Mature B cell

Question 1

Marginal zone diagnosis

Answer 1

Diagnosis requires either spleen histology or, alternatively, the documentation of a typical cell morphology and immunophenotype on blood cells coupled with the detection of intrasinusoidal infiltration by CD20+ cells in the bone marrow. Among B-cell tumors, deletion of 7q and NOTCh 2 is specific

Question 2

Presenting features Splenic MZL

Answer 2

in advanced-stage SMZL, symptomatic splenomegaly and cytopenia can be the presenting features
Lymphocytosis
Approximately 20% of patients show an autoimmune manifestation,1 including autoimmune hemolytic anemia, immune thrombocytopenia, cold agglutinin disease, circulating anticoagulants, acquired von Willebrand disease, or angioedema as a result of acquired C1-esterase inhibitor deficiency

Question 3

Lymphocytes in SMZL

Answer 3

typical cell morphology - round nuclei, condensed chromatin, and basophilic cytoplasm with polar short villi (so-called “villous lymphocytes”).
Heterogeneity in blood morphology is common,-small lymphoid cells without specific features, to various degrees of monocytoid and plasmacytoid differentiation.
Large cells, although rare, may suggest disease transformation into a large-cell lymphoma.15

Question 4

Bone marrow trephine MZL

Answer 4

sinusoidal pattern of infiltration is often detectable, usually combined with an interstitial and nodular component.
this pattern may also be observed, in several low-grade B-cell lymphomas.
a search for the dendritic meshwork more commonly present and disrupted in SMZL,18 is helpful in many instances.

Question 5

Extra nodal marginal malt associations

Answer 5

Hep c
H pylori gut
C psitticae eye
Sjorgens mouth
Hashimotos
Skin (assoc with Borrelia borgdorferi),

Question 6

DDx for IgM paraprotein

Answer 6

Waldenstroms
CLL
Marginal zone

Question 7

Trisomy 12 CLL

Answer 7

Atypical morphology
Inc prolymphocytic circulating cells
Decreased score on CLL flow - CD79b, FMC7 and surface Ig mod/strong
Assoc w worse prognosis esp when w notch p mutations

Question 8

DDx for splenic based lymphoma

Answer 8

SMZL
HCL
lPL
B-PLL
FOLLICULAR
McL

Question 9

Trisomy 12 CLL

Answer 9

atypical morphology
increased circulating prolymphocytoid cells low CLL scores on immunophenotyping due to expression of CD79b, FMC7 and surface Ig mod/strong ; this has been referred to as variant CLL 1. Furthermore, it may also be associated with a less favourable prognosis particularly in cases with associated NOTCH1 mutations 2.

Question 10

If matut s < 3- how do you rule out mantle cell

Answer 10

Immunostaining of bone marrow trephine sections or lymph node for nuclear cyclin D1
(ii) FISH for t(11:14) using blood, BM or lymph node

Question 11

Loss of tp 53

Answer 11

strongly associated with poor
response to alkylating agents and purine analogues.

Question 12

Diagnostic criteria for b pll

Answer 12

Lymphocytosis with more than 55% prolymphocytes
Immunophenotype SmIgM/D strong, CD20 strong,
CD22/CD79b strong, FMC7+, CD5+ in 33% patients.
CD23 usually negative.
Immunohistochemistry for Cyclin D1 in all cases to
exclude MCL and/or FISH for t(11;14).

Question 13

Peripheral blood film in lpl

Answer 13

marked rouleaux formation and variable
numbers of small lymphocytes, lymphoplasmacytoid cells and plasma cells, the
latter rarely present in high numbers. Anaemia, neutropenia and
thrombocytopenia may occur because of hypersplenism and/or bone marrow
failure

Question 14

Flow for LPL

Answer 14

Immunophenotype: Sm IgM+ CD5- CD10- CD19+
CD20+ CD22+ CD23- CD25+ CD27+ FMC7+
CD103- CD138-.
Additional inves

Question 15

Other tests in LPL

Answer 15

Plasma viscosity, tests of renal and hepatic function
Direct antiglobulin test, cold agglutinin titre and
cryoglobulins
Beta 2 microglobulin

Question 16

Cytogenetic is SMZL

Answer 16

Loss of 7q21-32 has been reported in up to 40% of cases of SMZL
Other described abnormalities include trisomy
3, deletion 13q and deletions of TP5

Question 17

Immunophenotype for SMZL

Answer 17

Marker Reaction
SmIg Strong, IgM and IgD
CD5, CD10, CD23 neg
CD19, CD20, FMC7 pos 
CD22 & CD79b pos
CD11c pos 
CD25 neg
CD43, CD103, CD123neg
Cyclin D1neg

Question 18

Prognostications Ng CLL

Answer 18

Rai or Binet stage
IGHV mutational status,
serum β2m,
presence of del(17p) and/or TP53 mutation, complex karyotype

Question 19

Flow for cll

Answer 19

Dim surface light chains
    B-cell surface antigens (CD19, CD20dim, CD23)
    CD5 surface antigen
CD 38 can be expressed or no and inc risk if it is

Question 20

Trans for mcl

Answer 20

11:14

Question 21

Indolent mantle zone

Answer 21

Leukaemuc, minmial ln, igh unmutated, Sox 11 neg

Question 22

Genetic profiles marginal zone lymphomas

Answer 22

Lacks translocation thus can differentiate it from irher types
Hetero del 7qm gain 3q notch2 and klf2