Heme and GI Flashcards

(86 cards)

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
Aplastic anemia
Hypocellular bone marrow | Cells replaced by fat and fibrous tissue
26
Itchy after shower
polycythemia (decreased ESR)
27
Filgastrim
cGSF neutropenia common side effect
28
IL-5
Eosinophilia by TH2
29
Mono
EBV or CVM Saliva CD8+ t cells (paracortex) Monspot EBV: pos CMV: neg Atypical lymphocytes
30
ALL
Tdt pos blasts > 20% Down syndrome T cell version: mediastinal mass
31
AML
t(15;17) RAR-PML translocation Auer rods Risk for DIC Myeloperoxidase stain tx: all trans retinoid acid
32
CML
B cells express CD5, CD19, CD 20 Smudge cells Mainly mature lymphocytes
33
hairy cell leukemi
TRAP pos Lack lymphaenopathy dry, fibrotic marrow tx: adenosine deaminase inihbitor j
34
Mycosis fungoides
cerebiform nuclei t cell lymphoma
35
CML
t(9;22) BCR;ABL Increased tyr kinase inhibitor Increased basophils and granulocytes tx: imatinib (blocks tyr kinase) Leukocyte alkaline phos negative
36
Polycythemia vera
Jak2 mut Itchy after shower, h/a, blurry vision Increased platelets and granulocytes tx: Phlebotomy or hydroxyurea Normal O2 sat, low EPO
37
Non Hodgkins lymphoma
1. follicular lymphoma 2. mantle cell 3. burkitt
38
Follicular lymphoma
t(14;18) Bcl-2 to Ig heavy chain tx: rituximab (anti DC 20)\ centrocytes and monoclonal cells (20:1 light chain ratio)
39
Mantle cell lymphoma
t(11;14) cyclin D1
40
Burkitt Lymphoma
EBV, africa, jaw t(8;14) c-myc activation high mitotic rate (Ki-67) tx: chemo starry sky on histo
41
Hodgkin Lymphoma
Reedsternburg cells (CD15 and CD30) Painless, lymphedema, night sweats, pruritis nodular sclerosing most common type
42
Multiple myeloma
50-60 yrs old Increased IL-6, activated RANKL Lytic lesions hyperCa M spike of IgG Rouleaux formation
43
Langerhans cells
Dendrtitic Express CD1a and S-100 Birbeck granules (tennis racket)
44
Intravascular hemolysis
decreased haptoglobin Increased UCB
45
Pleomorphic adenoma
In parotid | often recurrs
46
Warthin tumors
Germinal centers in parotid
47
Tracheo-esophageal fistual
aspiration choking, vomit, regurgitation Can lead to polyhydraminos in fetus
48
Esophageal web
protrusion of mucosa Can lead to obstruction Food will rot and smell risk for SCC Common with Plummer vinson syndrome
49
Plummer Vinson syndrome
1. Fe def 2. atrophic glossitis 3. esophageal webs
50
Zenker diverticulum
Out pouching of the esophagus-pharynx junction Cricopharyngeous muscle dysfunction
51
Mallory Weis
Violent coughing or vomiting Linear lacerations at GE junction Painful hematemesis and epigastric pain
52
HIV pts
Candida CMV -> linear ulcerations HSV -> punched out ulcers
53
Esophageal varices
from portal HTN Painless unless they rupture Left gastric and azygous vein
54
Scleroderma
dysphagia from atrophy of smooth muscle of lower 2/3 of esophagus Incompetence of LES
55
Achalasia
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
Cancers of Esophagus
1. Adenocarcinoma (most common in US due to reflux) | 2. SCC (most common in world) middle 1/3 of esphagus
57
Omphalocele
herniation into umbilical cord covered with periosteum
58
Pyloric stenosis
1. hypertrophy of smooth muscle wall at pyloric valve 2 projectile, non-billous vomit with visible peristalsis 3. olive mass
59
Curling ulcer
from stress/burns/trauma
60
Cushing ulcer
from high ICP
61
Tx of peptic ulcer disease
Amoxicillin Carithromycin Omeprazole
62
Gastric carcinoma
1. Nitrosamines 2 signet ring 3. linitis plastic (thick, leathery wall of stomach)
63
Kurkenberg tumor
signet rings (from gastric carcinoma) in ovaries
64
Virchow node
Left supraclavicular node from gastric carcinoma metastasis
65
Mary joseph node
Sub cutaneous periumbilical nodes from gastric carcinoma metastasis
66
Leser Trela sign
Acute presence of seborrheic keratosis with gastric carcinoma
67
Meckel diverticulum
failure of vitelline duct to involute 1. meconium through umbilicus 2 Can have gastric and pancreatic tissue 3. volvulus and inussusception
68
Duodenal atresia
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
Direct hernia
Medial to inf epigastric artery defect in transversalis fascia
70
Indirect hernia
Lat to inf epigastric artery most common Through internal inguinal ring
71
Gastrinoma
1. islet cell tumors of the pancreas 2. lead to ZE and duodenal ulcers 3. Alson in MEN 1
72
Celiac
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
Whipple disease
Tropheryma Whippleii 1, lamina propria with foamy macrophages 2. Schiff pos lysosomal granules 3. arthritis and malabs
74
Abetalipoproteinemia
1. AR 2. Def of Apo B proteins 3. Failure to absorb fatty acids 4. cannot form chylomicrons and LDL is decreased
75
Carcinoid tumor
1. Tumor that secretes 5HT 2. 5-HiAA in urine 3. wheezing, diarrhea, flushing of sin, RHF tx: octreotide (somatostatin)
76
Ulcerative colitis
1. crypt abscesses 2. continuous ulcerations 3. HLA B27 (ankylosing spondylitis) 4. p-ANCA PSC 5. tx: sulfasalazine and mesalamine
77
Crohns
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
Hirschsprung disease
1. DS 2. Failure of ganglionic cells to descend from neural crest 3. absent ganglion cells in rectum 4. failure to pass meconium
79
Arteriovenous malformation
hereditary hemorrhagic telangiectasia
80
Colonic diverticula
1. elderly with hematochezia 2. fe def anemia prone 3. sx similar to appendicitis on L side
81
FAP
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
HNPCC
Lunch sx AD DNA mismatch repair mutation Microsatelite instability
83
Peutz Jegher
1. hamartomatous polyps 2. melanin spots in lips and oral mucosa 3. AD Can lead to colorectal cancer
84
Type 2 DM
amyloid deposits in pancreatic islet cells
85
Kidney stones in croihns
Cholesteramine
86
LipoProtein Lipase
ApoC | Absence will lead to elevated chylomicrons after a meal