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Uworld > Hematology/Oncology > Flashcards

Hematology/Oncology Flashcards

1
Q

In what organelle (within erythrocyte) does heme synthesis take place

A

Mitochondria

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2
Q

What deficiency is associated with warfarin skin necrosis

A

Protein C/ S deficiency- warfarin inhibits these factors since dependent on vitamin K and get a hypercoagulable state, this leads to microvascular occlusion and hemorrhagic skin necrosis ( Factor 7 and protein C have the shortest half lives)

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3
Q

Benefits of COX 2 inhibitors vs. having COX 1 inhibition?

A

COX 2 inhibition have potent anti-inflammatory with out GI ulceration side effects, COX 1: inhibits platelet aggregation and can lead to GI ulceration

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4
Q

How are myoglobin and hemoglobin related

A

Structurally analogous, myoglobin is not sigmoid and would be similar to 4 beta monomers—> most left shift curve possible and myoglobin is momomeric

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5
Q

What features are associated with hair cell leukemia?

A

Dry tap (unsuccessful bone marrow aspiration), lymphocytes with cytoplasmic projections, TRAP positive stain

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6
Q

What are the atypical cells seen on blood smear in EBV infection?

A

CD8+ T cells, although EBV preferentially infects B cells

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7
Q

What does von Willebrand factor bind to?

A

Platelet glycoproteins (Gp1b) and collagen

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8
Q

What is dactylitis and what disease is it associated with?

A

Painful swelling of hands and feet, Sickle cell disease

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9
Q

What does haptoglobin do?

A

Binds circulating hemoglobin and reduced free renal excretion of free hemoglobin preventing tubular injury

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10
Q

5 Ps Acute intermittent porphyria and what enzyme is deficient and what is treatment?

A 
Painful abdomen
Port wine urine
Polyneuropathy
Psychological disturbances 
Precipitated by drugs ( alcohol, starvation)

Porphobilinogen deaminase

Tx: glucose and heme which INHIBIT ALA-synthase (rate limiting step)

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11
Q

What is extramedullary hematopoiesis and what are bone marrow signs?

A

Presence of erythroid precursors in liver and spleen, EPO hyperplastic marrow

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12
Q

Atypical venous thrombosis in adults <50, which no aPTT response after giving protein C

A

20% FACTOR V Leiden—> factor V resistance to protein C so—> hypercoagulable

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13
Q

Heme oxygenase

A

( found in macrophages) Converts heme into biliverdin( green color after bruise), CO, ferrous iron and then bilverdin is broken down by biliverdin reductase to yellow bilirubin which is then transported by albumin to liver

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14
Q

Burkitt lymphoma

A

t(8;14), cMYC overexpression=transcription activator

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15
Q

Mantle cell lymphoma

A

t(11;14), cyclin D1 overexpression=promoter of G1–>S phase

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16
Q

Follicular lymphoma

A

t(14;18), BCL2 overexpression=INHIB apoptosis

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17
Q

Chronic myelogneous leukemia (CML)

A

t(9;22), ABL gene from chr 9–> INC tyrosine kinase activity

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18
Q

Polycythemia vera (describe dz and associated mutation with function)

A

Myeloproliferative disorder characterized by UNCONTROLLED RBC production, mutation in JAK2 ( cytoplasmic tyrosine kinase)= hematopoietic cells more sensitive to growth factors, also see: splenomegaly, aquagenic pruritus(histamine from basophils), facial plethora, gout (inc cell turnover)

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19
Q

Aplastic anemia triad

A

Low hemoglobin, thrombocytopenia, ABSENT hematopoietic stem cells in bone marrow( high lipid content), would see and INC in EPO if also have normal renal function

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20
Q

How can you treat hemophilia?

A

Give thrombin because no factor 8 or 9 to convert factor 10 into factor 2 (thrombin)

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21
Q

CD 14 is a cell marker for and what disease can it be seen in that has caseating granulomas

A

Monocyte macrophage lineage with pale pink granular cytoplasm and secondary TB

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22
Q

Why is amyloid associated with multiple myeloma?

A

Forms from accumulation of mono colonial immunoglobulin light chains

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23
Q

What enzyme deficiency can present similar to glucose 6 phosphate dehydrogenase deficiency

A

Glutathione reductase deficiency: if don’t have, can’t use NADPH to reduce glutathione to get rid of free rads

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24
Q

Basophillic stippling and microcytic hypochromic anemia are associated with

A

Lead poisoning ( can also see abdominal pain and blue lead lines in teeth)

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25
Q

B cells mature in the _____ and undergo what process there

A

Bone marrow, VDJ recombination

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26
Q

What process are B cells undergoing in the follicles

A

Isotype switching and requires interaction of CD40 receptors on B cells with CD40 ligand of activated T cells (CD154)

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27
Q

Peru ate kinase deficiency leads to ______ anemia. How does the spleen react to this

A

Hemolytic, red pulp hyperplasia because has to work overtime to get rid of lysed RBCs

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28
Q

The anal canal drains to which lymph nodes

A

Superficial inguinal

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29
Q

Lymph from superior bladder drains into

A

External iliac nodes

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30
Q

Lymph from prostate drains into

A

Internal iliac nodes

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31
Q

Lymph from upper 1/3 rectum drains into

A

Inferior mesenteric nodes

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32
Q

Myeloblasts stain positive for

A

Peroxidase

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33
Q

Sickling in sickle cell disease is promoted by situations with ______ oxygen, ______ acidity, _____ blood volume, _____ levels of 2,3 bisphosphoglycerate

A

Low oxygen, increased acidity, low blood volume, increased

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34
Q

Patients who receive more than 5-6 L of blood transfusion have high levels of _____ in the blood and this can chelate ____ and leads to parathesias

A

Citrate, calcium

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35
Q

What is the translocation associated with APML and how do you treat?

A

15;17

15: PML
17: RARA

Tx with all trans retinoic acid

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36
Q

Methemoglobinemia happens because _____ cause poisoning by inducing the conversion of oxidized Fe3+ state. The _____ of oxygen remains unchanged

A

Nitrates, partial pressure (amount of oxygen dissolved in the plasma)

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37
Q

Inhibition of proteasomes may lead to an _____ of proapoptotic proteins

A

Excess

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38
Q

G6PD deficiency can lead to hemolytic anemia. What conversion is inhibited

A

Glucose 6 phosphate to 6 phosphogluconate

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39
Q

Hemoglobin F does not bind to 2,3 bisphosphoglycerate and as a result has a _____ affinity for O2 than Hemoglobin A

A

higher, this allows HbF to extract lots of oxygen from mom

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40
Q

Hemoglobin S leads to ______ interactions that cause sickling

A

hydrophobic, glu switched for a valine (nonpolar)

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41
Q

Clinical features of back pain at night, not relieved by rest or analgesics, advanced age, and systemic symptoms suggest what kind of cause

A

metastasis to bone

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42
Q

DIC is associated with sepsis and how does it affect aPTT and PT, platelets, fibrinogen

A 
BOTH PROLONGED (ONLY ONE THAT does this when compared to ITP and TTP)
-dec platelets, dec fibrinogen
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43
Q

The key growth factors for angiogenesis are?

A

VEGF: vascular endothelial growth factor, fibroblast growth factor

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44
Q

The ________ in basement membranes poses a physical barrier to the sprouting of new blood vessels

A

laminin

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45
Q

In a post menopausal women with microcytic anemia, need to rule out ________

A

occult blood loss (Ex: from GI malignancy)

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46
Q

Renal cortical cells sense hypoxia and respond by synthesizing and releasing _______. This leads to an elevated hematocrit

A

EPO, esp in sleep apnea

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47
Q

how does uremic platelet dysfunction affect PT, aPTT, bleeding time, platelet count?

A

platelet count=dec, everything else normal

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48
Q

Pure red cell aplasia is associated with ______, lymphocytic leukemia, parvovirus B19

A

thymomas and have preserved myeloid and megakaryotic elements

49
Q

Factor V Leiden is a defect in ______ and predisposes you to _______ events

A

protein C, thrombotic

50
Q

_______ defiiciency is a megaloblastic anemia that can occur in weeks in alcholics

A

folate

51
Q

PT is affected by _____ and if levels are really low sometimes vitamin K supplementation still won’t help because not enough around to be activated

A

factor 7

52
Q

bony pain in and older man who smokes is ______ cancer

A

prostate

53
Q

how does vwF disease affect PTT, bleeding time, PT

A

PTT: prolonged, PT: normal, bleeding time: prlonged

54
Q

_________ is the most common indolent non-Hodgkin lymphoma in adults. It typically has a waxing and waning course

A

follicular lymphoma

55
Q

Tumor cells detach from neighboring cells with dec expression of

A

E-cadherin

56
Q

Tumor cells adhere to the basement membrane which is facilitated by inc expression of

A

laminin

57
Q

Tumor cells invade the basement membrane with inc expression of

A

proteolytic enzymes (metalloproteinases, cathepsin D protease)

58
Q

The major chronic pathologic changes in the spleen of patients with sickle cell results from?

A

microvessel occulsion

-this leads to a fibrotic, shrunken, brown (excess hemosiderosis from extravascular hemolysis of RBCs)

59
Q

________ is defined by a hematocrit leve >52% in men and 48% in women

A

erythrocytosis

60
Q

Erythrocytosis with and increase in RBC mass indicates

A

absolute erythrocytosis

61
Q

Erythrocytosis with a normal RBC mass indicates

A

relative erythrocytosis

62
Q

Primary erythrocytosis is associated with ____ levels and is caused by

A

low EPO, myeloproliferative disorders (like polycythemia vera)

63
Q

Secondary erythrocytosis is associated with ___ levels and is caused by

A

high EPO, chronic hypoxia (COPD, high altitude, smoking)

64
Q

Activating mutations of the KRAS gene lead to constuitive activation of the _________ pathway leading to?

A

epidermal growth receptor pathway (EGFR), increased cell proliferation and growth
-tumors with KRAS mutation are resistant to anti EGFR drugs

65
Q

Large eosinophillic casts with Bence Jones proteins and a pt. w/ fatigue, back pain, elevated serum protein, and azotemia lead to?

A

multiple myeloma

66
Q

SLE induced antiphospholipid syndrome is made by the presence of _________. How does this affect PTT and why? Will it be corrected with FFP?

A

lupus anticoagulant, prolongs PTT because it is an antiphospholipid antibody and it binds the reagent to the test, no

67
Q

Which cancer is associated with auer rods and what’s the translocation

A

APML, t(15;17)

68
Q

What precursor ALL has TdT+, CD 10, CD19

A

precursor B-ALL

69
Q

What precursor ALL has TdT+, CD 2,3,4,5,7,8

A

precursor T-ALL

70
Q

__-ALL is more likely to present with a large anterior mediastinal mass that can compress the great vessels, causing _________. It can also compress the esophagus/trachea leading to

A

T, superior vena cava syndrome (edema of face and arms), dysphagia, inspiratory stridor/dyspnea

71
Q

___ thalasemia minor is common in people of Mediterranean descent. You can see inc level of which hemoglobin? What is the initial step in pathogenesis of this disorder?

A

beta, HbA2: b/c dec beta chain synthesis. mRNA of the beta globin gene is disrupted

72
Q

Patients with sickle cell disease have inc requirement of what vitamin and what can this lead to?

A

folic acid (b/c of inc erythrocyte turnover), macrocytic anemia

73
Q

If you have a low reticulocyte count that signifies a _____ response to anemia and lack of ________

A

inadequate, erythropoiesis

74
Q

The most common for DIC in pregnancy is release of ___________ from an injured placenta into maternal circulation

A

tissue factor, DIC presents with bleeding from incision sites, and IV sites from MASSIVE consumption of platelets and clotting factors

75
Q

Dysplasia is a _______ change in epithelial cells

A

reversible, once it invades BM=carcinoma and not reversible

76
Q

Schistocytes can occur from mechanical trauma such as damage with ______

A

prosthetic valves

77
Q

What does haptoglobin do? When do levels decrease?

A

binds free Hgb and promotes its uptake by the reticuloendothelial system, when lots of free Hgb is released into the serum such as in intravascular hemolysis

78
Q

Paroxysmal nocturnal hemoglobinuria has an associated deficiency of which two CD proteins? It is associated with _______ anemia and _______thrombosis. What is its clinical triad? How does this affect the kidney

A

CD55/59. aplastic, hepatic vein,

  • hypercoagulability, pancytopenia,hemolytic anemia
  • hemosiderosis in the kidney from chronic hemolysis with breakdown of iron containing erythrocytes
79
Q

Migratory ______ is suggestive for visceral cancer. Why does hypercoagulability develop?

A

thrombophlebitis, b.c adenocarcinomas produce a thromboplastin like substance capable of causing intravascular coagulations

80
Q

How are these levels in multiple myeloma: RBCs, platelets, Ca, ESR, urine protein, serum protein, serum albumin

A
  • dec RBCs and platelets(b/c plasma cells infiltrate the bone marrow)
  • elevated Ca and ESR( systemic inflammation and proteolytic bone lesions)
  • inc urine and serum protein but DEC serum albumin (b/c plasma cells make M protein–> rouleaux formation (stacks of RBCs) so get an inc in total SERUM protein but albumin typically dec
81
Q

The gene for heavy chain of Igs is found on which chromosome?

A

14

82
Q

_______calcemia is a common finding after blood transfusions and why? What are symptoms?

A

Hypocalcemia,

Citrate in the blood will bind calcium

Vague muscle weakness, irritability

83
Q

Diffuse B cell lymphoma has cells that are ____ positive

A

CD5, presents with B symptoms

84
Q

In sickle cell trait, only _____ of hgb is abnormal. And what is a symptom they might have?

A

35-40%, episodic hematuria (sickling of RBCs in the renal medulla from thromboembolic events)

85
Q

Newborn born at homewith umbilical stump bleeding probably has a _______ deficiency

A

Vitamin K, so only PT and aPTT will be prolonged

-baby didn’t get vitamin K shot in the hospital

86
Q

von willibrand disease leads to ____ PTT and ____ bleeding time

A

prolonged, prolonged

vWF is a cofactor for factor 8 as well

87
Q

sporadic forms of _______ lymphoma can present with highly mitotic, basophilic lymphocytes surrounding clear zones of macrophages. most common presentation in north america is:

A

burkitt lymphoma, constipation, abdominal mass( enlarged LNs)

88
Q

What is WAGR syndrome?

A

Wilms tumor, Aniridia(partial or complete lack of the iris), Genital abnormalities, mental Retardation

89
Q

unfractionated heparin is better at blocking ____ than regular heaprin

A

thrombin

-both affect factor Xa but unfractionated can also attack Xa AND thrombin

90
Q

Rat poison has ______ in it so if someone overdoses good to treat with

A

warfarin, FFP to replace clotting factors

91
Q

Cryoprecipitate has what factors

A

8,13,vWF,fibrinogen

92
Q

Eculizimab blocks the conversion of ______

A

C5 and MAC complex

93
Q

What is somatic hypermnutation

A

component of affinity maturation, its a randomized mutation process of variable regions, then follicular dendritic cells present antigen and B cells that prod the antibodies with highest affinity are positively selected

94
Q

________ presents as hypoparathyroidism, adrenal insufficiency, chronic mucocutaneous candidiasis, skin dystrophy

A

AIRE gene dysfunction

95
Q

________ presents with Bierbeck granules, painful bone lytic lesions, eczematous rash. Cells are ____ positive and express _____ protein

A

LangerHans histiocytosis, CD1a, S100

96
Q

Hemolytic disease of the newborn typically results from ________

A

erythrocyte opsonization from maternal antibodies (hydros fetalis)

97
Q

Mutations that cause production of hemoglobin with higher oxygen affinity ____ the ability of Hgb to release oxygen to the peripheral tissues so get a _______

A

compensatory erythrocytosis that helps maintain oxygen delivery

98
Q

Hereditary spehrocytosis results in ____ membrane stability –>______ erythrocyte osmotic fragility

A

dec, inc

99
Q

Sickle cell can cause _______ induced hemolysis due to Hgb S polymerizing and causing damage

A

hypoxia

100
Q

Red blood cell fragments of burr and helmet cells are significant for

A

mechanical trauma

-look for “just got a heart valve”

101
Q

isoniazid inhibits pyroxidine phosphokinase–> ___ deficiency. This is a cofactor for __________ and when dec can lead to ________anemia

A

B6, ALA synthase, sideroblastic

102
Q

Lysing of RBCs in glycerol situation w/ inc osmotic fragility leads you to think

A

hereditary spherocytosis

103
Q

__________ is a mutation in the HFE gene and typically interacts with the _______ receptor that acts as a sensor for iron stores. They’re at inc risk of developing __________ and _________

A

primary hemochromatosis, transferrin

  • this causes receptors to detect falsely low levels
  • hepatocellular carcinoma, liver failure
104
Q

Integrins interact with the ECM by binding to which 3 proteins?

A

collagen, fibronectin, laminin

105
Q

reticulocytes contain a bluish cytoplasm and why is it blue?

A

because of ribosomal RNA

106
Q

_____ and ______ cause a WBC>50,000 with an inc in___________. How is the leukocyte (neutrophil) alkaline phosphatase leveL?

A

CML, leukemia reaction

  • precursor forms( bands, metamyelocytes, myelocytes)
  • CML: Dec (b/c WMCs are cytochemically abnormal), leukemoid rxn: inc
107
Q

Phosphorylated Rb leads to _____ transcription

A

inc

108
Q

erythroblastalis fetalis can lead to high levels of unconjugated bilirubin and SEVERE consequence of this is

A

kernicterus (can damage basal ganglia, thalamus)

109
Q

Hereditary spherocytosis can lead to __________ unconjugated bilirubin—>

A

inc, gallstones

110
Q

reverse anticoagulation (heparin use) with

A

protamine sulfate

111
Q

replace warfarin overdose with

A

fresh frozen plasma

112
Q

reverse low fibrinogen levels with

A

cryoprecipitate (fibrinogen, factor 7, vWF)

113
Q

heparin doesn’t cross the placenta because its _____phillic

A

hydro (high water solubility)

114
Q

What are three hematological abnormalities in PNH?

A
  • hemolytic anemia (C5b-9 MAC formation),
  • hypercoagulable state (complement attack on platelets–> release of thrombogenic molecules
  • pancytopenia (aplastic anemia from lack of CD55,59)
115
Q

________ are the only drugs used to treat brain tumors

A

nitrosureas

116
Q

CA 19-9 is for ______ cancer

A

pancreatic

117
Q

CA-125 is for _______ cancer

A

ovarian/endometrial cancer

118
Q

abnormally large platelets on peripheral blood smear

A

Bernard Soulier (absence GP lb)

119
Q

von willi brand disease you will see ___ PT, _____ platelet count, _____ PTT, ______ bleeding time

A

normal PT, normal platelet count, prolonged PTT (factor 8), prolonged bleeding time

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