Pathology: Hematopathology: White Blood Cell Disorders, Block I

Front 1

Severe reduction in neutrophils: drug toxictity most common cause

Back 1

Agranuocytosis

Front 2

Malignancy of blood forming organs, arrested/distorted wbc maturation

Back 2

Leukemia

Front 3

Malignant disorder of lymphoid tissue

Back 3

Lymphoma

Front 4

Reduction in neutrophils that may be due to supression of bone marrow production (tumors, granulomatous infections) or destruction of neutrophils (SLE, RA, drug interactions).

Back 4

Neutropenia

Front 5

This condition of an enlarged spleen leading to sequestration of rbcs, wbcs and platelets alone and in combination, is especially prevalent in alcoholics who have spelenic congestion and enlargement due to liver cirrhosis

Back 5

Hyperspelism

Front 6

Name four conditions that may lead to destruction of neutrophils and neutropenia

Back 6

1. Immune disoreders (SLE, RA)
2. Splenomegaly
3. Hypersplenism (alcoholics)
4. Increased peripheral utilization (bacteial or fungal infections)

Front 7

Elevated white blood cell counts with circulating immature white cells, stimulating myeloid leukemia

Back 7

Leukemoid Reaction

Front 8

Characterized by wbc toxic granulations, cytoplsmic vacuoles, Dohle bodies, and absence of circulating blasts and nucleated red blood cells:

Back 8

Leukemoid reaction

Front 9

Increased lymphocytes on CBC- seen with viral infections, especially infectious mononucleosis (IM) and CMV- peripheral smear shows large lymphocytes with abundant pale blue cytoplasm

Back 9

Lymphocytosis

Front 10

Cancer of immature cells that arise from the myeloid progenitor cells of the bone marrow

Back 10

Acute Myeloid Leukemia

Front 11

Cancer characterized by the proliferation of immature cells that arise from the myeloid progenitor cells of the bone marrow

Back 11

Acture Myeloid Leukemia

Front 12

This disease causes anemia, neutropenia, thrombocytopenia, and an outpouring of immature cells, including blasts and nucleated RBCs into the peripheral blood

Back 12

AML

Front 13

Gingival hyperplasia may occur in this disease because monoblasts often show solid organ infiltration

Back 13

AML

Front 14

In AML, blasts sometimes accumultae in extramedullary tissue to produce tumors referred to as

Back 14

Myeloid Sarcoma - such tumors can appear anywhere in the body and may precede other manifestations of AML by several months

Front 15

This condition presents with anemia and thrombocytopenia, lekuocyte count that is high, low or normal, and a blast count greater than 20% in peripheral blood

Back 15

AML

Front 16

Distinctive red-staining, rod-like cytoplasmic structures occuring only in myeloblasts- stain strongly for MPO.

Back 16

Auer Rods - most reliable morphologc feature for distinguishing AML from ALL.

Front 17

_______ greater than 20% is required for the diagnosis of AML.

Back 17

Blasts

Front 18

The presence of MPO or Sudan Black stain is evidence of what?

Back 18

Myeloid Origin

Front 19

______ activity is used to identify cells of monocytic lineage.

Back 19

Nonspecific Esterase (NSE)

Front 20

High levels of which three things reflect cell mass and increased rate of lysis of abnormal cells?

Back 20

1. Serum Uric Acic
2. Serum LDH
3. B2-microglobulin
Uric acid indicates cell death b/c its the end priduct of nucleic acid breakdown.

Front 21

This type of AML can be treated with all trans retinoic acid, which matures cells by inhibiting the effects of the PML-RAR alpha receptor.

Back 21

M3

Front 22

A malignant bone marrow neoplasm of lymphocyte precursors

Back 22

Acute Lymphoblastic Leukemia (ALL)

Front 23

Most common malignancy in childhood, 75% of cases under the age of 6 years

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ALL

Front 24

The clinical features of this disease are palor, weakness, anemia, petechial hemorrhage, bone pain, and fever (mimics viral infection).

Back 24

ALL - clinical features relate directly to the replacement of normal bone marrow elements by lymphoblast.

Front 25

This disease has hypercellular bone marrow with >25% blasts and no MPO activity.

Back 25

ALL

Front 26

What are the three classifications of ALL?

Back 26

1. Morphologic (FAB)
2. Immunologic
3. Cytogenic

Front 27

The use of ______ and a panel of monoclonal antibodies to T cell and B cell associated antigens will identify almost all cases of ALL.

Back 27

TdT assay

Front 28

About 85% of all cases of ALL are which type?

Back 28

Precursor B cell type

Front 29

Which cell marker is present in about 80% of cases of B-ALL and associated with a good prognosis?

Back 29

CD10

Front 30

The lymphoblasts in precursor T-Cell ALL are derived from cells in which stage of T-cell development?

Back 30

Thymocyte stage

Front 31

Which T cell markers are expressed in a high percentage of precursor T-cell ALL?

Back 31

CD2, CD5, and CD7.

Front 32

Lymphoblastic neoplasms of both precursor T cell and B cell type may present primarily as what?

Back 32

Extramedullary Tumors

Front 33

_______ mutations involve a loss of function, or balanced translocations producing fusion proteins that drive lymphoblast proliferation.

Back 33

B-ALL - hyperdiploidy is the most common chromosomal change and is associated with favorable prognosis.

Front 34

40-50% of T-cell ALL involve which chromosomal abnormality?

Back 34

Translocations - up to 70% have gain of function mutations

Front 35

______ involvement is the most significant extramedullary manifestation of ALL.

Back 35

CNS involvement - usually seen in T-ALL . The CNS is the most common site of relapse so it must be frequently monitored.

Front 36

An acquired chronic lymphoproliferative disorder of clonal B lymphocytes.

Back 36

Chronic Lymphocytic Leukemia (CLL) - most common leukemis in US and Europe - usually elderly males

Front 37

Neoplastic counterpart of a subpopulation of B cells co-expressing B cell markers (CD19 and CD20) and T-cell marker CD-5

Back 37

CLL

Front 38

The hallmark of this disease is the presence of persistent peripheral blood lymphocytosis consisting of mature appearing lymphocytes:

Back 38

CLL

Front 39

Patients with this disease complain of weakness, nightsweats, weight loss, are prone to pneumonia due to altered immunity, lymph adenopathy, spenomegaly, and hyperslenism contributing to pancytopenia:

Back 39

CLL

Front 40

Occurs in 5-10% of cases of CLL and is characterized by development of rapidly progressive lymphoma - transformation is heralded by fever, weight loss, abdominal pain, lymphadeopathy and worsening cytopenias:

Back 40

Richter Syndrome

Front 41

Most common transformation occuring in 15% of CLL patients which causes a gradual increase in circulating prolymphocytes which is the stage between lymphoblast and mature lymphocyte

Back 41

Prolymphocytoid Transformation of CLL

Front 42

A neoplasm of mature B lymphocytes named for characteristic fine cytoplasmic projections - 2% of all leukemias - usually affects middle aged white males

Back 42

Hairy Cell Leukemia

Front 43

This disease presents with often massive splenomegaly, pancytopenia, and dry tap bone marrow aspirations (due to reticulin deposition- a type of bone marrow fibrosis)

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Hairy Cell Leukemia