MATURE LYMPHOID NEOPLASM

MATURE LYMPHOID NEOPLASM  Peripheral Blood

 Lymphomas – are neoplasms of the lymphoid

system.
o Diagnosis is based on a combination of
biologic features:
 Morphology
 Immunophenotype
 Molecular Genetic
characteristics
 Clinical Information

 Chromosomal abnormalities
 Ritcher Syndrome
MATURE B-CELL LYMPHOMA
 Derived from various stages of B cell
differentiation.
 All B cell lymphomas produce monoclonal light
chain immunoglobulins, clonal immunoglobulin
gene rearrangements, or both
CHRONIC LYMPHOCYTIC LEUKEMIA (CLL) / SMALL
LYMPHOCYTIC LYMPHOMA (SLL)
 Characterized by accumulation of small
lymphoid cells in peripheral blood, bone
marrow, and lymphoid organs
 Most common type of Leukemia in Western
Hemisphere
 Pathophysiology
 Morphology:
• Bone marrow and peripheral blood
films show small lymphoid cells with a
characteristically coarse chromatin
(“soccer-ball pattern)
• Absent or inconspicuous nucleoli, and
scant cytoplasm
• Gain of extra chromosome 12
• Translocation to the end of long arm of
chromosome 14 at band 14q32 (but
break in 11q13 seems to be common)
• Rare transformation of CLL
characterized by a more aggressive
form of large cell lymphoma
• [PB- Increased lymphocytes w/the
presence of Basket Cell/Smudge Cell]
• Problem is in Lymphoid linage (Mature
Lymphocytes are seen)- CML
• Immature Lymphocytes are seen in ALL
• Mature Lymphocytes- incompetent and
useless in the circulation
• SLL- If solid lymph modal Mass is
present, we can call it as Small
Lymphocytic Leukemia
• Start from leukemia will lead to
lymphoma Present anemia
/Thrombocytopenia → Severe CLL
• Proliferation and accumulation of
lymphocytes (Bcells) that are relatively
unresponsive to antigenic stimuli [even
T helper cell signals that there is
infection happening, B cell uncapable of
producing antibody]
 MATURE LYMPHOID NEOPLASM

• Dormant and accumulate in peripheral • Low CD30 level

blood, bone marrow, lymph nodes and  Staging
spleen
 Clinical Presentation Binet staging system for CLL
• Affects elderly >50 years old Stage Median Survival
• Common in Men A: Hgb >10 g/dL; plt >120 months
• Anemia (marked fatigue, bruising, pallor >100 x 109 /L;
or jaundice) B: Hgb >10 g/dL; plt 61 months
• Lymph Node obstruction (fever, >100 x 109 /L; >3
recurrent or persistent infection, bone anatomic sites involved
tenderness, weight loss and edema C: Hgb <10 g/dL; plt 32 months
 Physical Finding <100 x 109
• Enlarged lymph nodes /L
• Splenomegaly
• Hepatomegaly
 Laboratory findings:
• Immunophenotype of CLL by flow Rai Staging system for CLL
cytometry Stage Risk and Median
• Surface lg dim or negative Survival
• CD5 (+) Stage 0: lymphocytosis Low; 10 years
• CD19 (+) in PB and BM only
• CD20 weak (+) (CD19 expression > Stage I: lymphocytosis Intermediate, 6 years
CD20 expression is virtually plus enlarged lymph
pathognomonic) nodes
• CD22 weak (+) Stage II: lymphocytosis Intermediate; 6 years
• CD79a (+) plus enlarged liver
• CD23 (+) and/or spleen
• CD11c weak (+) Stage III: lymphocytosis High; 2 years
• FMC7 (-) plus anemia (Hgb
Recurrent chromosomal abnormalities in CLL Stage IV: lymphocytosis High; 2 years
Genetic Abnormality Description and thrombocytopenia,
anemia and
Trisomy 12 Present in 20%; atypical
organomegaly may be
morphology; aggressive
present
clinical course
• Treatment
Deletions at 13q14 Present in 50%;
o Early stage
associated with long
 no treatment (just follow up)
survival
o Symptomatic, higher risk, bone
Deletions at 11q22-23 Present in 20%;
marrow failure
extensive
 chemotherapy (Fludarabine,
lymphadenopathy;
cyclophosphamide, rituximab)
poor survival
 Alkylating agents (chlorambucil,
Deletions at 6q21 Present in 5%
Bentamustine or
Deletions at 17p13 Present in 10% cyclophosphamide)
Ig heavy chain variable Associated with better o Bone marrow failure with autoimmune
region gene mutation prognosis cytopenias
TP53 abnormalities Associated with poor  STEROIDS
prognosis o Lymphadenopathy
• Good prognosis  RADIATION (DEBULKING)
• Low ZAP70 o Splenomegaly
 MATURE LYMPHOID NEOPLASM
 SPLENECTOMY
o Stem Cell Transplant
PROLYMPHOCYTIC LEUKEMIA (PLL)
 Is a rare mature lymphoid leukemia that can be
derived from B or T cells.
 Diagnosis requires more than 55% of circulating
lymphoid cells that have the morphology of a
prolymphocyte
 Morphology
o The pathogenic cell of B cell PLL is a
prolymphocyte of medium size with
HAIRY CELL LEUKEMIA (PLL)
round nucleus, moderately abundant
cytoplasm and distinct “punched-out”
nucleus.
 Peripheral Blood
 Pathophysiology
o Proliferation and accumulation of
abnormal lymphoid cells in the spleen,
bone marrow and liver
 Clinical Presentation
o Men (mean age of presentation is 70
years)
o Fatigue, weakness, weight loss, sweats,
and fever.
o Generally acute in onset
 Physical Findings
o Enlargement of spleen and less often
the liver
o Lymphadenopathy is uncommon
 Immunohistochemistry
o CD 20, Bcl-1 negative stain
 Flow Cytometry
o CD19, CD20, CD 22, CD79a, CD79b and
FMC7
 Genetics
o t(11;14) (q13;q32) abnormality has
been reported to be positive in 20%
o Deletions of 11q23 and 13q14
 Treatment:
o Goal of therapy:
 Reduce the lymphocyte mass in
the blood, marrow, and tissue
 Reduce symptoms
 Improve hematopoiesis
o Responds poorly [alkylating agents]
 Characterized by small B lymphocyte with
abundant cytoplasm and fine (“hairy”)
cytoplasmic projections
 OLD NAME: Histocytec; Leukemic Endotheliosis
 Rare neoplasm
 Middle aged to elderly.
 Male to Female ratio of 5:1
 Treatable and curable
 Hallmark: Cytopenias
 1923 Ewald described the disease as “Leukemic
reticuloendotheliosis”
 Two common characteristics
o Large spleen
o Presence of mononuclear cells with
numerous cytoplasmic projections
 Morphology:
o Predominantly found in the bone
marrow and the red pulp of the spleen
o Lymph node involvement is rare
o Neoplastic cells display an oval or
indented nucleus, abundant cytoplasm,
and fine, hairlike cytoplasmic
projections
 Peripheral Blood
 Laboratory Findings:
o Presence of Hairy Cells
 MATURE LYMPHOID NEOPLASM

 Scanty to abundant, agranular

cytoplasm
 Chromatin is loose and lacy
 (+) B- cell Markers – CD19,
CD20, CD22
 (+) TRAP (Tartrate Resistant
Acid Phosphatase)
 CD123 and Annexin A1 – most
specific marker for HCL
 Other Mature B-cell Lymphoma:
o Mantle Cell Lymphoma
o Follicular Lymphoma
o Extranodal Marginal Zone Lymphoma of
Mucosa-associated Lymphoid Tissues
o Plasma Cell Neoplasms
o Diffuse Large B Cell Lymphoma
o Burkitt Lymphoma