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MOHD3 Exam 2 > Myeloid Lymphoid Bone Marrow Disorders > Flashcards

Myeloid Lymphoid Bone Marrow Disorders Flashcards

1
Q

Increase in number of circulating neutrophils

A

Neutrophilia

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2
Q

Cytoplasmic findings of neutrophils associated with bacteria infections (3)

A
  1. Toxic granulation
  2. Dohle bodies: rough ER
  3. Cytoplasmic vacuolation
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3
Q

Extreme leukocytosis resulting in high WBC that simulates leukemia but is due to infection or another underlying disorder.

May resemble chronic myelogenous leukemia

A

Leukemoid reaction

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4
Q

Malignant clonal neoplasm of multipotent myeloid stem cells characterized by accumulation of myeloblasts in bone marrow and blood

A

Acute Myeloid Leukemia

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5
Q

Block in differentiation of leukemic stem cells

A

Acute myeloid leukemia

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6
Q

t (15;17) seen in ____

Treatment by ___

A

Acute promyelocytic leukemia

Treatment by all-trans-retinoic acid (ATRA)

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7
Q

Clinical features of acute myeloid leukemia

A

Fatigue: anemia
Infection: granulocytopenia
Bleeding: thrombocytopenia

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8
Q

Disseminated intravascular coagulation (DIC) common in ___

A

acute promyelocytic leukemia with t(15;17)

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9
Q

Infiltration of gums seen in ____

A

acute myelomonocytic leukemia

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10
Q

Prognosis of AML
Initial:
Long term survival:

A

60-80% reach initial remission w/ combination chemotherapy BUT relapses common
Long term survival: 1/3 pt age 18-60.
Pt w/ t(15;17) have 80% survival.
Unfavorable cytogenetics have

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11
Q

Positive cytochemical stains for myeloperoxidase

A

Acute Myeloid Leukemia

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12
Q

Negative cytochemical stain for myeloperoxidase

A

Acute Lymphoblastic Leukemia

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13
Q

Most (80%) acute lymphoblastic leukemia have precursor B cells that express:

A

CD19, CD10, TdT

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14
Q

Less common acute lymphoblastic leukemia have precursor T cells that express:

A

CD7, CD2, TdT

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15
Q

Prognosis for B-ALL w/ hyperdiploidy

A

Favorable

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16
Q

Prognosis of B-ALL with t(12;21) TEL-AML1

A

Favorable

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17
Q

Prognosis of B-ALL with t(9;22) BCR-ABL1, 11q23 rearrangements

A

Unfavorable

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18
Q

Prognosis of B-ALL with hypodiploidy

A

Unfavorable

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19
Q

Most common childhood tumor

A

Acute lymphoblastic leukemia

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20
Q

Clinical presentation of ____:
Fatigue, infections, bleeding
Bone pain, lymphadenopathy, hepatosplenomegaly, CNS symptoms

A

Acute lymphoblastic leukemia

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21
Q

Chronic myeloproliferative neoplasm characterized by predominately granulocytic differentiation. Increased neutrophils granulocytic precursors (some), basophila, eosinophilia.

A

Chronic Myelogenous Leukemia

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22
Q

15% of leukemia in adults, rare in children. Onset of 55 y/o.

A

Chronic Myelogenous Leukemia

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23
Q

Often asymptomatic at diagnosis. Lethargy, fatigue, weight loss. May have splenomegaly.

A

Chronic Myelogenous Leukemia

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24
Q

Left shift with full range of myeloid precursors circulating in peripheral blood. Basophilia. Eosinophilia.

A

Chronic Myelogenous Leukemia

25
Q

Morphology of bone marrow in CML

A

Hypercellular bone marrow. Increased number of maturing myeloid cells. Normal myeloid cell morphology. NO increase in blast cells. Barely any fat.

26
Q

marked neutropenia, usually

A

Agranulocytosis

Predisposition to infection

27
Q

Has Philadelphia chromosome from t(9;22)(q34q11) resulting in long arm chromosome 9 and short chromosome 22

A

CML

28
Q

Genes that are fused in CML

Leads to:

A

BCR/ABL

Production of abnormal tyrosine kinase with altered activity

29
Q

Clinical manifestation: 50% are asymptomatic
Lethargy, fatigue, weight loss
Splenomegaly (80%)

A

Chronic Myelogenous Leukemia

30
Q

Treatment of CML

A 
Imatinib mesylate (Gleevec): blocks tyrosine kinase 
Continue indefinitely 
90% pt go into remission
31
Q

Blast phase of CML =
Symptoms:
Lineage:
Treatment:

A

> 20% blasts in peripheral blood or marrow

Fever, night sweats, bone pain, hemorrhage, anemia

80%: myeloid lineage
20%: lymphoid lineage

Similar to AML: chemotherapy

32
Q

Clonal lymphoproliferative disorder of morphologically “mature” lymphocytes

If only involves lymph nodes =

A

Chronic Lymphocytic Leukemia

Small Lymphocytic Lymphoma

33
Q

Morphology of Chronic Lymphocytic Leukemia:

Characteristic feature of CLL:

A

Small, condensed nuclear chromatin, monomorphic cells

B cells express CD5 (T cell marker)

34
Q

CLL seen usually in:

A

Pt greater than 60 y/o

More common in males

35
Q

Clinical features of chronic lymphocytic leukemia

A

Susceptibility to infection
Anemia
Thrombocytopenia

36
Q

Dyspoiesis =

A

abnormal morphologic features in maturing myeloid, erythroid and megakaryocytic cells

37
Q

Clonal myeloid stem cell disorders characterized by peripheral blood cytopenias, ineffective hematopoiesis and dyspoiesis

A

Myelodysplastic Syndrome (MDS)

38
Q

Trisomies and deletions are common in ___

A

Myelodysplastic Syndromes

39
Q

Morphology of Myelodysplastic Syndromes

A

Pancytopenia w/ dyspoietic features of RBC, granulocytes and platelets
Dyspoietic features of granulocytic precursors, erythroid precursors, megakaryocytes
Increased myeloblasts BUT always less than 20%
Hypercellular bone marrow

40
Q

Chronic Myeloproliferative Neoplasms caused by:

A

mutation in JAK2 gene (Jak2 V617F) –> constituativelly on pathway even without ligands –> no proliferative control of stem cells
See in all polycythemia
Half in essential thrombocythemia and primary myelofibrosis

41
Q

Clonal proliferation of multipotent myeloid stem cells that result in increased number of differentiated granulocytes, red blood cells or platelets.

A

Chronic Myeloproliferative Neoplasms

42
Q

Types of Chronic Myeloproliferative Neoplasms

A

Chronic myelogenous leukemia (CML)
Polycythemia vera
Essential thrombocythemia
Primary myelofibrosis

43
Q

Chronic myeloproliferative neoplasm associated w/ thrombocytosis and increased megakaryocytes in bone marrow.

A

Essential thrombocythemia

44
Q

Least likely to transform to AML or myelofibrosis

A

Essential Thrombocythemia

45
Q

Clinical features of essential thrombocythemia:

Typically seen in:

A

Thrombosis or hemorrhage
Splenomegaly

Adults (50-60 y/o)

46
Q

Morphology of essential thrombocythemia

A

Increase platelet count
Variation in size of platelets
increased large atypical megakaryocytes in bone marrow

47
Q

Characteristic feature of primary myelofibrosis

A

Tear drop RBC

48
Q

A clonal myeloproliverative neoplasm characterized by proliferation of predominantly megakaryocytes and granulocytes in the bone marrow. Reactive deposition of fibrous connective tissue with extramedullary hematopoiesis.

A

Primary myelofibrosis

49
Q

Extramedullary hematopoiesis in:

A

primary myelofibrosis

50
Q

Morphology of primary myelofibrosis:

A

Marked splenomegaly: extramedullary hematopoiesis
Tear drop RBC
Leukoerythroblastic reaction: immature myeloid cells and nucleated RBC in blood

51
Q

Morphology of bone marrow in primary myelofibrosis

A

Cellular stage: trilineage hyperplasia –> hypercelluar marrow
Increased neutrophils and clusters of large atypical megakaryocytes

Fibrotic stage: Hypocellular marrow

52
Q

Clinical features of primary myelofibrosis:
Seen in:
Survival:

A

Splenomegaly
Insidious onset

Middle age and elderly

Survival 3 years: 5-20% progress to acute leukemia

53
Q

Chronic myeloproliferative neoplasm characterized by increased red cells in peripheral blood and bone marrow. Possible leukocytosis and thrombocytosis.

A

Polycythemia vera

54
Q

Diseases characterized by clonal proliferation of terminally differentiated B cells capable of secreting immunoglobulin or portions of immunoglobulin (M-protein).

A

Plasma cell neoplasms

55
Q

Bone marrow based plasma cell neoplasm associated with an M-protein in serum and/or urine.

A

Plasma cell myeloma (multiple myeloma)

56
Q

Symptoms of symptomatic myeloma

A 
End organ damage
"CRAB": 
hyperCalcemia
Renal insufficiency
Aneima
Bone or lytic lesions
57
Q

Clinical features of plasma cell myeloma:
Seen in:
Survival:

A

Renal damage
Recurrent infections
Bone pain and fractures due to lytic lesions

50+ y/o

Incurable: 5 year survival

58
Q

Pathological features of plasma cell myeloma:

A

Blood smear: rouleaux formation

Bone marrow: increased number of clonal plasma cells

MOHD3 Exam 2 (13 decks)

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