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UWorld > Heme > Flashcards

Heme Flashcards

1
Q

Aquagenic pruritis, facial plethora, and splenomegaly are symptoms of…

A

PV

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2
Q

… is associated with JAK2 mutations, making the RBC cell more sensitive to growth factors.

A

PV

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3
Q

Cells with increased cytoplasm, bilobed nuclei, eosinophilic nuclei

A

Reed Sternberg cells

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4
Q

Reed Sternberg cells are seen in…

A

Hodgkin’s lymphoma

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5
Q

B symptoms are:

A

fever, night sweat, weight loss

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6
Q

The peripheral blood smear is normal in what type of lymphoma?

A

Hodgkin’s

Lymph node biopsy to diagnose

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7
Q

Binds free Hbg to promote uptake by reticuloendothelial system

A

Haptoglobin

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8
Q

With intracellular hemolysis, haptoglobin (increases/decreases).

A

Increases

also increased LDH, bili

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9
Q

Patients with protein C or S deficiency treated with warfarn are at risk for…

A

Warfarin-induced Skin Necrosis

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10
Q

Cell type seen in G6PD deficiency

A

Bite cells

Heinz bodies

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11
Q

What drugs cause G6PD anemia?

A

Dapsone, Antimalarials, TMP-SMX

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12
Q

What causes can induce G6PD anemia?

A

Drugs
DKA
Infection
Fava beans

(DDIF is the differential)

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13
Q

Deficiency of G6PD and glutathione reductase cause what in the RBC?

A

Oxidative stress > bite cells, heinz bodies

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14
Q

Pyruvate kinase deficiency causes what in the RBC?

A

Failure of glycolysis

  • Cannot produce ATP bc do not have mitochondria
  • Cannot maintain structure
  • Destroyed by spleen > splenic work hypertrophy
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15
Q

Bleeding time and Prothrombin time in Immune Thrombocytopenic Purpura

A 
Increased BT (bc decreased platelets)
Normal PT (bc normal fibrinogen)
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16
Q

Unfractionated heparin binds…

A

thrombin, Xa equally well

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17
Q

LMWH binds…

A

Xa&raquo_space;> thrombin

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18
Q

What does EBV bind to infect B cells?

A

CD21

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19
Q

Atypical CD8 T cells with a large cytoplasm, eccentric nucleus, and cell membrane that conforms to the border of adjacent cells are seen in response to …

A

EBV infection

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20
Q

An ADP Receptor blocker like Clopidogrel

A

Ticlopidene

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21
Q

Serious side effect of Ticlopidene

A

Neutropenia: fever & mouth ulcers

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22
Q

What lab values differentiates absolute vs. relative polycythemia?

A

RBC mass

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23
Q

Polycythemia with normal RBC mass could be caused by:

A

1) dehydration

2) excessive diuresis: plasma volume contraction

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24
Q

Polycythemia with increased RBC mass could be caused by:

A

1) PV

2) Secondary polycythemia

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25
Q

What differentiates PV from secondary polycythemia?

A

PV: all 3 cell lines increased
Secondary: only RBC increased

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26
Q

What are the causes of secondary polycythemia?

A

Hypoxia

EPO-tumor

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27
Q

…is the central regulator of iron, produced by the liver.

A

Hepcidin

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28
Q

What is the MOA of hepcidin?

A

When the body has a lot of iron, hepcidin binds ferroportin and stop iron absorption

Ferroportin is then degraded

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29
Q

In hypoxia, hepcidin is (increased/decreased).

A

Decreased

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30
Q

What anticoagulant can be used in pregnancy

A

HEPregnancy

not Warfarin

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31
Q

Inheritance of G6PD deficiency

A

X linked recessive

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32
Q

Disease characterized by defective binding of RBC cytoskeleton to plasma membrane due to mutations in structural proteins

A

Hereditary Spherocytosis

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33
Q

What structural proteins are mutated in Hereditary Spherocytosis?

A

Ankyrin, band 3, spectrin

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34
Q

Small RBC with no central pallor and increased MCHC

A

Spherocytes

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35
Q

Inheritance of hereditary spherocytosis

A

Autosomal dominant

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36
Q

Non-jaw presentation of Burkitt’s

A

GI mass

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37
Q

Function of c-myc

A

TF that controls cell proliferation, differentiation, apoptosis

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38
Q

The mutation that causes beta-thal is a defect in…

A

mRNA transcription, processing, translation

NOT folding

39
Q

Beta-thal causes what kind of anemia?

A

Microcytic

40
Q

Increased HbgF, HbgA2, and target cells indicate

A

Beta-thal

41
Q

CD14 is a marker for what lineage?

A

Monocyte/macrophage

42
Q

What is the Haldane effect?

A

Binding O2 in lungs drives Hbg to release H+ (as H2O) and CO2

43
Q

What is the Bohr effect?

A

Increased CO2 and H+ in the periphery facilitates O2 unloading

44
Q

What disease is caused by a deficiency of HMB synthase?

A

Acute intermittent porphyria

45
Q

What substances build up in acute intermittent porphyria?

A

d-Ala and porphobilinogen > cause symptoms

46
Q

What disease is characterized by…

  • Intermittent episodes of abdominal pain
  • Neuro manifestations (muscle weakness commonly)
  • After exposure to offending med
  • Urine darkens on standing in sunlight
A

Acute Intermittent Porphyria

Note: no skin photosensitivity

47
Q

In what disease to RBCs show osmotic fragility on exposure to saline?

A

Hereditary spherocytosis

  • Increased membrane lysis in hypotonic saline or NaCL
48
Q

What is a secondary improvement post-splenectomy in patients with Hereditary Spherocytosis?

A

Decreased gallstones

  • Decreased hemolysis
  • Decreased bili
49
Q

What is the action of heparin?

A

Bind and activate antithrombin III

50
Q

Increased urinary excretion of radio-labeled B12 on Schilling test indicates…

A

B12 deficiency in diet

51
Q

Increased urinary excretion of radio-labeled B12 on Schilling test only after addition of IF indicates…

A

Pernicious anemia

52
Q

Low excretion with both B12 and IF on Schilling test indicates…

A

Malabsorption

53
Q

Three meds that cause aplastic anemia:

A

Chloramphenicol (antibiotic)
Carbamazepine
Sulfas

54
Q

Increased: PTT, PT, D-dimer
Decreased: fibrinogen, platelets, Factor V and VIII

Indicates…

A

DIC: disseminated intravascular coagulation

55
Q

What is the MOA of vinca alkaloids?

A

Inhibit microtubule formation in axons–M phase inhibitor

56
Q

Adverse effects of chemo agents:

Vincristine

A

Neurotox: finger numbness and tingling

57
Q

Adverse effects of chemo agents:

Doxorubicin

A

Cardiotox

58
Q

Adverse effects of chemo agents:

Bleomycin

A

Dry cough + DOE

59
Q

Adverse effects of chemo agents:

Alkylating agents

A

Tarry stool + fatigue

60
Q

Adverse effects of chemo agents:

6-MP

A

Jaundice + abdominal pain (cholestasis, hepatitis)

61
Q

Adverse effects of chemo agents:

Cyclophosphamide

A

Hemorrhagic cystitis

62
Q

How does hypocalcemia result from transfusion?

A

Citrate is found in packed red blood cells
Citrate chelates calcium
Hypocalcemia > paresthesias

63
Q

Golden yellow brown substance, granular or crystalline, that accumulates in hemolytic anemia

A

Hemosiderin

64
Q

Acute renal failure + thrombocytopenia in a child

A

HUS: hemolytic uremic syndrome

65
Q

Decreased: RBC, Hct, platelets
Increased: BT, LDH, BUN/Cr

Diagnosis?

A

HUS

66
Q

What is indicated by increased phosphate, K, uric acid and low Ca in a patient with leukemia/lymphoma?

A

Tumor lysis syndrome

67
Q

How can tumor lysis syndrome be prevented?

A

Hydration + hypouricemic drugs (alloprinol, rasburicase)

68
Q

What is the point of the HMP shunt?

A

Regenerate NADPH to maintaine glutathione in a reduced state

- This reduces oxidative stress

69
Q

The HMP shunt involves what enzyme in the oxidative step?

A

G6PD is the rate limiting enzyme

G6P to R5P, make 2 NADPH

70
Q

What splenic syndrome can occur in children with sickle cell?

A

Splenic sequestration crisis due to vasocclusion and splenic pooling of RBCs

71
Q

What ist he spleen like in an adult with sickle cell?

A

Functional asplenia from repeated infarcts

- Fibrosis and atrophy

72
Q

What is a microangiopathic hemolytic anemia that occurs along with AKI?

A

HUS

73
Q

What bugs cause HUS?

A

Shiga toxin producing organisms

E Coli O157:H7, Shigella

74
Q

How does folate deficiency cause megaloblastosis?

A

Decreased DNA synth because of lack of dTMP

- Give thymidine to activate the salvage pathway

75
Q

What is the translocation and oncogene in Follicular Lymphoma?

A

t(14,18), bcl2

76
Q

What is fibronectin?

A

Large glycoprotein produced by fibroblasts
Binds integrins, matrix collagen, glycosaminoglycans
Mediator of normal cell adhesion and migration that is lost in cancer

77
Q

What is the mutation in HbS and what does it cause?

A

Glut > val in beta chain
Sickling under hypoxic conditions
Aggregation of beta chains after unloading

78
Q

What is the mutation in paroxysmal nocturnal hemoglobinuria?

A

PIGA gene causes decreased G1P anchor

Increased complemented mediated hemolysis of RBCs

79
Q

How does phosphorylated Rb work?

A

pRB allows progression from G1 > S

This is constitutive in tumors

80
Q

What illnesses are associated with pure red cell aplasia?

A

Thymoma, lymphocytic leukemia, parvo B19

81
Q

What is the mitochondrial enzyme that activates caspasases, causing cell death through the intrinsic pathway?

A

Cytochrome C

82
Q

What is the fx of haptoglobin?

A

Will bind free Hbg in blood & be excreted

Low levels in sickle cell (bc lots of hemolysis)

83
Q

Leukemia that presents as mediastinal mass, with respiratory sx, dysphagia, SVC syndrome

A

T cell ALL

84
Q

Why do retics appear bluish on smear?

A

Have lost nucleus but retain residual rRNA

85
Q

Why is Heparin preferred to Warfarin in pregnancy?

A

Heparin is water soluble and therefore cannot cross placenta, while Warfarin in lipophilic and can cross

86
Q

What two substances come together to make porphyrin (1st step in heme synth)?

A

Glycine + Succinyl co A ( via ALA-S) > ALA

87
Q

Where does the first rxn in heme synth occur?

A

Mito of liver cells

88
Q

What deficiency is indicated in patients who get Warfarin Skin Necrosis?

A

Protein C def.

89
Q

AD predisposition to many cancers, esp. breast, brain, adrenal cortex tumors. Associated with p53.

A

Li-Fraumeni

90
Q

Tumor markers:

CEA

A

Colorectal & pancreatic

91
Q

Tumor markers:

CA 19-9

A

Pancreatic

92
Q

Tumor markers:

CA 125

A

OVarin

93
Q

Tumor markers:

AFP

A

HCC of liver

Nonseminoma germ cell tumors (ex. yolk sac)

94
Q

Tumor markers:

b-HCG

A

Choriocarcinoma
Trophoblastic tumor of pregnancy
Hydatidiform mole

UWorld (15 decks)

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