HBT and the Pancreas Flashcards
Which drug characteristically causes valvular plaques usual causing MR?
Fenfluramine + phentermine (fen-phen.
Plaque is similar to that caused by carcinoid heart disease and methysergide / ergot therapy.
Nonbacterial thromboembolis is increased in underlying malignancy particularly this histologic type
Mucinous adenocarcinoma
Hypertrophic cardiomyopathy is associated with ventricular obstruction. The obstruction is dynamic caused by which leaflet of which valve.
Anterior leaflet, MV
Most commonly mutated gene in hypertrophic cardiomyopathy affects synthesis of which protein.
B-myosin heavy chain
Most common form of restrictive cardiomyopathy worldwide
Endomyocardial fibrosis
Form of endomyocarditis associated with peripheral hyper eosinophilia, large mural thrombi a d release of major basic protein
Loeffler endomyocarditis
Most common helminth with associated cardiac involvement
Trichinosis
Most common tumor metastatic to the heart
Lung Ca
Most common location of the most common primary tumor of the heart
Myxoma of the left atrium
Cold agglutinins develop in which infections
Mycoplasma pneumonia
Infectious mono
Single most important long-term limitation for cardiac transplantation
Graft coronary arteriosclerosis
The earliest change in peripheral blood of patients with megaloblastic anemias
Hyper segmented neutrophils
When do you see monocytosis?
Chronic infections Bacterial endocarditis Rickettsiosis and malaria Collagen vascular diseases Inflammatory bowel diseases
When do you see basophilic Leukocytosis?
Rare! But may be seen in myeloproliferative disease (CML)
Acute bacterial infection that causes lymphocytosis
Bordetella pertussis
Causes of follicular hyperplasia
Rheumatoid arthritis
Toxoplasmosis
Early stage HIV
ALL is also known as ____.CLL is also known as ____.
ALL = precursor B-cell and T-cell lymphoblastic leukemia
CLL = small lymphocytic leukemia
Staining characteristics of ALL vs AML
ALL is Periodic acid Schiff positive.
AML is Periodic acid Schiff negative
Lymphoid Neoplasm Quiz!Most common in adolescent males.Thymic involvement, mediastinal mass.NOTCH1
Precursor T cell lymphoma
Aka T-cell ALL
Lymphoid Neoplasm Quiz!85% of childhood acute leukemia
Precursor B-cell lymphoblastic leukemia aka
B-cell ALL
Splenomegaly is greatest in which hemolytic condition
Hereditary spherocytosis
Lymphoid Neoplasm Quiz!30% of all leukemiasOccurs in older adults
Chronic lymphocytic lymphoma
Lymphoid Neoplasm Quiz!Small “cleaved” cells mixed with large cells.t(14;18).CD10+, BCL2+
Follicular lymphoma
Lymphoid Neoplasm Quiz!CD+ mature B cells that express cyclin D1 and have surface Ig.t(11;14).Moderately aggressive.
Mantle cell lymphoma
B cells home to epithelium creating lymphoepithelial lesions.CD5-, CD10-.Cured by local excision.Association with autoimmune conditions.H pylori associated, associated with MALT
Extra nodal marginal zone lymphoma
Intermediate sized round cells with several nucleoli; diffuse tissue involvement with apoptosis (“starry sky”).CD10+.Endemic in Africa.Visceral involvement.
Burkitt lymphoma
40-50% of adult lymphomasMature B cellsAggressive; often arise at extra nod areas
Diffuse large B-cell lymphoma
Lymphoid Neoplasm Quiz!Most common lymphoid neoplasm in adults.Disseminated bone disease with lytic lesions
Plasmacytoma aka plasma cell myeloma
Lymphoid Neoplasm Quiz!Most common cutaneous lymphoid malignancy.Lymphoid cells with convoluted nuclei infiltrating the epidermis (Pautrier microabscess).CD4+ mature T-cells.Association with Sezary syndrome.
Mycosis fungoides
Most common adult T-cell lymphoma.Mature T-cell phenotype.
Peripheral T-cell lymphoma, NOS
Most common type of Hodgkin lymphoma.Adolescent males
HL, nodular sclerosis type
Most common plasma cell dyscrasia
Monoclonal gammopathy of undetermined significance
Lesions in multiple myeloma are most commonly found in which bone.
Vertebral column
The single most common genetic abnormality in myeloproliferative disorders other than CML (PCV, essential thrombocytemia, myelofibrosis)2
JAK2 kinase
What is Hand-Schuller-Christian triad?
Calvarial bone defects
Diabetes insipidus
Exophthalmos
Seen in multifocal Langerhans cell histiocytosis
Thromboplastic substances are released by:
Placenta in obstetric complications.
Cytoplasmic granules of acute promyelocytic leukemia.
Mucin-secreting adenocarcinoma cells.
Drugs that can cause selective thrombocytopenia
Alcohol
Thiazides
Cytotoxic drugs
Infections that can cause thrombocytopenia
Measles
HIV
CML characterized by which chromosomal translocation
(9;22) translocation moving ABL from chromosome 9 to chromosome 22
Useful bioassay for vWF
Ristocetin-dependent platelet agglutination
What are Curschmann spirals? Charcot-Leyden crystals? They are seen in which condition.
Curschmann spirals - whirls of shed epithelium seen in mucus plugs
Charcot-Leyden crystals - crystalloids made of eosinophils proteinsIn asthma
Drugs that cause pneumonitis and fibrosis
Bleomycin
Amiodarone
‘May well be’ the most common inherited bleeding disorder
Von willebrand disease
Causes of noncaseating granulomas
Mycobacterial infections
Fungal infections
Berylliosis
Sarcoidosis
This pulmonary disease has a higher prevalence among nonsmokers!
Sarcoidosis
Most pulmonary emboli arise from thrombi within ___
Deep veins of lower legs
Most common bacterial cause of acute exacerbation
Haemophilus influenzae
Complications much more likely with pneumococci serotype ___
3
Most common cause of gram negative bacterial pneumonia
Klebsiella pneumonia
Most common cause of death resulting from a single infectious agent. It causes death in ___% of a deaths worldwide.
TB!6%
How many TB bacilli are required for smear positivity? Pcr?Gold standard for TB diagnosis
10,000 organisms.
10 organisms.
TB culture
- Most common opportunistic viral pathogen in AIDS2. Most common cause of a CNS lesion
- CMV
2. Toxoplasma
How many % of certain African and Asian countries are tuberculin positive?
80
Most common form of opportunistic CMV disease
Retinitis
SCLC is characterized by which gene mutation. NSLCs? Lung adenocarcinoma?
SCLC: RB
NSLC: p16/CDKN2A
Adenocarcinoma: KRAS and EGFR
Risk for lung CA is how many times increased for heavy smokers? In passive smoking?
60x.
2x.
Causes of cornea verticillata
- Fabry’s disease (a-galactosidase deficiency causing accumulation globotriaosylceramide)
- Amiodarone
‘Soap bubble lesions’ in the brain are seen in which infection
Cryptococcosis. Due to expansion of perivascular Virchow-Robin spaces
Experimental model of membranous nephritis
Heymann nephritis.
Induced in animals by immunization with renal tubular brush border proteins
HIstologic type of heroin nephropathy and HIV nephropathy
FSGS
Most characteristic change in post infectious GN under light microscopy
Uniformly increased cellularity of glomerular tufts
Drugs that cause interstitial non necrotizing granulomas in kidneys
Thiazides
Methicillin
Rifampin
Aside from Berger’s disease, name another disease characterized by IgA deposition in the mesangium.
HSP. However this is a systemic syndrome involving skin, GI, joints and kidneys.
Pathophysiology of Cushing ulcers
Increased ICP cause direct stimulation of vagus which increased gastric acid secretion
Favored region of gastric carcinomas
Lesser curvature of the antropyloric region
Hirschsprung characterized by mutations in the:
RET gene
2 other viruses that cause diarrhea
Adenovirus (ad40, ad41)
Astrovirus
Most common location of diverticulosis
Sigmoid colon
Major cause of endemic bacillary dysentery in locations of poor hygiene in developing and developed countries
Shigella flexneri
EHEC produces most severe disease in the:
Right colon
Diagnosis of lactose intolerance is made by:
Breath hydrogen tests
Reflects bacterial overgrowth in the presence of excess intraluminal carbohydrate
Major cause of childhood diarrhea
Cryptosporidiosis
Hallmark of Whipple disease
Distended PAS+ macrophages in the lamina propia
Crohn’s disease is most common in:
Terminal ileum
Villous adenomas can produce this electrolyte abnormality
Hypokalemia. (And hypoproteinemia.)
They secrete mucoid material rich in K and protein.
Peutz-Jeghers syndrome is characterized by germ line mutations in this gene which codes for what.
LKB1.
Serine threonine kinase.
PTEN is a tumor suppressor. How does it work?
Encodes a phosphatase.
- Interrupts signals from several tyrosine kinase receptors (used by growth factors)
- Favors apoptosis through BAD/BCL2
NSAIDs may reduce incidence of colorectal Ca. How?
Inhibition of COX-2 (over expressed in 90% of colorectal Ca.)
PGE2 increased by COX2 action favor epithelial cell proliferation, inhibit apoptosis, enhance angiogenesis (by increasing VEGF.)
Although the vast majority of hyper plastic polyps have no malignant potential, ___ which is found in the right side of the colon may be precursors of colorectal Ca.
Sessile serrated adenoma
Earliest event in the formation of adenomas
Loss of the APC tumor suppressor gene
How does APC function as a tumor suppressor gene?
APC increases degradation of beta catenin. The latter can translocation to the nucleus and activate transcription of genes such as MYC and cyclin D1.
What is the antecedent lesion in colorectal Ca developing via the APC/beta-catenin pathway? In the DNA mismatch pathway?
APC: tubular/villous adenomas (leads to differentiated adenocarcinoma in the left colon)
MSI: sessile serrated adenomas (leads to mucinous Ca in the right colon)
How does microsatellite instability lead to colorectal CA?
Some microsatellite sequences are located in coding or promoter regions of type 2 TGFB and BAX.
- TGFB2 inhibits colonic epithelial growth.
- BAX is a pro-apoptotic protein.
How does mutation of K-RAS lead to cancer formation?
Mutated RAS (G protein) is trapped in an activated state (bound to GTP) that delivers mitotic signals and prevents apoptosis.
Most GISTs are characterized by a somatic mutation in the ___ gene which encodes a ____. Drug of choice?
c-KIT aka CD117
Tyrosine kinase receptor
Imatinib mesylate
Most SI adenocarcinoma arise in the:
Duodenum (including the ampulla of Vater)
Appendiceal inflammation may be due to a ball of these worms.
Oxyuriasis vermicularis
Histologic criterion for the diagnosis of appendicitis
Neutrophilic infiltration of the muscularis propia
Cells of the ___ constitute a reserve compartment of progenitor cells for hepatocytes and bile duct cells. What do you call these cells?
Canals of Hering.
Oval cells
What underlying genetic abnormality is seen in clear cell Ca? Papillary renal cell Ca? What do these genes normally encode?
Clear cell CA.
VHL (3p25) encodes protein involved in limiting angiogenic response to hypoxia.
Papillary renal cell CA.
MET (7q31) is a tyrosine kinase receptor for hepatocyte growth factor.
Bilateral complete ureteral obstruction causes anuria. Bilateral partial ureteral obstruction causes ____.
Polyuria!
Due to defects in tubular concentrating mechanisms and this may obscure the true nature of the disturbance.
Feathery degeneration in liver is due to:
Retained biliary material.Diffuse, foamy, swollen hepatocytes.
How does CKD and hyperparathyroidism cause peptic ulcers?
Hypercalcemia stimulates gastrin production and acid secretion
Wilm’s tumor contains a variety of cell and tissue components all derived from ____
Mesoderm
Occupational exposure to ___ increases risk of renal cell Ca.
Cadmium
What is the effect of pH on kidney stone formation?
High pH favors Ca stone, struvite stone formation.
Low pH favors urate, cystine stones
Nephron segments most susceptible to ischemia
Straight portion, PCT
Ascending thick limb
Avitaminosis A can cause renal stones! How?
Desquamated cells from the metaplastic epithelium of the collecting system act as nidi.
Which arteries undergo onion skinning in malignant nephrosclerosis
Interlobular arteries and larger vessels
Most common genetic cause of end-stage renal disease in children and young adults.What is the underlying abnormality?
Nephronophthisis-medullary cystic disease
Mutation in NPHP which encode components of the epithelial cell cilia
The underlying defect in cystic diseases of the kidney is a defect in the:
Cilia centrosome complex
Causes of hepatic dysfunction WITHOUT overt necrosis
Tetracycline toxicity
Reye Syndrome
Acute fatty liver of pregnancy
Major source of excess collagen in cirrhosis are the ___
Stellate cells (formerly the Ito Cells); produce TGF-B
Conversion of heme to bilirubin occurs where
Mononuclear phagocytes
Histologic hallmark of serious liver damage
Deposition of fibrous tissue
Name one antibiotic that causes noncaseating epithelioid granuloma.
Sulfonamide
Liver biopsy reveals numerous blood-filled cavities NOT lined by endothelial cells. What is this condition? What two drugs cause this pattern of hepatic injury?
Peliosis hepatis.
- Anabolic steroids
- Tamoxifen
Two drugs which cause microvesicular steatohepatitis with Mallory bodies
Ethanol
Amiodarone
Three agents associated with liver angiosarcoma
Thorotrast
Vinyl chloride
Arsenic
Most common metabolic liver disease
Non-alcoholic fatty liver disease
Non-alcoholic fatty liver disease and non-alcoholic steatohepatitis is associated with which underlying metabolic abnormality
Insulin resistance
Asymptomatic fatboy had routine serum electrolytes done. Aminotransferases were elevated. What is the cause?
Non-alcoholic fatty liver disease is the MOST COMMON cause of incidental elevation of serum transaminases.
3 mechanisms by which insulin resistance causes hepatic steatosis.
- Impaired oxidation of fatty acids
- Increased synthesis and uptake of fatty acids
- Decreased hepatic secretion of VLDL
Micronodular pattern of cirrhosis is seen in which two conditions.
- Alcoholic cirrhosis
2. Hereditary hemochromatosis
Reye syndrome is heralded by:
Pernicious vomiting!
With irritability, or lethargy and hepatomegaly.
Key pathologic finding in the liver of patients with Reye syndrome
Microvesicular steatosis
Primary biliary cirrhosis vs primary sclerosing cholangitis
- 95% AMA positive
- ‘Beading’ on cholangiography
- Association with IBD, pancreatitis and idiopathic fibrosing disease
- Association with Sjogren, scleroderma, and thyroid disease
- PBC (PSC is 80% p-ANCA positive)
- PSC
- PSC
- PBC
This condition is due to toxic injury to the sinusoidal endothelium causing release of RBCs into the space of Disse leading to proliferation of stellate cells and fibrosis of terminal branches of the hepatic vein. What are the causes of this condition?
Sinusoidal obstruction syndrome
(aka veno-occlusive disease)
Drinking bush-tea with pyrrolizidine alkaloid (in Jamaicans)
Cyclophosphamide and total body irradiation (in post bone marrow transplant patients)
Most common benign lesion of the liver
Cavernous hemangioma
How does aflatoxin cause CA?
It binds COVALENTLY with DNA and cause p53 mutations.
Liver specimen contains a single, large hard “scirrhous tumor.” Histology reveals well-differentiated polygonal cells growing in nests or cords and separated by lamellae of dense collagen bundles. Diagnosis?
Fibrolamellar carcinoma, a variant of HCC
Radioopaque or radiolucent?
Cholesterol gallstone
Brown stone
Black stone
20% of cholesterol stones are radioopaque.
50-75% of black strones are radioopaque.
Brown stones are radiolucent.
Route of entry of bacteria in cholangitis
Through the Sphincter of Oddi,
NOT via the hematogenous route
Parasitic causes of cholangitis
- Fasciola hepatica
- Schistosomiasis
- Clonorchis sinensis
- Opisthorchis viverrini
Most common clinically significant congenital pancreatic anomaly
Pancreas divisum
How does pancreas divisum predispose patient to develop pancreatitis?
In pancreas divisum, the fetal duct systems have failed to fuse. The main duct of Wirsung is thus very short, draining only a small portion of the head; while the accessory duct drains the bulk of the pancreas. This relative stenosis caused by the bulk of secretions passing through the minor papilla increases risk of pancreatitis.
Name drugs that can cause pancreatitis.
Thiazides Azathioprine Estrogen Sulfonamide Furosemide Methyldopa Pentamidine Procainamide
Name three infectious causes of pancreatitis.
Mumps
Coxsackie
Mycoplasma pneumoniae
Metabolic derangements which can cause pancreatitis
Hypercalcemia
Hypertriglyceridemia
Hyperparathyroidism
Most frequently altered oncogene in pancreatic CA
K-RAS
Most frequently inactivated tumor suppressor gene in pancreatic CA
p16 (CDKN2A)
Blocks RB phosphorylation by blocking cyclinD-CDK4 complex
Firs mutation in pancreatic CA sequence
According to the figure in p683, telomerase shortening and then mutations of the K-RAS
Most pancreatic cancers arise in the:
Head (60%)
Body (15%)
Tail (5%)
Diffuse (20%)
AIDS has the tat protein while Hep B has this protein which is a transcriptional transactivator. Required for viral infectivity
HBV-X protein
Average asymptomatic incubation period for hepatitis B
120 days
