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uworld > Hematology and Oncology > Flashcards

Hematology and Oncology Flashcards

1
Q

List the 3 most common cancers in women

A
  1. breast
  2. lung (most deaths)
  3. colorectal
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2
Q

Cancer immunotherapy often includes administration of monoclonal antibodies against a target overexpressed on the cancer cell surface. The Fc portion of the antibody binds ____ cells via CD___ leading to cytotoxicity

A

natural killer

CD16

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3
Q

What clotting factors are produced initially by the liver in an inactive form and then activated by vitamin K dependent carboxylatoin

A

II
VII
IX
X

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4
Q

PT will be prolonged in cirrhosis primarily due to what deficiency

A

factor VII (shortest half life, produced by the liver, and part of the extrinsic pathway which effects PT)

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5
Q

Nitrates are oxidizing agents that are effective in treating ___ poisoning due to their ability to induce methemoglobinemia which contains ___ instead of _____

A

cyanide
ferric (FE3+)
ferrous (Fe4+)
(cyanide binds ferric iron m ore avidly than to mitochondrial cytochrome enzymes, diminshing cyanides toxic effect)

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6
Q

Patients with sickle cell disease become functionally asplenic within the first few years of like which dramatically increases the risk for infections with encapsulated organsims, particularly ____

A

streptococcus pneumoniae

also at increased risk for salmonella osteolmyelits

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7
Q

What medications are most commonly known to cause pancytopenia

A

antiseizure: carbamazepine, valproic acid
sulfonamides
nifedipine

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8
Q

Thrombotic thrombocytopenic purpura is a thrombotic microangiopathy resulting in ____ and ____ which are both findings that are essential for making the diagnosis

A

microangiopathic hemolytic anemia

thrombocytopenia

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9
Q

Thrombotic thrombocytopenia purpura is triggered by a severe deficiency in ______

A

ADAMTS13

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10
Q

What kind of receptor is HER2

A

tyrosine kinase

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11
Q

Tx for HER2 positive breast cancer

A

trastuzumab

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12
Q

What is the result of mycoplasma pneumoniae causing cross-reacting IgM antibodies

A

mild, transient hemolytic anemia

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13
Q

CD19 is only expressed by what cells

A

B cells

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14
Q

Vincristine has what classic toxicity and it stops the cell cycle at what stage

A 
neurotoxicity (peripheral neuropathy)
M phase (disrupts neuronal microtubules)
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15
Q

HbF contains __ globin instead of ___ globin seen in HbA

A

gamma

beta

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16
Q

Diagnostic lab values for DIC

A

prolonged PT/PTT

thrombocytopenia (due to consumption of coagulation factors and platelets)

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17
Q

Erythropoietin signal transduction is primarily mediated by _____ which promotes erythrocyte precursor survival

A

JAK2/STAT

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18
Q

Porphyria cutanea tarda is causes photosensitivity (blistering) and hyperpigmentation and is caused by a deficiency in what enzyme

A

late steps in porphyrin synthesis: uroporphyrinogen decarboxylase

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19
Q

acute intermittent porphyria causes abdominal and neurological symptoms and is caused by a deficiency in what enzyme

A

early steps of porphyrin synthesis: porphobilinogen deaminase

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20
Q

Parvovirus is what type of virus? (DNA/RNA, positive sense, negative sense, single strand, double strand, enveloped, nonenveloped)

A

nonenveloped sing-stranded DNA virus

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21
Q

In Von Willebrand disease, how platelet levels, PT and PTT be affected

A

platelets and PT normal

PTT prolonged due to low levels of factor VIII

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22
Q

What are the main side effects of cyclin dependent kinase inhibitors

A

inhibit cellular replication in rapidly dividing cells (bone marrow, neutropenia, leukopenia, anemia)

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23
Q

What can cause peripheral neuromuscular excitability (paresthesia, muscle spasms) following a blood transfusion

A

citrate anticoagulants can chelate plasma calcium –> hypocalcemia

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24
Q

What is the underlying pathology of primary/hereditary hemochromatosis

A

mutation in HFE protein that causes enterocytes and hepatocytes to detect falsely low iron levels –> increased DMT1 expression on enterocytes to increased iron intestinal absorption and hepatocytes decrease hepcidin synthesis which increases ferroportin expression allowing increased iron secretion into circulation

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25
Q

what are the clinical features of primary hemochromatosis

A

micronodular cirrhosis
diabetes mellitus
skin pigmentation (bronze diabetes)
increased risk for hepatocellular carcinoma, congestive heart failure, and testicular atrophy

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26
Q

enoxaparin MOA

A

low molecular weight heparin

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27
Q

What type of leukemia can cause respiratory symptoms such as a child presenting with dyspnea, tachypnea, and inspiratory stridor as well as dysphagia with solid foods

A

T-cell acute lymphoblastic leukemia (ALL)

can cause a mediastinal mass

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28
Q

What is the MOA of emicizumab treating hemophilia A

A

mimics activity of factor VII
bispecific monoclonal antibody that binds both factor IXa and factor X bringing them in close proximity to allow for factor X activiation

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29
Q

Sickle cell anemia results from a point mutation that substitutes ____ in the place of ____

A

valine

glutamic acid

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30
Q

Why does unfractionated heparin have equal activity against factor Xa and thrombin while LMWH has greater activity against factor Xa than thrombin

A

only unfractionated heparin has pentasaccharide chain long enough to bind to both antithrombin and thrombin which is required to inactivate thrombin
both of them can bind anitithrombin, though, which is all that is needed to increase its ability to inactivate factor Xa

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31
Q

Symptoms of colicky abdominal pain, constipation, and peripheral neuropathy in the presence of a microcytic anemia with normal iron studies suggests what diagnosis

A

lead poisoning

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32
Q

what does a peripheral smear in lead poisoning show

A

microcytic anemia that may reveal basophilic stippling

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33
Q

What are causes of target cells (RBCs)

A

reduced cell volume: thalassemia, iron deficiency

excessive cell membrane: obstructive liver disease, postsplenectomy

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34
Q

How does carbon monoxide poisoning affect O2 binding to hemoglobin

A

heme binds CO stronger than O2 but it also decreases hemoglobin O2 unloading

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35
Q

A 50 yr old man with painless mass in his neck that fluctuates in size and has a t(14;18) chromosomal translocation has overexpression of what gene and type of lymphoma is this

A 
BCL2 (18)
follicular lymphoma (a non hodgkin lymphoma)
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36
Q

Apixaban blocks the conversion of ___ to ____

A

prothrombin
thrombin
(direct factor Xa inhibitor)

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37
Q

In erythrocytosis, a normal red blood cell mass indicates what as the cause of polycythemia

A

plasma volume contraction (relative erythrocytosis)

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38
Q

How does multiple myeloma cause bone pain

A

secretes RANKL and destroys osteoprotegerin –> increased RANK activity –> osteoclast differentiation and inhibition of osteoblasts –> purely radiolucent/osteolytic bone lesions

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39
Q

What chemotherapy agent can cause lung toxicity that usually manifests as pulmonary fibrosis

A

bleomycin

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40
Q

what is the MOA of bleomycin

A

binds to iron and oxygen molecules to create free radicals that cause DNA strand breaks

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41
Q 
What location of enlarged lymph nodes is unlikely to be caused by common childhood infections and is concerning for malignancy: 
anterior cervical 
posterior cervical 
submandibular
submental
supraclavicular
A

supraclavicular

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42
Q

How does the BCL2 protein normally inhibit apoptosis

A

blocking the release of proapoptotic factors (cytochrome C protein) from the mitochondria

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43
Q

What lymphoma presents with a painless waxing and waning (fluctuating) lymphadenopathy

A 
follicular lymphoma
(most common indolent (painless) non hodgkin lymphoma in adults)
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44
Q

Vitamin B12 is an essential cofactor in the recycling of ____ to ____ which is required for amino acid, purine, and thymidine synthesis

A

5-methyl-tetrahydrofolate
tetrahydrofolate
(B12 = colbalamin)

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45
Q

____ is a polypeptide synthesized by the _____ that acts as a regulator of intestinal iron absorption and iron release by macrophages

A

hepcidin
liver
(hypoxia and high erythropoiesis –> low hepcidin –> increased intestinal absorption and stimulate iron release from macrophages)

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46
Q

what complication of multiple myeloma can lead to renal failure, heart failure, and neurologic dysfunction

A

amyloidosis (light chains form insoluble fibrils)

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47
Q

Acute promyelocytic leukemia is associated with what chromosomal translocation

A

t(15;17)

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48
Q

Auer rods = ______

A

acute myelogenous leukemia (includes acute promyelocytic leukemia)

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49
Q 
Tumor lysis syndrome has what effect on the following:
potassium
uric acid
phosphorus
lactate dehydrogenase
A

potassium: increased (–> hypocalcemia)
uric acid: increased
phosphorous: increase
lactate dehydrogenase: increased

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50
Q

What causes the delay in warfarin’s therapeutic effect

A

it only blocks the generation of new clotting factors, therapeutic effect is delayed 3-5 days until preexisting clotting factors are consumed

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51
Q

What 3 things can cause a left shift in the hemoglobin oxygen dissociation curve

A

increased pH
decreased 2,3-bisphosphoglycerate (increased in low O2 and decreases O2 affinity allowing release in peripheral tissues)
decreased
temperature (stabilizes bonds)

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52
Q

hemolytic anemia, hypercoagulability, and pancytopenia are characteristic of what disease

A

paroxysmal nocturnal hemoglobinuria

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53
Q

Paroxysmal nocturnal hemoglobinuria is caused by an acquired mutation of the ___ gene which is involved in the synthesis of _______

A

PIGA

glycosylphosphatidylinositol (GPI) anchor

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54
Q

what is the pathogenesis of paroxysmal nocturnal hemoglobinuria

A

mutation in gene for GPI anchor synthesis –> CD55 (decay-accelerating factor) and CD59 (MAC inhibitory protein) deficiencies –> complement mediated hemolysis (no inhibition of complement on RBC)

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55
Q

flow cytometry is the gold standard for diagnosis of paroxysmal nocturnal hemoglobinuria which will show absence of ___ and ____

A

CD55

CD59

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56
Q

megaloblastic anemia with neurologic dysfunction is due to what deficiency

A

B12

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57
Q

What lymphoma has presence of macrophages and apoptotic bodies in a see of medium-sized lymphocytes

A

Burkitt

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58
Q

Burkitt lymphoma is associated with what chromosome translocation

A

t(8;14)

8: c-Myc

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59
Q

Acute intermittent porphyria can cause neurologic symptoms and abdominal pain due to deficiency of what enzyme and can be treated by decreasing activity of what enzyme

A 
porphobilinogen deaminase
aminolevulinate synthase (rate limiting enzyme in hepatic pathway of heme)
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60
Q

Methotrexate is a _____ antagonist which competitively inhibits the enzyme _____ which catalyzes the synthesis of ______.

A

folic acid
dihydrofolate (DHF) reductase
tetrahydrofolate

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61
Q

Lecovorin/folinic acid has what affect on methotrexate vs 5-FU chemotherapeutic effects

A

methotrexate: inhibited (bc leucovorin bypasses the DHF reductase step that is inhibited by methotrexate)
5-FU: potentiates toxicity (5-FU requires presence of reduced folate inorder to form complexes with thymidylate synthase)

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62
Q

list 3 B cell markers

A

CD19
CD20
CD23

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63
Q

A patient with anemia, thrombocytopenia, and leukocytosis with a predominance of CD-20 positive cells has high suspicion of what diagnosis

A

chronic lymphocytic leukemia (CLL)

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64
Q

The restocetin cofactor assay measures platelet agglutination via binding of ____ receptors to ____. It is abnormal in what deficiency?

A

glycoprotein 1b

vWF deficiency

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65
Q

The primary immune response to EBV is through what cells which appear in the peripheral blood smear as what

A

CD8 T lymphocytes

reactive (atypical) CD8 T cells

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66
Q

jaundice, dark urine, normocytic anemia, reticulocytosis, and bite cells are indicative of what diagnosis

A

glucose 6 phosphate dehydrogenase deficiency

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67
Q

Rasburicase MOA

A

recombinant version of urate oxidase
converts uric acid into allantoin that is more soluble
used in tumor lysis syndrome

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68
Q

Erythrocytes always convert glucose to pyruvate but have what alternate pathway of doing so that does not generate ATP

A

bisphosphoglycerate mutase converts 1,3BPG into 2,3BPG

2,3BPG is converted to 3 phosphoglycerate by bisphosphoglycerate phosphatase and neither of these steps yield ATP

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69
Q

vWF functions as a promotor of platelet adhesion at sites of vascular injury by binding ____ to ____

A 
platelet glycoproteins (primarily Gp1b)
subendothelial collagen on injured blood vessel walls
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70
Q

Hemoglobin C is caused by a ___ mutation that results in a ___ residue being substituted by ____ in the beta globin chain

A

missense
glutamate
lysine

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71
Q

Rank the speed of hemoglobin movement during gel electrophoresis:
hemoglobin A
hemoglobin S
hemoglobin C

A

hemoglobin A> HbS>HbC

72
Q

Deferoxamine MOA in blood transfusion

A

chelation therapy

prevents iron-induced cardiotoxicity and congestive heart failure from developing

73
Q

chromosome translocation t(15;17) causes what leukemia and forms a fusion of what genes

A

acute promyelocytic leukemia (M3 variant of AML)
15 = PML gene
17 = RARA gene (retinoic acid receptor alpha)

74
Q

____ is a monoclonal antibody directed against the CD20 antigen

A

rituximab

75
Q

If a patient has elevated WBC with an increase in myelocytes and decreased leukocyte alkaline phosphatase, what is the diagnosis

A

CML (chronic myelogenous leukemia)

76
Q

Isoniazid inhibits pyridoxine phosphokinase leading to impaired activation of pyridoxine (B6) which is a cofactor for ____. Inhibition of this could cause _____ anemia

A

delta aminolevulinic acid synthase (catalyzes rate limiting step of heme synthesis)
sideroblastic (anemia caused by impaired heme production)

77
Q

explain how HUS causes thrombocytopenia and hemolytic anemia

A

shiga toxin causes capillary endothelial damage –> platelet activation –> microthrombi –> erythrocyte damage

78
Q

How does G6PD effect the following: reticulocytes, lactate dehydrogenase, haptoglobin

A

reticulocytes: increased
lactate dehydrogenase: increased
haptoglobin: decreased (binds free circulating hemoglobin)
(all due to RBC lysis)

79
Q

Multiple myeloma is a malignancy of what cell type

A

plasma cells

80
Q

What is the diagnosis of a patient presenting with thrombocytopenia, microangiopathic hemolytic anemia, renal insufficiency, neurologic symptoms and fever

A

TTP (thrombotic thrombocytopenic purpura)

81
Q

Describe the pathogenesis of thrombotic thrombocytopenic purpura (TTP)

A

formation of autoantibody inhibitors against ADAMTS13 which usually cleaves ultralarge vWF –> aggregation and activation of platelets

82
Q

Which is more suggestive of malignancy: polyclonal proliferation of lymphocytes or monoclonal

A

monoclonal

83
Q

How does demsmopressin help treat patients with symptomatic vWF disease

A

transiently increases vWF and factor VIII release from endothelial cells

84
Q

Burkitt lymphoma is associated with what chromosome translocation

A

t(8;14)

Myc overexpression which functions as a transcription activator –> cell growth and proliferation

85
Q

What causes a increase in mean corpuscular hemoglobin concentration in spherocytosis

A

mild dehydration of the red blood cells

86
Q

Precursors to what cells stain positive for Tdt, CD10, CD19

A

Bcell

87
Q

What is the pathogenesis of disseminated intravascular coagulation (DIC)

A

widespread formation of microvascular thrombi leads to consumption of platelets, coagulation factors, and fibrinogen. Subsequent activation of anticoagulant proteins leads to low protein C/S. Most patients have bleeding complications and end organ damage to lungs or kidneys

88
Q

How does myoglobin oxygen binding differ from hemoglobin

A

myoglobin reaches saturation at a much lower partial pressure of oxygen than hemoglobin (1 mm Hg vs 26) and it does not experience heme-heme interactions bc it only has a single heme group so does not have sigmoid shape curve

89
Q

A patient on LMWH that develops thrombocytopenia and deep venous thrombosis 5-10 days after starting LMWH after a right hip arthroplasty is suspicious of what diagnosis

A

HIT type 2

heparin induced thrombocytopenia

90
Q

what causes thrombocytopenia in HIT type 2

A

destruction of antibody coated platelets by splenic macrophages

91
Q

what platelet receptor normally promotes platelet binding binding to fibrinogen

A

glycoprotein iib/iiia

92
Q

The factor V leiden mutation, causes factor Va resistance to what? which causes atypical venous thrombosis

A

resistant to inactivation by activated protein C

93
Q

Multiple myeloma is sometimes treated with lenalidomide, what is its MOA

A

increases the binding affinity of E3 ubiquitin ligase complex to specific transcription factors that are overexpressed in myeloma cells –> destruction by proteasome

94
Q 
How are the following affected in anemia secondary to menstrual blood loss: 
serum ferritin
circulating transferrin
MCV
hypersegmented neutrophils
serum folate
A 
serum ferritin: low
circulating transferrin: high
MCV: low
hypersegmented neutrophils: none
serum folate: normal
95
Q

What type of leukemia is a B cell neoplasm that infiltrates bone marrow causing fibrosis and bone marrow failure and infiltrates the splenic red pulp causing massive splenomegaly

A

hairy cell leukemia

lymphocytes have cytoplasmic projections

96
Q

What is the best test to monitor the anticoagulation effect of warfarin? Heparin?

A

warfarin: PT (or INR)
heparin: PTT

97
Q

In DIC due to gram-negative sepsis, the coagulation cascade is activated by ____ leading to widespread deposition and the consumption of coagulation factors and platelets with eventual bleeding

A

bacterial endotoxins

98
Q

What is seen on peripheral blood smear of DIC

A

schistocytes

99
Q

What effect does anemia of chronic disease have on total iron binding capacity

A

its either normal or low

100
Q

PCR requires what 4 things

A

primers that are complementary to the regions of DNA flanking the segment of interest
thermostable DNA polymerase
deoxynucleotide triphosphates
source DNA template strand

101
Q

What is the most common cause of cancer death in both women and men

A

lung cancer

102
Q

How does intravenous glucose help treat acute intermittent porphyria which may present with abdominal pain, peripheral neuropathy, neuropsych symptoms, and reddish brown urine

A

downregulates ALA synthase activity

103
Q

how does methemoglobinemia affect oxygen binding

A

methemoglobin is unable to carry oxygen

104
Q

how is PO2 of arterial blood altered in CO poisoning, anemia, and polycythemia

A

it is unchanged in all of these conditions bc PO2 is a measure of dissolved O2 in blood and does not account for O2 bound to hemogobin

105
Q

A patient with factor VII deficiency likely has normal or abnormal of the following: PT, PTT, bleeding time

A

PT: prolonged

PTT and bleeding time: normal

106
Q

What effects does unfractionated heparin have on thrombin time
PTT
activity of factor Xa

A

thrombin time: prolonged
PTT: prolonged
Activity of factor Xa: decreased

107
Q

What is the genetic cause of beta thalassemia

A

DNA mutations that effect the transcription, processing, and translation of beta globin mRNA most commonly causing aberrant precursor mRNA splicing or premature chain termination during translation –> beta globin deficiency

108
Q

In a patient with beta thalassemia who has routine blood cell transfusions a liver biopsy shows Kupffer cells containing coarse, yellow-brown cytoplasmic granules which are most likely composed of what

A

hemosiderin (iron storage complex)

109
Q

MOA hydroxyurea used in sickle cell patients

A

increases expression of gamma globin chains –> increased circulating fetal hemoglobin

110
Q

What has the classic histopatholgy description of uniform, round, medium sized tumor cells with basophilic cytoplasm and a proliferation fraction of >99%

A 
Burkitt lymphoma
(basophilic= lymphocytes)
111
Q

What chromosome translocation is seen in CML

A

t(9;22)

BCR-ABL –> constitutive tyrosine kinase activity

112
Q

____ converts heme to _____, a pigment that causes the greenish color to develop in bruises several days after injury

A

heme oxygenase

biliverdin

113
Q

Patients of advanced age have increased bone marrow ___ and reduced bone marrow ____, leading to impaired bone marrow reserve

A

fat

mass

114
Q

What is the role of haptoglobin

A

binds circulating hemoglobin and reduces renal excretion of free hemoglobin, preventing tubular injury

115
Q

How might cystic fibrosis cause coagulopathy

A

decreased fat soluble vitamin absorption –> low vit K –> decreased activation of II, VII, IX, X

116
Q

6-mercaptopurine (6-MP) is an immunosuppression drug that is inactivated by what 2 enzymes?
reduced function of either of these enzymes causes increased levels of active metabolites of 6-MP, leading to increased risk for toxicity

A

xanthine oxidase

thiopurine methyltransferase

117
Q

Pyruvate kinase deficiency causes splenomegaly how?

A

failure of glycolysis –> insufficient ATP to maintain erythrocyte structure –> spleen parenchyma removes deformed erythrocytes –> red pulp hyperplasia

118
Q

What anticoagulant prolong both the PTT and PT but have no affect on the thrombin time

A

direct factor Xa inhibitors

119
Q

what medications prolong the thrombin time

A

medications that directly or indirectly inhibit thrombin (unfractionated heparin and dabigatran)

120
Q

Desmopressin can alleviate bleeding through vWF release from what cells?

A

endothelial

121
Q

What inherited bleeding disorder causes prolonged bleeding time and prolonged PTT but does not affect the PT

A

von Willebrand disease

122
Q

If a 2 wk old baby has 70% Hemoglobin F, 20% hemoglobin A, and 10% hemoglobin S do they have sickle cell or sickle cell trait

A

sickle cell trait

123
Q

Paroxysmal nocturnal hemoglobinuria can cause what renal pathology

A

hemosiderosis

iron deposition in kidney from chronic hemolysis

124
Q

what cause of hemolytic anemia will display an absence of CD55 on RBC

A

paroxysmal nocturnal hemoglobinuria

125
Q

What causes autosplenectomy in sickle cell patients

A

repetitive splenic infarctions caused by microvessel occlusion –> small, firm splenic remnant

126
Q

Anemia of chronic disease is caused by elevated inflammatory cytokine levels, mainly _______

A

hepcidin

127
Q

How does hepcidin cause anemia of chronic disease

A

increases sequestration of iron within the reticuloendothelial system and reduce circulating iron levels

128
Q

In anemia of chronic disease, how are circulating iron levels and bone marrow iron levels affected

A

circulating: low

bone marrow: high

129
Q

What is the pathogenesis of primary myelofibrosis

A

atypical megakaryocytic hyperplasia which stimulates fibroblast proliferation, resulting in progressive replacement of the marrow space by extensive collagen deposition

130
Q

Apart from chronic myelogenous leukemia, the chronic myeloproliferative disorders frequently harbor a mutation in what

A

nonreceptor cytoplasmic tyrosine kinase, JAK2

–> constitutive tyrosine phosphorylation activity –> STAT activation and enters nucleus –> transcription

131
Q

What cause of hepatocellular carcinoma will show intranuclear fragments of foreign DNA within hepatic lesions

A 
Hepatitis B 
(integrates into host genome and produces oncogenic viral protein: HBx)
132
Q

_____ deficiency is associated with coiled hair, perifollicular keratosis, hemorrhage, easy bruising, bleeding gums and loose teeth. The PT, PTT, and platelet count are normal.

A

vitamin C

133
Q

Primary myelofibrosis causes massive splenomegaly how?

A

extramedullary hematopoiesis –> expansion of red pulp

134
Q

____ is characterized by aggregates of packed follicles that obscure the normal lymph node architecture

A

follicular lymphoma

135
Q

90% of patients with follicular lymphoma have the ___ translocation, which causes the overexpression of the ___ protein

A

t(14;18)

BCL-2 (antiapoptotic)

136
Q

Primary myelofibrosis is a myeloproliferative disorder associated with the clonal expansion of ____

A

megakaryoctes

137
Q

Pure red cell aplasia is associated with what 3 pathologies

A

thymoma
lymphocytic leukemias
parvovirus B19

138
Q

Howell-Jolly bodies on peripheral smear reflect what?

A 
splenic dysfunction
(seen in sickle cell)
139
Q

Where does uric acid precipitate in the nephron as seen in tumor lysis syndrome

A

distal tubules and collecting duct

acidic environments –> precipitate; alkalization of urine prevents crystallization

140
Q 
Which of the following would decrease warfarin's therapeutic effects: 
amiodarone
cimetidine
phenytoin
phenobarbital
metronidazole
rifampin
carbamazepine
A 
carbamazepine
phenytoin
phenobarbital
rifampin
(the others listed are CYP450 inhibitors which would increase warfarin effects)
141
Q

Megaloblastic anemia in a patient who developed a heavy drinking problem in this past year is due to what

A 
folate deficiency
(B12 deficiency would take years to deplete)
142
Q

What lysosomal storage disorder presents with bone pain, hepatosplenomegaly, and cytopenias and is due to a beta glucocerebrosidase deficiency

A

Gaucher

143
Q

What causes rouleaux formation and what pathologies is it seen in

A

elevated levels of circulating proteins which disrupts the repulsive electrostatic charge on the erythrocyte surface and causes stacked aggregation
can been seen in inflammatory conditions that increase acute phase reactants (fibrinogen), it is classicaly linked with multiple myeloma and Waldenstrom macroglobunemia which generate high levels of monoclonal paraprotein (immunoglobulins)

144
Q

How do proteasome inhibitors (bortezomib) treat cancer

A

they blog the degradation of ubiquitinated proteins by the proteasome, which leads to accumulation of abnormally folded proteins that trigger cellular apoptosis

145
Q

Cancer cells avoid immune recognition by overexpressing _____ which binds its receptor on cytotoxic T cells and inhibits their response (T-cell exhaustion)

A

programmed death ligand 1 (PD-L1)

146
Q

The most common cause of elevated platelet counts in all age groups is what?

A

reactive thromobocytosis (high levels of inflammatory cytokines prompt liver to release thrombopoietin. It is seen in chronic infection, rheumatologic disease, and burns)

147
Q

What is seen on blood smear of leukemoid reaction

A

numerous mature neutrophils which may have reactive morphologic features such as Dohle bodies, toxic granulation, and cytoplasmic vacuoles

148
Q

what lab value can help distinguish leukemoid reaction from CML

A

leukocyte alkaline phosphatase ( enzyme found in maturing neutrophils)
leukemoid reaction: normal or increased
CML: decreased (bc abnormal maturing neutrophils have decreased levels)

149
Q

95% of patients with polycythemia vera have what gene mutation

A

V617F mutation involving the JAK2 gene –> hematopoietic cells more sensitive to growth factors

150
Q

what are the clinical manifestations of polycythemia vera

A

increased RBC and plasma volume and low EPO levels
also can have elevated platelet and/or WBC count
thrombotic events from blood hyperviscocity
peptic ulceration and aquagenic pruritus from histamine release form neutrophils
gouty arthritis from increased cell turnover

151
Q

what is seen on peripheral blood smear of lead poisoning

A

basophilic stippling

on a background of hypochromic microcytic anemia

152
Q

EBV infection induces _______ which react to antigens from animal erythrocytes

A

heterophile antibodies

153
Q

EBV is associated with what malignancies

A

Burkitt lymphoma
nasopharyngeal carcinoma
(and central nervous system lymphoma in HIV positive patients)

154
Q

Why is nephrotic syndrome a hypercoaguble state

A

loss of anticoagulant factors, especially antithrombin III

155
Q

LMWH binds _____ and predominately acts on _____

A

antithrombin III

factor Xa

156
Q

etoposide MOA

A

inhibits sealing activity of topoisomerase II

157
Q

what are topoisomerase I inhibitors

A

irinotecan

topotecan

158
Q

An anterior mediastinal mass with elevated serum levels of alpha fetoprotein and beta hCG is classic for a ____ tumor

A

nonseminomatous germ cell tumor

159
Q

After exposure to various anticancer agents, tumor cells can become resistant due to what cell function

A

human multidrug resistance (MDR1) gene codes for P-glycoprotein, a transmembrane ATP-dependent efflux pump protein that has a broad specificity for hydrophobic compounds that can both reduce influx of drugs into cytosol and increase efflux from the cytosol thereby preventing the action of chemotherapeutic agents

160
Q

what are two characteristic markings of myelodysplastic syndrome

A

1 or more cytopenias

dysplasia of erythrocytes and neutrophils

161
Q

What deficiency presents with dysphagia (from esophageal web formation), koilonychia (spoon-shaped nails), and a shiny red tongue

A

iron deficiency

162
Q

enoxaparin MOA

A

a LMWH that functions like heparin in that it binds and activated antithrombin III

163
Q

What causes DIC in abruptio placentae (placental bleeding between uterine wall and placenta)

A

decidual damage and ischemia –> tissue factor release into maternal circulation –> coagulation

164
Q

How can ceftriaxone lead to a hemolytic anemia

A

penicillins and cephalosporins are common triggers for drug-induced hemolytic anemia because they can bind to the erythrocyte surface and act as haptens for IgG attachement –> clearance by splenic macrophages

165
Q

Auer rods are deformed azurophilic granules found in the cytoplasm of myeloblasts that stain positively for ______

A

myeloperoxidase

166
Q

Hemophilia is an X linked recessive bleeding disorder due to decreased levels of ____ (hemophilia A) or ____ (hemophilia B)

A

A: factor VIII
B: factor IX

167
Q

A patient’s red blood cells are incubated in a hypotonic solution with glycerol and hemoglobin is released, the control sample does not release hemoglobin. What is the diagnosis

A

hereditary spherocytosis

168
Q

what test of hemostatic function is generally abnormal in hemophilia

A

partial thromboplastin time (PTT)

169
Q

What is the purpose of the Kozak consesnus sequence position before and AUG codon

A

identifies the specific AUG codon that serves as the initiator of translation, mutations affecting the Kozak consensus sequence can significantly impair protein translation

170
Q

with increased bone marrow erythropoiesis, accelerated release of immature RBC into the blood stream cause them to contain bluish cytoplasm due to what

A

residual ribosomal RNA

171
Q

diminished thymidine synthesis is likely due to wat cause of megaloblastic anemia

A

vitamin B12 deficiency

172
Q

Parvovirus primarily attacks erythroid progenitor cells due to tropism for an erythrocyte cell surface receptor, infection of the erythroid progenitor cells prevents RBC maturation leading to formation of ______ which can be seen on blood smear

A

giant pronormoblasts (several times larger than surrounding RBCs with glassy, intranuclear viral inclusion

173
Q

Auer rods = ____

A

AML

174
Q

How does renal disease cause excessive bleeding

A

uremic toxins in circulation impair platelet aggregation and adhesion –> qualitative platelet disorder (numbers are normal)

175
Q

30% of patients with SLE have _______ antibodies which can caue paradoxical aPTT prolongation and a false positive RPR/VRDL

A

antiphospholipid

176
Q

Patients with antiphospholipid antibodies are at risk for ____ and ____

A

venous and arterial thromboembolism

unexplained, recurrent pregnancy loss

177
Q

all trans retinoic acid induces remission in about 90% of patients with what cancer

A

acute promyelocytic leukemia (M3 variant of acute myelogenous leukemia)

uworld (19 decks)

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