General Flashcards by Yurie Sekigami

t(11;14)

mantle cell lymphoma

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t(11;18)

MALToma (extranodal marginal zone lymphoma)

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t(8;14)

Burkitt lymphoma

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what does the SCF colony-stimulating factor do?

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

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what CFU does thrombopoietin induce?

CFU-meg

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where is thrombopoietin made?

liver

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what is the Mpl receptor?

receptor used by platelets and megakaryocytes to bind thrombopoietin

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what is the unique side effect of thrombopoietin receptor agonists?

myelofibrosis

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what cells make G-CSF? (4)

monocytes, macrophages, fibroblasts, endothelial cells

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what is filgrastim and what is it used for?

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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unique side effect of filgrastim

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

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what conditions is basophilia seen in?

CML and chronic kidney disease

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what kind of diseases have JAK2 mutations?

myeloproliferative neoplasms other than CML

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

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

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dasitinib and imatinib

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

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pruritis after bathing

polycythemia vera

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clusters of atypical megakaryocytes

primary myelofibrosis

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

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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teardrop cells

primary myelofibrosis

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what can primary myelofibrosis turn into?

AML

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name the 4 myeloproliferative neoplasms

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

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common features of myeloproliferative neoplasms (5)

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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abnormal megakaryocytes are found in what two conditions?

primary myelofibrosis and essential thrombocythemia

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essential thrombocytopenia: mutation, expected histological findings and treatment

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

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why are myelodysplastic syndromes worse than myeloproliferative neoplasms?

1. increased risk of developing AML | 2. cytopenias cause mortality via infection or bleeding; only possible cure is stem cell transplant

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

myelodysplastic syndromes and AML

chromosomal abnormality --> would you expect cytoses or cytopenia?

cytopenia (characteristic of myelodysplastic syndromes)

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

impaired mitochondrial metabolism; myelodysplastic syndromes

treatments for myelodysplastic syndromes

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

markers of immaturity in AML

CD34(+), CD117(+)

markers of immaturity in ALL

CD34(+), TdT(+)

CD117

marker of immaturity in myeloid lineage (found in AML)

which cytogenetic abnormalities cause AML?

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

APL: disease mechanism

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

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

Langerhans histiocytosis is the diagnosis; CD1a; BRAF

causes of primary and secondary HLH (hemophagocytic lymphohistiocytosis)

primary: defects in perforin gene secondary: EBV, lymphomas

what are the major findings in HLH? (4)

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

HLH (hemophagocytic lymphohistiocytosis)

which antibody isotypes are part of the naive BCR?

IgD and IgM

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

isotypic and allotypic: constant region of heavy chain | idiotypic: antigen binding region

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

IL-7

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

pre-B

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

positive selection: thymic cortex | negative selection: thymic medulla

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

lymphocytes; CMV, adenovirus, toxoplasmosis

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

lymphocytes (transient stress lymphocytosis)

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

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

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

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

two malignant conditions caused by t(9;22)

CML and B-ALL

soccer ball chromatin

CLL

smudge cells

CLL

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

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

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

hairy cell leukemia is the diagnosis; splenomegaly; indolent

name the 2 mature B cell lymphoid leukemias

CLL and hairy cell leukemia

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

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

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

Sezary syndrome

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

ATLL; "flower cell"; hypercaclemia, lymphadenopathy, hepatosplenomegaly

T cell neoplasm associated with autoimmune disease (RA)

large granular lymphocytic leukemia (LGLL)

name the 3 mature T cell lymphoid leukemias

ATLL, Sezary syndrome, LGLL

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

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

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

APL

end-organ damage of multiple myeloma

CRAB: hypercalcemia, renal inusufficiency, anemia, bone disease

most common cause of death in multiple myeloma

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

localized growth of monoclonal plasma cells in upper aerodigestive tract

plasmacytoma

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

lymphoplasmacytic lymphoma

benign but precursor to myeloma

MGUS

lymphoplasmacytic lymphoma: mechanism of disease

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

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

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

diagnose: ileocecal mass in adolescent

sporatic (not EBV-related) Burkitt lymphoma

starry sky pattern

Burkitt lymphoma

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

DLBCL

follicular lymphoma: mechanism of disease

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

t(14;18) --> BCL2 overexpression

follicular lymphoma

t(11;14) --> cyclin D1 overexpression

mantle cell lymphoma

mantle cell lymphoma: mechanism of disease

t(11;14) --> cyclin D1 overexpression

how does nodular sclerosis Hodgkin lymphoma present?

mediastinal mass in young adult

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

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

Reed-Sternberg cells

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