UWorld 2017 Flashcards

1
Q

Complication of Chronic Granulomatous Disease

A

Recurrent suppurative infections with Catalase-positive organisms

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2
Q

2 consequences of C1 deficiency

A

Increased susceptibility to infection with encapsulated bacteria
Predisposes to SLE

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3
Q

C3 deficiency predisposes to

A

Recurrent infections with encapsulated bacteria

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4
Q

C5-9 deficiency leads to

A

Infections with N. meningitidis and N. gonorrhoeae

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5
Q

Defect in type I interferon release leads to

A

Increased susceptibility to viral infections

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6
Q

5 causes of pancytopenia without splenomegaly

A
Aplastic anemia
Vit B12 deficiency anemia
Folic acid deficiency anemia
Acute leukemias
Certain forms of myelodysplastic syndrome
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7
Q

Primary thrombotic microangiopathy syndromes

A

Platelet activation + diffuse microthrombosis in arterioles/capillaries
Hemolytic anemia w/ schistocytes
Thrombocytopenia
Organ injury (brain, kidney, heart)

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8
Q

Pentad of Thrombocytopenic thrombotic purpura

A
Fever
Neuro symptoms (progr lethargy)
Renal failure
Anemia
Thrombocytopenia
In the setting of GI illness + normal TP, aPTT, BT
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9
Q

Vinca alkaloids

A

Act on M phase of cell cycle

Neurotoxicity (peripheral neuropathy)

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10
Q

Sickle cell anemia

A

Point mutation: glutamic acid instead of valine (in beta-chain of Hb)
Exertional dyspnea
Pneumonia w/ life-threatening acute chest syndrome
Recurrent abdo pain

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11
Q

Heparin-induced thrombocytopenia

A

HIT treated w/ direct thrombin inhibitors (argatroban)

Avoid HMWH + LMWH

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12
Q

Chronic myeloproliferative disorders

A

Mutation in JAK2 (cytoplasmic tyrosine kinase)

Persistent activation of signal transducers + activators of transcription (STAT) proteins

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13
Q

Side effects of Dapsone (prophylactic ttt of HIV+ for pneumocystic jiroveci pneumonia in case of TMP-SMX allergy)

A

Fever, rash, methemoglobinemia, oxidative stress

If G6PD deficiency, hemolytic anemia

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14
Q

Immunophenotyping of lymphoblasts in precursors B-ALL and T-ALL

A

B-ALL: TdT+, CD10+, CD19+

T-ALL: TdT+, CD1a+, CD2, CD3, CD4, CD5, CD7, CD8

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15
Q

Intravascular hemolytic anemia

A

Decreased serum haptoglobin
Increased LDH
Increased indirect bilirubin

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16
Q

Cause of Polycythemia vera

A

Majority have JAK2 V617F mutation

Hematopoietic stem cells more sensitive to growth factors

17
Q

Labo in HUS

A

Decreased hemoglobin, platelets

Increased bleeding time, lactate dehydrogenase, bilirubin, BUN, creatinine

18
Q

Activating mutation of KRAS gene

A

Constitutive activation of EGFR
Increased cell prolif + growth
Resistant to chemotherapy w/ anti-EGFR drugs (cetuximab, panitumumab)

19
Q

Labo in lead poisoning

A

Microcytic anemia w/ normal iron studies

Basophilic stippling

20
Q

Serious complication of chronic hemolytic anemia + frequent blood transfusion

A

Hemosiderosis

Hemosiderin (brown-yellowish pigments) in Kupffer cells in liver

21
Q

After various anticancer agents, tumor cells can become resistant

A

Human multidrug resistance (MDR1) gene
P-glycoprotein: transmembrane ATP-dep efflux pump protein
P-GP: reduce influx of drugs into cytosol, increase efflux from cytosol
So prevent action of CT

22
Q

Growth factors for angiogenesis in neoplastic + granulation tissue

A

VEGF and FGF
Proinfl cytokines (IL1, IFN-G) indirectly by increased VEGF
Laminin in basement membranes: barrier to new Vx formation

23
Q

Chronic vs acute leukemias on peripheral blood smears

A

Chronic: prevalence of mature cells
Acute: prevalence of blasts

24
Q

Protein C deficiency

A

Exaggerate hypercoagulability after Warfarin: short 1/2 life of prot C + def of prot C
Thrombotic occlusion of microvasculature w/ skin necrosis

25
Q

Hairy cell leukemia

A

Indolent B-cell neoplasm
Bone marrow failure
Infiltration in reticuloendoth system: massive splenomegaly
Dry tap of BM + lymphocytes w/ cytoplasmic projections

26
Q

Splenic changes in sickle cell ds

A

Repeated splenic infarctions
Then splenic atrophy + fibrosis (late ado): autosplenectomy
Then inf w/ encaps bact

27
Q

Megaloblastic anemia

A
Severe macrocytosis (MCV > 110)
Folic acid or vit B12 def
Chronic hemolytic anemia: increased erythrocyte turnover so increased folic acid requirements; risk of macrocytosis
28
Q

Aplastic anemia

A

Triad: low Hb, thrombocytopenia, absent hematopoietic cells in BM
Increased circulating EPO if normal renal fct

29
Q

Pure red cell aplasia

A

Rare form of marrow failure
Severe hypoplasia of marrow erythroid elements
Normal granulopoiesis + thrombopoiesis
Ass w/ thymoma, lymphocytic leukemias, parvo B19 inf

30
Q

DIC

A

Complication of G- bact sepsis, acute pancreatitis, burn injury, APL
G- bact endotoxins activate coag cascade
Schistocytes, thrombocytopenia
Decreased fibrinogen, prolonged PT + PTT

31
Q

AML, M3 presentation

A

Can present w/ persistent inf + coagulopathy: hgic signs
BM biopsy: promyelocytes w/ Auer rods
t(15;17) translocation: fusion of retinoic acid receptor-alpha gene + promyelocyte leukemia gene

32
Q

Follicular lymphoma

A

Overexpression of bcl-2

Increased secretion of Bcl-2 prot: inhibits apoptosis

33
Q

Infections in sickle cell ds

A

Strepto pneumoniae
Hemophilus influenza
Osteomyelitis: #1 Salmonella, #2 Staph aureus and E coli

34
Q

Sickle cell trait

A

Asymptomatic
Relative protection from malaria
Normal Hb, reticulocyte, RBC index values
Life expectancy = gen pop

35
Q

B-cell vs T-cell ALL

A

B-cell: 70-80%, fever, malaise, bleeding, bone pain, HSM

T-cell: 15-17%, mediastinal mass, resp sympt, dysphagia, SVC sd

36
Q

Ttt of hereditary spherocytosis

A

Splenectomy + blood transfusions

Folic acid supplementation

37
Q

Hemophilia

A

Low fact VIII or IX: failure to convert prothrombin to thrombin
Adding thrombin to blood: clotting