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USMLE Step 1 > Hematopoietic & Lymphoreticular > Flashcards

Hematopoietic & Lymphoreticular Flashcards

(123 cards)

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1
Q

t(9;22)

A

Chronic myelogenous leukemia (CML)

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2
Q

t(12;21)

A

B-cell acute lymhocytic leukemia (ALL)

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3
Q

t(14;18)

A

Follicular lymphoma

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4
Q

t(15;17)

A

Acute promyelocytic leukemia (APL)

Translocation of retinoic acid receptor alpha gene (chromosome 17) and promyelocytic leukemia gene (chromosome 15)

Auer rods (see picture)

Tx: all-trans retinoic acid

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5
Q

Composition of platelet granules

A

Dense granules: ADP, calcium

α granules: vWF, fibrinogen

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6
Q

Bernard-Soulier syndrome

A

Deficiency of GpIb decreases platelet-to-vWF adhesion

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7
Q

von Willebrand disease

A

AD deficiency in von Willebrand factor prevents platelet adhesion and inhibits intrinsic pathway (vWF carries/protects factor VIII)

Dx: negative ristocetin test, increased BT and PTT

Tx: DDAVP (releases stored vWF from endothelial cells)

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8
Q

Glanzmann’s thromboasthenia

A

Deficiency of GpIIb/IIIa prevents platelet-to-platelet aggregation

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9
Q

Ticlopidine

Clopidogrel

Prasugrel

Ticalgrelor

A

Inhibits ADP-induced expression of GpIIb/IIIa inhibiting platelet aggregation

Indicated for ACS, coronary stending

SA: neutropenia (ticlopidine), TTP/HUS

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10
Q

Abciximab

Eptifibatide

Tirofiban

A

Inhibits GpIIb/IIIa directly preventing platelet aggregation

Indicated for unstable angina, angioplasty

SA: bleeding, thrombocytopenia

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11
Q

Vitamin K associated factors

A

II, VII, IX, X, C, S

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12
Q

Factor V Leiden

A

Mutant factor V is resistant to degradation by activated protein C → pro-thrombotic

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13
Q

β-thalassemia

A

Decreased synthesis of β globin chain due to point mutation in splice sites and promoter sequences (normally 2 genes on chromosome 11)

β-thalassemia minor (heterozygote): can be asymptomatic

β-thalassemia major (homozygote): severe anemia, HbA2, HbF, marrow expansion, extramedullary hematopoiesis

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14
Q

α-thalassemia

A

Decreased synthesis of α globin chain due to gene deletion (normally 4 genes on chromosome 16)

cis deletion in Asian populations (more severe disease in offspring)

Trans mutation in African populations

4 deletions: Hb Barts (γ2γ2), fetal hydrops

3 deletions: HbH (β2β2)

1-2 deletions: asymptomatic

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15
Q

Paroxysmal nocturnal hemoglobinuria

A

Acquired defect in myeloid stem cells resulting in absent glycosylphosphatidylinositol (GPI) anchoring protein → inability to bind DAF allows complement-mediated damage to RBCs, WBCs, and platelets

Hemolysis occurs at night when shallow breathing → acidosis → complement activation

Triad: Coombs - hemolytic anemia, pancytopenia, venous thrombosis

Labs: lack of CD55/59 (DAF)

Tx: eculizumab

A/w acute leukemias

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16
Q

G6PD deficiency

A

X-linked recessive disorder characterized by decreased half-life of glucose-6-phosphate dehydrogenase → reduced production of NADPH → reduced glutathione regeneration → RBCs become susceptible to oxidative stress

Findings: Heinz bodies, bite cells

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17
Q

Hereditary spherocytosis

A

Inherited defect of RBC cytoskeleton-membrane tethering proteins (ankyrin, spectrin, band 3.1, protein 4.2)

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18
Q

Autoimmune hemolytic anemia

A

Warm agglutinin (IgG): SLE, CLL, drugs

Cold agglutinin (IgM): CLL, Mycoplasma pneumonia, infectious mononucleosis

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19
Q

Immune thrombocytopenic purpura (ITP)

A

Autoantibody mediated destruction of platelets (typically IgG directed against GPIb or GPIIb/IIIa)

Increased megakaryocytes on bone marrow biopsy

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20
Q

Thrombotic Thrombocytopenic Purpura (TTP)

A

Deficiency of ADAMTS13 → reduced degradation of vWF multimers → accumulation of multimers promotes platelet activation and aggregation → microthrombi

Characterized by neurologic and renal symptoms, fever, thrombocytopenia, and microangiopathic hemolytic anemia

Tx: exchange transfusion and steroids

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21
Q

Disseminated intravascular coagulation (DIC)

A

Widespread activation of clotting leads to deficiency in clotting factors

Causes: sepsis (gram -), trauma, obstetric complications, acute pancreatitis, malignancy, nephrotic syndrome, transfusion

Labs: increased BT, PT, PTT, and fibrin split products (D-dimer), decreased platelets, fibrinogen, factors V and VIII

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22
Q

Hemophilia

A

Hemophilia A: defect in factor VIII

Hemophilia B: defect in factor IX

Both increase PTT and cause hemarthroses, easy bruising

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23
Q

Protein C or S deficiency

A

Reduced ability to inactivate factors V and VIII

Increased risk of thrombotic skin necrosis with hemorrhage following administration of warfarin

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24
Q

Which coagulation factors are activated by FVIIa?

A

IX, X

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25
Which coagulation factors are activated by FIIa (thrombin)?
XI, VIII, V, XIII, activates platelets
26
Which coagulation factors require Vitamin K?
II (thrombin), VII, IX, X (2-7-9-10), protein C, and protein S
27
Which coagulation factors are found in cryoprecipitate?
I (fibrinogen), VIII, XIII, vWF, fibronectin
28
Which coagulation factor is produced outside of the liver in addition to within the liver?
Factor VIII is produced by endothelial cells
29
Which coagulation factors are inhibited by antithrombin III?
Inhibits thrombin and serine protease inhibitors (serpins) Potentiated by heparin XII, XI, IX, X, II (thrombin) 9-10-11-12 + 2 (thrombin)
30
Which coagulation factors are inactivated by the protein C and S complex?
Factors Va and VIIIa
31
What is Bernard-Soulier syndrome?
Bleeding disorder caused by genetic deficiency of GPIb --\> prevents platelet adhesion
32
What is Glanzmann thrombasthenia?
Bleeding disorder caused by genetic deficiency of GPIIb/IIIa --\> prevents platelet aggregation
33
What is HbH?
α-Thalassemia (three gene deletion) β2β2 hemoglobin
34
What is HbF?
Fetal hemoglobin α2γ2
35
What is HbA?
Normal adult hemoglobin α2β2
36
What is Hb Barts?
α-Thalassemia (four gene deletion) γ2γ2
37
What is HbA2?
Increased in β-thalassemia α2δ2
38
How many genes encode hemoglobin α chains and on which chromosome are they?
4 genes on chromosome 16
39
How many genes encode hemoglobin β chains and on which chromosome are they?
2 genes on chromosome 11
40
Which anemias results in extramedullary hematopoiesis (e.g. hematopoiesis moves into skull --\> crew-cut x-ray, facial bones --\> chimpmunk facies, liver, spleen --\> hepatosplenomegaly)?
β-thalassemia major Sickle cell anemia
41
How do you diagnose specific thalassemias?
Gel electrophoresis α-thalassemia: HbH (β2β2) or Hb Barts (γ2γ2) β-thalassemia minor: reduced HbA (α2β2), increased HbA2 (α2δ2), increased HbF (α2γ2) β-thalassemia major: no HbA (α2β2), increased HbA2 (α2δ2), increased HbF (α2γ2)
42
Where is iron, folate, and vitamin B12 absorbed?
Duodenum, jejunum, and ileum, respectively
43
What is anisocytosis?
Variations in the size of RBCs
44
What is poikilocytosis?
Variations in the shape of RBCs
45
What are Howell-Jolly bodies?
Fragments of DNA left in RBCs that are usually removed by the spleen Associated with hereditary spherocytosis (treated with splenectomy), sickle cell anemia (autosplenectomy)
46
What is the difference in the gene mutation for sickle cell anemia and hemoglobin C?
A glutamic acid residue is replaced by valine in sickle cell anemia and lysine in hemoglobin C
47
What is CD55?
CD55 is decay accelerating factor (DAF) which is absent in paroxysmal nocturnal hemoglobinuria
48
What is a Heinz body?
Precipitated hemoglobin due to oxidative stress G6PD deficiency Heinz bodies are removed by splenic macrophages resulting in bite cells
49
Which diseases are associated with target cells?
Thalassemias Hemoglobinopathies (sickle cell, HbC)
50
Which neoplasia is associated with t(12;21)?
B-cell acute lymphoblastic leukemia (B-ALL) Good prognosis, more common in children
51
Which neoplasia is associated with t(9;22)?
Philadelphia chromosome --\> BCR-ABL fusion protein --\> increased tyrosine kinase activity --\> treat with imantinib B-cell acute lymphoblastic leukemia (B-ALL); poor prognosis, more common in adults Chronic myeloid leukemia (CML)
52
Which neoplasia is associated with t(15;17)?
Acute promyelocytic leukemia The retinoic acid receptor (RAR) on chromosome 17 is translocated to chromosome 15 RAR disruption blocks maturation and promyelocytes accumulate (Auer rods) Treat with all-trans-retinoic acid (ATRA) --\> vitamin A derivative
53
Which neoplasia is associated with t(14;18)?
Follicular lymphoma Diffuse large B-cell lymphoma (most common type of NHL) BCL2 on chromosome 18 translocates to the Ig heavy chain locus --\> overexpression of Bcl2 (inhibits apoptosis)
54
Which neoplasia is associated with t(11;14)?
Mantle cell lymphoma Cyclin D1 gene on chromosome 11 translocates to Ig heavy chain locus --\> overexpression of cyclin D1 --\> promotes G1/S cell cycle transition --\> neoplastic proliferation CD5+
55
Which neoplasia is associated with t(8;14)?
Burkitt lymphoma (B cells CD20+) C-MYC on chromosome 8 translocates to Ig heavy chain locus --\> overexpression of c-myc oncogene Associated with EBV African form → jaw Sporadic form → abdomen, pelvis Histology: “starry-sky”
56
Which neoplasias are associated with lytic bone lesions?
Adult T-cell lymphoma Multiple myeloma
57
Which neoplasias are CD5+ and CD20+
CLL/SLL Mantle cell lymphoma Langerhans cell histiocytosis
58
What diseases are associated with ineffective hematopoiesis?
Megaloblastic anemia Hemolytic anemia β-Thalassemia
59
What diseases are associated with tear-drop RBCs?
Extramedullary hematopoiesis (β-Thalassemia, sickle cell anemia) Myelophthisic process Myelofibrosis
60
What does tissue factor pathway inhibitor inhibit?
TF-VIIa complex and Xa
61
How do endothelial cells inhibit platelet activation and aggregation?
PGI2, NO, ecto-ADPase
62
Melphalan
Nitrogen mustard derivative bifunctional alkylating agent Alkylates DNA resulting in breaks and cross-linking
63
Chlorambucil
Nitrogen mustard derivative bifunctional alkylating agent Alkylates DNA resulting in breaks and cross-linking
64
Cyclophosphamide Ifosfamide
Nitrogen mustard derivative bifunctional alkylating agent Alkylates DNA resulting in cross-linking at guanine N-7 Must be activated by the liver Side effects: _hemorrhagic cystitis_, myocardial necrosis, myelosuppression Partially prevented with _mesna_
65
Carmustine
Nitrosurea alkylating agent Alkylates DNA resulting in breaks and cross-linking Can penetrate CNS Can be used as implantable wafer
66
Lomustine
Nitrosurea alkylating agent Alkylates DNA resulting in breaks and cross-linking Can penetrate CNS
67
Cisplatin
Platinum alkylating agent Cross-links DNA Side effects: nephrotoxicity (prevent with amifostine and chloride diuresis), ototoxicity
68
Oxaliplatin
Platinum alkylating agent Cross-links DNA Side effects: neurotoxicity Treats colorectal cancer
69
Carboplatin
Platinum alkylating agent Cross-links DNA
70
Procarbazine
Nonspecific alkylating agent Treats non-Hodgkin lymphoma Side effects: MAOI (eat less tyramine)
71
Vincristine
Binds to tubulin, prevents polymerization into microtubules, arrests cells in M-phase Side effects: neurotoxicity (areflexia, peripherla neuritis), paralystic ileus
72
Vinblastine
Binds to tubulin, prevents polymerization into microtubules, arrests cells in M-phase Side effects: neurotoxicity, mucositis, _myelosuppression_
73
Paclitaxel
Promotes polymerization and stability of microtubules and inhibits disassembly (prevents anaphase) Side effects: neutropenia, mucositis, alopecia
74
Docetaxel
Promotes polymerization and stability of microtubules and inhibits disassembly Affect G2/M-phase Side effects: neutropenia, mucositis
75
Ixabepilone
Binds β-tubulin and promotes polymerization and stability of microtubules and inhibits disassembly Affect G2/M-phase Side effects: neutropenia, cardiotoxicity, peripheral neuropathy
76
Etoposide
Inhibits topoisomerase II and increases DNA degradation Affects G2/S-phase Side effects: myelosuppression
77
Teniposide
Inhibits topoisomerase II and increases DNA degradation Affects G2/S-phase Side effects: myelosuppression
78
Irinotecan
Inhibits topoisomerase I and prevents DNA unwinding and replication Affects S-phase Side effects: neutropenia, diarrhea
79
Topotecan
Inhibits topoisomerase I and prevents DNA unwinding and replication Affects S-phase Side effects: neutropenia, diarrhea
80
Daunorubicin
Anthracycline antibiotic: 1) intercalates in DNA inhibiting DNA and RNA polymerase 2) induces free radical formation which breaks DNA 3) inhibits DNA topoisomerase II Side effects: cardiotoxicity (dilated cardiomyopathy from free radical damage), myelosuppression, alopecia Dexrazoxane (iron chelator) may reduce cardiac damage
81
Doxorubicin
Anthracycline antibiotic: 1) intercalates in DNA inhibiting DNA and RNA polymerase 2) induces free radical formation which breaks DNA 3) inhibits DNA topoisomerase II Side effects: cardiotoxicity (dilated cardiomyopathy from free radical damage), myelosuppression, alopecia Dexrazoxane (iron chelator) may reduce cardiac damage
82
Dexrazoxane
Iron chelator inhibits free radical formation and reduces cardiac damage associated with daunorubicin and doxorubicin (anthracycline antibiotics) Affects G2-phase
83
Bleomycin
Forms DNA-bleomycin-Fe complex that is oxidized --\> free radicals break DNA Affects G2-phase Side effects: _pulmonary fibrosis, hyperpigmentation of hands_, mucositis
84
Methotrexate
Competitively inhibits dihydrofolate reductase: 1) prevents formation of dTMP 2) decreases synthesis of purine ring structure Affects S-phase Side effects: myelosuppression, mucositis, hepatotoxicity (high dose), abortifacient Can use leucovorin (folinic acid) to reverse side effects
85
5-fluorouracil
Pyrimidine analogue converted to 5-FdUMP which inhibits thymidylate synthase; 5-FdUTP incorporated into RNA resulting in defective transcripts Side effects: myelosuppression, photosensitivity Rescue with uridine
86
Capecitabine
5-FU prodrug whose final set of activation is catalyzed by thymidine phosphorylase, and enzyme elevated in tumor cells
87
Cytarabine
Cytidine analogue converted to Ara CTP which inhibits DNA polymerase and causes DNA chain termination when incorporated Causes pancytopenia
88
Gemcitabine
Cytidine analogue inhibits DNA polymerase and ribonucleotide reductase
89
Azacitidine
Pyrimidine nucleoside analog gets incorporated into RNA and inhibits processing and function Treats myelodysplastic syndrome
90
6-mercaptopurine
Purine analogue: 1) inhibits de novo purine synthesis 2) competitively inhibits biosynthetic reactions 3) incorporated into DNA and RNA Side effects: myelosuppression, hepatotoxicity, increased toxicity with allopurinal (metabolized by xanthine oxidase)
91
6-thioguanine
Purine analogue: 1) inhibits de novo purine synthesis 2) competitively inhibits biosynthetic reactions 3) incorporated into DNA and RNA Side effects: myelosuppression, hepatotoxicity
92
Asparaginase
Catalyzes conversion of asparagine to aspartate to inhibit protein synthesis (asparagine levels are low in tumor cells) Side effects: reduced protein synthesis (clotting factors, insulin, albumin), hypersensitivity reactions, hepatotoxicity
93
Imatinib mesylate
Competitively blocks ATP-binding side of c-abl tyrosine kinase Inhibits c-kit and PDGF-associated tyrosine kinases Treats CML and Ph+ ALL Side effects: myelosuppression and CHF
94
Erlotinib
Inhibits EGF receptor-associated tyrosine kinase Side effects: CHF
95
Gefitinib
Inhibits EGF receptor-associated tyrosine kinase Side effects: CHF
96
Vemurafenib
Inhibits B-raf serine-threonine kinase with V600E mutation Treats melanoma
97
Cetuximab
Antibody against EGF receptor
98
Trastuzumab (Herceptin)
Inhibitor of HER2 tyrosine kinase receptor (extracellular domain) SA: cardiotoxicity
99
Bevacizumab
Antibody against VEGF --\> inhibits angiogenesis
100
Rituximab
Anti-CD20 antibody that promotes complement-mediated lysis of malignant B cells May cause reactivation of HBV, increases risk of progressive multifocal leukoencephalopathy (a/w JC virus)
101
Ipilimumab
Antibody against CTLA-4 that blocks inhibitory activity Treats malignant melanoma
102
Pembrolizumab
Bind to PD-1 receptor on T cells
103
Gamtuzumab ozogamicin
Antibody against CD33 (myeloid surface antigen)
104
Bortezomib
Proteasome inhibitor Treats multiple myeloma and mantle cell lymphoma
105
all-trans-retinoic acid
Treats promyelocytic leukemia by causing blasts to mature
106
Heparin
Anticoagulant Activates antithrombin --\> inactivates thrombin and factor Xa Indicated for PE, ACS, MI, DVT; safe during pregnancy (highly water soluble so doesn't cross the placenta) Monitor with PTT Can reverse with protamine sulfate Can cause heparin-induce thrombocytopenia --\> IgG develops against heparin bound to platelet factor 4 --\> activates platelets --\> thrombosis and thrombocytopenia
107
Warfarin (Coumadin)
Inactivates vitamin K epoxide reductase --\> reduced gamma-carboxylation of factors II, VII, IX, X, proteins C and S Monitor with PT/INR Contraindicated in pregnancy (highly lipophilic and crosses placenta) May cause tissue necrosis Give vitamin K or FFP for reversal
108
Smudge cells
Chronic lymphocytic leukemia (CLL)/Small lymphocytic lymphoma (SLL)
109
TRAP +
Hairy cell leukemia
110
Enoxaparin
Low-molecular weight heparin (acts more on factor Xa) Better bioavailability, 2-4 times longer half-life, can be administed subcutaneously and without laboratory monitoring
111
Dalteparin
Low-molecular weight heparin (acts more on factor Xa) Better bioavailability, 2-4 times longer half-life, can be administed subcutaneously and without laboratory monitoring
112
Argatroban
Inhibits thrombin directly (derived from leeches) Indicated for anticoagulation in patients with HIT
113
Bivalirudin
Inhibits thrombin directly (derived from leeches) Indicated for anticoagulation in patients with HIT
114
Apixaban
Directly inhibits Xa Indicated for stroke prophylaxis in patients with afib Don't require coagulation monitorng
115
Rivaroxaban
Directly inhibits Xa Indicated for stroke prophylaxis in patients with afib, treatment and prophylaxis for DVT and PE Don't require coagulation monitorng
116
Alteplase (tPA) Reteplase (rPA) Tenecteplase (TNK-tPA)
Aids conversion of plasminogen to plasmin which cleaves thrombin and fibrin clots (↑ PT and PTT) Indicated for early MI, stroke, severe PE SA: bleeding Treat toxicity with aminocaproic acid (fibrinolytic inhibitor), FFP, cryoprecipitate
117
Azathioprine
Purine analogue: 1) inhibits de novo purine synthesis 2) competitively inhibits biosynthetic reactions 3) incorporated into DNA and RNA Side effects: myelosuppression, hepatotoxicity, increased toxicity with allopurinal (metabolized by xanthine oxidase)
118
Dactinomycin
Intercalates in DNA SA: myelosupression
119
Hydroxyurea
Inhibits ribonucleotide reductase to decrease DNA synthesis
120
Imantinib
Tyrosine kinase inhibitor of brc-abl (Philadephia chromosome fusion gene in CML) and c-kit Indicated for CMG, GI stromal tumors SA: fluid retention
121
Common chemotoxicities
122
Rasburicase
Recombinant version of urate oxidase, and enzyme that converts uric acid into more soluble metabolites to prevent hyperuricemia from tumor lysis syndrome
123
Pure reb cell aplasia
Associated with parvovirus B19 infection, thymomas, or lymphocytic leukemias

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