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USMLE > Heme/Onc > Flashcards

Heme/Onc Flashcards

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USMLE® Step 1 flashcards
1
Q

What are the dense granules of thrombocytes?

A

ADP, Ca+2

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2
Q

What are the alpha granules of thrombocytes?

A

vWF, fibrinogen, fibronectin

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3
Q

Where is 1/3 of platelet pool synthesized?

A

Spleen

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4
Q

Which leukocytes are granulocytes?

A

Neutrophils, eosinophils, basophils

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5
Q

Which leukocytes are mononuclear cells?

A

Monocytes (macrophage in tissue), Lymphocytes (T and B cells)

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6
Q

In an acute inflammmatory response, which leukocyte is predominant?

A

Neutrophil

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7
Q

List 5 chemotactic factors for neutrophils

A

IL-8, LTB-4, kallikrein, platelet-activating factor, C5a

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8
Q

Which cytokine activates macrophages in tissues?

A

gamma-interferon

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9
Q

which leukocyte is important in granuloma formation?

A

macrophage

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10
Q

How do macrophages initiate septic shock?

A

CD14 binds Lipid A from bacterial LPS

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11
Q

Which cells do macrophage present antigens to/

A

CD4 (via MHC II)

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12
Q

List the causes of eosinophilia (NAACP)

A

Neoplasia, Asthma, Allergies, Chronic Adrenal Insufficiency, Parasites

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13
Q

Basophilia can be a sign of which hematologic problem?

A

CML (myeloproliferative disease)

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14
Q

Which leukocyte produces major basic protein?

A

Eosinophil

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15
Q

Describe Type I hypersensitivity reaction (allergic response)

A

Mast cell binds Fc portion of IgE, IgE cross-links, degranulation, release of histamine, heparin, tryptase and eosinophil chemotactic factors

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16
Q

Cromolyn sodium is used to prevent

A

Degranulation of mast cells (allergic prophylaxis)

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17
Q

Which cell serves as the link between innate and adaptive immunity?

A

Dendritic cell

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18
Q

Which MHC do dendritic cells express?

A

MHC II

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19
Q

Which blood cell is affected in multiple myeloma?

A

plasma cell

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20
Q

Which blood cells make up the majority of circulating lymphocytes

A

T cells

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21
Q

Which blood type is a universal donor of plasma?

A

AB

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22
Q

Which blood type is universal recipient of plasma?

A

Type O

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23
Q

What is the purpose of administering RhoGAM to mothers/

A

prevents maternal anti-d IgG production in Rh- mothers against Rh+ positive fetus (prevents hemolytic disease of newborn)

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24
Q

What is ABO hemolytic disease of newborn

A

Hemolytic anemia of A, B, or AB fetus in type O mother. Presents as mild jaundice in first 24 hours of birth, treatment is phototherapy or exchange transfusion

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25
Q

Why can ABO hemolytic disease of newborn happen in first pregnancy and not Rh disease?

A

Anti-ABO antibodies are formed early in life while anti-D IgGs usually form after exposure to Rh

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26
Q

Which aspects of inflammation does PGE4 mediate?

A

Vasodilation, increased vascular permeability, pain and fever

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27
Q

Leukotrienes are primarily involved in which inflammatory response?

A

Anaphylaxis

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28
Q

What is the immediate response of mast cells in acute inflammation?

A

Release histamine to increase vasodilation of arterioles and vascular permeability

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29
Q

What is the delayed response of mast cells in acute inflammmation?

A

production of arachidonic acid metabolites, especially Leukotrienes

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30
Q

Which factors are involved in the Classical pathway for Complement activation?

A

IgM and IgG bound to antigen (bind C1)

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31
Q

Which factors activate the alternative pathway

A

microbial products

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32
Q

Which factors activate the Mannose-binding lectin pathway

A

MBL binds mannose on microorganisms to activate complement

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33
Q

Which complement factors are part of the MAC complex

A

C5b, C6-C9

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34
Q

Which complement protein is chemotactic for neutrophils

A

C5a

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35
Q

Which complement protein is an opsonin for phagocytosis?

A

C3b

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36
Q

Describe the function of Hageman factor (Factor XII)

A

Activates coagulation and fibrinolysis, complement and kinin system

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37
Q

Which inflammatory molecules are responsible for redness and warmth?

A

Histamine, Prostaglandins D2, E2 and I2, and bradykinin (vasodilators)

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38
Q

What are the key mediators of swelling in inflammmation?

A

Histamine (endothelial cell contraction) and tissue damage

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39
Q

Key mediators of pain in inflammation

A

Bradykinin and PGE2 (sensitize sensory nerve endings)

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40
Q

Key mediators of fever in inflammation?

A

Pyrogens that cause macrophage to release IL-1 and TNF (increase COX in perivascular cells of hypothalamus)

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41
Q

Which prostaglandin mediates fever in the hypothalamus?

A

PGE2

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42
Q

What are the features of autosomal recessive integrin deficiency? (CD18)

A

Delayed separation of umbilical cord, increased circulating neutrophils and recurrent bacterial infections that lack pus formation

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43
Q

What are the features of Chediak-Higashi syndrome?

A

Increased risk of pyogenic infections, neutropenia, giant granules on leukocytes, albinism, peripheral neuropathy, defective primary hemostasis

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44
Q

Which cellular mechanism is defective in Chediak-Higashi syndrome?

A

Cell-trafficking and phagolysosome formation

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45
Q

Which enzyme is defective in Chronic Granulomatous Disease?

A

NADPH Oxidase (converts O2 –> Superoxide radical)

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46
Q

People with CGD are prone to infections by which organisms?

A

Catalase-positive (S. aureus, Pseudomonas, Serratia, Nocardia, Aspergillus)

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47
Q

Which test is used to screen for CGD

A

Nitroblue tetrazolium (leukocytes turn blue if normal, stay colorless if NADPH oxidase is defective)

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48
Q

MPO deficiency increases risk of which infections?

A

Candida infections

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49
Q

What are the anti-inflammatory cytokines produced by macrophages?

A

IL-10 and TGF-beta

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50
Q

Which cytokine from macrophages recruits additional neutrophils?

A

IL-8

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51
Q

Which leukocytes mediate abscess formation?

A

Macrophages via fibrosis

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52
Q

Which T cells are involved in chronic inflammation?

A

CD4+ T cells activated by MHCII of macrophages

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53
Q

Which cells express MHC I and what do they present?

A

All nucleated cells, intracellular antigens (viruses)

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54
Q

Which cells express MHC II and what do they present?

A

Antigen-presenting cells (dendritic, macrophage), extracellular antigens (parasites, bacteria, etc)

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55
Q

Which two interactions activate CD4+ helper T cells?

A

B7 on APC to CD28 on T cell and MHCII on APC to TCR (+CD3) on T cell

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56
Q

What are the two subsets that helper T cells divide into?

A

TH1 and TH2

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57
Q

Which cytokines are secreted by TH1 cells?

A

IFN-gamma to activate macrophage and promote B cell class-switch to IgG, IL-2 to activate CD8+ T cells (and inhibit TH2)

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58
Q

Which cytokines do TH2 cells secrete and what is their function?

A

IL-4 (B cell class switch to IgE); IL-5 (recruit eosinophils); IL-13 (B cell class switch); IL-10 (inhibit TH2)

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59
Q

What is the second activation signal for CD8+ T cells?

A

IL-2 secreted by TH1 cells

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60
Q

How do CD8+ T cells kill infected cells? (2 ways)

A

Secrete perforin and granzyme to activate apoptosis; express FasL to bind Fas on target cells and activate apoptosis

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61
Q

List 3 ways to activate apoptosis

A

cyt c, Fas-FasL, granzyme via CD8+

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62
Q

Which two signals activate B cells to become plasma cells?

A

CD40 on B binds CD40L on helper T cell; BCR-MHCII

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63
Q

What 2 cytokines released by helper T cell promote B cell class switch?

A

IL-4 and IL-5

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64
Q

Describe what a granuloma looks like

A

epithelioid histiocytes (macrophage with abundant pink cytoplasm) surrounded by giant cells and rim of lymphocytes

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65
Q

Difference between caseating and non-caseating granulomas

A

Non-caseating granulomas lack central necrosis; caseating granulomas are characteristic of tuberculous and fungal infections

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66
Q

Which cytokine induces helper T cells to turn into TH1 subtype?

A

IL-12

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67
Q

Describe how granulomas form

A

Macrophage ingest pathogen and present to CD4 T cell on MHC II–> macrophage secrete IL-12 to differentiate T cell into TH1 cell–> TH1 cell secretes IFN-gamma to activate macrophages into epithelioid histiocytes and giant cells

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68
Q

List the clinical presentation of DiGeorge syndrome

A

T-cell deficiency (thymic aplasia), hypocalcemia (parathyroid aplasia), abnormalities of heart, great vessels and face

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69
Q

What are three major causes of SCID?

A

Cytokine receptor defects (most common); ADA deficiency (leads to oxidative death of lymphocytes); MHC II deficiency (cannot activate T cells and cytokine production)

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70
Q

X-linked agammaglobulinemia is a mutation in which enzyme?

A

Bruton tyrosine kinase

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71
Q

What is the defect in X-linked Agammaglobulinemia?

A

Pre- and pro-B cells cannot mature to plasma cells (complete lack of immunoglobulins)

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72
Q

Presentation of X-linked Agammaglobulinemia

A

presents after 6 months of life with recurrent bacterial, enterovirus and Giardia infections

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73
Q

Why does X-linked agammaglobulinemia present after 6 months?

A

Infant no longer has protective maternal IgGs

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74
Q

Patients with Common variable immunodeficiency are at increased risk of which diseases?

A

Autoimmmune diseases and lymphoma

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75
Q

What is the difference in presentation between X-linked agammaglobluinemia and common variable immunodeficiency?

A

CVID arises in late childhood (not after 6 months) and blood tests show low Ig count

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76
Q

Which protein is mutated in hyper-IgM syndrome?

A

CD40L (on helper T cells) or CD40 receptor (on B cells)

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77
Q

What is the Ig presentation of hyper-IgM syndrome?

A

elevated IgM, low IgA, IgG and IgE (recurrent pyogenic infections) at mucosal sites

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78
Q

Which gene is mutated in Wiskott-Aldrich syndrome?

A

WASP (XLR)

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79
Q

Clinical presentation of Wiskott-Aldrich Syndrome?

A

Eczema, thrombocytopenia (low platelet count), and recurrent infections

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80
Q

What is the major cause of death in Wiskott-Aldrich syndrome?

A

Bleeding

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81
Q

Patients who are prone to Neisseria infections are deficient in which immune component?

A

Complement C5-C9 (MAC complex)

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82
Q

What causes hereditary angioedema (inflammation around orbitals)

A

C1 deficiency (persistent vasodilation)

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83
Q

What does central tolerance lead to?

A

T cell apoptosis or generation of regulatory T cells

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84
Q

Which mutation leads to autoimmune polyendocrine syndrome (hypoparathyroidism, adrenal failure, chronic candida infections)

A

AIRE

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85
Q

Which 2 autoimmune conditions are polymorphisms in CD25 associated with?

A

MS and T1DM

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86
Q

What is a the phenotype of Treg cells?

A

CD3+ CD4+ CD25+ FOXP3+

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87
Q

IPEX syndrome is a mutation in which protein?

A

FOXP3 on Treg cells

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88
Q

Describe symptoms of IPEX syndrome

A

Immune dysregulation
Polyendocrinopathy
Etneropathy
X-linked

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89
Q

What type of Hypersensitivity reaction is SLE?

A

Type III (Antigen-Antibody complexes)

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90
Q

Deficiency of which complement proteins associated with SLE?

A

C1q, C4 and C2 (needed to clear Ab-Ag complexes)

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91
Q

Which antibodies are highly specific for SLE?

A

Anti-Sm (mitochondrial) and Anti-dsDNA

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92
Q

Which antiphospholipid antibodies are elevated in SLE?

A

anticardiolipin, anti-beta2-glycoprotein I, and lupus anticoagulant (falsely-elevated PTT)

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93
Q

What is a complication of antiphospholipid syndrome?

A

recurrent miscarriages

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94
Q

What is antihistone antibody specific for?

A

Drug-induced lupus

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95
Q

anti-SSA/Ro and anti-SSB/La associated with which disorder

A

Sjogren syndrome

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96
Q

What type of HS reaction is Sjogrens

A

Type IV Lymphocyte-mediated damage with fibrosis

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97
Q

Which malignancy commonly presents with Sjogrens

A

Rheumatoid arthritis

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98
Q

What risks exists for babies with mother’s who have anti-SSA?

A

neonatal lupus, congenital heart block

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99
Q

B cell (marginal) zone lymphoma risk is increased in which condition?

A

Sjogren’s

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100
Q

What is the diagnostic criteria for Sjogren’s?

A

Lymphocytic sialedenitis on lip biopsy

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101
Q

What are the two types of scleroderma and what are their antibodies?

A 
Limited type (CREST syndrome): anti-centromere
Diffuse type (any organ, especially lungs): anti-Scl70 (DNA Topoisomerase I)
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102
Q

What is primary intention?

A

Wound edges brought together, minimal scar formation

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103
Q

What is secondary intention wound healing?

A

Edges not approximated–granulation tissue fills defect; myofibroblasts contract–scar formation

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104
Q

Deficiencies that cause delayed wound healing?

A

Vitamin C (hydroxylation of proline and lysine of procollagen– collagen cross-linking)
Copper (cofactor for lysyl oxidase to cross-link collagen)
Zinc (cofactor for collagenase)

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105
Q

How do disorders in primary hemostasis typically manifest?

A

Mucosal or skin bleeding

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106
Q

What is the most common symptom of primary hemostasis disorders

A

epistaxis

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107
Q

List symptoms of mucosal and skin bleeding common to primary hemostasis disorders

A

epistaxis (nosebleeds), hemoptysis, GI bleeds, petechiae, purpura, easy bruising, echhymoses

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108
Q

What is Immune Thrombocytopenic Purpura?

A

Splenic generation of autoantibodies against platelets (IgG)

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109
Q

What are the two forms of ITP?

A

Acute (in children, usually after viral infection)

Chronic (in women, usually pregnant, secondary to autoimmune dz, can lead to ITP in kids)

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110
Q

Lab findings of ITP?

A

thrombocytopenia (<50k), normal PT/PTT, megakaryocytosis

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111
Q

What is treatment of ITP for refractory cases?

A

Splenectomy

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112
Q

What types of cells can be seen in microangiopathic hemolytic anemia?

A

Schistocytes (helmet cells)

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113
Q

What are two major causes of Microangiopathic Hemolytic Anemia?

A

HUS and TTP

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114
Q

Mutation in which enzyme causes thrombotic thrombocytopenic purpura (TTP)

A

ADAMTS13

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115
Q

What is the function of ADAMTS13?

A

Cleaves vWF multimers (polymeric vWF cause abnormal aggregation and microthrombi)

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116
Q

How is deficiency of ADAMTS13 acquired?

A

Autoimmunity (autoantibody), more common in females

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117
Q

What causes hemolytic uremic syndrome (HUS)?

A

endothelial damage by drugs or infection

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118
Q

What is the most common cause of HUS?

A

E Coli O157:H7 (undercooked beef); verotoxin damages endothelial cells

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119
Q

Clinical findings of HUS/TTP

A

Skin/mucosal bleeding, hemolytic anemia (schistocytes), fever

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120
Q

Clinical finding specific to HUS

A

Renal insufficiency (elevated Creatinine/BUN)

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121
Q

Clinical finding specific to HUS

A

CNS involvement (hydroencephaly, confusion, etc)

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122
Q

Lab findings of HUS/TTP

A

Thrombocytopenia, longer bleeding time, normal PT/PTT, anemia with schistocytes, megakaryocytosis

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123
Q

What is the treatment for TTP/HUS?

A

Plasmapharesis (protein removal) and corticosteroids

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124
Q

What is the defect in Bernard-Soulier syndrome?

A

defective GPIb so platelet cannot adhere to endothelial cell

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125
Q

What is the defect in Glanzmann thrombasthenia?

A

GPIIb/IIIa deficient so platelet aggregation is impaired

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126
Q

How does aspirin affect platelet aggregation?

A

Inhibits COX irreversibly, impairing aggregation

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127
Q

What are the clinical features of secondary hemostasis disorders (coagulation cascade)

A

Deep tissue bleeding into muscles and joints; rebleeding after surgeries

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128
Q

Which pathway/factors does prothrombin time measure?

A

Extrinsic: 7; Common: 2, 5, 10, fibrinogen

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129
Q

Which pathway/factors does partial thromboplastin time measure?

A

Intrinsic (12, 11, 9, 8) and common (2, 5, 10, fibrinogen)

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130
Q

How is Hemophilia A inherited?

A

XLR or de novo

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131
Q

Which factor is deficient in Hemophilia A?

A

VIII of intrinsic pathway

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132
Q

Lab findings of Hemophilia A

A

Increased PTT; normal PT
Decreased Factor VIII
Normal platelet count and bleeding time

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133
Q

Symptoms of Hemophilia A

A

Deep tissue bleeding; rebleeding after surgical procedures

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134
Q

Which factor is deficient in Hemophilia B (Christmas disease)?

A

Factor IX

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135
Q

How to distinguish between Coagulation Factor Inhibitor and Hemophilia A?

A

In CFI, PTT does not correct after mixing normal plasma with patient’s plasma (bc antibodies impair VIII function, there is no deficiency)

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136
Q

What is von Willebrand Disease?

A

Genetic vWF deficiency, most common inherited coagulation disorder

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137
Q

How is the most common type of vWF disease inherited?

A

AD

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138
Q

Lab findings of vWF disease?

A

Increased bleeding time, increased PTT (decreased VIII deficiency), abnormal ristocetin test (impaired agglutination)

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139
Q

Describe the ristocetin test

A

Induces platelet agglutination by having vWF bind platelet GPIb; checks for vWF disease

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140
Q

Treatment for von Willebrand disease?

A

Desmopressin (ADH analogue that stimulates vWF production from Weibel-Palade bodies of endothelial cells)

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141
Q

Where is Vitamin K produced

A

gut flora

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142
Q

How does vitamin K deficiency lead to hemorrhage?

A

Vitamin K is needed to gamma carboxylate factors (activate) 2, 7, 9, 1 and Proteins C and S, all of which are needed for coagulation; deficiency keeps them inactive, so coagulation can’t occur

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143
Q

Who is at greatest risk of Vitamin K deficiency?

A

Newborns (sterile gut)
Pts with Malabsorption syndromes
Pts on long-term broad-spectrum antibiotics

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144
Q

How does liver failure lead to thrombocytopenia and abnormal hemostasis?

A

Decreased production of coagulation factors (monitor PT)

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145
Q

Laboratory findings of disseminated intravascular coagulation

A

thrombocytopenia (decreased platelet count)
increased PT/PTT
decreased fibrinogen
hemolytic anemia (schistocytes)

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146
Q

Which test confirms DIC dx?

A

Elevated D-dimer (split products of fibrin)

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147
Q

How is a clot removed?

A

fibrin broken down via plasmin, coagulation factors destroyed, platelet aggregation inhibited

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148
Q

What activate plasmin from plasminogen?

A

tPA

149
Q

What inactivates plasmin?

A

alpha2-antiplasmin

150
Q

What do disorders in fibrinolysis resemble?

A

DIC

151
Q

How to distinguish disorders in fibrinolysis from DIC?

A

D-dimers in DIC, only fibrinogen split products in fibrinolysis disorder

152
Q

Treatment for disorder in fibrinolysis?

A

aminocaproic acid

153
Q

Labs for disorder in fibrinolysis?

A

increased bleeding time (clotting factors destroyed by overactive plasmin), increased PT/PTT

154
Q

What is the most common site for thrombi development?

A

Deep veins below the knee

155
Q

How to distinguish thrombus from postmortem clot?

A

Lines of Zahn and attachment to vessel wall

156
Q

What is Virchow’s triad?

A

3 risk factors for thrombosis: disruption of blood flow, endothelial cell damage, hypercoagulable state

157
Q

3 main causes of endothelial damage

A

atherosclerosis, vasculitis, homocysteine

158
Q

How do folate and B12 deficiency increase the risk of thrombosis?

A

Lead to mild elevation in homocysteine, which damages endothelial cells and can promote clotting cascade

159
Q

Which congenital disorder leads to high levels of homocysteine with homocysteinuria?

A

Cystathionine beta synthase deficiency

160
Q

Clinical features of cystathione beta synthase deficiency?

A

vessel thrombosis, mental retardation, lens dislocation, long slender fingers, death at young age

161
Q

Three main sites of DVTs?

A

Deep veins of legs, hepatic and cerebral veins

162
Q

What clinical feature is an indication of hypercoagulable state?

A

Recurrent DVTs, DVTs at a young age

163
Q

Which disorder is the most common inherited cause of hypercoagulable state?

A

Factor V Leiden (cannot be inactivated by Protein C or S bc lacks cleavage site)

164
Q

How does PTT behave in ATIII deficiency with Heparin dosing?

A

does not increase like it should

165
Q

How are oral contraceptives related to a hypercoagulable state?

A

Estrogen increases synthesis of coagulation factors

166
Q

On histology, what do you find in an atherosclerotic embolus?

A

Cholesterol clefts

167
Q

What increases the risk of fat embolus?

A

Bone fractures

168
Q

Laprascopic surgery increases the risk of which embolus?

A

Gas embolus

169
Q

How does an amniotic fluid embolus present?

A

SOB, neuro symptoms, DIC; characterized by squamous cells and keratin debris from fetal skin

170
Q

A saddle embolus that causes sudden death likely originated from which part of the body?

A

Deep veins below the knee

171
Q

What are the presenting signs and symptoms of anemia?

A

Weakness, fatigue, and dyspnea
Pale conjunctiva and skin
Headache and lightheadedness
Angina

172
Q

List 4 microcytic anemias

A

Iron-deficiency anemia, sideroblastic anemia, thalassemia, anemia of chronic disease

173
Q

Where does iron absorption occur in gut?

A

Duodenum

174
Q

Why does iron deficiency cause microcytic anemia?

A

Low iron = low hemoglobin so RBCs divide and extra time to maintain hemoglobin concentration

175
Q

Which two hookworms are linked to iron-deficiency anemia?

A

Ancylostoma duodenalle; Necator americanus

176
Q

What are the lab values of early stages of iron deficiency?

A

Low ferritin (stored iron is depleted first), increased TIBC (measure of transferrin molecules in blood)

177
Q

What are lab values for iron-deficiency anemia after ferritin is depleted?

A

decreased serum iron, decreased % saturation (% of transferrin bound by iron)

178
Q

Lab findings of iron-deficiency anemia

A

microcytic, hypochromic RBCs with increased red cell distribution width; decreased ferritin, increased TIBC, decreased % saturation, decreased serum iron; increased free erythrocyte protoporphyrin

179
Q

How does Plummer-Vinson Syndrome present?

A

Beefy-red tongue, dysphagia, iron-deficiency anemia due to esophageal web and atrophic glossitis

180
Q

Which enzyme is involved in anemia of chronic disease?

A

Hepcidin

181
Q

Lab values of anemia of chronic disease?

A

increased ferritin (sequestered iron), decreased TIBC, decreased serum iron, decreased % saturation, increased FEP

182
Q

Which vitamin deficiency can lead to sideroblastic anemia?

A

B6

183
Q

What causes sideroblastic anemia?

A

Decreased protoporphyrin synthesis

184
Q

What are sideroblasts?

A

RBCs with iron-laden mitochondria forming ring around nucleus

185
Q

List three causes of acquired sideroblastic anemia

A
  1. Lead poisoning
  2. Alcoholism
  3. B6 deficiency
186
Q

How does lead poisoning cause sideroblastic (microcytic) anemia?

A

inhibits ALAD and ferrochelatase

187
Q

How does alcoholism cause sideroblastic anemia?

A

Poisons mitochondria- site of protoporphyrin synthesis

188
Q

How does B6 deficiency lead to sideroblastic anemia?

A

ALAS (rate-limiting step) can only function with B6 as a cofactor
commonly a side-effect of isoniazid therapy

189
Q

Lab findings of sideroblastic anemia

A

increased ferritin, decreased TIBC, increased serum iron, increased %saturation

190
Q

Which chromosome has beta globin genes?

A

Chromosome 11

191
Q

Which chromosome has alpha globin genes

A

Chromosome 16

192
Q

How many copies of beta globin are there?

A

2

193
Q

How many copies of alpha globin gene are there?

A

4

194
Q

Which genetic error causes alpha thalassemias?

A

gene deletion

195
Q

Which genetic mutation causes beta thalassemias?

A

gene mutation

196
Q

clinical presentation of alpha thalassemia where two genes are deleted?

A

mild anemia with increased RBC count; cis deletion in Asians, trans in Africans

197
Q

Cis deletions of alpha globin are problematic because

A

increase rate of spontaneous abortions and inheritance in offspring

198
Q

Clinical presentation when three genes of alpha globin are deleted

A

Severe anemia, beta chains form HbH that damage RBCs

199
Q

Clinical presentation when four alpha genes are deleted

A

lethal in utero (hydrops fetalis); gamma chains form tetramer (Hb Barts)

200
Q

Presentation of beta thalassemia minor?

A

usually asymptomatic with mild RBC increase

201
Q

Histology and electrophoresis of beta thalassemia minor

A

microcytic, hypochromic RBCs with target cells

slightly increased HbA2 and slightly decreased HbA

202
Q

Parvovirus B19 causes aplastic anemia crisis in which patients?

A

Beta thalassemia, sickle cell patients

203
Q

List major features of beta thalassemia major

A

Severe anemia a few months after birth, extravascular hemolysis, “crewcut” Xray and chipmunk facies, risk of secondary hemochromatosis

204
Q

Blood smear of beta thalassemia major

A

microcytic, hypochromic RBCs with target cells and nucleated red blood cells

205
Q

What causes the appearance of nucleated red blood cells in thalassemia?

A

Synthesis of RBCs in abnormal locations (liver, spleen, skull) allows nucleated ones to escape

206
Q

What causes extravascular hemolysis in beta thalassemia

A

precipitation of alpha chains that damage RBC membranes

207
Q

Electrophoresis of beta thalassemia major

A

Little to no HbA, Increased HbA2 and HbF

208
Q

Two major causes of macrocytic anemia

A

B12 or Folate deficiency

209
Q

What causes megaloblastic cells in macrocytic anemia

A

Impaired DNA synthesis leads to one less division (larger cells)

210
Q

What causes hypersegmented neutrophils in macrocytic anemia

A

Impaired DNA synthesis leads to impaired division of granulocytic precursors

211
Q

List 5 causes of folate deficiency

A

poor diet (alcoholism, elderly), pregnancy, cancer, hemolytic anemia, and methotrexate

212
Q

Where is folate absorbed

A

Jejunum

213
Q

List 5 clinical and lab findings of folate deficiency

A 
Macrocytic RBCs with hypersegmented neutrophils
Glossitis
decreased serum folate
increased serum homocysteine
normal methylmalonic acid
214
Q

Where is B12 absorbed

A

ileum

215
Q

What is pernicious anemia?

A

Autoimmune destruction of parietal cells that leads to intrinsic factor deficiency

216
Q

List 5 common causes of B12 deficiency

A

Pernicious anemia, pancreatic insufficiency, Crohn’s, Diphyllobothrium latum (fish tapeworm) and veganism

217
Q

How does B12 deficiency lead to spinal cord degeneration?

A

B12 is a cofactor for converting MMA to succinyl coA. Low B12 allows methylmalonic acid to buildup, which impairs spinal cord myelinization

218
Q

Clinical findings of B12 deficiency macrocytic anemia

A

Macrocytic RBCs with hypersegmented neutrophils
Glossitis
Spinal cord degeneration

219
Q

Lab findings of B12 deficiency

A

decreased serum B12
increased serum homocysteine
increased methylmalonic acid

220
Q

What are reticulocytes?

A

Young RBCs released from bone marrow; bluish cytoplasm on smear due to residual RNA

221
Q

How does reticulocyte count distinguish between hemolytic normocytic anemia and underproduction normocytic anemia?

A

RCT >3% implies hemolytic; RCT <3% implies marrow underproduction

222
Q

List the clinical findings of extravascular hemolysis

A

splenomegaly, jaundice, RCT >3% due to marrow hyperplasia

223
Q

Where does extravascular hemolysis occur

A

Spleen, liver, lymph nodes (reticuloendothelial system)

224
Q

What causes jaundice in extravascular hemolysis

A

unconjugated bilirubin from broken down protoporphyrin overwhelms liver, which conjugates bilirubin into bile

225
Q

What causes splenomegaly in extravascular hemolysis?

A

Increased activity of spleen causes hyperplasia

226
Q

Clinical findings of intravacular hemolysis

A

Hemoglobinemia, hemoglobinuria, hemosiderinuria, decreased serum haptoglobin

227
Q

Why does haptoglobin decrease in intravascular hemolysis?

A

More of it is bound to Hemoglobin from lysed RBCs in blood vessels

228
Q

List three disorders with predominant extravascular hemolysis

A

Hereditary spherocytosis, Sickle Cell Anemia and Hemoglobin C

229
Q

How is hereditary spherocytosis diagnosed?

A

Osmotic fragility test

230
Q

Why does splenectomy lead to RBCs with Howell-Jolly bodies?

A

Spleen removes extra genetic material in RBCs; without spleen, extra genetic material remains as Howell-Jolly bodies

231
Q

Which globin chain is mutated in sickle cell anemia?

A

beta chain

232
Q

What three factors promoted sickling of cells?

A

Hypoxemia, dehydration and acidosis

233
Q

What are clinical features of sickle cell anemia

A

Extravascular hemolysis (jaundice, splenomegaly), intravascular hemolysis (hemoglobinuria), massive erythroid hyperplasia so crewcut/chipmunk faces, hepatomegaly

234
Q

What is a common presenting sign of sickle cell anemia in infants

A

Dactylitis

235
Q

Why are sickle cell patients prone to infections from encapsulated organisms?

A

Due to autosplenectomy (fibrotic spleen)

236
Q

What is the mutation in Hemoglobin C disease?

A

beta globin glutamic acid replaced with lysine

237
Q

How to RBCs protect themselves against Complement

A

GPI on RBCs anchors DAF (decay accelerating factor) and MIRL (membrane inhibitor of reactive lysis) to inactivate complement C3 convertase

238
Q

What is paroxysmal nocturnal hemoglobinuria a defect in?

A

GPI

239
Q

Why does intravascular hemolysis in PNH occur primarily at night?

A

We breathe shallow at night, leads to increase CO2, respiratory acidosis, activates complement

240
Q

What is the screening test and confirmatory test for PNH

A

Sucrose test to screen; Acidified serum test for CD55 (DAF)

241
Q

What is the main cause of death in PNH?

A

Thrombosis of hepatic, portal or cerebral veins

242
Q

Which cell is specifically mutated in PNH? What does this increase the risk for

A

Myeloid cell –> Acute myeloid leukemia (AML)

243
Q

How does G6PD present clinically?

A

Hemoglobinuria and back pain hours after exposure to oxidative stress

244
Q

IgG mediated immune hemolytic anemia involves

A

extravascular hemoolysis

245
Q

IgM mediated immune hemolytic anemia involves

A

intravascular hemolysis

246
Q

What is IgG mediated immune hemolytic anemia associated with?

A

SLE, CLL, and penicllin and cephalosporins

247
Q

What is IgM mediated immune hemolytic anemia associated with

A

Mycoplasma pneumoniae and infectious mononucleosis

248
Q

Describe the direct Coomb’s test

A

Add antibodies (IgG) to patient’s RBCs. Agglutination confirms presence of IgG and IHA

249
Q

Describe indirect Coomb’s test

A

Add anti-IgG and test RBCs to patient’s serum. Agglutination confirms serum antibodies

250
Q

How does IgM cause intravascular hemolysis in IHA?

A

IgM binds and fixes complements in cold temperatures; residual C3b acts as opsonin for splenic macrophages

251
Q

Three common causes of microangiopathic hemolytic anemia (intravascular hemolysis)

A

HUS/TTP, prosthetic heart valves, aortic stenosis

252
Q

How do the fevers in P falciparum malaria and P vivax and ovale differ?

A

P falciparum– fever everyday

P vivax and ovale- fever every other day

253
Q

List three major causes of anemia due to underproduction

A 
Parvovirus B19 (infects progenitor RBCs and halts erythropoeisis)
Aplastic anemia  (damage to hematopoetic stem cells; biopsy shows fatty marrow)
Myelophthisic process (replacement of marrow with metastatic cancer)
254
Q

List two causes of neutropenia

A 
Drug toxicity (damage to stem cells)
Severe infection (increased neutrophils in tissues, decreased circulating)
255
Q

What is the treatment for neutropenia?

A

GM-CSF or G-CSF

256
Q

4 causes of lymphopenia

A

Immunodeficiency
High cortisol state
Autoimmune destruction (SLE)
Whole body radiation

257
Q

What characterizes immature neutrophils?

A

decreased CD16 (Fc receptors)

258
Q

Which lymphoma exhibits eosinophilia?

A

Hodgkin Lymphoma

259
Q

Which cancer has increased circulating basophils?

A

Chronic myeloid leukemia

260
Q

What causes lymphocytic leukocytosis

A

Viral infections and bordatella pertussis infection

261
Q

How does EBV cause splenomegaly

A

Increased T cell proliferation in periarterial lymphatic sheath of spleen (PALS)

262
Q

Which T cells are predominant in infectious mononucleosis?

A

CD8+ T cells

263
Q

List three complications of Infectious mononucleosis

A
  1. Increased risk of splenic rupture
  2. Rash if exposed to ampicillin
  3. Increased risk of recurrence and B cell lymphoma
264
Q

What is the marker for Lymphoblasts?

A

TdT (DNA Polymerase)

265
Q

What are the markers for B-ALL

A

Tdt CD10, CD19, CD20

266
Q

Who is at risk of developing Acute Lymphoblastic Leukemia?

A

Children (especially with Down Syndrome)

267
Q

What is the translocation in children with B-ALL?

A

t(12;21) (TEL-AML1); good prognosis

268
Q

What is the translocation in adults with B-ALL?

A

t(9;22); poor prognosis BCR-ABL (Philadelphia)

269
Q

What are the markers for T-ALL

A

TdT, CD2 to CD28

270
Q

What is the genetic mutation in T-ALL?

A

11q23

271
Q

Children <5 yo with Down’s are at increased risk of developing which WBC cancer?

A

Acute megakaryoblastic leukemia

272
Q

Children >50 with Down’s are at increased risk of developing which WBC malignancy

A

Acute Lymphoblastic Leukemia

273
Q

What are the identifying features of Acute Myeloid Leukemia?

A

positive staining for MPO
Auer rods, CD13, CD 33
commonly arises in 50-60 yo adults

274
Q

What is the translocation mutation in Acute Promyelocytic Leukemia?

A

t(15;17); RAR moves to 15 and inhibits blast maturation

275
Q

How is Acute Promyelocytic Leukemia treated?

A

ATRA

276
Q

What is the translocation mutation in Acute Monocytic Leukemia?

A

t(9;11)

277
Q

What is characteristic about Acute Monocytic Leukemia?

A

Blasts infiltrate gums

278
Q

How do myelodysplastic syndromes present?

A

Cytopenias, hypercellular bone marrow, abnormal maturation of cells and blasts <20%

279
Q

Which AMLs lack MPO?

A

Acute Promyelocytic Leukemia and Acute Megakaryoblastic leukemia

280
Q

What are the markers for Chronic lymphocytic Leukemia?

A

CD5, CD20 (naive B cells)

281
Q

Smudge cells on a blood smear indicate which Leukemia?

A

CLL

282
Q

List 3 complications of CLL

A

Hypogammaglobulinemia (prone to infections)
Hemolytic Anemia
Transformation into B cell lymphoma

283
Q

What is the marker for Hairy Cell Leukemia?

A

TRAP+ of mature B cells

284
Q

Clinical features of Hairy Cell Leukemmia

A

Splenomegaly, dry-tap bone marrow (fibrosis), pancytopenia, lymphocytes with cytoplasmic projections

285
Q

Cladribine (2-CDA) treats which leukemia?

A

Hairy cell leukemia (inhibits ADA)

286
Q

What is the prognosis of Adult T Cell Leukemia/Lymphoma?

A

Poor, death in a few months

287
Q

Which cells proliferation in ATLL

A

Mature CD4 T cells

288
Q

Who is at risk of acquiring ATLL

A

HTLV-1 infected people (Japan, Caribbean)

289
Q

Clinical features of ATLL

A

Rash, generalized lymphadenopathy, lytic bone lesions with hypercalcemia

290
Q

What does a smear for Mycosis Fungoides present?

A

CD4 Lymphocytes with cerebroid nuclei (Sezary Cells)

291
Q

Clinical feature of Mycosis Fungoides?

A

Skin rash, plaques and nodules

292
Q

Which cells proliferate in Myeloproliferative disorders?

A

Mature cells of myeloid lineage

293
Q

Complications of Myeloproliferative disorders

A

Hyperuricemia (gout) due to high turnover of cells

Progression to marrow fibrosis and acute leukemia

294
Q

What is the translocation and mutation in Chronic Myelogenous Leukemia?

A

Philadelphia chromosome t(9;22) BCR:ABL leading to increased tyrosine kinase activity

295
Q

Which cells are characteristically increased in CML?

A

Basophils

296
Q

What is the first line treatment for CML?

A

Imatinib (blocks tyrosine kinase activity)

297
Q

List two complications of CML

A

Can transform to AML or ALL since mutation is in pluripotent stem cells

298
Q

Common clinical finding of CML

A

Splenomegaly

299
Q

What are 3 distinguishing features of CML from leukemoid reaction

A

Negative LAP stain, Increased basophils, t(9;22)

300
Q

Which mutation is associated with Polycythemia Vera?

A

JAK2 Kinase

301
Q

Four symptoms of Polycythemia vera

A

Blurry vision and headache; increased risk of thrombosis; facial flushing; itching after bathing

302
Q

Distinguishing feature of PV from reactive polycythemia

A

Decreased EPO, normal SaO2

303
Q

What is the mutation in essential thrombocytopenia?

A

Jak2 Kinase

304
Q

Clinical features of Myelofibrosis

A

Splenomegaly, Tear cells on smear, increased risk of infection, thrombosis and bleeding

305
Q

Myelofibrosis is a mutation of Jak2kinase leading to proliferation of

A

megakaryocytes

306
Q

Follicular cortex hyperplasia (B cell region) is associated with

A

rheumatoid arthritis and early stages of HIV

307
Q

Paracortex hyperplasia (T cell region) is seen in

A

Viral infections and Mononucleosis

308
Q

Hyperplasia of sinus histiocytes is seen in

A

cancers

309
Q

List 5 Non-Hodgkin Lymphomas

A

Follicular Lymphoma, Mantle Cell Lymphoma, Marginal Zone Lymphoma, Burkitt Lymphoma

310
Q

Proliferation of small B cells CD20+ is characteristic of

A

Follicular cell Lymphoma

311
Q

t(14;18)

A

Follicular Lymphoma

312
Q

Bcl2 in follicular lymphomas

A

inhibits apoptosis of defective B cell, leads to monoclonality

313
Q

Treatment of Follicular lymphoma

A

Rituximab (anti-CD20 antibody)

314
Q

Four distinguishing features of Follicular lymphoma from hyperplasia

A
  1. Disruption of architecture
  2. Lack of tingible body macrophages
  3. Bcl2 expression
  4. Monoclonality
315
Q

Which lymphoma is characterized by t(11;14)

A

Mantle cell lymphoma

316
Q

Which gene is mutated in mantle cell lymphoma

A

Cyclin D1, promotes G1/S transition

317
Q

Which gene is mutated in Burkitt Lymphoma

A

c-myc; promotes cell growth (transcription activation)

318
Q

t(8;14) is which lymphoma

A

Burkitt

319
Q

African vs sporadic presentation of Burkitt lymphoma

A

African child- extranodal mass on jaw
sporadic- extranodal mass on abdomen
due to EBV

320
Q

Histology of Burkitt lymphoma

A

Starry sky

321
Q

Gene mutation in diffuse large cell B lymphoma

A

BCL-6

322
Q

Which infection is diffuse large B-cell lymphoma associated with

A

HIV

323
Q

What are Reed-Sternberg cells?

A

Large B cells with multilobed nuclei with prominent nucleoli; positive for CD15 and CD30

324
Q

What causes fever, chill, weight loss and night sweat symptoms in HL?

A

RS cells secrete cytokines to recruit reactive lymphocytes, plasma cells, macrophages and eosinophils

325
Q

Most common type of HL

A

Nodular sclerosis

326
Q

Which HL has best prognosis

A

Lymphocyte rich

327
Q

Which HL has worse prognosis

A

Lymphocyte depleted

328
Q

Which cytokine may be abundant in multiple myeloma?

A

IL-6 (growth factor for plasma cells)

329
Q

6 clinical features of MM

A 
bone pain with hypercalcemia
elevated serum protein
Increased risk of infection 
Rouleux formation of RBCs (chains)
AL Amyloidosis 
Proteinuria
330
Q

What is Waldenstrom Macroglobulinemia

A

B cell lymphoma with monoclonal IgM production

331
Q

4 clinical features of Waldenstroms Macroglobulinemia

A
  1. Generalized lymphadenopathy
  2. M spike
  3. Visual and neurologic deficits (retinal hemorrhage)
  4. Bleeding
332
Q

What is the histological presentation of Langerhans cell histiocytosis

A

Birbeck granules (tennis rackets) on EM

333
Q

Marker for Langerhans Histiocytosis

A

CD1a+ and S100+

334
Q

Letterer-Siwe Dz clinical presentation

A

Langerhans cell malignant proliferation

Skin rash and cystic skeletal defects <2 yo

335
Q

Which disease is included in differential of osteosarcoma of young adult?

A

Eosinophilic granuloma (benign proliferation of Langerhans cells)

336
Q

Hand-Schuller-Christian disease

A

Scalp rash, lytic skull defects, diabetes insipidus, exhophthalmos <3; malignant Langerhans cell proliferation

337
Q

Which congenital disorder would lead to atrophic lymph node paracortex

A

DiGeorge Syndrome (Thymic aplasia)

338
Q

Which condition would lead to atrophic follicular cortex in lymph node?

A

Agammaglobulinemia

339
Q

Two ways ionizing radiation treats cancer cells

A

DNA double strand breakage, free radical formation

340
Q

Which clotting factors are produced by liver?

A

II, VII, IX, X (inactive form; activated by Vitamin K)

341
Q

Test to confirm Vitamin K deficiency?

A

Supplementation with Vit K improves Prothrombin time

342
Q

Which cells synthesize factor VIII?

A

Endothelial cells

343
Q

Why might PTT be affected in von Willebrand disease?

A

vWF is needed to form VIII

344
Q

Describe holiday heart syndrome

A

Atrial fibrillation after consumption of excessive alcohol (absent P waves)

345
Q

What causes high QRS voltage on an EKG?

A

Ventricular hypertrophy (prolonged untreated hypertension)

346
Q

Prolongation of QRS interval raises suspicions of

A

Fascicular or bundle branch block

347
Q

Where are troponin and calmodulin found, respectively?

A

Troponin in cardiac and skeletal muscle, calmodulin in smooth muscle

348
Q

Which muscles have T tubules and what is their function?

A

skeletal and cardiac muscle; allow depolarization and rapid release of intracellular calcium stores

349
Q

What is the normal kappa:lambda light chain ratio

A

3:1

350
Q

> 6:1 kappa:lambda light chain ratio indicates

A

lymphoma

351
Q

Three most common adult cancers by incidence

A

breast/prostate, lung, colorectal

352
Q

Three most common adult cancers by mortality

A

Lung, breast/prostate, colorectal

353
Q

What are oncogenes?

A

Mutated proto-oncogenes (transcription regulators) that lead to uncontrolled cell growth

354
Q

How does intravascular hemolysis decrease haptoglobin levels?

A

Hemolysis causes an increase in free hemoglobin, which binds free haptoglobin and decreases its levels

355
Q

Which antibiotic can cause pancytopenia?

A

Chloramphenicol

356
Q

The majority of hemoglobin in neonates up to 6 months is

A

HbF (alpha2gamma2)

357
Q

Bcr-abl codes for

A

Tyrosine kinase

358
Q

Over-expression of Erb-B2 gene is associated with which cancers

A

Breast and ovarian

359
Q

Microcytic anemia with normal ferritin and transferring levels suggest

A

alpha or beta thalassemia

360
Q

Normal MCV, low hemoglobin, low tranfserring, high serum ferritin and CRP suggest

A

Anemia of chronic disease

361
Q

Hypersegmented neutrophils are characterstic of which anemias

A

Megaloblastic anemias (folate or B12 deficiency)

362
Q

Bcr-abl codes for

A

Tyrosine kinase

363
Q

Over-expression of Erb-B2 gene is associated with which cancers

A

Breast and ovarian

364
Q

Microcytic anemia with normal ferritin and transferring levels suggest

A

alpha or beta thalassemia

365
Q

Normal MCV, low hemoglobin, low tranfserring, high serum ferritin and CRP suggest

A

Anemia of chronic disease

366
Q

Hypersegmented neutrophils are characterstic of which anemias

A

Megaloblastic anemias (folate or B12 deficiency)

367
Q

CD15 is a marker for

A

REed STernberg cells in Hodgkin Lymphoma

368
Q

CD16 is a marker for

A

NK cells, neutrophils and macrophages (low affinity Fc receptor)

USMLE (36 decks)

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