acute l Flashcards by skug LU

The peak inci
dence of __in children is between 2 and 5 years of age.

ACUTE LYMPHOBLASTIC LEUKEMIA

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, there may be a large mass in the mediastinum
leading to compromise of regional anatomic structures.

T cell ALL

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Bone pain, mucocutaneous bleeding

B cell ALL`

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most common type of lymphoblast seen

small lymphoblast (1.0 to
2.5 times the size of a normal lymphocyte)

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ALL cells can be confused with cells in

AML

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are the strongest predic
tor of adverse treatment outcomes for children and adults

Chromosomal translocations are the strongest predic
tor of adverse treatment outcomes for children and adults

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mutations involving the NOTCH1 gene,

T-ALL

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has the worst prog
nosis among ALLs.

B-lymphoblastic leukemia/
lymphoma with the t(9;22)(q34;q11.2);BCR-ABL1 mutation
(Philadelphia chromosome–positive ALL)

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, which has shown success in treating chronic
myeloid leukemia,

Imatinib

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in B-lymphoblastic leukemia/
lymphoma is common in childhood B-ALL,

Hyperdiploidy

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is a
group of metabolic complications that can occur in patients
with malignancy, most notably lymphomas and leukemias, with
and without treatment of the malignancy

Tumor lysis syndrome

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Tumor lysis syndrome is characterized by

hyperkalemia,
hyperphosphatemia, hyperuricemia and hyperuricosuria, and
hypocalcemia.

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AML with this translocation has myeloblasts with dysplastic granular cytoplasm, Auer rods, and some maturation

Acute myeloid leukemia with t(8;21)(q22;q22.1);RUNX1/
RUNX1T

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Acute myeloid leukemia with inv(16)(p13.1q22) or t(16;16)
(p13.1;q22);CBFB-MYH11.

Myeloblasts, monoblasts, and promyelocytes IN Pb and bm along with eosinp[hilia with dysplastic changes
central nervous system is a common site for relapse
remission rate is good, but only one half of
patients are cured.

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characterized by a differentiation block
at the promyelocytic stage.

Acute promyelocytic leukemia with PML-RARA.

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Detection of the 15;17 translocation is sufficient for
diagnosis regardless of blast count

Acute promyelocytic leukemia with PML-RARA

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their procoagulant activity initiates
disseminated intravascular coagulation; however, thromboem
bolic events may occur at presentation and during treatment

Acute promyelocytic leukemia with PML-RARA.

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“butterfly” or “coin-on-coin” nucleus,

Acute promyelocytic leukemia with PML-RARA.

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PML RARA

Treatment
includes all-trans-retinoic acid (ATRA) and arsenic trioxide

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induces differentiation of
the malignant promyelocytes

atra

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is a rare leukemia (6% of AML cases) that presents with an
increase in monoblasts and immature monocytes

Acute myeloid leukemia with t(9;11)(p22;q23);KMT2A (MLL)
MLLT3.

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Typically this disease occurs in children and may be associated
with gingival and skin involvement and/or disseminated intravas
cular coagulation.

Acute myeloid leukemia with t(9;11)(p22;q23);KMT2A (MLL)
MLLT3

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a,l with myodeplasia-related changes are has specific MDS-associated cytogenetic abnormality except

del(9q).

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There must also be absence of AML with recurrent genetic ab
normalities, NPM1 and biallelic mutation of CEBPA

Acute Myeloid Leukemia with Myelodysplasia-Related
Changes

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-rearranged is more common in very young infants and translocation may occur in the utero (has very poor prognosis)

t(v;11q23);KMT2A(MLL)

– common in children accounting for 25% of cases (more than 46 chromosomes) w/ favorable prognosis

Hyperdiploidy

– less than 46 chromosomes (poor prognosis)

Hypodiploidy

derived from a B cell progenitor rather than hematopoietic stem cells

t(12;21) (p13;q22);ETV6-RUNX1 translocation – derived from a B cell progenitor rather than hematopoietic stem cells

rare clinically aggressive tumor derived from precursors of plasmacytoid dendritic cells. skin lesions and may ultimately progress to involve peripheral blood and bone marrow

Blastic Plasmacytoid Dendritic Cell Neoplasm

 refers to extramedullary proliferation of blasts of one or more myeloid lineages that disrupts tissue architecture.

Myeloid Sarcoma

Fiftyfold increased incidence of AML during the first 5 years of life

Myeloid Proliferation related to Down Syndrome

 Similar to FAB M2

1. t(8;21)(q22;q22.1) – RUNX1/RUNX1T1 mutation

 Myeloblasts with dysplastic granular cytoplasm, Auer rods  BM eosinophilia

t(8;21)(q22;q22.1) – RUNX1/RUNX1T1 mutation

 Eosinophilia with dysplastic changes in the BM  High incidence of extramedullary disease  CNS is a common site for relapse  Favorable prognosis

Inv(16)(p13.1q22) / t(16;16) – CBFB-MYH11

Differentiation block at the promyelocytic stage (hypergranular with Auer rods)

APML with PML-RARA

“butterfly” or “coin-on-coin” nucleus

PML with PML-RARA

faggot cells)

Acute Promyelocytic Leukemia (APL; FAB M3)

CD13 and CD33 positive

Acute Promyelocytic Leukemia (APL; FAB M3)

inc in monoblasts and immature monocytes

t(9;11)(p22;q23) – KMT2A

 Motile blast cells with pseudopodia  Associated with gingival and skin involvement, DIC

t(9;11)(p22;q23) – KMT2A

20% blasts + multilineage dysplasia + history of MDS or MDS/MPN or MDS-associated cytogenetic abnormality except del(9q)

Acute Myeloid Leukemia with Myelodysplasia-related

M0- AML with Minimal Differentiation

 No Auer rods  No clear evidence of cellular maturation  Negative results with Myeloperoxidase and Sudan Black B

 Have Auer rods (fused primary granules)  90% of nonerythroid cells in the bone marrow (marrow myeloblast) Fewer than 10% of WBC show maturation to promyelocte stage or beyond

M1- AML without Maturation

Presents with greater than 20% blasts, at least maturing cells of neutrophil lineage  Fewer than 20% precursors with m

M2- AML with Maturation

may have Auer rods; chromosome abnormality t(8;21)

(+) CD13, CD33, CD65, CD11b, CD15

M2- AML with Maturation

pan myeloid markers)

CD13 and CD33 positive (

t(15;17)- results in fusion between PML gene and retinoic acid alpha

M3- Acute Promyelocytic Leukemia

Most aggressive of acute leukemia with severe bleeding tendency

Promyelocytes- Predominating cell type

Presence of myeloid and monocytoid cells in PBS and BM

Positive for the myeloid antigens CD13 and CD33 and the monocytic antigens CD14, CD4, CD11b, CD11c, CD64, and CD36.

 FAB M5a - most common in children with >80% monoblasts in the bone marrow  FAB M5B - middle-aged adults with <80% monoblasts in the bone marrow.

FAB M5a vsFAB M5b

These cells are CD14+, CD4+, CD11b+, CD11c+, CD36+, CD64+, and CD68+

Also referred to as erythremic myelosis or Di Guglielmo’s syndrome

7. M6- Pure Erythroid Leukemia/Erythroleukemia

Chr 5 and 7

 Proliferation of both immature granulocytic and erythrocytic cell types  Megaloblastoid erythropoiesis  Nucleated RBCs in PB- >50% of the total number of nucleated cells  Ringed sideroblasts, Howell-Jolly bodies are present

proliferation of megakaryoblasts and atypical megakaryocytes in BM

Dx requires- at least 20% blasts, of which at least 50% must be of megakaryocyte origin

 Dry tap; pancytopenia with thrombocytosis  Usually have cytopenias  With Dysplastic features

CD41, CD42, and CD61 (platelet markers) positive

Associated with lytic bone lesions and hyperhistaminemia

ACUTE BASOPHILIC LEUKEMIA

BM → hypercellular with diffusely fibrotic stroma, inc cell precursors, dysplastic blasts

ACUTE PANMYELOSIS WITH MYELOFIBROSIS

Extramedullary proliferation of blasts of one or more myeloid lineages that disrupts tissue architecture

MYELOID SARCOMA

Transient myeloproliferative disorder (TMD) - associated with GATA1 mutations

AML RELATED TO DOWN SYNDROME

Enzyme found in the primary granules of granulocytic cells.

Myeloperoxidase

Differentiate myeloblasts and neutrophilic granulocytes from cells of monocytic origin

Esterases

acute l Flashcards

(67 cards)