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Pathoma Flashcards

1
Q

Can be used as a Tx for neutropenia to boost granulocyte production?

A

GM-CSF or G-CSF

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2
Q

Lymphocytic leukocytosis is usually caused by viral infections but can be caused by which bacteria?

A

Bordetella pertussis infection (produces lymphocytosis-promoting factor)

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3
Q

Mono is usually caused by EBV infection, what is a less common cause?

A

CMV infection (monospot test will be negative)

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4
Q

CD8+ T-cell response to EBV leads to generalized lymphadenopathy due to?

A

T-cell hyperplasia in the LN paracortex

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5
Q

CD8+ T-cell response to EBV leads to splenomegaly due to?

A

T-cell hyperplasia in the periarterial lymphatic sheath (PALS)

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6
Q

How can you differentiate AML and ALL?

A

ALL: positive nuclear staining for TdT (a DNA polymerase)
AML: positive cytoplasmic staining for MPO, or crystal aggregates of MPO may be seen as Auer rods

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7
Q

Acute Promyelocytic Leukemia (APL) is characterized by?

A

t(15;17) translocation of retinoic acid receptor (RAR) on Ch 17 to Ch15 -> RAR disruption blocks maturation, promyelocytes accumulate;
Incr risk for DIC

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8
Q

Tx of APL?

A

all-trans-retinoic acid which causes the blasts to mature (and eventually die)

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9
Q

pts w Down Syndrome have an increased risk of Acute Leukemia: which type would typically arise before age 5?
which type AFTER the age of 5?

A

Acute Megakaryoblastic leukemia;

ALL

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10
Q

Blasts characteristically infiltrate gums in what type of leukemia?

A

Acute Monocytic Leukemia (proliferation of monoblasts which usually lack MPO)

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11
Q

Hairy Cell Leukemia is a?

A

neoplastic proliferation of mature B-cells characterized by hairy cytoplasmic processes, cells are positive for tartrate-resistant acid phosphatase (TRAP)

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12
Q

Hairy Cell Leukemia clinical features?

A

splenomegaly (due to accum. of hairy cells in RED pulp) and dry tap due to marrow fibrosis. Lymphadenopathy is usually absent.

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13
Q

Hairy Cell Leukemia has an excellent response to what Tx?

A

2-CDA (cladribine)- an adenosine deaminase inhibitor -> adenosine accum to toxic levels in the neoplastic B cells

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14
Q

Which Leukemia is assoc. w/ HTLV-1 and MC seen in Japan and the Caribbean?
clinical features?

A

Adult T-Cell Leukemia/Lymphoma (ATLL);

Rash, gen lymphadenopathy, hepatosplenomegaly, lytic (punched-out) bone lesions w. Hypercalcemia

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15
Q

Polycythemia Vera assoc. with what mutation?

A

JAK2 Kinase mutation!

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16
Q

What features of CML help distinguish it from a leukemoid rxn (reactive neutrophilic leukocytosis)?

A

Negative leukocyte alkaline phosphatase (LAP) stain, Increased Basophils!, t(9;22) present

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17
Q

Essential Thrombocythemia assoc. with what kind of mutation?

A

JAK2 Kinase mutation

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18
Q

Polycythemia Vera (PV) must be distinguished from reactive polycythemia, differentiating features?

A

In PV: EPO levels are decreased, and SaO2 is normal

In reactive polycythemia due to high altitude or lung disease: SaO2 is low and EPO is incr
In reactive polycythemia due to ectopic EPO production from renal cell carcinoma: EPO is high and SaO2 is normal

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19
Q

MC involved artery in MI?

2nd MC?

A

Left Ant. Descending -leads to infarction of the ant. wall and ant. septum of the LV;
RCA -> infarction of post. wall, post. septum and papillary mm. of the LV

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20
Q

Hypospadias is due to failure of?

A

the urethral folds to close

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21
Q

Epispadias is due to?

A

abnormal positioning of the genital tubercle

is assoc. with bladder exstrophy

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22
Q

3 in situ carcinomas that are precursor lesions to SCC of the penile skin

A

1) Bowen disease- presents as leukoplakia
2) Erythroplasia of Queyrat- Erythroplakia
3) Bowenoid Papulosis: multiple reddish papules

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23
Q

Congenital failure of testes to attach to the inner lining of the scrotom, via processus vaginalis, increases risk for?

A

Testicular torsion, presents in adolescents w/ sudden testicular pain and absent cremasteric reflex

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24
Q

Dilation of the spermatic v. due to impaired drainage, presents as scrotal swelling w “bag of worms” appearance?
usually on what side?

A

Varicocele;

L side since L testicular v. drains into the L renal v. (extra step in drainage), is assoc. w L-sided RCC

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25
Q

MC type of testicular tumor?

RFs inc cryptorchidism and Klinefelter Syndrome

A

Germ Cell tumors (divided into Seminomas and non-seminomas: Embryonal Ca., Yolk sac tumor, Choriocarcinoma, Teratoma)

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26
Q

Type of testicular tumors that usually occur in men 15-40yoa?

MC type of testicular mass in males over 60yo?

A

Germ cell tumors;

Lymphoma (usually diffuse large B-cell type and bilateral)

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27
Q

Malignant testicular tumor, forms a homogenous mass w No hemorrhage nor necrosis! good prognosis

A

Seminoma

radiosensitive, great prognosis

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28
Q

Malignant testicular tumor c/o primitive cells that may produce glands, forms hemorrhagic mass w necrosis

A

Embryonal Carcinoma

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29
Q

MC testicular tumor in kids? characteristic findings?

A 
Yolk Sac (endodermal sinus) tumor -> elevated AFP, 
Histo: Schiller-Duval bodies that resemble glomeruli
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30
Q

Which cells of Choriocarcinomas make beta-hCG? which may lead to what Sxs?

A

Syncytiotrophoblasts;

hyperthyroidism or gynecomastia (alpha-subunit of hCG is similar to that of FSH, LH and TSH)

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31
Q

Type of tumor cmpsd of mature fetal tissue that is benign in females but malignant in males?

A

Teratoma

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32
Q

S/Sx of Acute Prostatitis ?

A

Presents as dysuria, F/C,

Prostate is tender/boggy on DRE, secretions show WBCs; MC due to bacteria- reveal w culture

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33
Q

BPH MC occurs in what zone of the prostate?

A

central periurethral (which -> Sx due to obstruction of urethra)

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34
Q

Tx for BPH?

A

alpha1-antagonists (ie Terazosin) to relax smooth m., also lowers BP; Selective alpha1A-antagonists (Tamsolusin) used in normotensive pts; and
5alpha-reductase inhibitors

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35
Q

Prostate adenocarcinoma usually arises in what area of the prostate?

A

Peripheral, Posterior
(hence does NOT produce urinary Sxs early on)
Biopsy required to confirm Ca.

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36
Q

Fine-needle biopsy of the thyroid shows “tumor cells in an amyloid background” , Dx?

A

Medullary carcinoma of the thyroid- tumor cells produce Calcitonin which –> localized amyloidosis

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37
Q

Dialysis-assoc. Amyloidosis- fibrils are cmpsd of?

May present as?

A

beta2-microglobulin, which deposits in joints ;

Carpel tunnel syndrome

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38
Q

beta-amyloid is derived from beta-amyloid precursor protein (b-APP), deposits in brain forming ?

Gene for b-APP is on what Chr?

A

Amyloid plaques (Alzheimer disease);

Chr 21 (thus most pts w Trisomy 21 develop Alzheimer disease by age 40)

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39
Q

Age-related (Senile) cardiac Amyloidosis is due to deposition of?

A

Normal (non-mutated) transthyretin

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40
Q

Systemic Amyloidosis can involve almost any tissue, but what is the MC organ involved ?

A

Kidney (classically –> NephrOtic syndrome)

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41
Q

Due to dysfunction of neutrophils, AR, presents w episodes of fever and acute serosal inflamm. (can mimic appendicitis, arthritis, or MI); High SAA during attacks deposits as AA amyloid in tissues

A

Familial Mediterranean fever (FMF) which occurs in persons of Mediterranean origin, assoc. with secondary Amyloidosis

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42
Q

Primary Amyloidosis is systemic deposition of ?

Assoc. with?

A

AL amyloid, which is derived from Ig light chain ;

Plasma cell dyscrasias (ie Multiple Myeloma)

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43
Q

Multiple proteins can deposit as amyloid, shared features include?

A

beta-pleated sheet configuration, congo red staining and apple-green birefringence

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44
Q

Under persistent stress, metaplasia can progress to dysplasia and eventually result in cancer, a notable exception to this?

A

Apocrine metaplasia of the breast- carries no increased risk for cancer

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45
Q

Apoptosis is mediated by ______ that activate proteases and endonucleases

A

Caspases

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46
Q

Presents as unilateral, painful cystic lesion at the lower vestibule adjacent to the vaginal canal, usually in women of reproductive age

A

Bartholin cyst- cystic dilation due to inflamm. and obstruction of bartholin gland

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47
Q

Hallmark histo feature of HPV infected cells?

A

Koilocytes
(“rasinoid cells”, pyknotic, immature squamous cells w dense irregularly staining cytoplasm and perinuclear halo-like clearing)

48
Q

Condyloma acuminatum MC due to?

A

HPV type 6 or 11 (low-risk types)

49
Q

Vulvar carcinoma may be HPV related or non-HPV related, Distinguish between them

A

HPV related is due to high-risk HPV types 16 and 18 and gen occurs in women of reproductive age;

Non-HPV: MC due to long-standing lichen sclerosis, gen seen in elderly women (usually over 70yo)

50
Q

Imp distinction btwn Paget disease of the nipple vs extramammary Paget disease

A

both c/o malignant epithelial cells in the epidermis,
Paget of the nipple is almost always assoc. w an underlying carcinoma;

Extramammary- usually no underlying carcinoma

51
Q

If there are malignant epithelial cells in the epidermis of the vulva, must distinguish between extramammary Paget disease and Melanoma, by..

A

Paget cells: PAS+, keratin +, and S100-

Melanoma is PAS -, keratin -, S100+

52
Q

Presents with bleeding and a clear, grape-like, polypoid mass protruding from the vagina or penis of a child less than 5?

A

Sarcoma Botryoides AKA Embryonal Rhabdomyosarcoma- malignant mesenchymal proliferation of immature skeletal m., stains Desmin + and myogenin +

53
Q

When spread to regional LNs occurs, cancer from the lower 1/3 of vagina goes to?

and cancer from upper 2/3 goes to ?

A

superficial Inguinal nodes ;

Internal iliac nodes

54
Q

MOST imp. predictor for progression of endometrial hyperplasia to carcinoma?

A

the presence of cellular atypia

55
Q

Compare these proliferations of smooth m. arising from myometrium: benign Leiomyoma vs malignant Leiomyosarcoma

A

Leiomyoma: estrogen sensitive, may enlarge in pregnancy and shrink after menopause, MULTIPLE white, whorled masses, usually ASx;

-sarcoma: arises de novo, in POST-menopausal women, SINGLE lesion w areas of hemorrhage and necrosis

56
Q

Endometrial polyp can arise as an ADR of?

A

Tamoxifen (has anti-estrogenic effects on breast but weak pro-estrogenic effects on the endometrium)

57
Q

MC site of involvement of endometriosis? which classically forms?

A

ovary- appears as endometrioma= blood-filled “chocolate cyst”

58
Q

BRCA1 mutation carriers have an increased risk for what type of tumor of the ovary and fallopian tube?

A

Serous carcinoma (a surface epithelial tumor)

59
Q

Useful serum marker to monitor Tx response and screen for recurrence of ovarian surface epithelial tumors?

A

CA-125

60
Q

Metastatic mucinous tumor that invs both ovaries, MC due to metastatic (diffuse type) gastric Ca. with signet cells

A

Krukenberg tumor

61
Q

Moderate global ischemia leads to infarcts in watershed areas and damage to highly vulnerable areas such as?

A

Pyramidal neurons of cerebral cortex (leads to laminar necrosis), Pyramidal neurons of hippocampus (temporal lobe, imp. in long-term memory), Purkinje layer of the cerebellum

62
Q

What type of stroke –> pale infarct at the periphery of the cortex

A

thrombotic (type of ischemic) stroke, due to rupture of an atherosclerotic plaque

63
Q

What type of stroke –> hemorrhagic infarct at the periphery of the cortex

A

Embolic (type of ischemic) stroke

MC source of emboli is L side of heart- a fib; usually involves MCA

64
Q

Ischemic stroke results in what type of necrosis?

what early microscopic change can be seen from 12 to 24hrs after infarction?

A 
Liquefactive;
Red neurons (eosinophilic)
65
Q

Intracerebral hemorrhage ie. bleeding into brain parenchyma is classically due to? what is the MC site?
presents as?

A

rupture of Charcot-Bouchard microaneurysms of the lenticulostriate vessels (complication of HTN);
Basal ganglia;
HA/N/V, eventual coma

66
Q

“worst HA of my life” with nuchal rigidity classic presentation of ? lumbar puncture will show?

A

Subarachnoid hemorrhage;

Xanthochromia (yellow hue due to bilirubin)

67
Q

Subarachnoid hemorrhages are MC due to ? (specifically)

assoc. with what med conditions?

A

Berry aneurysms- most frequently in the Ant. circle of Willis at branch points of the Ant. communicating a. ;
Assoc. w Marfans and ADPCKD

68
Q

Subacute sclerosing panencephalitis (invs both gray and white matter) is progressively debilitating; due to?

A

slowly progressing, persistent infection of the brain by MEASLES virus (infection occurs in infancy then neurologic signs present yrs later such as visual loss, weakness, dementia)

69
Q

Central pontine myelinolysis is focal demyelination of the pons (anterior brain stem) due to ? classically presents as?

A

rapid IV correction of hyponatremia in severely malnourished pts. (ie pts w liver disease/alcoholics);
acute bilateral paralysis (‘locked in syndrome’)

70
Q

Neuromyelitits optica is an autoimmune disease of CNS, test for Abs against?

A

Aquaporin channel 4 (AQP4-IgG)

71
Q

Progressive Multifocal Leukoencephalopathy presents w rapidly progressive neurologic signs( vision loss, weakness, dementia..) due to?

A

immunosuppression -> reactivation of latent JC virus infection
(Natalizumab, a drug for MS -> increased risk)

72
Q

malignant high-grade tumor of astrocytes; regions of necrosis surrounded by tumor cells (=pseudopalisading) and endothelial cell proliferation, usually arises in cerebral hemisphere? tumor cells are positive for?

A

GBM (characteristically crosses the corpus callosum ie “butterfly lesion”);
GFAP

73
Q

In kids, CNS tumors are usually where?

A

infratentorial (ie cerebellum, 4th ventricle)

74
Q

Meningioma is a benign tumor of arachnoid cells, MC seen in ? may present as?

A 
adult women (tumor expresses estrogen receptor);
Seizures (tumor compresses but does not invade the cortex)
75
Q

Meningioma: histo? imaging?

A

whorled pattern, if these calcify ->psammoma bodies;

round mass attached to the dura

76
Q

Schwannomas are positive for what tumor marker?

Bilateral tumors are seen assoc. with what condition?

A

S-100+

Neurofibromatosis type 2

77
Q

Schwannoma is a benign tumor of schwann cells that can inv. cranial or spinal nn., within the cranium they MC involve?

A

CN VIII at the cerebellopontine angle (presents as hearing loss and tinnitus)

78
Q

Oligodendroglioma: malignant tumor of oligodendrocytes, imaging reveals?

A

calcified tumor in the white matter, usually involving the frontal lobe (may present w seizures; fried egg appearance of cells on Bx)

79
Q

MC CNS tumor in kids? usually arises where?
imaging reveals?
Bx shows?

A

Pilocystic astrocytoma- cerebellum
cystic lesion with a mural nodule!;
Rosenthal fibers (thick eosinophilic processes of astrocytes) and eosinophilic granular bodies

80
Q

3 MC CNS tumors in kids?

A

Pilocystic astrocytoma, ependymoma, medulloblastoma

81
Q

Medulloblastoma is a malignant tumor w poor prognosis, usually arises in kids, derived from?
histo?

A

Neuroectoderm;
small, round blue cells, +/- Homer-Wright rosettes
(grows rapidly, spreads via CSF, see ‘drop metastasis’ to the cauda equina)

82
Q

*Craniopharyngioma is a benign tumor but tends to recur after resection, presents as ? arises from?
what is commonly seen on imaging?

A

supratentorial mass in kids/young adults, may compress optic chiasm -> bitemporal hemianopsia!!;
epithelial remnants of Rathke’s pouch;
calcifications

83
Q

Ependymoma is a malignant tumor of ependymal cells, usually seen in ? MC arises where? may present as?
histo?

A

kids;
4th ventricle;
hydrocephalus;
Perivascular pseudorosettes

84
Q

Devel. malformation, pancreas forms a ring around the duodenum-> risk of duodenal obstruction and nonbilious vomiting

A 
Annular pancreas 
(ventral pancreatic bud abnormally encircles duodenum)
85
Q

Acute pancreatitis presents with elevated serum lipase and amylase, which is more specific for pancreatic damage? why?

A

lipase;

b/c amylase could also be elevated if there is damage to salivary gland

86
Q

Acute pancreatitis -> hypocalcemia, due to?

A

Ca++ is consumed during saponification in fat necrosis

87
Q

one complication of acute pancreatitis is Pancreatic abscess which is often due to? and presents as?

A

E. coli;

abd pain, high fever, persistently elevated amylase

88
Q

If a thin elderly patient (~70yo) develops DM you should consider what Dx?

A

Pancreatic carcinoma

since tumors in body/tail can -> 2ndary DM and this is not the typical demographic for DM

89
Q

Trousseau syndrome?

A

migratory thrombophlebitis in the extremeties assoc. with visceral carcinoma; presents as swelling, erythema, tenderness in extremities
(seen in ~10% of pts w pancreatic carcinoma)

90
Q

Serum tumor marker of pancreatic carcinoma?

A

CA 19-9

91
Q

MC type of gallstones are? do they show up on x-ray?

A

Cholesterol;

No they are usually radiolucent (10% are radiopaque due to assoc. Ca++ and these could show up)

92
Q

What are some risk factors for Bilirubin stones?

A

extravascular hemolysis (increased bilirubin in bile) and biliary tract infection (ie E. coli, Ascaris roundworms in areas of poor sanitation; and C. sinensis- Chinese liver fluke)

93
Q

Waxing and waning RUQ pain is known as? due to?

A

Biliary colic ;

gallbladder contracting against a stone lodged in the cystic duct

94
Q

Acute cholecystitis (acute inflamm. of the gallbladder wall) presents with?

A

RUQ pain, often radiates to Right SCAPULA!, F/N/V, incr WBC count, and incr serum alk phos (from duct damage)

95
Q

Rokitansky-Aschoff sinuses are? characteristic of?

A

invaginations of gallbladder mucosa into the muscular wall ;

Chronic cholecystitis (chronic inflamm of the gallbladder)

96
Q

Porcelain gallbladder?

A

Shrunken, hard, calcified gallbladder, a late complication of Chronic cholecystitis

97
Q

Gallbladder adenocarcinoma has a poor prognosis and classically presents as?
a major RF?

A

cholecystitis in an elderly woman;

RF: Gallstones, esp when complicated by porcelain gallbladder

98
Q

Extravascular hemolysis and ineffective erythropoiesis both -> high levels of UCB, incr risk for pigmented bilirubin gallstones and dark urine, what makes the urine dark?

A

dark urine due to incr urine urobilinogen

NOT due to UCB since it is not H2O-soluble it is absent from urine

99
Q

Tx for physiologic jaundice of the newborn?

A

Phototherapy- makes the UCB water-soluble!

does NOT conjugate it

100
Q

AR deficiency of bilirubin canalicular transport protein? clinical manif?

A

Dubin-Johnson Syndrome;
increased CB and grossly black liver, otherwise ASx
(dark liver usually seen during surgery for smthng else)

101
Q

this infection in pregnant women is assoc. w Fulminant hepatitis ie liver failure w massive liver necrosis

A

HEV

102
Q

Cirrhosis is end-stage liver damage w disruption of normal hepatic parenchyma by bands of fibrosis/regen. nodules of hepatocytes, the fibrosis is mediated by?

A

TGF-beta from stellate cells!

103
Q

Alcoholic hepatitis -> acute inflamm, necrosis, and swelling of hepatocytes w formation of Mallory bodies which are??

A

damaged intermediate/ cytokeratin filaments within hepatocytes

104
Q

In Hemochromatosis- tissue damage is mediated by?

A

generation of free radicals (from excess iron)

105
Q

Primary biliary cirrhosis= autoimmune granulomatous destruction of intrahepatic bile ducts, classically arises in?
marker of disease found in serum?

A

women (avg age is 40);

Antimitochondrial Ab

106
Q

what -> “onion-skin” Periductal fibrosis, “beads on a string” w contrast imaging; p-ANCA often + ?
assoc w what condition?

A

Primary Sclerosing Cholangitis;

assoc w Ulcerative colitis

107
Q

Benign tumor of hepatocytes assoc. with OC use? risk of rupture and intraperitoneal bleeding esp. during?

A

Hepatic adenoma;

Pregnancy (since tumors grow w exposure to estrogen)

108
Q

Serum tumor marker for HCC?

A

alpha-fetoprotein

109
Q

Aflatoxins are derived from?

incr risk for what Ca?

A

Aspergillus!;

HCC

110
Q

Only nephrotic syndrome that has an excellent response to corticosteroid Tx?

A

Minimal change disease

111
Q

If nephrotic syndrome presents in a pt. with HIV, SCD or heroin abuse, be sure to consider ?

A

Focal segmental glomerulosclerosis

112
Q

this NephrOtic Sx due to Ag-Ab cmplx deposition is usually idiopathic but can be assoc. with SLE, solid tumors, HBV/HCV, or drugs? EM findings?

A

Membranous nephropathy;

subepithelial deposits w/ “spike and dome”

113
Q

Sjogrens can be primary or assoc w another AI disorder, esp which one?

A

RA (rheumatoid factor is often present even when RA is not)

114
Q

pregnant women w anti-SSA (crosses placenta) are at risk for delivering babies with? what pts would have this Ab?

A

neonatal lupus and congenital heart block ;

pts w Sjogrens, and a subset of pts w SLE

115
Q

c/b ANA plus anti-U1 ribonucleoprotein (RNP) Abs? features?

A

Mixed connective tissue disease (AI mediated tissue damage w features of SLE, systemic sclerosis, polymyositis, but lack severe CNS/renal involvement)

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