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June 8th Flashcards

1
Q

WAGR Complex

A

Wilms Tumor
Aniridia
GU malformations
Retardation (motor and mental)

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2
Q

Metabolic panel in young asthmatic

A

They’re on a Beta agonist which would shift K into cells

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3
Q

IF: Granular pattern of immune complex deposition LM: diffuse capillary thickening

A

Membranous glomerulonephritis
Diffuse Proliferative glomerulonephritis (Lupus nephritis)
Remember membranous = thickening of BM & diffuse proliferative!

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4
Q

IF: Granular pattern of immune complex deposition LM: hyper cellular glomeruli

A

Acute PSGN

Hypercellularity = inflammatory cells

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5
Q

LM: segmental sclerosis and hyalinosis

A

Focal Segmental Glomerulonephritis

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6
Q

IF: IgG, IgM, IgA and C3 deposition in the mesangium

A

Acute PSGN

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7
Q

Anticoagulant during pregnancy

A

Heparin/LMWH

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8
Q

Heparin antidote

A

Protamine sulfate

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9
Q

Warfarin antidote

A

Fresh frozen plasma

Vit K oral

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10
Q

Fibrinolytic agent (streptokinase,urokinase, alteplase) antidote

A

Aminocaproic Acid

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11
Q

Veteran describing war in detail with no emotion

A

Isolation

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12
Q

A child abuser was himself abused as a child

A

Identification

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13
Q

Underlies all other defense mechanisms

A

Repression

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14
Q

May lead to multiple personalities

A

Dissociation

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15
Q

Deficiency of Aldolase B

A

Fructose intolerance

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16
Q

Deficiency of cystathione synthase

A

Homocysteinuria

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17
Q

Galactose 1 phosphate uridyltransferase deficiency&raquo_space; intellectual disability, hepatosplenomegaly and cataracts

A

Classic Galactosemia

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18
Q

Deficient in tyrosine

A

Albinism

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19
Q

Protein responsible for biconcave shape and flexibility of RBCs

A

Spectrin

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20
Q

Anisocytosis

A

RBC of varying sizes

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21
Q

Poikilocytosis

A

RBC of varying shapes

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22
Q

Reticulocyte

A

Immature RBC

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23
Q

Acanthocyte

A

Spur cells
Liver disease & Abetalipoproteinemia
Irregular spiked

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24
Q

Basophilic stippling

A

Lead poisoning

Inhibition of RNA breakdown

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25
Q

Degmacyte

A

Bite cell

G6PD deficiency

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26
Q

Echinocyte

A

Burr cell
Uremia (renal failure)
Regular spikes

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27
Q

Ringed sideroblasts

A

Excessive iron due to disorders of heme synthesis found in bone marrow

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28
Q

Schistocyte

A

Helmet cell
Fragmented RBCs
DIC TTP/HUS HELLP
Mechanical hemolysis

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29
Q

Dacrocyte

A

Teardrop cell

Myelofibrosis and bone marrow infiltration

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30
Q

Target cells

A 
"THAL" 
Thalassemia 
Hemoglobin C disease 
Asplenia 
Liver disease
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31
Q

Heinz bodies

A

Oxidized Hb fragments
G6PD deficiency
Many in one cell&raquo_space; “Heinz 57 ketchup”

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32
Q

Howell-Jolly bodies

A

Basophilic nuclear remnant found in RBCs only 1 per cell
Asplenic patients
Sickle cell disease

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33
Q

How does mom/fetus transfer O2?

A

Fetal Hb (alpha2gamma2) has less affinity for 2,3-DPG which gives it a higher affinity for O2 allowing the Fetal Hb to extract O2 from the maternal Hb (alpha2beta2)

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34
Q

Rate limiting enzyme of heme synthesis

A

ALA-synthease

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35
Q

Acute Intermittent Porphyuria

A

Defect in porphobilinogen deaminase/uroporphyrinogen I synthase

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36
Q

Porphyria Cutaenous Tarda

A

Defect in URO decarboxylase

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37
Q

Sites of erythropoiesis in embryo/fetal?

A 
"Young Livers Synthesize Blood" 
Yolk sac 
Liver 
Spleen 
Bone marrow
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38
Q

Causes of polycythemia

A

EPO producing tumors “Potentially Really High Hematocrit” (Pheo, RenalCC, Hepatic CC, Hemangioblastoma)
Polycythemia vera
Trisomy 21
Elevated altitude (chronic hypoxia)

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39
Q

Lead poisoning Sx

A 
Encephalopathy 
Memory loss 
Renal failure 
Wrist and foot drop 
Lead lines in bones and gums 
Basophilic stippling and macrocytic anemia
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40
Q

Hexokinase vs. Glucokinase

A 
Hexokinase find everywhere
Insulin independent 
Low Km/ Low Vmax
Glucokinase only in liver and B cells of pancreas 
Induced by insulin 
High Km/High Vmaz
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41
Q

Inhibitor of alcohol dehydrogenase

A

Fomepizole

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42
Q

Inhibitor of aldehyde dehydrogenase

A

Disulfram

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43
Q

Iron poisoning inhibits which enzymes in Heme synthesis?

A

ALA dehyrdataes

Ferrochelatase

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44
Q

Test to dx beta thalassemia minor

A

Hb electrophoresis

Increased HbA2

45
Q

Iron deficiency anemia in pt >50

A

Rule out colon cancer

46
Q

Which has neuro deficits B12/B9?

A

B12 has peripheral neuropathy

Folate does not

47
Q

Megaloblastic anemia with increased methylmalonic acid

A

B12 deficiency

48
Q

Megaloblastic anemia with no hyperammonemia

A

Orotic aciduria

Refractory to B12 and folate

49
Q

Hypo cellular bone marrow with fatty infiltration

A

Aplastic anemia

50
Q

Causes of aplastic anemia

A

Radiation
Drugs (benzene, chloramphenicol, alkylating agents)
Viral (parvo B19, HIV, EBV)
Following acute hepatitis

51
Q

Cause of anemia of chronic disease

A

Increased inflamm&raquo_space; increased hepcidin&raquo_space; binds ferroportin on intestinal mucosa and macrophages inhibiting iron transport

52
Q

Labs for anemia of chronic disease

A

decreased serum iron
decreased TIBC
normal or increased ferritin
normal transferrin saturation >18%

53
Q

Hair on end skull appearance

A

Beta thalassemia
Sickle cell
(due to marrow proliferation)

54
Q

Microcytic anemia reversible with B6

A

Sideroblastic anemia

55
Q

Anemia in Mediterranean population

56
Q

Anemia in African/Asian

A

Alpha thal

57
Q

Beta thalassemia major increased type of Hb

58
Q

Beta thalassemia minor increased type of Hb

A

HbA2 (alpha delta)

59
Q

Thalassemia with target cells

A

Beta thalassemia

60
Q

Difficulty swallowing
Glossitis
Microcytic anemia

A

Plummer Vinson Syndrome

Iron deficiency anemia

61
Q

Alpha thalassemia type

A

Missing 1 allele = Asx
Missing 2 allele = Alpha thal trait&raquo_space; minimal anemia
Missing 3 allele = HbH disease very little alpha, excess B forms B4 = HbH
Missing 4 alleles = Hb Barts no alpha globin so forms y4, hydrops fetalis&raquo_space; incompatible with life

62
Q

Hb Barts

A

Alpha thal with 4 allele deletion

y4

63
Q

HbH disease

A

Alpha thal with 3 allele deletion

B4

64
Q

RLS of beta oxidation of fatty acids

A

Carnitine acyltransferase 1

65
Q

Cofactors for pyruvate dehydrogenase

What other enzyme uses the same cofactors

A 
"TLC For Nancy"
TPP 
Lipoid acid 
CoA 
FAD
NAD+ 
Also needed for alpha ketoglutarate
66
Q

Result of glycolytic enzyme deficiency

A

Hemolytic anemia

67
Q

Result of pyruvate dehydrogenase

A

Neuro deficits

68
Q

Tx of sickle cell anemia

A

Hydroxyurea

69
Q

Hams test +

A

Paroxysmal nocturnal hemoglobinuria

increases pH to increase complement and cause red cell lysis

70
Q

+ osmotic fragility test

A

hereditary spherocytosis

71
Q

Causes of sickle cell crisis

A

Low O2
Acidosis
Dehydration

72
Q

Sickle cell osteomyelitis

A

Salmonella

73
Q

Sickle cell mutation

A

Glutamic acid (Glu)&raquo_space; Valine (Val) at position 6 for the B chain

74
Q

HbC mutation

A

Glutamic acid (Glu)&raquo_space; Lysine (Lys) at position 6 for the B chain

75
Q

Cold agglutinins Ab

76
Q

Warm agglutinins Ab

77
Q

Causes of microangiopathic hemolytic anemia

A

DIC
TTP/HUS
SLE
Malignant HTN

78
Q

Causes of macroangiopathic hemolytic anemia

A

Prosthetic heart valve

Aortic stenosis

79
Q

Direct coombs test

A

Checking for Ab on the RBC with Ab which cause RBC agglutination

80
Q

Indirect coombs test

A

Checking for Ab in the serum to normal RBC

Test blood prior to transfusion

81
Q

Cause of cold agglutinins

A

EBV
Mycoplasma
CLL

82
Q

Cause of warm agglutinins

A

HIV/EBV
SLE
CLL & NH Lymphoma
Congenital immune abnormalities

83
Q

Waterhouse Freidrichson caused by

What does it lead to

A

Caused by meningococcemia

Leads to adrenal insufficiency (after the adrenal hemorrhage)

84
Q

Causes of hemolytic anemia in G6PD

A 
"Spleen Purges Nasty Inclusions From Damaged Cells" 
Sulfonamides 
Primiquine 
Nitrofuratoin (UTI) 
INH (TB) 
Fava beans 
Dapsone (Leprosy) 
Chloroquine
85
Q

Causes of hemolytic anemia in G6PD

A 
"Spleen Purges Nasty Inclusions From Damaged Cells" 
Sulfonamides 
Primiquine 
Nitrofuratoin (UTI) 
INH (TB) 
Fava beans 
Dapsone (Leprosy) 
Chloroquine
86
Q

Pentad of TTP

A 
"Nasty Fever Torched His Kidneys" 
Neuro Sx 
Fever 
Thorombocytopenia 
Hemolysis/Anemia 
Kideny insufficiency
87
Q

Triad of HUS

A

Thrombocytopenia
Hemolysis
Renal insufficiency

88
Q

Triad of HUS

A

Thrombocytopenia
Hemolysis
Renal insufficiency

89
Q

Causes of DIC

A 
"STOP Making Thrombi"
Sepsis (gram -) 
Trauma 
Obstetric complications (amniotic fluid emboli and abruptio placenta) 
acute Pancreatitis 
Malignancy 
Transfusion 
(Nephrotic syndrome has been excluded)
90
Q

Labs in DIC and PT/PTT/BT/PC

A

PT/PTT/BT are all increased
PC is decreased
increased fibrin split products (D-dimer)
Decreased fibrinogen***

91
Q

Labs in DIC and PT/PTT/BT/PC

A

PT/PTT/BT are all increased
PC is decreased
increased fibrin split products (D-dimer)
Decreased fibrinogen***

92
Q

2 yr old girl with increase in abdominal girth, failure to thrive and skin/hair depigmentation

93
Q

Enzyme deficient in Essential Fructosuria

A

Fructokinase

94
Q

Enzyme deficient in Classic Galactosemia

A

Galactose 1 phosphate uridytransferase

95
Q

Thyroid cells with optically clear nuclei

A

Papillary cell cancer of the thyroid

96
Q

Hemosiderinuria + Thrombosis

A

Paroxysmal nocturnal hemoglobinuria

97
Q

Hemosiderinuria + Thrombosis

A

Paroxysmal nocturnal hemoglobinuria

98
Q

Cancer most commonly associated with noninfectious fever

A

Hodgkin lymphoma

99
Q

Large B cells with bilobed nuclei and prominent owl eye inclusions

A

Reed Sternberg cells of Hodgkin lymphoma

100
Q

Most common lymphoma in US

A

Diffuse Large B Cell Lymphoma

101
Q

Most common lymphoma in kids

A

Lymphoblastic lymphoma

102
Q

Age of Hodgkin lymphoma

A

Bimodal distribution 20/65

103
Q

Associated with long term celiac disease

A

Enteropathy/Intestinal T Cell Lymphoma

104
Q

Associated with Sjogrens, Hashimoto’s thyroiditis and H. pylori

A

MALToma (B cell)

105
Q

Starry sky pattern

106
Q

Follicular lymphoma mutation

A

14,18 BCL-2 on 18

107
Q

Burkitt lymphoma mutation

A

8,14

c-myc on 8

108
Q

Mantle cell lymphoma mutation

A

11,14

Cyclin D1 on 11

DIT/Boards (22 decks)

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