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UWORLD > Hematology > Flashcards

Hematology Flashcards

1
Q

What clinical features are necessary to consider Thrombotic Thrombocytopenic purpura?

A

Microangiopathic hemolytic anemia and Thrombocytopenia

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2
Q

What causes Thrombotic Thrombocytopenic purpura?

A

Inhibition or deficiency of ADAMTS13 (cleaves vWF), leading to vWF multimers

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3
Q

What is the deficiency in Acute Intermittent Porphyria?

A

Porphobilinogen deaminase

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4
Q

What is the treatment for Acute Intermittent Porphyria?

A

Hemin- downregulates aminolevulinate synthase

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5
Q

What mutations are common in hereditary spherocytosis?

A

Scaffolding proteins- ankyrin and spectrin

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6
Q

What are the symptoms associated with hereditary spherocytosis?

A

hemolytic anemia, jaundice, and splenomegaly. Infection can trigger acute hemolytic crisis

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7
Q

Why can’t mature erythrocytes produce heme?

A

Mitochondria are required for the first and last 3 steps of heme synthesis

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8
Q

How does posttranscriptional silencing work?

A

miRNAs and siRNAs are incorporated into RNA-induced silencing complexes. If the RISC and mRNA are a perfect match, they mRNA will be cleaves. If they are a partial match, there will be translational repression, because the ribosome cannot bind

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9
Q

What are the major mechanisms of cell death via ionizing radiation?

A

DNA double strand break

Free radical formation

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10
Q

What is the surface marker for monocyte-macrophage lineage?

A

CD14

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11
Q

What is pure red cell aplasia? With which diseases is it associated?

A

Rare form of marrow failure characterized by hypoplasia of marrow erythroid elements in the setting of normal granulopoiesis and thrombopoiesis.
Associated with thymoma, lymphocytic leukemias, and parvo b19

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12
Q

Why is desmopressin beneficial to patients with von willebrand disease?

A

Increases vWF factor release from endothelial cells. Also raises circulating levels of factor VIII (hemophilia A)

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13
Q

What is the drug of choice in treatment of heparin-induced thrombocytopenia?

A

Direct thrombin inhibitor (-rudin, argatroban, dabigatran)

also, stop heparin

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14
Q

Which myeloproliferative disorders have a mutation in JAK2?

A

Essential thrombocytosis
Polycythemia vera
Primary Myelofibrosis

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15
Q

What is primary myelofibrosis?

A

Megakaryocytic hyperplasia stimulates fibroblasts, leading to replacement of marrow space with collagen. Hepatosplenomegaly is due to extramedullary hematopoiesis. Teardrop cells

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16
Q

Name the steps and special proteins involved in inflammatory leukocyte migration

A
  1. Margination
  2. Rolling: Sialylated carb groups bind to L-Selectin on neutrophils or E/P-selectin on endothelial cells
  3. Activation
  4. Tight adhesion and crawling: Neutrophilic CD 18 beta 2 integrins bind to endothelial ICAM-1
  5. Transmigration: Neutrophil integrin attachment to PECAM-1
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17
Q

What are the types of leukocyte adhesion deficiencies?

A

Type 1: absence of CD18, recurrent skin infections sans pus, delayed detachment of umbilical cord
Type 2: less severe
Type 3: similar to type 1

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18
Q

What are the symptoms associated with polycythemia vera?

A

aquagenic pruritis, facial plethora, splenomegaly

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19
Q

What causes polycythemia vera?

A

mutation in JAK2, a cytoplasmic (non-receptor) tyrosine kinase

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20
Q

What are heterophile antibodies?

A

Abs which react to antigens from animal erythrocytes

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21
Q

Which malignancies are associated with EBV?

A 
Burkitt Lymphoma (especially endemic)
Nasopharyngeal carcinoma
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22
Q

What are the stop codons?

A

UAA, UGA, UAG

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23
Q

What determines the prognosis of colorectal adenocarcinoma?

A

Tumor stage

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24
Q

What is the histological appearance of the spleen in SS patients after repeated infarction?

A

significant scarring, fibrosis, and atrophy

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25
Q

Why do SS patient have increased folic acid demand?

A

Increased erythrocyte turnover

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26
Q

What is a common histochemical marker for epithelial-derived tumors?

A

cytokeratin

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27
Q

What does the HER2 oncogene do?

A

codes for a human epidermal growth factor receptor with tyrosine kinase activity in the intracellular domain- activates epithelial growth and differentiation, reduces apoptosis.

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28
Q

What drug can be used for HER2 + carcinomas?

A

Trastuzumab

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29
Q

What are the steps involved in basement membrane penetration?

A
  1. detach by reducing E-cadherin expression
  2. Attach to BM by increasing Laminin
  3. Invade by releasing proteolyic enzymes
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30
Q

What is haptoglobin?

A

The thing in blood that binds to hemoglobin after hemolysis

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31
Q

What happens when there is a point mutation in the kozak consensus sequence of the beta globin?

A

ribosome does not assemble–> translation is not initiated–> beta thalassemia

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32
Q

What symptoms are worrisome for spinal metastases?

A

advanced age, progressive and persistent pain that is worse at night, not relieved by rest or analgesics, and is accompanied by systemic symptoms

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33
Q

Addition of what substance to a folate-deficient medium can reduce apoptosis?

A

Thymidine

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34
Q

What are the symptoms of infectious mononucleosis?

A

sore throat, myalgias, malaise, lymphadenopathy, splenomegaly, and fever

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35
Q

How does EBV infect lymphocytes?

A

binds to CD-21 on B-cells and infects them. Reactive (atypical) CD 8 T-cells then kill the virally infected B cells

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36
Q

How do reactive (atypical) T-cells look?

A

Larger with abundant cytoplasm, eccentrically placed nucleus, and cells membrane that conforms to border of other cells.

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37
Q

What stage of the cell cycle do Vinka alkaloids inhibit?

A

M phase by binding to beta tubulin and preventing polymerization of microtubule proteins

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38
Q

What are the signs and symptoms of multiple myeloma?

A

bone pain, fatigue, anemia, kidney dz, and hypercalcemia

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39
Q

What is the mechanism of action of bortezomib (boronic acid containing peptide) in MM?

A

In MM too much Ig is being made- bortezomib inhibits the proteasome, so there is accumulation of toxic intracellular proteins and proapoptotic proteins

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40
Q

Where does complement (C1) bind the Fc portion of IgG or IgM?

A

The Fc portion closer to the hinge region

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41
Q

What is a common cause for bleeding complications in neonates that do not receive normal care?

A

Vitamin K deficiency- need prophylactic supplementation. Vit K needed for gamma carboxylation

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42
Q

Which other enzyme deficiency can present similarly to G6PD deficiency?

A

Glutathione reductase- Along with NADPH, it is necessary for reduction of oxidized glutathione.

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43
Q

What is the acquired mutation in paroxysmal nocturnal hemoglobinuria?

A

PIGA gene that is involved in synthesis of GPI anchor that is needed for CD55 and CD59 attachment. CD55 and CD59 are need to inactivate complement and prevent MAC forming on normal cells.

44
Q

What are the s/s of paroxysmal nocturnal hemoglobinuria?

A

hemolytic anemia, hypercoagulability (due to release of free Hgb and other prothrombotic factors from lysed red blood cells), and pancytopenia.
More hemolysis at night due to lower blood pH.

45
Q

What is the effect of pyruvate kinase deficiency on RBCs?

A

Can’t produce ATP, so lose concentration gradients and defective maintenance of cell membrane architecture–> hemolysis.

46
Q

What effect do damaged red blood cells have on the spleen?

A

Hyperplasia of the red pulp since reticuloendothelial cells are involved in the removal of damaged RBCs

47
Q

What does integrin bind to in the ECM?

A

fibronectin, collagen, and laminin

48
Q

What are some extrapulmonary manifestations of Mycoplasma pneumoniae?

A

Hemolytic anemia from cold agglutinins, SJS, joint pains, encephalitis, cardiac rhythm disturbances, bullous myringitis

49
Q

What are the symptoms associated with B12 deficiency?

A

megaloblastic anemia, paresthesias, weakness, ataxic gait (dorsal and lateral columns)

50
Q

What are some causes of aplastic anemia?

A

Autoimmune
Drug: Carbamazepine, chloramphenicol, sulfonamides
Infection: Parvo B19, EBV
Exposure to radiation or toxins- benzene, solvents

51
Q

What is the presentation of aplastic anemia?

A

pancytopenia without splenomegaly

Dry tap- bone marrow is hypocellular with hematopoietic elements replaced by fat and bone marrow stroma

52
Q

How does protein c restrict clot formation?

A

inactivates Va and VIIIa

53
Q

What are the lab values associated with uremic platelet dysfunction?

A

Normal PT, PTT, and platelet count

Increased bleeding time

54
Q

What type of anemia is seen in SLE?

A

Autoimmune hemolysis due to warm IgG antibodies against RBCs.
Spherocytosis, + direct coombs test, extravascular hemolysis
Can also get leukopenia and thrombocytopenia
Can lead to IC deposit and Lupus nephritis

55
Q

Why does t(15;17) cause malignancy?

A

Leads to abnormal RARareceptor that can’t signal for myeloid differentiation at physiologic levels of retinoic acid

56
Q

Discuss the steps in the activation of the mTOR pathway

A
  1. growth factor binds receptor tyrosine kinase–> autophosphorylation
  2. phosphotyrosine residues activate PI3K
  3. Phosphorylates PIP2–>PIP3
  4. Activation of Akt
  5. Activates mTOR
  6. Translocates to nucleus and induces genes for survival, antiapoptosis, and angiogenesis
57
Q

What histologic feature indicates myeloid differentiation?

A

Auer rods

Stain for peroxidase

58
Q

What are the clinical s/s of AML and what causes them?

A

anemia, thrombocytopenia, and neutropenia due to marrow replacement by leukopenic cells

59
Q

Which of the most common non hodgkin lymphomas is indolent with a waxing and waning course?

A

follicular lymphoma- most often present with painless lymph node enlargement

60
Q

What types of mutations cause beta thalassemia?

A

ones that result in defective transcription, processing, and translation of Beta-globin mRNA

61
Q

Which symptoms are associated with platelet deficiencies versus factor deficiencies?

A

Platelet: Epistaxis, gingival bleeding
Factor: hemarthrosis, bruising

62
Q

How does HIV become resistant to protease inhibitors, NRTIs, and NNRTI?

A

Pol gene mutation

63
Q

What is the peau d’orange rash and what causes it?

A

Itchy, erythematous rash with firm and coarse skin texture- rapidly progressive
peau d’orange + breast edema=inflammatory breast cancer
The rash is caused by cancerous cells obstructing lymph drainage

64
Q

What are characteristic lab abnormalities in HUS?

A

decreased hemoglobin and platelet count and increased bleeding time, LDH, bilirubin, BUN, and creatinine

65
Q

What is Wiskott-Aldrich syndrome and what are the three cardinal findings?

A

XLR B cell and Tcell disorder
Eczema, recurrent infections (especially polysaccharide capsular organisms), and thrombocytopenia (petechiae, purpura, hematemesis)

66
Q

What causes a right shift of the oxygen-hemoglobin dissociation curve

A

increased H+, 2-3BPG, Temp,

67
Q

Which antiemetic drugs and receptors are useful in the treatment of chemotherapy induced nausea?

A

5-HT3 receptor antagonist: ondansetron- blocks vagus mediated nausea and central 5HT receptors in the area postrema
NK1 receptor antagonist: aprepitant- inhibits substance P
DA antagonist: metoclopramide

68
Q

What mutation causes X-linked sideroblastic anemia? What vitamin is a cofactor for this enzyme?

A

ALA synthase

B6–> pyridoxal 5’ phosphate

69
Q

What are the symptoms of B6 deficiency?

A

dermatitis, stomatitis, neuropathy, confusion, and sideroblastic anemia

70
Q

What are the treatments for cyanide poisoning?

A

Inhaled amyl nitrate: changes hemoglobin into methemoglobin, which bind better to CN than O2.

Hydroxycobalamin and sodium thiosulfate- turn CN into relatively nontoxic metabolites

71
Q

What are the symptoms of CN poisoning?

A

Reddish skin discoloration, tachypnea, headache, and tachycardia, often accompanied by N/V, confusion, and weakness

72
Q

What happens in CCl4 poisoning?

A

CYP 450 metabolizes CCl4 to toxic CCl3. This form free radicals that attack the the plasma membrane via lipid peroxidation, which leads to further damage from the peroxide created in the process

73
Q

Where does B cell VDJ recombination take place?

A

bone marrow

74
Q

Where does isotype switching occur?

A

in germinal centers within the follicles of the lymph node

75
Q

What are the skin findings in dermatomyositis?

A

heliotrope rash in the peri-orbital area and cheeks

Gottron’s papules- raised erythematous papules over joints and bony prominences of the hands

76
Q

With which malignancies is dermatomyositis associated?

A

ovarian, lung, colorectal, and non-hodgkin lymphoma

77
Q

What is a pathognomonic blood smear finding in AML?

A

Auer rods

78
Q

Why does overexpression of BCL2 lead to malignancy?

A

inhibits apoptosis of tumor cells

79
Q

What is the most common brain tumor in adults? What are the histological findings?

A

Glioblastoma multiforme
Gross: areas of necrosis and hemorrhage
Light microscopy: psudopalisading tumor cells and areas of necrosis

80
Q

What are the markers in ALL vs. AML?

A

AML: peroxidase positive granules (auer rods)
ALL: TdT+ and PAS+

81
Q

What are the precursor B cell markers?

A

CD10, CD19

82
Q

What are the precursor T cell markers?

A

CD 1, 2, 3, 4, 5, 7, 8

83
Q

What downregulates the T-cell response to tumor cells?

A

Binding of PD-1 to PD-1L. MABs blocking PD-1 can promote apoptosis of tumor cells

84
Q

Why would erythrocytes have not net gain of ATP in glycolysis?

A

If they were making 2,3-BPG during a hypoxic state- detours from the step that would generate ATP

85
Q

What is the treatment for carcinoid syndrome?

A

octreotide for symptomatic patients

surgery for liver metastases

86
Q

What is the primary mechanism of iron deficiency anemia?

A

blood loss. In the GI tract it is most often occult blood loss

87
Q

What is the product of c-myc?

A

a nuclear phosphoprotein that functions as a transcription activator controlling cell proliferation, differentiation, and apoptosis

88
Q

What is the leukemoid reaction? What are leukocyte alkaline phosphatase levels? What is seen on peripheral smear?

A

Leukemoid reaction is a benign leukocytosis in response to severe infection
The leukocyte alkaline phosphatase levels are normal or increased (decreased in CML)
There are basophilic inclusions in mature neutrophils- ribosomes bound with RER

89
Q

What is the action of the Rb tumor suppresor gene

A

Rb is active in its hypophosphorylated state and prevent the cell from moving from G1 to S

90
Q

What happens with an activating mutation in a KRAS gene?

A

Constitutive downstream signaling in the absence of EGFR–> increased cell proliferation and growth that is resistant to anti-EGFR meds.

91
Q

Which drugs cause aplastic anemia?

A

carbamazepine, chloramphenicol, sulfonamides

92
Q

When do T cells stop expressing both CD4 and CD8?

A

After negative selection is complete

93
Q

What causes the greenish color from a bruise after a few days?

A

Erythrocyte destruction–> release of iron-containing heme molecules that are degraded by heme oxygenase to biliverdin, CO2, and Fe. Biliverdin is green and is then further reduced to the yellow pigment bilirubin, whichis then transferred to the liver bound to albumin

94
Q

WHy is a reticulocyte bluer than an erythrocyte?

A

Because it retains its basophilic, reticular network of residual rRNA

95
Q

What nail changes are seen in iron deficiency?

A

koilonychia- spoon-shaped nails

96
Q

What causes primary hemochromatosis?

A

HFE gene mutation- normally complexes with transferrin to sense iron stores, but mutation causes it to detect falsely low iron levels.
This causes enterocytes to increase apical expression of DMT 1, which increases iron absorption from the intestinal lumen. Hepatocytes decrease hepcidin synthesis, resulting in increased ferroportin expression on the basolateral surface of enterocytes, leading to increased iron absorption

97
Q

What is the main mediator of neoplastic cachexia?

A

TNF-alpha:

  • hypothalamus: appetite suppression
  • Increases basal metabolic rate
98
Q

What are the characteristic s/s/pathology of Hairy cell leukemia?

A

dry tap with marrow fibrosis, massive splenomegaly

lymphocytes with filamentous, hair-like cytoplasmic projections. TRAP stain

99
Q

What is the treatment for hairy cell leukemia?

A

Cladribine and and pentostatin (adenosine analogs)

100
Q

What are the classic histological findings in Burkitt’s lymphoma?

A

Diffuse medium-sized lymphocytes and high proliferation index represented by high Ki-67 fraction
Starry sky appearance (macrophages)

101
Q

How does the ristocetin test work?

A

Ristocetin causes platelets to express GP1b. If platelets do not aggregate, then vWF level is decreased.

102
Q

What is the the first line therapy for menorrhagia due to vWF disease?

A

COCs

103
Q

What does the JAK2 V617F mutation do in polycythemia vera?

A

Makes hematopoietic cells more sensitive to growth factors

104
Q

Discuss the phases of apoptosis

A

Initiation: Bcl-2 and Bcl-x are replaced with proapoptotic Bak, Bax, and Bim, allowing for increased permebility of the mitochondria and release of cytochrome c, which activates caspase.
Execution: Caspases cleave cellular proteins and activate DNAses

105
Q

What is the most common cause of aplastic anemia in a patient with sickle cell?

A

Parvovirus B19

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