WBC 5: Chronic Lymphoproliferative/Lymphoma; Multiple Myeloma/Plasma Cell Disorders

LDH and b2-MG are indicators of ____.

tumor load

what is the gold standard for lymphoma diagnosis?

biopsy

which mature lymphoid neoplasm is associated with 13q14 del?

CLL

which mature lymphoid neoplasm is associated with t(8;14), MYC?

PLL

which mature lymphoid neoplasm is associated with BRAF V(600)E?

HCL

what is the most common leukemia in adults?

CLL

what kind of cell disorder is CLL?

B cell

the median age of CLL is ____.

72

the following characteristics are associated with ____:

• >85% of lymphocytes appear as small and mature with scant cytoplasm and a dense nucleus with a condensed chromatin pattern without a defined nucleolus (“cobblestone” or “soccer ball” appearance)

• smudge cells (very important cells, must be counted as lymphocytes)

CLL

the following immunophenotyping markers are associated with ____:

• CD19

• CD20

• CD23

• T cell markers CD5 (aberrant)

CLL

the following expresses 1 point when scoring for ____ diagnosis (catovsky-matutes scoring)

• weak expression of surface immunoglobulin

• expression of CD5

• expression of CD23

• no expression of FMC7

• absent or weak expression of CD79b or CD22

CLL

a total catovsky-matutes score greater than or equal to 4 is consistent with ____.

CLL

a total catovsky-matutes score 1 or 2 (low scores) are consistent with ____.

non-hodgkin lymphoma

an intermediate catovsky-matutes score of ____ remains problematic and these cases may require biopsy of a node or molecular data to establish the diagnosis.

3

list the 2 worst prognostic indicators of CLL.

del(17p); TP53

what is the rai classification for CLL based on the following characteristics:

• lymphocytosis >5 × 10⁹/L

low risk

what is the rai classification for CLL based on the following characteristics:

• lymphocytosis >5 × 10⁹/L

• lymphadenopathy

• splenomegaly or hepatomegaly or both

intermediate risk 

what is the rai classification for CLL based on the following characteristics:

• lymphocytosis >5 × 10⁹/L

• hemoglobin <11 g/dL

high risk

what is the binet classification for CLL based on the following characteristics:

• hemoglobin >10 g/dL

• PLTs >100 × 10⁹/L

• <3 enlarged nodal areas

stage A

what is the binet classification for CLL based on the following characteristics:

• hemoglobin >10 g/dL

• PLTs >100 × 10⁹/L

• >3 enlarged nodal areas

stage B

what is the binet classification for CLL based on the following characteristics:

• hemoglobin >10 g/dL

• PLTs <100 × 10⁹/L

• any number of enlarged nodal areas

stage C

which gene mutation status is an important factor in predicting how CLL will behave/progress/etc.?

IGHV

if there is a mutation OR no mutation? in the IGHV gene, the CLL is associated with the pre-germinal center type of CLL and more aggressive clinical course.

no mutation 

if there is a mutation OR no mutation? in the IGHV gene, there is a much better prognosis and better response to drug treatment.

mutation

testing for ____ and ____ are correlated with IGHV mutational status for CLL.

ZAP70; CD38

which mature lymphoid neoplasm is associated with the following characteristics:

• rare

• median age of 70

• >75% cases B cell

• very aggressive

PLL

which mature lymphoid neoplasm is associated with the following characteristics:

• massive splenomegaly

• marked absolute lymphocytosis (>100 × 10⁹/L)

PLL

a rapid rising WBC count may be a clue that the diagnosis is not CLL and that it is most likely ____.

PLL

which mature lymphoid neoplasm is associated with the following characteristics:

• medium to large lymphs with ample cytoplasm

• more open chromatin

• prominent nucleolus

PLL

which mature lymphoid neoplasm is associated with the following characteristics:

• CD7 +

• inv(14) in approximately 70% of patients (using FISH) 

• hematology analyzer

  • increased WBC

  • increased lymph

  • blast of atypical or variant lymph flag must be followed with a PBS

PLL

indolent disease of B cell lineage most commonly found in middle age men (median age, 50); mostly males:

HCL

major focus of ____ is in spleen, blood, and BM splenomegaly and cytopenias (fibrotic BM due to fibrosis).

HCL

which mature lymphoid neoplasm is associated with dry tap due to fibrotic BM (fibrosis) and requires a biopsy?

HCL

monoclonal anti-CD20 an annexin A using immunohistochemistry are diagnostic staining tests for the diagnosis of which mature lymphoid neoplasm?

HCL

which condition is a clonal expansion of cytotoxic T cells expressing:

• CD3

• CD8

• CD57

T cell LGL

_____ is CD3 negative but CD56 positive.

NK cell LGL

associated with retroviral infection by the human T lymphotropic virus type 1 (HTLV-1):

ATLL

what virus is endemic in Japan, Caribbean islands, Africa, South America, the Middle East, and northern Oceania.

HTLV-1

what virus is transmitted via placental circulation, breastfeeding, blood transfusion, or sex?

HTLV-1

the estimated risk of developing ATLL among HTLV-1 carriers is about ____ in women over a latency period of 55 to 70 years.

2.5%

the estimated risk of developing ATLL among HTLV-1 carriers is about ____ in men over a latency period of 55 to 70 years.

5%

there is marked leukocytosis in the ____ phase of ATLL.

leukemic 

what cells have the following characteristics:

• medium to large in size and have accentuated, convoluted nuclei

• coarsely clumped chromatin

• deeply basophilic cytoplasm

• “flower cell”

ATLL

the ____ immunophenotype is generally consistent with T helper cells (CD3 and CD4 are expressed while CD7 and CD8 are absent).

ATLL

CD25 and CCR4 are highly expressed in which condition?

ATLL

the soluble form of ____ receptors have prognostic significance and can be used as a tumor marker for assessing ATLL status.

IL-2

aggressive cancer of mature B cells associated with a fulminant clinical presentation:

burkitt lymphoma/leukemia

which burkitt lymphoma/leukemia subtype is associated with the following characteristics:

• found primarily in childhood in equatorial Africa 

• extranodal involvement is common with orbits and mandible as typical sites of disease 

• EBV genome has been present in neoplastic cells 

endemic

which burkitt lymphoma/leukemia subtype is associated with the following characteristics:

• presents mostly as an abdominal disease 

• extranodal involvement is not uncommon 

sporadic 

which burkitt lymphoma/leukemia subtype is associated with the following characteristics:

• profound immunosuppression

• blood and BM are primary sites of disease

HIV

what cells have the following characteristics:

• medium to large in size

• finely clumped chromatin

• deeply basophilic cytoplasm with distinct vacuoles

• BM and lymph biopsies

  • “starry sky” appearance

    • interspersed tangible body macrophages suspended in a “sea” of malignant cells

burkitt lymphoma/leukemia

what cells have the following characteristics:

• sIgG (+)

• CD10 (+)

• CD34 (-)

• Tdt (-)

burkitt lymphoma/leukemia

neoplastic disorder of germinal B cells and account for about 12% of non hodgkin lymphoma cases:

follicular lymphoma

which mature lymphoid neoplasm is associated with the following characteristics:

• middle to older age groups

• has an indolent course but it is most often incurable with current therapies

• most patients are asymptomatic

  • sometimes painless lymphadenopathy

follicular lymphoma

which mature lymphoid neoplasm is associated with the following characteristics:

• peripheral blood involvement in 10% cases

  • circulating cells have a condense chromatin pattern with distinct nuclear clefts

• BM may be involved

follicular lymphoma

which mature lymphoid neoplasm is associated with the following characteristics:

• about 3-6% of non hodgkin lmyphoma cases

• median age 68

• male predominance

• clinically aggressive

• extensive lymphadenopathy

• diagnosis based on morphology alone is challenging

mantle cell lymphoma

which mature lymphoid neoplasm is associated with the following characteristics:

• small cell forms can be mistaken for CLL

• blastoid variants can be mistaken for ALL

• some morphologic changes can be confused with hairy cells or prolymphocytes

mantle cell lymphoma

an accurate diagnosis of ____ requires demonstration of either t(11;14) via FISH or overexpression of cyclin D1 by immunohistochemistry.

mantle cell lymphoma 

most common form of cutaneous T cell lymphoma in elderly, male predominance.

MF/SS

which is more common (70%) and confined to the skin: MF or SS?

MF

malignant T cells in ____ have the following characteristics:

• abnormal appearance with scant cytoplasm and cerebriform

• folded nucleus

• variably condensed chromatin inconspicuous nucleoli

MF/SS

both MF and SS are CD+ ____ cell disorders.

t helper cell

which cutaneous T cell lymphoma (MF or SS) refers to psoriatic-like skin lesions, skin biopsy needed?

MF

which cutaneous T cell lymphoma (MF or SS) refers to erythroderma, generalized lymphadenopathy, and presence of clonal T cells in skin, lymph nodes, and peripheral blood, and severe pruritus (itching)?

SS

____ refers to neoplasm of terminally differentiated B cells.

plasma cell neoplasms

monoclonal gammopathy of undetermined significance (MGUS) is the precursor state of _____.

multiple myeloma

multiple myeloma is a ____ cell neoplasm with a median age of 69.

plasma

an M spike in SPE helps predicting the risk for ____ transformation from MGUS.

multiple myeloma

multiple myeloma usually stays in the bone marrow. true or false?

true

plasma cell leukemia (PCL) is common and less aggressive form where more than 20% of plasma cells are found in the blood (outside of the BM). true or false?

false

in multiple myeloma, abnormal proteins called ____ in the blood can cause RBCs to stick together, forming stacks called rouleaux.

paraproteins

to tell the difference between multiple myeloma plasma cells from normal/reactive plasma cells are through immunohistochemistry with ____.

CD138; kappa/lambda clonality 

what cells strongly express the following:

• CD38

• CD138

• CD56 (aberrant)

multiple myeloma

low grade lymphoplasmacytic lymphoma associated with aberrant secretion of IgM:

WM

high levels of IgM can result in hyper-viscosity syndrome requiring emergent plasmapheresis to alleviate symptoms in what condition?

WM

somatic mutation in what gene is found in over 90% of patients with WM?

MYD88

hodgkin lymphoma is a ____ based lymphoma.

nodal

hodgkin lymphoma (both types) are based on ____.

node biopsy 

which hodgkin lymphoma is associated with the following characteristics:

• >85% of cases usually in young adults

• responds well to chemotherapy

classic

which hodgkin lymphoma is associated with the following characteristics:

• RS cells are of B cell origin

  • do not express most B cell markers

  • CD30 (+)

  • CD15 (+)

  • never seen in peripheral blood

classic

which hodgkin lymphoma is associated with the following characteristics:

• no RS cells 

• malignant cell is a lymphocytic histiocytic (L&H) cell = “popcorn” cell

  • CD15 (-)

  • CD30 (-)

lymphocyte-predominant