General Flashcards

1
Q

t(11;14)

A

mantle cell lymphoma

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2
Q

t(11;18)

A

MALToma (extranodal marginal zone lymphoma)

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3
Q

t(8;14)

A

Burkitt lymphoma

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4
Q

what does the SCF colony-stimulating factor do?

A

binds c-Kit (CD117) on the surface of HSCs to induce all myeloid and lymphoid CFUs

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5
Q

what CFU does thrombopoietin induce?

A

CFU-meg

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6
Q

where is thrombopoietin made?

A

liver

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7
Q

what is the Mpl receptor?

A

receptor used by platelets and megakaryocytes to bind thrombopoietin

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8
Q

what is the unique side effect of thrombopoietin receptor agonists?

A

myelofibrosis

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9
Q

what cells make G-CSF? (4)

A

monocytes, macrophages, fibroblasts, endothelial cells

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10
Q

what is filgrastim and what is it used for?

A

recombinant G-CSF used for prevention of infection and treatment of fevers in patients with neutropenia and mobilization of stem cells prior to bone marrow transplant

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11
Q

unique side effect of filgrastim

A

bone pain (commonly in pelvis, hips) related to medullary expansion

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12
Q

what conditions is basophilia seen in?

A

CML and chronic kidney disease

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13
Q

what kind of diseases have JAK2 mutations?

A

myeloproliferative neoplasms other than CML

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14
Q

BCR-ABL fusion gene: what chromosomal abnormality and what two diseases?

A

t(9;22); CML and B-ALL

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15
Q

dasitinib and imatinib

A

BCR-ABL tyrosine kinase inhibitors used for CML and B-ALL

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16
Q

pruritis after bathing

A

polycythemia vera

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17
Q

clusters of atypical megakaryocytes

A

primary myelofibrosis

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18
Q

what is primary myelofibrosis? name the mutation and unique histological findings

A

rapid development of bone marrow fibrosis and extramedullary hematopoiesis in the spleen, liver and lymph nodes; JAK2 mutation; clusters of atypical megakaryocytes and teardrop cells

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19
Q

teardrop cells

A

primary myelofibrosis

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20
Q

what can primary myelofibrosis turn into?

A

AML

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21
Q

name the 4 myeloproliferative neoplasms

A

CML (chronic myelogenous leukemia), polycythemia vera, primary myelofibrosis, and essential thrombocythemia

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22
Q

common features of myeloproliferative neoplasms (5)

A

proliferation of one or more of the myeloid lineages, hypercellular bone marrow with effective hematopoiesis, splenomegaly or hepatomegaly, potential for progression, JAK2 mutations are implicated in all of them except CML

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23
Q

abnormal megakaryocytes are found in what two conditions?

A

primary myelofibrosis and essential thrombocythemia

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24
Q

essential thrombocytopenia: mutation, expected histological findings and treatment

A

JAK2 mutations; large, hypogranular platelets and enlarged megakaryocytes; alkylating agents to lower platelet count

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25
Q

why are myelodysplastic syndromes worse than myeloproliferative neoplasms?

A
  1. increased risk of developing AML

2. cytopenias cause mortality via infection or bleeding; only possible cure is stem cell transplant

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26
Q

what two types of diseases are differentiated based on 20% blasts?

A

myelodysplastic syndromes and AML

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27
Q

chromosomal abnormality –> would you expect cytoses or cytopenia?

A

cytopenia (characteristic of myelodysplastic syndromes)

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28
Q

ring sideroblasts: what are they caused by, and what diseases are they seen in?

A

impaired mitochondrial metabolism; myelodysplastic syndromes

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29
Q

treatments for myelodysplastic syndromes

A

hypomethylating agents (allows for gene expression but not curative), stem cell transplant

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30
Q

markers of immaturity in AML

A

CD34(+), CD117(+)

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31
Q

markers of immaturity in ALL

A

CD34(+), TdT(+)

32
Q

CD117

A

marker of immaturity in myeloid lineage (found in AML)

33
Q

which cytogenetic abnormalities cause AML?

A

t(8;21), inv(16), t(15;17)

34
Q

APL: disease mechanism

A

t(15;17) produces PML-RARalpha fusion gene –> arrests myeloid cells at promyelocyte stage; frequent DIC at diagnosis; responds to ATRA which overcomes the block and allows cells to mature

35
Q

Birbeck granules (“tennis racket” appearance) on EM = what CD molecule is expressed and what gene is mutated?

A

Langerhans histiocytosis is the diagnosis; CD1a; BRAF

36
Q

causes of primary and secondary HLH (hemophagocytic lymphohistiocytosis)

A

primary: defects in perforin gene
secondary: EBV, lymphomas

37
Q

what are the major findings in HLH? (4)

A
  1. very high ferritin
  2. cytopenia
  3. hypertriglyceridemia
  4. low fibrinogen
38
Q
  1. very high ferritin
  2. cytopenia
  3. hypertriglyceridemia
  4. low fibrinogen
A

HLH (hemophagocytic lymphohistiocytosis)

39
Q

which antibody isotypes are part of the naive BCR?

A

IgD and IgM

40
Q

where are isotypic, allotypic, and idiotypic differences in antibodies located?

A

isotypic and allotypic: constant region of heavy chain

idiotypic: antigen binding region

41
Q

what cytokine is important for the proliferation of pro-B/T cells?

A

IL-7

42
Q

at what stage is there only a heavy chain on the B cell surface?

A

pre-B

43
Q

where do T cell positive and negative selection occur, respectively?

A

positive selection: thymic cortex

negative selection: thymic medulla

44
Q

which type of cells proliferate in infectious mononucleosis? what other conditions can cause this?

A

lymphocytes; CMV, adenovirus, toxoplasmosis

45
Q

what type of cells can transiently proliferate after trauma, MI, or seizures?

A

lymphocytes (transient stress lymphocytosis)

46
Q

lymphocytes with large, abundant, lightly basophilic cytoplasm that encroaches on neighboring RBCs: diagnosis and what kind of cells are these?

A

infectious mononucleosis (a reactive lymphocytosis); cytotoxic (CD8) T cells

47
Q

B-ALL: population, indicators of good/poor prognosis

A

children
good prognosis: hyperdiploidy, t(12:21)
poor prognosis: hypodiploidy, t(9:22) = Philadelphia chromosome, treated with dasatinib

48
Q

two malignant conditions caused by t(9;22)

A

CML and B-ALL

49
Q

soccer ball chromatin

A

CLL

50
Q

smudge cells

A

CLL

51
Q

CLL: unique histological findings, indicators of good/poor prognosis, aggressive/indolent?

A

“soccer ball” chromatin, smudge cells
good prognosis: mutated IgHV, del 13q
poor prognosis: unmutated IgHV, del 17p (contains p53)
indolent (chronic)

52
Q

lymphocytes with hairy cytoplasmic projections = expected physical finding, aggressive/indolent?

A

hairy cell leukemia is the diagnosis; splenomegaly; indolent

53
Q

name the 2 mature B cell lymphoid leukemias

A

CLL and hairy cell leukemia

54
Q

“flower cells” = diagnose, important facts, symptoms (4), aggressive/indolent?

A

adult T cell leukemia (ATLL); HTLV-1 associated, endemic in Japan and Caribbean; skin lesions, lymphadenopathy, hepatosplenomegaly, hypercalcemia; aggressive

55
Q

“cerebriform” nuclei, no travel outside US, erythroderma and lymphadenopathy: diagnose

A

Sezary syndrome

56
Q

HTLV-1 associated T cell neoplasm: diagnosis, expected histological finding, symptoms

A

ATLL; “flower cell”; hypercaclemia, lymphadenopathy, hepatosplenomegaly

57
Q

T cell neoplasm associated with autoimmune disease (RA)

A

large granular lymphocytic leukemia (LGLL)

58
Q

name the 3 mature T cell lymphoid leukemias

A

ATLL, Sezary syndrome, LGLL

59
Q

large granular lymphocytic leukemia (LGLL): what cell is involved, symptoms, association, aggressive/indolent, expected histologic finding

A

cytotoxic T cells; neutropenia, anemia, splenomegaly; associated with autoimmune disease (RA); indolent; lymphocytes with eosinophilic granules

60
Q

diagnose: t(15;17) produces PML-RARalpha fusion gene –> arrests myeloid cells at promyelocyte stage

A

APL

61
Q

end-organ damage of multiple myeloma

A

CRAB: hypercalcemia, renal inusufficiency, anemia, bone disease

62
Q

most common cause of death in multiple myeloma

A

infections (production of normal antibodies are suppressed with M protein)

63
Q

localized growth of monoclonal plasma cells in upper aerodigestive tract

A

plasmacytoma

64
Q

IgM pentamers causing visual/neurological impairment; cryoglobulinemia (Raynaud phenomenon)

A

lymphoplasmacytic lymphoma

65
Q

benign but precursor to myeloma

A

MGUS

66
Q

lymphoplasmacytic lymphoma: mechanism of disease

A

IgM pentamers causing visual/neurological impairment; cryoglobulinemia (Raynaud phenomenon)

67
Q

Burkitt lymphoma: mechanism of disease; population; histological finding; aggressive/indolent?

A

t(8;14) –> c-myc overexpression; adolescents; starry-sky pattern; aggressive

68
Q

diagnose: ileocecal mass in adolescent

A

sporatic (not EBV-related) Burkitt lymphoma

69
Q

starry sky pattern

A

Burkitt lymphoma

70
Q

B cell lymphoma that presents as a rapidly growing mass/lymphadenopathy in adults

A

DLBCL

71
Q

follicular lymphoma: mechanism of disease

A

t(14;18) –> BCL2 overexpression = no apoptosis; generally indolent but 40% transform to DLBCL

72
Q

t(14;18) –> BCL2 overexpression

A

follicular lymphoma

73
Q

t(11;14) –> cyclin D1 overexpression

A

mantle cell lymphoma

74
Q

mantle cell lymphoma: mechanism of disease

A

t(11;14) –> cyclin D1 overexpression

75
Q

how does nodular sclerosis Hodgkin lymphoma present?

A

mediastinal mass in young adult

76
Q

popcorn cells, L&H cells = diagnosis, aggressive/indolent, where would you expect to see mass?

A

nodular lymphocyte predominant Hodgkin lymphoma; indolent; cervical, axillary mass in adult

77
Q

Reed-Sternberg cells

A

“owl-eye” appearance, found in Hodgkin lymphoma, B cell lineage but do not express CD20