC or chrons disease CD: cobble stone appearance of mucosa, creeping fat and strictures

CD

Ulcerative colitis UC or chrons disease CD: associated with ankylosing spondylitis, migratory polyarthritis, erythema nodosum

CD

Ulcerative colitis UC or chrons disease CD: smoking increases the risk

CD

Weak point in the colonic wall where colonic diverticula arises

Where vasa recta traverses the muscularis propria

Most common location of colonic diverticula

Sigmoid colon

Most common cause of ischemic colitis

Atherosclerosis of SMA

Most common site of ischemic colitis

Splenic flexure

Familial adenomatous polyposis with fibromatosis and osteomas

Gardner syndrome

Familial adenomatous polyposis asso with CNS tumors (medulloblastoma and glial tumors)

Turcot syndrome

Hamartomatous polyps throughout GI tract with mucocutaneuos hyperpigmentation of lips, oral mucosa and genital skin

Peutz Jeghers syndrome

3 carcinomas that HNPCC are at increase risks

Colorectal, ovarian, endometrial CA

Premature activation of this enzyme leads to activation of other enzymes in acute pancreatitis

Trypsin

2 most common cause of acute pancreatitis

Alcohol and gall stones

2 most common cause of chronic pancreatitis

Alcohol and cystic fibrosis

2 major risk factors for pancreatic carcinoma

Smoking and chronic pancreatitis

Why is estrogen a risk factor for gall stones

Estrogen increase activity of HMG CoA reductase -> increase cholesterol synthesis


Estrogen also increases lipoprotein receptors in the liver -> increasing cholesterol uptake

Rokitansky aschoff sinus

Chronic cholecystitis

Late complication of chronic cholecystitis

Porcelain gall bladder

Virus causing fulminant hepatitis in pregnant women (liver failure with massive liver necrosis)

HEV

Liver fibrosis is mediated by what cell which secretes TGF B

Stellate cells

Liver cirrhosis causes inefficient clearing of estrogen causing which symptoms of liver cirrhosis

Gynecomastia, palmar erythema, apider angiomata

Mallory body is asso with

Alcoholic hepatitis

Damaged intermediate filaments within the hepatocytes found in alcoholic hepatitis

Mallory bodies

Painful hepatomegaly, elevated liver enzymes (AST> ALT)

Alcoholic hepatitis (since alcohol is a mitondrial poison and AST is found in mitochondira)

Mutations in HFE gene most commonly C282Y causes

Primary hemochromatosis

How to distinguish brown pigment in hepatocytes as iron or lipofuscin

Prussian blue stain (iron will turn blue)

AR defect in ATP7B gene in ATP mediated hepatocyte copper transport

Wilson disease

Treatment for wilsons disease which is a copper chelating agent

D penicillamine

Autoimmune granulomatous destruction of intraheptic bile ducts

Primary biliary cirrhosis

Asso with antimitochondrial antibody

Primary biliary cirrhosis

Inflammation and fibrosis of intraheptic amd extrahepatic bile ducts

Primary sclerosing cholangitis (unlike primary biliary cirrhosis - intrahepatic only)

Periductal fibrosis with onion skin appearance

Primary sclerosing cholangitis

Fulminant lievr failure and encephalopathy in children with viral illness who take aspirin

Reye syndrome (mitochondrial damage)

Most common congenital renal anomaly

Horshoe kidney

Renal disease asso with berey aneurysm, hepatic cysts, mitral valve prolapse

ADPCKD

Why does glomerular disease cause hypercoagulable state

Decrease absorption of anti thrombin 3

Most common nephrotic syndrome in children

Minimal change disease

Nephrotic syndrome asso with Hodgkin lymphoma

Minimal change disease

Effacement of foot processes on EM

Minimal change disease

Selective proteinuria (loss of albumin but not Ig)

Minimal change disease

Only nephrotic syndrome with excellent response to steroids

Minimal change disease

Nephrotic syndrome asso with HIV, heroin use, sickle cell disease

Focal segmental glomerulosclerosis

Nephrotic syndrome asso with HBV, HCV, solid tumors, SLE, NSAIDs

Membranous Nephropathy

Subendothelial deposits with spike and dome appearance on EM

Membranous Nephropathy

Any nephropathy with "membranous" has (positive/negative) granular immunoflorescence

Positive since membranous = immune deposition

Thick capillary membrane with Tram Track appearance, granular immunofluorescence

Membranoproliferative GN

Type of Membranoproliferative GN asso with subendothelial immune deposits, HBV and HCV, most likely causong Tram Track appearance

Type 1 MPGN

Type of Membranoproliferative GN asso with intramembranous immune deposits and C3 nephritic factor (which stabilizes c3 convertase preventing degradation)

Type 2

Part of glomerulus that is preferentially affected in diabetic hyaline arteriolosclerosis

Efferent arteriole (constriction of EA results in increase glomerular filtration pressure, causing nephrotic syndrome)

Antihypertensive that decreases constriction of efferent arterioles in diabetic hyaline arteriolosclerosis

ACE inhibitors (decreases angiotensin 2 which constricts EA)

Sclerosis of the mesangium in diabetic nephropathy

Kimmelstiel Wilson nodules

Most commonly affected organ in systemic amyloidosis

Kidney

Apple green bifringence of mesangum deposits under polarized light

Systemic amyloidosis nephropathy

Hallamark of nephritic syndrome

Glomerular inflammation and bleeding

Virulence factor of S pyogenes causing PSGN

M protein

Asso with subepithelial hump on EM

PSGN

Crescents in RPGN is composed of

Fibrin and macrophages

Most common nephritic renal disease in SLE

Diffuse proliferative GN

Nephritic syndrome asso with linear immunoflourescence

Good pasteur syndrome

Nephritic syndrome asso with granular immunoflourescence

PSGN or Diffuse PGN

Nephritic syndrome asso with negative immunoflourescence

Wegener granulomatosis, microscopic polyangitis, churg strauss

Episodic hematuria with RBC casts usually following mucosal infections

IgA Nephropathy

Defect in Type 4 collagen, Thinning and spliting of glomerular basement membrane

Alport syndrome

Isolated hematuria, sensory hearng loss, ocular disturbances

Alport syndrome

Cortical scarring of the kidneys with blunted calyces

Chronic pyelonephritis

Scarring of upper and lower poles of thr kidney

Vesicoureteral reflux

Thyroidization of the kidney

Chronic pyelonephritis

Colicky pain with hematuria and unilateral flank tenderness

Nephrolithiasis

Type of stone causing staghorn calculus

Ammonium magnesium phosphate stone

Cause if staghorn calculus in children

Cysteinuria

Epo is produced in

Renal peritubular interstitial cells

Angiomyolipoma has increased frequency in patients with

Tuberous sclerosis

Hematuria, palpable mass and flank pain

Renal cell CA

Most common subtype of renal cell CA

clear cell type

Pathogenesis of Renal Cell CA is loss of what gene

VHL (3P) tumor suppressor gene

Increased risk for renal cell CA and hemangioblastoma

Von Hippel Lindau disease

Von Hippel Lindau disease is associated with (2 tumors)

renal cell CA and hemangioblastoma

Renal cell CA metastasizes to what lymph nodes

Retroperitoneal lymph nodes

Staging of T in renal cell CA is based in tumor size and imvolvement of what structure

Renal vein

Hallmark cell of Wilms tumor

Blastema (which produces primitive glomeruli, tubules and stromal cells)

What is WAGR syndrome

Wilms tumor


Aniridia


Genital abnormalities


Retardation (mental and motor)

Asso with wilms tumor, neonatal hypoglycemia, muscular hemihypertrophy, organomegaly

Beckwith Wiedemann syndrome

Major risk factor for urothelial CA

Smoking

Koilocytes

HPV

Thinning of the epideemis and fobrosis of the dermis of the vulva, post menauposal, leukoplakia with parchment like vulvar skin

Lichen sclerosis

Opposite of lichen sclerosis

Lichen simplex chronicus

Hyperplasia of vulvar epithelium, leukoplakia with thick leathery vulvar skin

Lichen simplex chronicus

Paget Disease or Melanoma: PAS +, Keratin +, S100 -

Paget disease

Paget Disease or Melanoma: PAS -, Keratin -, S100 +

Melanoma

Focal persistence of columnar epithelium in upper 1/3 of vagina

Adenosis

Malignant proliferation of glands in vagina with clear cytoplasm

Clear cell adenoCA

Cell with characteristic cytoplasmic cross striations

Rhabdomyoblast (in rhabdomyosarcoma)

Cell positive IHC staining for desmin and myoglobin

Rhabdomyoblast

Cancer of the lower 2/3 of vagina metastasize to what lymph nodes

Inguinal lymph nodes

Cancer of the upper 1/3 of vagina metastasize to what lymph nodes

Regional iliac lymph nodes

Proteins in high risk HPV that increases destruction of p53

E6

Proteins in high risk HPV that increases destruction of Rb gene

E7

Secondary amenorrhea due to loss of basalis (regenerative) layer of endometrium as a result of over aggressive D&C

Asherman syndrome

Fever, abdominal uterine bleeding, pelvic pain

Acute endometritis

Chracteristic cell in chronic endometritis

Plasma cell

Side effect of this drug causes endometrial polyp

Tamoxifen

Presence of endometriosis in the myometrium

Adenomyosis

Endometrial hyperplasia is a consequence of

Unopposed estrogen

Most important predictor for progression to endometrial CA

Cellular atypia

Polycystic ovarian syndrome is characterized by inc (FSH/LH) and dec (FSH/LH)

Increase LH


Decrease FSH


LH : FSH >2

Mutation of this gene has increased risk for serous carcinoma of ovary and fallopian tube

BRCA1 mutation (also causes breast CA)

Ovarian tumor that has cells that resemble urothelial cells

Brenner tumor

Ovarian tumor usually occur in women of reproductive age

Germ cell ovarian tumors

Most common germ cell tumor in females

Cystic teratoma

Cystic teratoma compose primarily of thyroid tissue

Stroma ovarii

Most common malignant germ cell tumor

Dysgerminoma

Ovarian tumor composed of large cells with clear cytoplasm and central nuclei

Dysgerminoma

What enzyme may be elevated in dysgerminoma

LDH (LDysgerminomaH)

Malignant tumor that mimics the yolk sac

Endodermal sinus tumor

Most common germ cell tumor in children

Endodermal sinus tumor

Schiller Duval bodies

Endodermal sinus tumor (glomeruloid structure)

Ovatian tumor composed of trophoblast and syncytiotrophobalst (villi are absent)

Choriocarcinoma - poor response to chemotherapy

Ovatian tumor composed of large primitive cells

Embryonal CA

Sex cord stromal ovarian tumor with signs of estrogen excess

Granulosa theca cell tumor

Reinke crystal

Leydig cells

Ovarian tumor that is fibroma, pleural effusion, ascites

Meigs syndrome

Necrotizing granulomatous inflammation of inguinal lymphatics and lymph nodes

Lymphogranuloma venereum (Chlamydia trachomatis)

Sub type of C trachomatis causing LGV

L1-L3

Most common congenital male repro abnormality

Cryptoorchidism

Scrotal swelling with "bag of worms" appearance

Varicocele

Varicocele is usually affected in what side

Left due to the connection to the ledt renal vein

Fluid collection within the tunica vaginalis of the scrotum

Hydrocele

Most common type of testicular tumor

Germ cell tumor

2 risk factors for testicular germ cell tumor

Cryptoorchidism and klinefelter syndrome

Most common testicular tumor in children

Yolk sac tumor

Schiller duval bodies

Yolk sac tumor

B hCG is produced

Synctiotrophoblast

Teratoma in females is (benign/malignant)

Benign

Teratoma in males is (benign/malignant)

Malignant

Most common cause of testicular mass in males >60 and often bilateral

Lymphoma usually diffuse large B cell type

BPH occurs in what psrt of prostate

Periurethral zone of prostate

Prolactin inhibits synthesis and release of which hormone

GnRH (causing amenorrhea, dec libido)

Prolactin is inhibited by what hormone

Dopamine (prolactinoma can be treated with dopamine agonist)

How will octreotide treat growth hormone adenoma

Inhib response of anterior pituitary to GHRH

Drug that would differentiate central from nephrogenic DI

Desmopressin (an ADH analog improve in central but not in nephrogenic)

Tumor that can produce ectopic ADH

small cell lung CA

Drug that blocks effect of ADH

Demeclocycline

Cause of increase BMR in hyperthyroidism

Increases synthesis of Na K ATPase

Thyroid hormone increases sympathetic nervous system by

Expression of B1 adrenergic receptors

HLA asso with hashimoto thyroiditis

HLA DR5

Anti microsomal antibodies

Hashimoto thyroiditis

Anti thyroglobulin antibody

Hashimoto thyroiditis

Hurtle cells

Hashimoto thyroiditis

Granulomatous thyroiditis following viral infection

Subacute (deQuervain) granulomatous thyroiditis

Chronic inflammation with extensive fibrosis of thyroid "hard as wood" nontender thyroid

Reidel fibrosing thyroiditis

Most common type of thyroid carcinoma

Papillary carcinoma of thyroid

Major risk factor for apillary carcinoma of thyroid

Exposure to ionizing radiation in childhood

Orphan annie nuclei cells

Papillary carcinoma of thyroid

Malignant proliferation of parafollicular C cells

Medullary CA

Mutation in Medullary carcinoma of thyroid

RET oncogene

Most common cause of primary hyperPTH

Pararhyroid adenoma

Non ezymatic glycation of large and medium sized vessels results to

Atherosclerosis

Cortisol upregulates what receptors in arterioles

A1 (causing HTN)

3 actions of cortisol in immunodeficiency

Dec Phos A2 (no arachidonic acid metabolites)


Inhib IL2 (no more T cell growth factor)


Inhib histamine release in mast cell

What tumor causes paraneoplastic ACTH secretion

Small cell lung CA

Most common cause of congenital adrenal hyperplasia

21 B hydroxylase def (which is needed to make aldosterone and cortisol so decrease of the 2 hormones and instead more sex steroids)

Enzyme deficiency with inc aldosterone but dec cortisol and sex steroids

17 hydroxylase (since this is needed in cortisol and sex steroid synthesis but not needed for aldosterone)

Enzyme deficiency with dec aldosterone and cortisol but inc sex steroids

21 B hydroxylase (which is needed to make aldosterone and cortisol so decrease of the 2 hormones and instead more sex steroids)

Functional unit of breast

Terminal duct-lobular unit

Ducts and lobules of the breasts are lined by what epithelium

Luminal cell layer


Myoepithelial layer

Most dense part of the breast

Upper outer quadrant of breast

Most common cause of acute mastitis

S aureus

Inflammation of the subareolar ducts, seen in smokers, subareolsr mass woth nipple retraction

Periductal mastitis

Inflammation with dilatation (ectasia) of subareolar ducts, green brown discharge

Mammary duct ectasia

How is obesity a risk factor for breast cancer

Adipose tissue converts androgen to Estrone (estrogen inc risk of breast cancer)

Type of invasive ductal CA of breast with blockage of the dermal lymphatics by cancer

Inflammatory CA

Good prognosis of the types of invasìve ductal breast CA

Tubular CA


Mucinous CA

Poor prognosis of the types of invasìve ductal breast CA

Medullary CA


Inflammatory CA

Subtype of invasive ductal CA asso with BRCA 1 mutation

Medullary CA

Subtype of invasive ductal CA asso with high grade malignant tumor cells with inflammatory cells in the background (lymphocytes, plasma cells)

Medullary CA

Malignant tumor of breast usually incidentally discovered and does not produce mass or calcification, often multifocal and bilateral

Lobular CA insitu

Malignant tumor of breast asso with dyscohesive cells lacking E cadherin

Lobular CA insitu

Her 2/neu amplification is associated with response to what drug

Trastuzumab

Her 2/nei is a receptor that is located in what part of the cell

Cytoplasm

BRCA mutation asso with breast and ovarian CA

BRCA1

BRCA mutation asso with breast CA in males

BRCA2

Congenital extension of cerebellar tonsils through the foramen magnum

Arnold-Chiari malformation

Syringomelia usually occurs what part of spinal cord

C8-T1 anterior horn

Part of the spinal cord affected in poliomyelitis

Anterior horn (where neurons to the muscle are located causing lower motor weakness

Inherited degeneration of anterior motor horn, presents as "floppy baby"

Werdnig Hoffman disease

Part of the spinal cord involved in amyotrophic lateral sclerosis

Anterior motor horn (LMN signs), lateral corticospinal tract (UMN signs)

Difference between ALS and syringomyelia

Lack of sensory impairment (+ in ALS, - in syringomyelia)

Disease caused by unstable trinucleotide repeat (GAA) in frataxin gene

Friedreich ataxia

Most common site of intracerebral hemorrhage

Basal ganglia

Most frequent location of berry aneurysm

Anterior circle of willis (branch of ACOM)

Berry aneurysm lacks what layer of the blood vesel

Media layer

Berry aneurysm is asso with (2 syndromes)

Marfan and ADPCKD

Round aggregates of tau protein in neurons of cortex

Pick disease

Pick disease is a degenerative disease of which part of the brain

Frontal and Temporal (spares occipital and parietal)

Huntington disease is the degeneration of GABAergic neurons in

Caudate nucleus of basal ganglia

Expanded trinucleotide repeat (CAG) in huntingtin gene

Huntington disease

Triad of urinary incontinence, gait instability and dementia (Wet, Wacky, Wabble)

Normal pressure hydrocephalus (due to stretching of corona radiata)

Most common metastasis to the CNS

Lung, breast, kidney CA

Malignant high grade tumor of astrocytes

Glioblastoma multiforme

Butterfly glioma

Glioblastoma multiforme (crosses the corpus callosum)

Benign tumor of the arachnoid cells

Meningioma

Tumor that arises usu from CN8 at the cerebellopontine angle (CPA)

Schwannoma

Schwannoma is positive for what marker

S100

Fried egg appearance of cells

Oligodendroglioma

Calcified tumor of the white matter usu involving frontal lobe

Oligodendroglioma

Most common CNS tumor in children

Pilocytic astrocytoma (usu in cerebellum)

Rosenthal fibers

Pilocytic astrocytoma

Malignant tumor from granular cells of cerebellum

Medulloblastoma

Small round blue cells, Homer Wright rosettes

Medulloblastoma

Perivascular pseudorosette

Ependymoma

Tumor from epithelial remnants of Rathke's pouch

Craniopharyngioma

Bitemporal hemianopsia in child

Craniopharyngioma

Activating mutation in FGFR3 (Fibroblast growth factor receptor 3), AD

Achondroplasia

Achondroplasia is due to a mutation in

FGFR3 (Fibroblast growth factor receptor 3),

Cause of blue sclera in Osteogenesis imperfecta

Reflection of choroidal veins

Enzyme usu mutated in osteopetrosis

Carbonic anhydrase II mutation (dec CA cause decrease H+ cause decrease acidic environment cause decrease resorption of bone)

Pigeon breast deformity, frontal bossing, rachitic rosary, bowing of legs

Rickets

Most common seeding side of osteomyelitis in children

Metaphysis

Most common seeding side of osteomyelitis in adults

Epiphysis

Causative bacteria of osteomyelitis in general

S aureus

Causative bacteria of osteomyelitis in sexually active

N gonorrhea

Causative bacteria of osteomyelitis in sickle cell anemia

Salmonella

Causative bacteria of osteomyelitis in diabetic or IV drug user

Pseudomonas

Lytic focus sorrounded by sclerosis on xray of bone

Osteomyelitis

Bone pain that resolves with aspirin

Osteoid osteoma

Usual site of osteoblastoma

Vertebrae

Usual site of osteoid osteoma

Cortex of diaphysis

Most common benign tumor of bone

Osteochondroma

Risk factor of osteosarcoma

Rb, Paget disease of bone, radiation exposure

Usual site of osteosarcoma

Metaphysis of long bones (usu distal femur or proximal tibia)

Usual site of giant cell tumor of bone

Epiphysis of long bones (distal femur or proximal tibia)

Soap bubble appearance on bone xray

Giant cell tumor

Malignant proliferation of poorly differentiated cells derived from neuroectoderm

Ewing sarcoma

Usual site of Ewing sarcoma

Diaphysis of long bones (male children)

Gene mutation in Ewing sarcoma

t(11:22)

Metastatic tumor causing osteoblastic lesions in bone

Prostate CA (sclerosis of bone instead of punched out lesions)

Chondroma is usually found in

Medulla of small bones of hands and feet

Chondrosarcoma usually found in

Cortex of axial bones

Hallmark of Rheumatoid arthritis

Synovitis leading to formation of pannus (inflammed granulation tissue)

Sausage finger or sausage toe (arthritis of DIP of hands and feet)

Psoriatic arthritis

Sausage finger or sausage toe (arthritis of DIP of hands and feet)

Psoriatic arthritis

Most common cause of infectious arthritis over all

N gonorrhea (usu young adults)


S aureus is second most common

Needle shaped crystals with negative birefringence under polarized light

Uric acid crystals

Deposition of calcium pyrophosphate

Pseudogout

Rhomboid shaped crystals with weak positive birefringence under polarized light

Paeudo gout

Parallel on polarized light is color

YeLLow (paraLLel)

Heliotrope rash

Dermatomyositis (rash upper eyelids)

Anti-Jo1 antibody

Dermatomyositis

Perimysial inflammation with perifascicular atrophy

Dermatomyositis

Endomysial inflammation with necrotic muscle fibers

Polymyositis

Replacement of skeletal muscle by adipose tissue

X linked muscular dystrophy

Calf pseudohypertrophy

X linked muscular dystrophy

Deletion of dystrophin

Duchene muscular dystrophy (Duchene=Deletion)

Mutation of dystrophin

Becker muscular distrophy

Amtibodies against presynptic calcium channels in NMJ, impairs release of Ach

Lamber-Eaton syndrome

Most common malignant soft tissue tumor in children

Rhabdomyosarcoma

Marker for rhabdomyosarcoma

Desmin positive

Most common site of rhabdomyosarcoma

Head and neck