Chapter 33 Leukocytic Disorders

Front 1

35yo woman completes a 50 mile marathon, what will be the effect on her neutrophil count?

Back 1

increased

Front 2

what type of neutrophil predominats in the marrow in Kostmann's disease?

Back 2

immature precursors ie...myeloblasts, promyeloctes

Front 3

patients with May-Hegglin anomaly have increased Dohle bodies
true or false

Back 3

False, the structures in May-Hegglin anomaly are probably altered RNA
May-Hegglin Anomaly: rare autosomal dominant condition involves the nonmuscle myosin heavy chain 9 gene (MYH9) linked to chromosome 22q12-13

Front 4

how do the neutrophils of the Pelger- Heut anomoly compare functionally with those in Alder-Reilly anomaly? how about those in Chediak-Higashi syndrome?

Back 4

both Pelger-Heut and Alder-Reilly neutrophils are functionally normal
Chediak-Higashi neutrophils are functionally impaired; Granulocytes, monocytes, and lymphocytes contain giant granules which appear to be abnormal lysosomes.

Alder-Reilly Anomaly: Dense, prominent, larger than normal azurophilic granulation in all white blood cells
Pelger-Huët Anomaly: hereditary, autosomal dominant condition involves failure of normal
segmentation of granulocytic nuclei. Most nuclei are bilobed and rounded
Chédiak-Higashi Syndrome: Partial oculocutaneous albinism, photophobia, immune deficiency, abnormally
large granules in leukocytes and other granule-containing cells,
neurologic defects, and frequent pyogenic infections characterize this rare,
autosomal recessive disorder

Front 5

features of tropical pulmonary eosinophilia?

Back 5

elevated peripheral eosinophils, serum IgE; found
mainly in India, Africa, Southeast Asia, and the South Pacific, Wuchereria bancrofti (a parasite of humans that inhabits the lymphatics) is the most
common and widely distributed cause of this disease, more common in males

Front 6

both basophilia and monocytosis should prompt a search for what possible disorder?

Back 6

basophilia may be seen in an allergic reaction, monocytosis seen in patients recovering from an infection; both can be seen in hematologic malignancies

Front 7

how does the cellular immune function in neonates compare with normal adults?

Back 7

cellar immunity of infants is comparable to that of adults

Front 8

list four viruses associated with acute infectious lymphocytosis(AIL)

Back 8

Acute infectious lymphocytosis (AIL) is a contagious condition characterized by lymphocytosis and occurring mainly in children. The incubation period is 12–21 days.
Antibody and viral studies have indicated a relationship between infectious lymphocytosis and
coxsackievirus A, coxsackievirus B6, echoviruses, and adenovirus type 12.

Front 9

what is the typical peripheral blood film in the human T lymphotropic leukemia virus 1 (HTLV-1) infection?

Back 9

The peripheral blood lymphocyte count is usually less than 20 × 109/L;
however, 10%–40% of these lymphocytes are immature forms. In most of
these cases, the lymphocytosis is monoclonal and, therefore, leukemic.
Although some patients with this infection progress to ATL, most (90%– 95%) patients with antibodies against HTLV-1 are symptom free.
Most individuals exhibit only a viral-like syndrome; however, others manifest a chronic progressive leukemia, and still others develop tropical spastic paraparesis. ATL typically develops decades after initial infection, and thus is usually seen in older patient

Front 10

what is the EBV site attachment to B cells?

Back 10

The virus attaches to C3d complement receptors (CD21) on B lymphoid cells and enters into the cells.

EBV then stimulates DNA synthesis in these B cells and induces the formation of several new antigens, including the viral capsid antigen (VCA), the membrane antigen (MA)....

Front 11

describe the principle underlying the heterophil antibody test for infectious mononucleosis?

Back 11

heterophil antibodies in patients with IM are absorbed by beef erythrocytes, in contrast to heterophil antibodies present in other disorders. so beef RBC stroma will absorb the heterophil antibodies of IM, then there is nothing to cause the horse RBC to agglutinate, so a negative result suggests IM. forssman's antigen is found on guinea pig kidney . as a parallel test incubate patient serum , guinea pig kidney, and horse RBC . the guinea pig kidney will absorb al those nonspecific antibodies, and the IM antibodies will cause the horse RBCs to agglutinate. a positive result suggests IM

Front 12

3 peripheral blood features that favor diagnosis of chronic myelogenous leukemia (CML) over leukemoid reaction?

Back 12

CML is associated with myelocyte peak most cells are myelocytes; basophilia; eosinophilia

Front 13

the french-american-british (FAB) criteria fro the diagnosis of AML required 30% blast in bone marrow or blood; the current WHO criteria?

Back 13

20% blast

Front 14

three cell surface markers that distinguish AML with minimal differentiation(M0) from a lymphoid malignancy?

Back 14

CD13, CD33 and CD117 are characteristic of the AML's; they are variably expressed in M0 through M7

Front 15

t*8;12)(q22;q22) is associated with which genes?
what is the prognostic significance?

Back 15

the genes are AML1 and ETO. this translocation is seen in only 25% of the cases of AML-M2, but is associated with a more favorable prognosis than if it is absent

Front 16

what translocation is seen in most cases of acute promyelocytic leukemia (APPL) hypergranular variant?
are Auer rod seen in M3?

Back 16

t(15;17)(q22;q21)
yes, but less common

Front 17

what is the prognostic significance of inv(16)(p13q22)?
what leukemia is it seen in?

Back 17

associated with a favorable prognosis;
eosinophilic variant of AML M4, called AML M4EO;
in this variant there may not be an abundance of eosinophils; only 1% in some cases

Front 18

CMML is a clonal stem cell disorder in which the predominant feature is persistent monocytosis
The marrow also shows monocytosis and often has increased promonocytes, which may be distinguished from abnormal myelocytes by nonspecific esterase (α-napthyl acetate, or α-napthylbutyrate esterase) staining or strong labeling with antilysozyme or CD68. Eosinophilia or basophilia may be present, and plasmacytoid-appearing monocytes are seen frequently. When eosinophils exceed 1.5 × 109/L, the WHO recommends the subcategory of CMML with eosinophilia.

Back 18

CMML is a clonal stem cell disorder in which the predominant feature is persistent monocytosis
The marrow also shows monocytosis and often has increased promonocytes, which may be distinguished from abnormal myelocytes by nonspecific esterase (α-napthyl acetate, or α-napthylbutyrate esterase) staining or strong labeling with antilysozyme or CD68. Eosinophilia or basophilia may be present, and plasmacytoid-appearing monocytes are seen frequently. When eosinophils exceed 1.5 × 109/L, the WHO recommends the subcategory of CMML with eosinophilia.

Front 19

what is the immunophenotypic profile for acute megakaryoblastic leukemia?

Back 19

CD13, CD41, CD61

Front 20

prognostic significance of chromosome band 11q23 abnormality in AML?

Back 20

associated with an unfavorable profnosis; also associated with leukemias secondary to chemotherapy for a previous malignancy

Front 21

in CML there is a marked increase in the number of cells of the neutrophil line. how are the other cell lines affected

Back 21

several other cell lines are proliferative including basophils, eosinophils, monocytes, and platelets
patients are usually anemic and a small percentage are thrombocytopenic rather than have increased platelets

Front 22

in polycythemia vera all three cell lines are proliferative and there is moderate neutrophilia. what is the neutrophil alkaline phosphatase level?

Back 22

neutrophil alkaline phosphatase and leukocyte alkaline phosphatase are equivalent terms;
neutrophil alkaline phosphatase is elevated in PV; absent in CML

Front 23

which of the myeloproliferative disorders may progress to acute leukemia?

Back 23

all, CML, PV, ET, myelofibrosis with myeloid metaplasia

Front 24

ET is a diagnosis of exclusion, particularly exclusion of other MPD.
what lab and biopsy findings are required for diagnosis?

Back 24

other MPD's must be ruled out
WHO requires a Hb of lesss than 13g/dL for the diagnosis of ET; requires the absence of Philadelphia chromosome; and finally no marrow fibrosis (this rules out CIM)

Front 25

what clinical finding to distinguish MDS from MPD?

Back 25

LACK of organomegaly=MDS

Front 26

MDS subcategories are based chiefly on the percentage of blast present in marrow and peripheral blood:
RA
RARS
RA with excess blast

Back 26

RA: <5% BM, <1% PB
RARS: <5% BM, <1% PB, >15% RS in BM
RAEB: 5-20% BM, <5% PB

Front 27

immunochemistry was used prior to flow cytometry to distinguish the blast of ALL from those of AML;
ALL blast?

Back 27

ALL blast are consistently negative for MPO and naphthyl ASD CAE and generally negative SBB (occasionally stining occurs); PAS gives a block positivity

Front 28

most frequent translocation in adults with ALL?

Back 28

in adults with ALL the t(9;22) translocation is the most common and contributes to the poorer prognosis in adults compared to children with this leukemia

Front 29

8 yog has acute leukemia which appears to be lymphoid in origin; flow is performed; distinguish between pre-B ALL and Burkitt

Back 29

TdT will be positive in early pre-B ALL and negative in Burkitt; CD20 and SIg will be negative in early pre-B ALL and positive in Burkitt

Front 30

compare CLL and PLL

Back 30

CLL PLL
lymphadenopathy:positive; negative
WBC: 30-200; >100
gender:male; male
prognosis:fair, indolent; poor
percent prolymphs: <10%; >55%

Front 31

what immunophenotypic differences distinguish CLL from PLL?

Back 31

CLL: weakly positive for surface Ig, positive for CD5, ocasionally CD11c, CD23, CD25
PLL: stongly positive for surface Ig, negative for CD5 and CD23

Front 32

sezary syndrome, what is the characteristic immunophenotype

Back 32

this is a T cell malignancy usually with T-helper differentiation (CD4); it often loses the pan T cell marker CD7. CD3 is positive.

Front 33

what are the two common chromosomal abnormalities in SLL?

Back 33

trisomy 12 is seen in 20% of cases, and 13q abnormalities are seen in 50%

Front 34

does a negative tset for t(11;14) or lack of overexpression of cyclin D1 rule out mantle cell lymphoma?

Back 34

no; there are false negatives with this translocation, so it is specific but not sensitive

Front 35

H.pylori is associated with what type of lymphoma?
what is the immunophenotype?

Back 35

marginal zone lymphoma; phenotype is CD19, CD20, CD22, CD79a; CD5, CD10, CD23 negative; it is sometimes driven to remission by treatment of H. pylori

Front 36

what is the most common lymphoma of the CNS?

Back 36

diffuse large B cell lymphoma

Front 37

9 yob for Burkitt lymphoma; he wants to perform an EBV test; which types of Burkitt are associated with EBV

Back 37

endemic form seen in African children, and the HIV-associated forms are EBV related; sporadic form is seen world wide is not EBV related

Front 38

what way might T lymphoblastic lymphoma present as a medical emergency?

Back 38

mediastinal mass; especially young men, it may cause airway compromise

Front 39

anaplastic large cell lymphoma (ALCL) is a disease of adolescents and young adults, as is hodgkin's lymphoma and the malignant cells of ALCL can mimic Reed-Sterburg cells, both morphologically and by immunohistochemistry, as both are CD30 positive; what cytogenetic study enables confident diagnosis of ALCL?

Back 39

t (2;5)(p23;q35) translocation which results in a fusion protein, NPM-ALK that combines a tyrosine kinase with a nucleolar protein, helps seal the diagnosis of ALCL

Front 40

Hodgkin's lymphoma is positive for what cell surface markers?

Back 40

RS cells are positive CD15 and CD30; the L&H popcorn cells of nodular lymphocyte predominant HD are positive for CD20 and CD45 but negative for CD15 and CD30

Front 41

lacunar cells in mediastinal lymphoid neoplasm in a 23yof would have an immunophenotype of?

Back 41

nodular sclerosis HD ; the lacunar cells are CD15 and CD30 positive

Front 42

etiologies of renal failure in multiple myeloma?

Back 42

light chains may cause obstructions with a resultant loss of nephrons; amyloid deposits in the glomeruli contribute to renal failure

Front 43

ostwald visometer used to measure serum viscosity in a waldenstrom macroglobulinemia patient; it is 6.3 ; the normal control run at the same time is 3.4; interpret the results?

Back 43

the control is abnormal; it should be 1.4 to 1.8; the viscometer must be recalibrated and the patients sample retested before the result can be relaesed

Front 44

34 yow recent immigrant from rural region of italy, presents with new onset diarrhea with fatty stools and recurrent colds; colon biopsy shows marked lymphoplasmacytic infiltrate; you suggest SPEP and UPEP; a faint band is in the beta-gamma region; diagnosis

Back 44

this is alpha heavy chain disease; routine SPEP and UPEP may be normal hindering diagnostic efforts, but sometimes show a faint alpha-chain bands
an immunofixation electropheresis would show just the heavy chain, without kappa or lambda component