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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
What differentiates PV from secondary polycythemia?
PV: all 3 cell lines increased Secondary: only RBC increased
26
What are the causes of secondary polycythemia?
Hypoxia | EPO-tumor
27
...is the central regulator of iron, produced by the liver.
Hepcidin
28
What is the MOA of hepcidin?
When the body has a lot of iron, hepcidin binds ferroportin and stop iron absorption Ferroportin is then degraded
29
In hypoxia, hepcidin is (increased/decreased).
Decreased
30
What anticoagulant can be used in pregnancy
HEPregnancy not Warfarin
31
Inheritance of G6PD deficiency
X linked recessive
32
Disease characterized by defective binding of RBC cytoskeleton to plasma membrane due to mutations in structural proteins
Hereditary Spherocytosis
33
What structural proteins are mutated in Hereditary Spherocytosis?
Ankyrin, band 3, spectrin
34
Small RBC with no central pallor and increased MCHC
Spherocytes
35
Inheritance of hereditary spherocytosis
Autosomal dominant
36
Non-jaw presentation of Burkitt's
GI mass
37
Function of c-myc
TF that controls cell proliferation, differentiation, apoptosis
38
The mutation that causes beta-thal is a defect in...
mRNA transcription, processing, translation NOT folding
39
Beta-thal causes what kind of anemia?
Microcytic
40
Increased HbgF, HbgA2, and target cells indicate
Beta-thal
41
CD14 is a marker for what lineage?
Monocyte/macrophage
42
What is the Haldane effect?
Binding O2 in lungs drives Hbg to release H+ (as H2O) and CO2
43
What is the Bohr effect?
Increased CO2 and H+ in the periphery facilitates O2 unloading
44
What disease is caused by a deficiency of HMB synthase?
Acute intermittent porphyria
45
What substances build up in acute intermittent porphyria?
d-Ala and porphobilinogen > cause symptoms
46
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
Acute Intermittent Porphyria Note: no skin photosensitivity
47
In what disease to RBCs show osmotic fragility on exposure to saline?
Hereditary spherocytosis - Increased membrane lysis in hypotonic saline or NaCL
48
What is a secondary improvement post-splenectomy in patients with Hereditary Spherocytosis?
Decreased gallstones - Decreased hemolysis - Decreased bili
49
What is the action of heparin?
Bind and activate antithrombin III
50
Increased urinary excretion of radio-labeled B12 on Schilling test indicates...
B12 deficiency in diet
51
Increased urinary excretion of radio-labeled B12 on Schilling test only after addition of IF indicates...
Pernicious anemia
52
Low excretion with both B12 and IF on Schilling test indicates...
Malabsorption
53
Three meds that cause aplastic anemia:
Chloramphenicol (antibiotic) Carbamazepine Sulfas
54
Increased: PTT, PT, D-dimer Decreased: fibrinogen, platelets, Factor V and VIII Indicates...
DIC: disseminated intravascular coagulation
55
What is the MOA of vinca alkaloids?
Inhibit microtubule formation in axons--M phase inhibitor
56
Adverse effects of chemo agents: Vincristine
Neurotox: finger numbness and tingling
57
Adverse effects of chemo agents: Doxorubicin
Cardiotox
58
Adverse effects of chemo agents: Bleomycin
Dry cough + DOE
59
Adverse effects of chemo agents: Alkylating agents
Tarry stool + fatigue
60
Adverse effects of chemo agents: 6-MP
Jaundice + abdominal pain (cholestasis, hepatitis)
61
Adverse effects of chemo agents: Cyclophosphamide
Hemorrhagic cystitis
62
How does hypocalcemia result from transfusion?
Citrate is found in packed red blood cells Citrate chelates calcium Hypocalcemia > paresthesias
63
Golden yellow brown substance, granular or crystalline, that accumulates in hemolytic anemia
Hemosiderin
64
Acute renal failure + thrombocytopenia in a child
HUS: hemolytic uremic syndrome
65
Decreased: RBC, Hct, platelets Increased: BT, LDH, BUN/Cr Diagnosis?
HUS
66
What is indicated by increased phosphate, K, uric acid and low Ca in a patient with leukemia/lymphoma?
Tumor lysis syndrome
67
How can tumor lysis syndrome be prevented?
Hydration + hypouricemic drugs (alloprinol, rasburicase)
68
What is the point of the HMP shunt?
Regenerate NADPH to maintaine glutathione in a reduced state | - This reduces oxidative stress
69
The HMP shunt involves what enzyme in the oxidative step?
G6PD is the rate limiting enzyme | G6P to R5P, make 2 NADPH
70
What splenic syndrome can occur in children with sickle cell?
Splenic sequestration crisis due to vasocclusion and splenic pooling of RBCs
71
What ist he spleen like in an adult with sickle cell?
Functional asplenia from repeated infarcts | - Fibrosis and atrophy
72
What is a microangiopathic hemolytic anemia that occurs along with AKI?
HUS
73
What bugs cause HUS?
Shiga toxin producing organisms E Coli O157:H7, Shigella
74
How does folate deficiency cause megaloblastosis?
Decreased DNA synth because of lack of dTMP | - Give thymidine to activate the salvage pathway
75
What is the translocation and oncogene in Follicular Lymphoma?
t(14,18), bcl2
76
What is fibronectin?
Large glycoprotein produced by fibroblasts Binds integrins, matrix collagen, glycosaminoglycans Mediator of normal cell adhesion and migration that is lost in cancer
77
What is the mutation in HbS and what does it cause?
Glut > val in beta chain Sickling under hypoxic conditions Aggregation of beta chains after unloading
78
What is the mutation in paroxysmal nocturnal hemoglobinuria?
PIGA gene causes decreased G1P anchor | Increased complemented mediated hemolysis of RBCs
79
How does phosphorylated Rb work?
pRB allows progression from G1 > S | This is constitutive in tumors
80
What illnesses are associated with pure red cell aplasia?
Thymoma, lymphocytic leukemia, parvo B19
81
What is the mitochondrial enzyme that activates caspasases, causing cell death through the intrinsic pathway?
Cytochrome C
82
What is the fx of haptoglobin?
Will bind free Hbg in blood & be excreted | Low levels in sickle cell (bc lots of hemolysis)
83
Leukemia that presents as mediastinal mass, with respiratory sx, dysphagia, SVC syndrome
T cell ALL
84
Why do retics appear bluish on smear?
Have lost nucleus but retain residual rRNA
85
Why is Heparin preferred to Warfarin in pregnancy?
Heparin is water soluble and therefore cannot cross placenta, while Warfarin in lipophilic and can cross
86
What two substances come together to make porphyrin (1st step in heme synth)?
Glycine + Succinyl co A ( via ALA-S) > ALA
87
Where does the first rxn in heme synth occur?
Mito of liver cells
88
What deficiency is indicated in patients who get Warfarin Skin Necrosis?
Protein C def.
89
AD predisposition to many cancers, esp. breast, brain, adrenal cortex tumors. Associated with p53.
Li-Fraumeni
90
Tumor markers: CEA
Colorectal & pancreatic
91
Tumor markers: CA 19-9
Pancreatic
92
Tumor markers: CA 125
OVarin
93
Tumor markers: AFP
HCC of liver Nonseminoma germ cell tumors (ex. yolk sac)
94
Tumor markers: b-HCG
Choriocarcinoma Trophoblastic tumor of pregnancy Hydatidiform mole

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