HEMA2 Lec 1st Shift Reviewer
Lymphoid & Plasma Cell Neoplasms
Prepared by: C3
MATURE B-CELL NEOPLASMS
• The World Health Organization (WHO)
Classification of Tumors of the
Hematopoietic and Lymphoid Tissues,
fourth edition, has enhanced the
classification of lymphoid neoplasms by
including immunophenotypic features
and genetic abnormalities to define
different disorders
CHRONIC LYMPHOCYTIC
LEUKEMIA/SMALL LYMPHOCYTIC
LYMPHOMA
• Chronic leukemias are generally
characterized by the presence of
leukocytosis with an increased number
of mature lymphocytes, lymphocytosis,
on a peripheral blood film
• Malignant lymphoproliferative disorders
are characterized by an accumulation of
lymphocytes
• Both CLL and SLL Neoplasms are
composed of small B Lymphocytes in
the peripheral blood, bone marrow,
spleen, and lymph nodes, mixed with
prolymphocytes and paraimmunoblasts
forming proliferation centers in tissue
infiltrates
• In contrast to CLL, SLL is used for
nonleukemic patients with the tissue
morphology and immunophenotyped of
CLL
Epidemiology
• CLL is the most common form of
leukemia in adults in Western countries,
but it is very rare in far Eastern countries
• CLL/SLL accounts for almost 7% of non-
Hodgkin lymphomas (NHLs) in biopsies
• The median age of onset is 65 years
o This form of leukemia is rare
before age 20 and uncommon
before age 50
o More males than females (1:5 to
2.1:1) are afflicted by this
disorder
• CLL has the highest genetic
predisposition of all hematologic
neoplasms
• A family predisposition can be
documented in 5 to 10% of patients with
CLL
o The overall risk is two to seven
times greater in first-degree
relatives of CLL patients
Etiology
• Classic CLL is usually a B-cell disorder
• Mature B-cell neoplasms are clonal
proliferations of B cells at various stages
of differentiation ranging from naïve B
cells to mature plasma cells
• Mature B-cell neoplasms comprise more
than 90% of lymphoid neoplasms
worldwide. These include:
o Chronic lymphocytic
leukemia/small lymphocytic
leukemia
o B-cell prolymphocytic leukemia
o Hairy cell leukemia
o Plasma cell myeloma
o Monoclonal gammopathy of
undetermined significance
(MGUS)
o Primary amyloidosis
o Heavy chain diseases
o Burkitt lymphoma/leukemia
• B-CLL is a biologically and clinically
heterogeneous hematologic malignancy
characterized by a gradually progressive
accumulation of morphologically mature
B lymphocytes in the blood, bone
marrow, and lymphatic tissues
TRIPLE C NOTES 1
 • Cells are characterized as CD5+,
CD19+, and CD23+ monoclonal B cells
• An excess of B cells is more likely to be
a result of decreased apoptosis and
deregulation of cell cycle control than of
an increased proliferation rate
• CLL cells are very resisant to apoptosis
o The anti-apoptopic BCL2 gene
is reported to be overexpressed
in 65% to 70% of B-cell CLLs
Cytogenetics
• CLL is heterogeneous at the clinical,
cellular, and molecular levels
• Chromosomal alterations occur in
approximately 80% of CLL cases; these
alterations include:
o 13q deletion
o 11q deletion
o Trisomy of chromosome 12
o 17p deletion
• The high rate of recurrence of the same
chromosomal abnormalities suggests
that these abnormalities may affect a
common pathway
• Molecular genetics has two major
applications in the analysis of chronic
lymphoid malignancies:
o Demonstration of the clonal
nature of a population of
lymphoid cells
o Detection of pathogenetically
important rearrangements, for
example, clonal IG or TCR gene
rearrangements, that are useful
in diagnosis of CLL
• Molecular genetic detection of genomic
rearrangements may not only assist with
the diagnosis but can also provide
important prognostic information. Many
of these rearrangements can act as
molecular markers for the detection of
low levels of residual disease
Molecular Genetics
Variable Region Genes
• New knowledge regarding the biology of
CLL has demonstrated that
approximately 50% to 70% of CLL cases
undergo immunoglobulin variable region
genes (IgVH) hypermutation
• The IgVH mutational status is important
in determining the prognosis of patients
with CLL
Zeta-chain, associated protein 70
• Another signaling associated molecule,
the zeta-chain-associated protein 70
(ZAP-70), was recently discovered to be
differentially expressed in the CLL
subgroup without IgVH mutation that had
poor outcomes
• ZAP-70, an enzyme normally expressed
in T lymphocytes, is critical for the
activation of T cells by antigen
Thymidine Kinase
• Another new finding is the correlation of
the serum value of thymidine kinase with
IgVH gene mutational status and also
with disease progression
• DNA microarray has demonstrated that
CLL exhibits a characteristic gene
expression profile closely related to
memory B cells and independent of the
presence of IgVH mutations
CD38
• Expression of CD38, a membrane
protein that marks cellular activation and
maturation and that has signaling
activity, often correlates with the
presence of IgVH mutations.
• CD38 surface expression on the
malignant cells is now viewed as an
independent marker of a patient’s
clinical outcome
MicroRNA
• MicroRNA (miRNA) expression profiles
can be used to distinguish normal B
cells from malignant B cells in CLL
patients
• A unique microRNA signature is
associated with prognostic factors and
disease progression in CLL
• MicroRNAs regulate the expression of
protein-coding genes and can act as
oncogenes, tumor suppressors, or both
TRIPLE C NOTES 2
 • Alterations in CLL can affect the
following:
o Evasion of apoptosis
o Self-sufficiency in growth
o Stimulation of angiogenesis and
dissemination
• Evasion of apoptosis is associated with
overexpression of the anti-apoptopic
protein bcl2
o BCL2 is responsible for
maintaining the delicate
homeostasis between
proliferation and apoptosis and
promotes cell survival by
inhibiting cell death
• Self-Sufficiency in growth demonstrates
that normal cells require growth stimuli
compared to cancer cells that are
capable of regenerating their own
growth signals without having to rely on
mitogens in the surrounding
environment in order to actively
proliferate
• Stimulation of Angiogenesis and
Dissemination is a characteristic of the
marrow and lymph nodes of patients
with CLL
o Patients show a high degree of
tissue neovascularization
Staging and Prognosis
• Clinical staging systems (Rai and Binet)
for assessing prognosis in CLL were
developed in the early 1980s, based on
easily obtainable biological and clinical
parameters
• The staging classification is:
o 0 – Bone marrow & blood
lymphocytosis
o I – Lymphocytosis with enlarged
nodes
o II – Lymphocytosis with
enlarged spleen or liver or both
o III – Lymphocytosis with anemia
o IV – Lymphocytosis with
thrombocytopenia
• Most patients with CLL now have Rai
stage 0 or I disease at diagnosis
• Prognosis is roughly related to the
extent of organ infiltration at the time of
diagnosis
• Since the introduction of clinical stages,
there has been a continuous effort to
identify new prognostic factors in CLL.
Parameters with demonstrated
independent prognostic value include:
o Number of lymphocytes in the
peripheral blood
o Degree of bone marrow
infiltration
o Proportion of abnormal
lymphoid cells in the peripheral
blood
o Lymphocyte doubling time
o Immunoglobulin heavy-chain
variable-region gene mutation
status
o Cytogenetic abnormalities
assessed by fluorescent in situ
hybridization
o Z-chain-associated protein
kinase-70 protein expression
Clinical Signs & Symptoms
• The typical patient with CLL is
asymptomatic, and the disease is
usually discovered at the time of a
routine physical examination
• The disease is typically suggested by
abnormal findings discovered on a
complete blood count (CBC) for the
evaluation of an unrelated illness
• Common symptoms include:
o Malaise
o Low-grade fever
o Night sweats
o Weakness
o Fatigue
o Anorexia
o Weight loss
TRIPLE C NOTES 3
 • Physical examination usually reveals
cervical and supraclavicular adenopathy
• Hepatosplenomegaly is also frequently
present

Laboratory Data
• Normal bone marrow elements get Figure 2. Two slides showing chronic lymphocytic
crowded out because of the excessive leukemia.
lymphoid production and packaging of
the marrow space by malignant
lymphocytes
o This infiltration by the leukemic
clone results in anemia,
thrombocytopenia, and
neutropenia
• Although leukocytosis may be observed,
it is less pronounced than in chronic
myelogenous leukemia
o Total leukocyte counts can
range from 30 to 200 x 109/L
• In one-third of patients, the total
leukocyte count is greater than 100 x
109/L
• Peripheral blood smears commonly
exhibit up to 80% or 90% small
lymphocytes
• Many of these cells have an overmature
look because of the hypercondensed
nuclear chromatin pattern
• An occasional large lymphoblast may be Figure 3. A: Hyperleukocytosis (left). An elevated
noted. Smudge cells are highly leukocyte concentration in a centrifuged peripheral
blood specimen from a patient with T-cell acute
characteristic
lymphoblastic leukemia (right). Pulmonary alveolar
• Both the granulocytes and platelets are capillaries expanded by leukocyte aggregates
normal indicative of leukostasis in a patient with acute
myeloid leukemia. B: Microarray analysis in chronic
lymphocytic leukemia (CLL) (left). The expression of
C247 signature genes (right). Differentiation of CLL
patients into those with or without mutations of the IgV
gene by the expresisons of 56 genes and Ig
immunoglobulins.

Treatment Options
• Previously, patients with CLL were only
treated for palliative reasons, but
numerous new treatment options are
now available for the treatment of CLL
• Allogenic hematopoietic stem cell
transplantation (alloHSCT) is the only
Figure 1. Chronic lymphocytic leukemia. Mature cells potentially curative treatment available
predominate in the chronic leukemias. In this blood for patients with B-cell CLL
smear, a typical increase in the number of smudge
cells is seen.

TRIPLE C NOTES 4
 • Newer treatment chemoimmunotherapy
was introduced in 2000
o This strategy incorporates the
use of monoclonal antibodies to
chemotherapy
o This strategy appears to provide
for a first time-observed survival
benefit but is not considered
curative
reaction that is not inhibited by tartaric
acid or tartrate-resistant acid
phosphatase (TRAP) stain
• The immunological markers include
CD19+, CD20+, CD22+, CD24+, and
CD25+ reactivity to the monoclonal
antibody that recognizes the interleukin-
2 (Tac) receptor

Minimal Residual Disease

• PCR-based and flow cytometry-based
assays are used to assess MRD in CLL
• Real-time PCR has become common for
quantification by PCR

HAIRY CELL LEUKEMIA

• HCL is an uncommon chronic
lymphoproliferative disorder of the B-
lymphocyte type
• This mature B-cell malignancy is
diagnosed based on clinical features,
morphology, and phenotyping and Figure 4. Hairy cell leukemia. A: Peripheral blood. B:
Bone marrow (H&E stain). C: Spleen (H&E stain).
generally treated with curative intent
• Much more common in males than in
Hairy Cell Leukemia Variant
females
• A variant form of HCL, hairy cell
o It has been suggested that a
leukemia variant (vHCL), was
locus on the X Chromosome
discovered in 1980. In addition, much
might be involved
rarer variants, Japanese variant, and
o Usually affects patients older
blastic variant, exist
than 30 years of age
• vHCL is a more aggressive type of HCL
• So named because of the appearance
and has different morphological
of fine, hair-like, irregular cytoplasmic
characteristics than typical HCL
projections that are characteristic of
• vHCL cells are smaller than the typical
lymphocytes in this disease
HCL cell with a central round nucleus,
o Cytoplasmic projections are not
prominent nucleoli, a larger nuclear-
always obvious in HCL and in
cytoplasmic ratio, and basophilic
some cases (e.g. artifactually in
cytoplasm with occasional cytoplasmic
other lymphoid neoplasms or
projections
reactive cells) are not specific
for HCL
• Morphologically, HCLs are large with
TRIPLE C PRO-TIPS: REMEMBERING HAIRY
moderately large nuclei CELL LEUKEMIA
o Sometimes the slate-blue
cytoplasm is vacuolated Hairy Cell Leukemia is CD 11c (+)
o The nucleus is frequently oval or The Italian word for “hair” is “Capelli” so…
slightly clefted and may be “Cape11i” = CD 11c (+)
convoluted with a homogeneous
chromatin pattern
• The cytochemical features of HCL
include a strong acid phosphatase

TRIPLE C NOTES 5
PROLYMPHOCYTIC LEUKEMIA
• B-cell prolymphocytic leukemia
represents malignancy of B
prolymphocytes affecting blood, bone
marrow, and spleen
• Characterized by a large number of
small lymphocytes with scant cytoplasm
and the immature features of
prolymphocytes in the peripheral blood
• The leukocytosis can exceed 100 x
109/L
• Prolymphocytes must exceed 55% of
lymphoid cells in the peripheral blood
MULTIPLE MYELOMA (PLASMA
CELL MYELOMA)
• Malignant bone marrow-based plasma
cell neoplasm associated with abnormal
protein production
• Plasma cell leukemia is an increased
number of plasma cells in the
peripheral blood and should be
considered a form of multiple myeloma
and not a separate entity
• Multiple myeloma accounts for
approximately 1% of all types of
malignant diseases and about 10% of
hematological malignancies
Epidemiology
• Multiple myeloma accounts for
approximately 1% of all types of
malignant diseases and about 10% of
hematological malignancies
• It usually evolves from an asymptomatic
premalignant stage of clonal plasma cell
proliferation called “monoclonal
gammopathy of undetermined
significance (MGUS)
Etiology
• In Multiple Myeloma, typically, the bone
marrow is involved, but the disorder may
involve other tissues as well
• The etiology is unknown; however,
radiation may be a factor, and the
possibility of a viral cause has been
suggested
Clinical Signs and Symptoms
• Symptoms of multiple myeloma include
bone pain (typically in the back or chest)
that is present at the time of diagnosis in
more than two thirds of patients,
weakness, and fatigue
• Weight loss and night swats are not
prominent until the disease is advanced
• Abnormal bleeding may be a prominent
feature
• In some patients, the major symptoms
result from acute infection, renal
insufficiency, hypercalcemia, or
amyloidosis
• This disorder runs a progressive course,
and most patients die in 1 to 3 years
• The major causes of death are infection
and renal insufficiency
• Multiple myeloma leads to a
compensatory decrease in synthesis
and increase in catabolism of normal
immunoglobulins
Laboratory Data
• Anemia is present at the time of
diagnosis in approximately two thirds of
patients
• Increased plasma volume caused by
monoclonal protein commonly produces
hypervolemia
• The leukocyte count can be normal,
although about one third of patients
have leukopenia
o Relative lymphocytosis is
usually present
o Sometimes, eosinophilia is
noted
• In rare cases in the terminal stages,
plasmablasts and plasma cells may
amount to 50% of the leukocytes in the
peripheral blood
o Rouleaux formation on
peripheral blood smears is
common
• Bleeding is common
• Platelet abnormalities, impaired
aggregation of platelets, and
interference with platelet function by the
abnormal monoclonal protein contribute
to the bleeding
TRIPLE C NOTES 6
 • Inhibitors of coagulation factors and
thrombocytopenia from marrow
infiltration of plasma cells or
chemotherapy may also contribute to
bleeding
• Some patients have a tendency toward
thrombosis, which may be manifested
by a shortened coagulation time,
increased fibrinogen, and increased
factor VIII
• Electrophoresis of serum usually
demonstrates the overproduction of IgM
(19S) antibodies
• Electrophoresis of the serum or urine Figure 6. Abnormal serum protein electrophoretic
patterns contrasted with a normal pattern. Polyclonal
reveals tall sharp peaks on the
hypergammaglobulinemia, characteristic of benign
densitometer tracing; a dense localized reactive processes; shows a broad-based increase in
band is seen in 75% of myeloma cases immunoglobulins, owing to immunoglobulin secretion
• A monoclonal serum protein is detected by a myriad of reactive plasma cells.
in 91% of patients
o The type of antibody is IgG in
the majority of patients
o Less frequently IgA is seen, and
rarely IgD is demonstrated

Figure 7. Protein electrophoresis in plasma cell

Figure 5. Various appearances of plasma cells in a
smear of normal bone marrow. A prominent pale
paranuclear zone and cytoplasmic vacuoles are seen
in (A) and (B). The cytoplasm in (C) has a reticular
appearance. The other cells in (A) are a
nonphagocytic reticular cell and a late polychromatic
erythroblast.
disease. Serum or urine is placed at one end of a strip
of gel‚ across which an electrical current is applied.
Most proteins have a negative charge and migrate
toward the positive pole at various speeds according
to their molecular weight and charge. The serum on
the left shows a narrow band in the gamma globulin
region. Serum protein has spilled into urine‚ which
demonstrates a matching band. In the scan of patient
serum on the right‚ the height of the tracing is
proportional to the amount of protein stained in each
band. Monoclonal protein appears as a tall‚ narrow
(monoclonal) “M-protein.”
Treatment
• Multiple myeloma is incurable with
conventional chemotherapy
• Thalidomide, lenalomide, and
bortezomib are novel agents that first
demonstrated efficacy in treating
relapsed and refractory MM, but they
are now all being utilized as treatment
for newly diagnosed disease

TRIPLE C NOTES 7
WALDENSTRÖM PRIMARY
MACROGLOBULINEMIA
(LYMPHOPLASMACYTIC
LYMPHOMA)
Epidemiology
• The condition of WM has an age-
specific incidence
o It is most commonly found in
older men; the median age of
onset varies between 63 and 68
years of age
o Onset is usually insidious
o The incidence of WM is higher
among whites
• Prognostic factors include the patient’s
age, β2-microglobulin level, monoclonal
protein level, hemoglobin concentration,
and platelet count
• The reported median survival of patients
with WM ranges between 5 and 10
years from the time of diagnosis
Etiology
• WM is a B-cell neoplasm characterized
by lymphoplasma proliferative disorder
with infiltration of the bone marrow and a
monoclonal immunoglobulin M (IgM)
protein
• This malignant lymphocyte-plasma cell
proliferative disorder is associated with
the production of abnormally large
amounts of gamma globulin of the 19S
or IgM type
Clinical Signs & Symptoms
• The symptoms of WM are due to the
extent of tumor infiltration and to
elevated IgM levels in the blood
circulation
• Symptoms include:
o Weakness
o Fatigue attributable to anemia
o Bleeding
• Bone pain is virtually non-existent
• About one-fourth of patients with WM
have neurological abnormalities
• Patients usually suffer from chronic
anemia and bleeding episodes
• Thrombocytopenia and hyperviscosity
may also contribute to the bleeding
disorder
Laboratory Data
• The most consistent feature of the bone
marrow or lymph nodes of WM patients
is the presence of pleomorphic B-
lineage cells at different stages of
maturation, such as:
o Small lymphocytes
o Lymphoplasmacytoid cells
(abundant basophilic cytoplasm
but lymphocyte-like nuclei)
o Plasma cells
• Characteristically, blood samples are
described as having hyperviscosity
• Detecting monoclonal gammopathies
usually involves serum protein
electrophoresis (SPEP) and
immunoelectrophoresis (IEP) to test
both serum and urine
• Additionally, cryoglobulins can be
detected in the patient’s serum
o Cryoglobulins are proteins that
precipitate or gel when cooled to
0°C and dissolve when heated
o In most cases, monoclonal
cryoglobulins are IgM or IgG
Figure 8. Waldenström. Bone marrow aspirate
showing malignant cells with lymphoid and
plasmacytoid morphology.
Treatment
• Therapy is postponed for asymptomatic
patients, and progressive anemia is the
most common indication for initiation of
treatment
TRIPLE C NOTES 8
 • Main therapeutic options include:
o Alkylating agents
o Nucleoside analogues
o Rituximab
• Novel agents, for example, vortezomib
show promise as a targeted therapy
options in WM
LYMPHOMAS
Relationship Between Lymphomas and
Leukemias
• The term lymphoproliferative disorder
includes the various forms of leukemias
and malignant lymphomas that are of
lymphoreticular origin
• The neoplastic cells of leukemia and
lymphoma have an intimate relationship
o Frequently, the neoplastic cells
of these two disorders are
identical
o It is characterized by the
infiltration of abnormal
lymphocytes and destruction of
the normal architecture of the
node
Categories
• The major forms of malignant
lymphomas are divided into Hodgkin
and non-Hodgkin (NHL) types
• The NHLs account for more than two-
thirds of all lymphomas and more than
75% of the fatalities due to lymphoma
• Rare forms of lymphoma include Burkitt
Lymphoma and Mycosis fungoides, a
variant of Sézary syndrome, which
demonstrates skin involvement

Characteristics
• The lymphomas are a group of closely
related disorders that are characterized
by the overproliferation of one or more
types of cells of the lymphoid systems
such as:
o Lymphoreticular stem cells
o Lymphocytes
o Reticulum cells
o Histiocytes
• Malignant lymphoma expresses itself as
a disorder of the lymph nodes

TRIPLE C NOTES 9
 Hodgkin Disease
• Hodgkin disease is characterized by a
persistent defect in the cellular immunity
with abnormalities in T lymphocytes, IL-
2 production, and increased sensitivity
to suppressor monocytes and normal T
suppressor cells
• The cellular origin of the Reed-
Sternberg cell is unknown, but Reed-
Sternberg cells have been shown to
Pathophysiology function as stimulatory cells in many
• Although the etiology of most lymphocyte reactions, as accessory
lymphomas is unknown, the potential cells in mitogen-induced T-cell
role of a virus in the pathogenesis of proliferation, and as antigen-presenting
lymphomas is strongly suspected cells in HLA-DR-restricted antigen-
• In humans, the development of B cells in specific T-cell activation
the bone marrow is initiated by the
assembly of genes for the variable
regions of the heavy and light chains of
antibodies in B-cell progenitors,
mediated by a process called V(D)J
recombination
• In this process, the DNA located
between the rearranging gene elements
is deleted from the chromosome (or
sometimes inverted)

Figure 9. Reed-Sternberg cell typical of Hodgkin

disease.

Precursor of Hodgkin Disease and B-Cell

Lymphomas
• Cytogenetic studies now suggest that
Reed-Sternberg cells arise from a single
clone, a common B-cell precursor
located in a germinal center
• Reed-Sternberg cells are a defining Figure 10. Hodgkin lymphocyte–rich “classic”
feature of Hodgkin disease, but less Hodgkin disease. The background is primarily
lymphocytes with Reed-Sternberg cells.
than 1% of cells in a sample of Hodgkin
disease tissue are Reed-Sternberg cells

TRIPLE C NOTES 10
Non-Hodgkin Lymphoma
• The most frequent type of NHL is diffuse
large B-cell lymphoma, which accounts
for approximately 40% of new cases of
lymphoma
• More than half of patients with diffuse
large B-cell lymphoma are older than 60
years of age
• 3 different types of diffuse large B-cell
lymphomas can be defined based on
gene expression subgroups:
o Germinal center, B-cell-like
lymphoma that expresses high
levels of genes characteristic of
germinal center
o Activated B-cell-like lymphoma,
which expresses genes
characteristic of mitogenically
activated blood B cells
o New subgroup, type 3 diffuse
large B-cell lymphoma, which
has a heterogeneous gene
expression that suggests it
includes more than one subtype
of lymphoma
Figure 11. Lymphomas. A: Non-Hodgkin lymphoma.
B: Reactive adenitis in a child. C: Mixed large and
small lymphocytes in a patient with Hodgkin disease.
D: Large B-cell lymphoma.
TRIPLE C PRO-TIPS: HODGKIN AND NON-
HODGKIN LYMPHOMA TREATMENT MNEMONICS
HODGKIN’s = A Big Ventricular Damage (ABVD)
• Adriamycin
• Bleomycin
• Vinblastine
• Dacarbazine
NON-HODGKIN’s = Raptor-CHOP
• Rituximab
• Cyclophosphamide
• Hydroxydaunorubicin
• Oncovin (Vincristine)
• Prednisolone
Characteristic of Other Forms
• In NHL, Reed-Sternberg cells are
absent
• The infiltrating cells may be of one type
or may have a mixed cell population of
lymphocytes, histiocytes, eosinophils,
and some plasma cells
Cytogenetic Analysis
• The chromosomal anomalies that have
been observed in hematological
malignant disease include structural
rearrangement as translocations and
deletions and numerical abnormalities
with respect to structural
rearrangements
Sézary Syndrome
• The leukemic phase of cutaneous T-cell
lymphoma, mycosis fungoides, is
called Sézary Syndrome
• Diagnosis of Sézary Syndrome is
dependent on the primary diagnosis of
mycosis fungoides in a skin biopsy
• Adults between 40 and 60 years old are
most frequently afflicted with skin
lesions that progress to the tumor stage
• In peripheral blood, the disease is
characterized by the presence of
abnormal circulating lymphocytes,
Sézary Cells
• A Sézary cell is typically the size of a
small lymphocyte and has a dark-
staining, clumped, nuclear chromatin
pattern
o The distinctive folded, groove-
like chromatin pattern is
described as cerebriform
TRIPLE C NOTES 11
Figure 12. Mycosis fungoides (cutaneous T-cell
lymphoma). Lesions have characteristic “smudgy,”
poorly defined patches and plaques in a typical
location.

Figure 13. Mycosis fungoides with focal interstitial

infiltration by small lymphocytes.

Figure 14. Sézary cells. Two circulating neoplastic T-

helper cells with irregular nuclei and a thin rim of
cytoplasm are seen.

TRIPLE C NOTES 12