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Heme/Onc Random Facts Flashcards

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1
Q

What is the life span of a thrombocytes.

A

8-10 days

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2
Q

What are the granules in platelets?

A

Dense granules-contain ADP and Ca, alpha-granules contain vWF and fibrinogen

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3
Q

Where is 1/3 of the platelet pool stored

A

In the spleen

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4
Q

What are the granules in neutrophils?

A

Specific granules contain ALP, collagenase, lysozyme, and lactoferrin. Azurophilic granules (lysosomes) contain proteases, acid phosphatase, myeloperoxidase, and beta-glucuronidase

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5
Q

How are macrophages involved in septic shock?

A

Lipid A from bacterial LPS binds CD 14 on macrophages to initiate septic shock

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6
Q

What activates macrophages?

A

Gamma-interferon

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7
Q

What does eosinophilic mean?

A

Pink

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8
Q

What does basophilic mean?

A

Blue/purple

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9
Q

What are the causes of eosinophilia?

A

Neoplasia, asthma, allergy, chronic adrenal insufficiency, parasites

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10
Q

What die the granules in basophils contain?

A

Heparin and histamine. Leukotrienes synthesized and released on demand

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11
Q

What can basophilia be a sign of?

A

Myeloproliferative disease, particularly CML

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12
Q

What’s the universal donor and universal recipient of plasma?

A

AB is the universal plasma donor (because no antibodies in their plasma), O is the universal recipient of plasma (because antibodies have nothing to react with)

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13
Q

How does warfarin work?

A

It inhibits vitamin K episode reductase, which is needed to activate the vitamin K dependent clotting factors

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14
Q

MOA of antithrombin

A

Inhibits activated forms of factors 2, 7, 9, 10, 11, and 12. Principle targets of antithrombin: thrombin and factor Xa

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15
Q

What does tPA do?

A

Converts plasminogen to plasmin

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16
Q

MOA of protein C and S

A

Protein C is converted to activated protein C by thrombin-thrombin insulin complex on endothelial cells, APC is activated by protein S to cleave and inactivate factor Va and VIIIa

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17
Q

MOA of clopidogrel, prasugrel, and ticlipidine

A

Inhibit ADP-induced expression of GpIIb/IIIa

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18
Q

MOA of abciximab, eptifibatide, and tirifiban

A

Inhibit GpIIb/IIIa directly

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19
Q

What is Bernard Soulier syndrome

A

A deficiency of GpIb

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20
Q

What is Glazmann thrombasthenia

A

Deficiency of GpIIb/IIIa

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21
Q

When might you see a failure of agglutination with ristocetun assay?

A

Von Willebrand disease and Bernard-Soulier syndrome

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22
Q

When might you see acanthocytes (“spur cells”)

A

Liver disease, abetalipoproteinemia

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23
Q

When might you see target cells

A

HbC disease, Asplenia, liver disease, thalassemia

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24
Q

What are Heinz bodies?

A

Oxidation do Hb-SH groups to -S–S- causing hemoglobin precipitation with subsequent damage to RBC membrane causing bite cells

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25
What might you see in a reticulocyte in Wright-Giemsa stain
Blue staining, represents the residual ribosomal RNA
26
Where does iron absorption occur?
In the duodenum and proximal jejunum so bypassing this in a gastrojejunostomy results in iron deficiency anemia
27
What is associated with koilonychia?
(Spoon nails) iron deficiency anemia
28
What happens with a four allele deletion in the alpha-globin gene?
Hemoglobin Barts (4 Gamma chains) forms. Is incompatible with life
29
What is hemoglobin H?
Four beta chains
30
What ethnicities are associated with alpha and beta thalassemia?
Alpha- Asian and African, beta-Mediterranean
31
How is beta thalassemia minor diagnosed?
Increased HbA2 on electrophoresis
32
MOA of lead poisoning
Inhibits ferrochatase and ALA dehydratase causing decreased heme synthesis and increased RBC protoporphyrin
33
Signs and symptoms of lead poisoning
Lead lines on gingivae and metaphysics of long bones on xray, encephalopathy and erythrocytes basophilic stippling, abdominal colic and sideroblastic anemia, drops-wrist and foot
34
What causes hereditary sideroblastic anemia?
X-linked defect in delta-ALA synthase gene
35
Causes of sideroblastic anemia
Genetic, acquired (myelodysplastic syndromes) and reversible (alcohol, lead, vitamin B6 deficiency, copper deficiency, isoniazid)
36
What is orotic aciduria?
AR defect in UMP synthase, presents as FTT, developmental delay, and megaloblastic anemia refractory to folate and B12. NO hyperammonemia (vs ornithine transcarbamylase deficiency)
37
Tx of orotic aciduria
Uridine monophosphate to bypass mutated enzyme
38
What might have an abnormal eosin-5-maleimide binding test
Hereditary spherocytosis
39
Pyruvate kinase deficiency
AR, causes decreased ATP causing rigid RBCs and extra vascular hemolytic anemia. Hyperplasia of the red pulp of the spleen
40
Mutation in HbC disease
Glutamic acid to lysine in beta-globin, causes extravascular hemolysis
41
Triad, labs, and tx in PNH
Triad: Coombs - hemolytic anemia, pancytopenia, and venous thrombosis, labs: CD55/59 neg RBCs on flow cytometry, tx: eculizumab
42
Warm agglutinin
Chronic anemia seen in SLE and CLL and with certain drugs (alpha-methyldopa), IgG
43
Cold agglutinin
IgM, acute anemia triggered by cold, seen in CLL, mycoplasma and infectious mononucleosis
44
What is the direct Coombs test
Anti-Ig antibody added to patient's blood. RBCs agglutinate if RBCs are coated with Ig
45
Indirect Coombs
Normal RBCs added to patients serum. If serum has anti-RBC surface Ig, RBCs agglutinate when Coombs reagent is added
46
Effect of corticosteroids on neutrophils, eosinophils, and lymphocytes
Neutrophilia from demargination, eosinopenia from sequestration in lymph nodes, lymphopenia from apoptosis
47
Enzyme inhibited in acute intermittent porphyria
Porphibiligen deaminase
48
Enzyme deficient in porphyria Cutanea tarda
Uropirphyrinogen decarboxylase
49
Pathogenesis of ttp
Inhibition or deficiency of ADAMTS13 (vWF metalloprotease) causes large vWF multimers which causes increased platelet adhesion, which causes platelet aggregation and thrombosis
50
Pentad of ttp
Neurologic and renal sx, fever, thrombocytopenia, and MAHA
51
When might you give cryoprecipitate
For coagulation factor deficiencies involving fibrinogen and factor VIII
52
High leukocyte alkaline phosphatase means what?
Leukemoid reaction
53
What are Dohle bodies?
Light blue (basophilic) peripheral granules in neutrophils seen in a leukemoid reaction
54
What is Hodgkin lymphoma associated with?
EBV
55
Cell markers in reed Sternberg cells
CD15 and CD30
56
Genetics of Burkitt lymphoma
T(8;14) translocation of c-myc and heavy-chain Ig
57
What is commonly involved in diffuse large B cell lymphoma
Waldeyer's ring (oropharyngeal lymphoid tissue) and GI tract
58
Genetics of follicular lymphoma
T(14;18)-translocation of heavy-chain Ig and Bcl-2. Cleaved nuclei on histo
59
Genetics of mantle cell lymphoma
t(11;14)-translocation of cyclin D1 and heavy chain Ig
60
Cerebriform nuclei
Mycoses fungoides/Sezary syndrome
61
Rouloux formation
Multiple myeloma
62
Neutrophils with bilobed nuclei seen after Chen
Pseudo-Pelger-Huet anomaly
63
Child with lytic bone lesions and skin rash or as recurrent otitis media with a mass involving mastoid bone
Langerhans cell histiocytosis. Calls express S100 (mesodermal origin) and CD1a. Bit beck granules (tennis rackets on EM)
64
T(12;21)
Better prognosis ALL
65
Smudge cells
Small lymphocytic lymphoma/CLL
66
Tx for CML
Imatinib (a small molecule inhibitor of the bcr-abl tyrosine kinase)
67
Mechanism of HIT
Development of IgG Ab against heparin bound platelet factor 4. The antibody-heparin-PF4 complex activates platelets causing thrombosis and thrombocytopenia
68
Direct thrombin inhibitors
Argatroban, bivalirudin, dabigatran
69
MOA of cilostazol, dipyridamole
Phosphodiesterase III inhibitor. Increases cAMP in platelets, resulting in inhibition of platelet aggregation; vasodilators
70
MOA of hydroxyurea for cancer
Inhibits ribonucleotide reductase
71
Clinical use for cytarabine
Leukemias (AML), lymphoma
72
Use and toxicity of Busulfan
CML. Also used to ablate patient's bone marrow before bone marrow transplantation. Toxicity: severe myelosuppression

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