Heme and GI Flashcards

1
Q

Microangiopathic hemolytic anemia

A
  1. microthrombi from platelets
  2. shistocytes and helmet cells

artificial heart valves
HUS (shigella or e. coli)

Increased LDH and decreased Haptoglobulin

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2
Q

ITP

A

Ab to Gp llb/IIIa

Female, 30’s isolated decrease platelets

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3
Q

Bernard Soulier syndrome

A

Gp ib receptor

Ristocetin test NORMAL

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4
Q

Glanzman thrombasthenia

A

GP IIb/IIIa def

abciximab mimics this

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5
Q

Aspirin

A

irreversible inhibits thromboxane A2

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6
Q

hemophilia A

A

Factor VII def

X linked (male, mat uncle)

Increased PTT

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7
Q

Hemophilia B

A

Factor IX def

Increased PTT

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8
Q

Von Willebrand disease

A

Increased bleeding time and PTT

ABNORMAL ristocetin test

tx: desmopression to increase VWF from endothelial cells

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9
Q

Vitamin K

A

inhibits epoxide reductase

factors II, VII, IX, X

Def: increase PT, PTT but normal BT

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10
Q

Liver failure

A

increased PT

coag factors synthesized in liver

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11
Q

DIC

A

decreased platelets

increased PT, PTT

low fibrinogen, increased D-Dimer (fibrin split products)

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12
Q

how to slow fibrinolysis

A

Aminocaproic acid blocks activation of plasminogen

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13
Q

Protein C or S def

A

Hypercoag state

Warfarin skin necrosis

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14
Q

Factor V leiden

A

EUROPEAN

resistance to protein C or S

PE

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15
Q

Iron def anemai

A
  1. iron abs in duodenum
  2. transferrin for transport
    3 ferritin for storage

Serum iron low, ferritin iron low, increased TIBC

Microcytic, hypochromic

Koiloychia (spoon finger nails)
Pica (chew dirt, ice, ricks) esp in pregnancy

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16
Q

Sideroblastic anemia

A
  1. ALA synthetase def (needs vit B6)
  2. Fe is stuck in mitochondria
  3. ringed sideroblasts with prussian blue stain (in marrow)
  4. Isoniazid (b6 def) can cause this
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17
Q

beta thal

A
  1. increased HBA2 and HBf

2. chronic transfusions overtime (cause hemochormatosisi and hemosiferin deposits)

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18
Q

alpha thal

A

4 gene: hydrops fetalis (Hb Barts)

3 gene: HbH

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19
Q

Hereditary spherocytosis

A
  1. cytoskeleton abnl (spectrin, ankyrin)
  2. lose central pallow
  3. high RDW, increased LDH, low haptoglobin
    4 increased bili (gall stone risk)
  4. pos osmotic fagility tst
  5. tx: splenectomy
  6. howell jolly bodies (nuclear DNA)
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20
Q

Sickle cell

A

glutamic acid -> val

sickling with low O2 (reversible )

tx: hydroxyurea (increases HbF)

At risk for renal papillary necrosis

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21
Q

Paroxysmal nocturna hemoglobinuria

A
  1. mediated by complement
  2. CD55 or CD59 ddef
  3. blood in urine when waking up
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22
Q

G6PD def

A

Cannot produce NADPH (glutathion reductase)

x-linked

Primaquine, sufla

Hb with precipitate as heinz body
bite cells

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23
Q

Pure red cell aplasia

A

thymoma

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24
Q

Auto immune hemolytic anemia

A

Coomb test pos

Igm: cold agglutination (m pneumonia)

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25
Q

Aplastic anemia

A

Hypocellular bone marrow

Cells replaced by fat and fibrous tissue

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26
Q

Itchy after shower

A

polycythemia (decreased ESR)

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27
Q

Filgastrim

A

cGSF

neutropenia common side effect

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28
Q

IL-5

A

Eosinophilia by TH2

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29
Q

Mono

A

EBV or CVM
Saliva
CD8+ t cells (paracortex)

Monspot
EBV: pos
CMV: neg

Atypical lymphocytes

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30
Q

ALL

A

Tdt pos

blasts > 20%
Down syndrome

T cell version: mediastinal mass

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31
Q

AML

A

t(15;17) RAR-PML translocation
Auer rods
Risk for DIC

Myeloperoxidase stain

tx: all trans retinoid acid

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32
Q

CML

A

B cells express CD5, CD19, CD 20

Smudge cells

Mainly mature lymphocytes

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33
Q

hairy cell leukemi

A

TRAP pos
Lack lymphaenopathy
dry, fibrotic marrow

tx: adenosine deaminase inihbitor j

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34
Q

Mycosis fungoides

A

cerebiform nuclei

t cell lymphoma

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35
Q

CML

A

t(9;22) BCR;ABL
Increased tyr kinase inhibitor

Increased basophils and granulocytes

tx: imatinib (blocks tyr kinase)

Leukocyte alkaline phos negative

36
Q

Polycythemia vera

A

Jak2 mut
Itchy after shower, h/a, blurry vision

Increased platelets and granulocytes

tx: Phlebotomy or hydroxyurea

Normal O2 sat, low EPO

37
Q

Non Hodgkins lymphoma

A
  1. follicular lymphoma
  2. mantle cell
  3. burkitt
38
Q

Follicular lymphoma

A

t(14;18) Bcl-2 to Ig heavy chain

tx: rituximab (anti DC 20)\

centrocytes and monoclonal cells (20:1 light chain ratio)

39
Q

Mantle cell lymphoma

A

t(11;14)

cyclin D1

40
Q

Burkitt Lymphoma

A

EBV, africa, jaw

t(8;14) c-myc activation

high mitotic rate (Ki-67)

tx: chemo

starry sky on histo

41
Q

Hodgkin Lymphoma

A

Reedsternburg cells (CD15 and CD30)

Painless, lymphedema, night sweats, pruritis

nodular sclerosing most common type

42
Q

Multiple myeloma

A

50-60 yrs old
Increased IL-6, activated RANKL

Lytic lesions hyperCa

M spike of IgG
Rouleaux formation

43
Q

Langerhans cells

A

Dendrtitic
Express CD1a and S-100

Birbeck granules (tennis racket)

44
Q

Intravascular hemolysis

A

decreased haptoglobin

Increased UCB

45
Q

Pleomorphic adenoma

A

In parotid

often recurrs

46
Q

Warthin tumors

A

Germinal centers in parotid

47
Q

Tracheo-esophageal fistual

A

aspiration
choking, vomit, regurgitation

Can lead to polyhydraminos in fetus

48
Q

Esophageal web

A

protrusion of mucosa

Can lead to obstruction

Food will rot and smell

risk for SCC

Common with Plummer vinson syndrome

49
Q

Plummer Vinson syndrome

A
  1. Fe def
  2. atrophic glossitis
  3. esophageal webs
50
Q

Zenker diverticulum

A

Out pouching of the esophagus-pharynx junction

Cricopharyngeous muscle dysfunction

51
Q

Mallory Weis

A

Violent coughing or vomiting
Linear lacerations at GE junction

Painful hematemesis and epigastric pain

52
Q

HIV pts

A

Candida
CMV -> linear ulcerations

HSV -> punched out ulcers

53
Q

Esophageal varices

A

from portal HTN
Painless unless they rupture

Left gastric and azygous vein

54
Q

Scleroderma

A

dysphagia from atrophy of smooth muscle of lower 2/3 of esophagus

Incompetence of LES

55
Q

Achalasia

A
  1. Inability to relax LES
  2. birds beak on imaging
  3. damaged ganglion cells in myenteric (auerbach plexus)
  4. call lead to sCC
    tx: Ca channel blockers (nifedipine)
56
Q

Cancers of Esophagus

A
  1. Adenocarcinoma (most common in US due to reflux)

2. SCC (most common in world) middle 1/3 of esphagus

57
Q

Omphalocele

A

herniation into umbilical cord

covered with periosteum

58
Q

Pyloric stenosis

A
  1. hypertrophy of smooth muscle wall at pyloric valve
    2 projectile,
    non-billous vomit with visible peristalsis
  2. olive mass
59
Q

Curling ulcer

A

from stress/burns/trauma

60
Q

Cushing ulcer

A

from high ICP

61
Q

Tx of peptic ulcer disease

A

Amoxicillin
Carithromycin
Omeprazole

62
Q

Gastric carcinoma

A
  1. Nitrosamines
    2 signet ring
  2. linitis plastic (thick, leathery wall of stomach)
63
Q

Kurkenberg tumor

A

signet rings (from gastric carcinoma) in ovaries

64
Q

Virchow node

A

Left supraclavicular node from gastric carcinoma metastasis

65
Q

Mary joseph node

A

Sub cutaneous periumbilical nodes from gastric carcinoma metastasis

66
Q

Leser Trela sign

A

Acute presence of seborrheic keratosis with gastric carcinoma

67
Q

Meckel diverticulum

A

failure of vitelline duct to involute

  1. meconium through umbilicus
    2 Can have gastric and pancreatic tissue
  2. volvulus and inussusception
68
Q

Duodenal atresia

A
  1. bilious emesis within hours of birth
  2. failure of recanalization of embryonic gut
  3. common in DS
  4. distention of stomach with tight pyloric sphincter and blind loop of duodenum

double bubble sign

69
Q

Direct hernia

A

Medial to inf epigastric artery

defect in transversalis fascia

70
Q

Indirect hernia

A

Lat to inf epigastric artery

most common

Through internal inguinal ring

71
Q

Gastrinoma

A
  1. islet cell tumors of the pancreas
  2. lead to ZE and duodenal ulcers
  3. Alson in MEN 1
72
Q

Celiac

A
  1. Blunting of small intestinal vili and inflamm in lamina propria of duodenum
  2. HLA-DQ2
  3. IgA and IgG to Gliadin, tissue transglutaminase and endomysial smooth muscle
73
Q

Whipple disease

A

Tropheryma Whippleii

1, lamina propria with foamy macrophages

  1. Schiff pos lysosomal granules
  2. arthritis and malabs
74
Q

Abetalipoproteinemia

A
  1. AR
  2. Def of Apo B proteins
  3. Failure to absorb fatty acids
  4. cannot form chylomicrons and LDL is decreased
75
Q

Carcinoid tumor

A
  1. Tumor that secretes 5HT
  2. 5-HiAA in urine
  3. wheezing, diarrhea, flushing of sin, RHF

tx: octreotide (somatostatin)

76
Q

Ulcerative colitis

A
  1. crypt abscesses
  2. continuous ulcerations
  3. HLA B27 (ankylosing spondylitis)
  4. p-ANCA PSC
  5. tx: sulfasalazine and mesalamine
77
Q

Crohns

A
  1. Ileum
  2. transmural inflammation
  3. Non-caseating granuloma (TH1)
  4. Skin lesions, cobble stone
  5. fistula
  6. risk for Ca stones

tx: infliximab (TNF alpha ab)

78
Q

Hirschsprung disease

A
  1. DS
  2. Failure of ganglionic cells to descend from neural crest
  3. absent ganglion cells in rectum
  4. failure to pass meconium
79
Q

Arteriovenous malformation

A

hereditary hemorrhagic telangiectasia

80
Q

Colonic diverticula

A
  1. elderly with hematochezia
  2. fe def anemia prone
  3. sx similar to appendicitis on L side
81
Q

FAP

A

Mut of APC gene on chr 5

  1. villous adenoma (fibrovascular stalk with tubular glands and villous components)
  2. Gardener sx: osteomas in jaw
  3. Turcot: glioblastoma and medulloblastoma
82
Q

HNPCC

A

Lunch sx
AD
DNA mismatch repair mutation

Microsatelite instability

83
Q

Peutz Jegher

A
  1. hamartomatous polyps
  2. melanin spots in lips and oral mucosa
  3. AD

Can lead to colorectal cancer

84
Q

Type 2 DM

A

amyloid deposits in pancreatic islet cells

85
Q

Kidney stones in croihns

A

Cholesteramine

86
Q

LipoProtein Lipase

A

ApoC

Absence will lead to elevated chylomicrons after a meal