Burkitt lymphoma

Hodgkin lymphoma

Primary effusion lymphoma (PEL)

CD19+

CD34+

TdT+

CD19+

CD20+

CD22+

SIg+

CD138+

CD20-

CIg+

CD45

- name

- type of cells

-Leukocyte common antigen (LCA)

- Found on most mature hematolymphoid cells

CD3, CD2, CD5, CD7

- type of cells

- notes

T-cell markers

May be lost in some T-cell neoplasms

May be gained in non-T-cell neoplasms

CD19, CD20, CD22

- cell type

CD10

- cell type

Kappa or Lambda

- what is it?

- what cells?

- Immunoglobulin light chains

- on most mature B-cells and plasma cells

Staging of Lymphomas

-Stage 1

-Stage 2

-Stage 3

-Stage 4

Stage 1

- involvement of single lymph node region

Stage 2

- involvement of 2+ lymph node regions on same side of diaphragm

Stage 3

- involvement of lymph node regions on both sides of diaphragm

Stage 4

- diffuse or disseminated involvment of 1+ extralymphatic organs (including bone marrow)

- Chronic lympocytic leukemia/Small lymphocytic lymphoma (CLL/SLL)

- Follicular lymphoma

- Extranodal Marginal zone lymphoma (MALT)

- Mantle cell lymphoma

- Burkitt lymphoma

- Diffuse large cell lymphoma

- peripheral T-cell lymphoma

- mycosis fungoides/Seazary syndrome

- anaplastic large cell lymphoma

Chronic lymphocytic Leukemia/ Small lymphocytic lymphoma (CLL/SLL)

-Symptoms/Age groups

-Morphology

-Tests

-Prognosis

-Treatment

Symptoms:

- old age

- splenomegaly

- hepatomegaly (SLL)

- immunosuppression

- hypogammaglobulinemia

- bone marrow failure

- organ filtration

Morphology:

- Peripheral blood = smudge cells, lymphocytosis

- Lymph node = Mature B-cells with proliferation centers

Tests:

- CD 19, CD5

Prognosis:

- indolent course, transforms to large cell lymphoma (Richter's syndrome)

- 13q = good

- Trisomy 12 = intermediate

- 17q13 & 11q23 = poor

Treatment:

- Alemtuzumab (CD52 on mature lymphocytes)

Follicular Lymphoma

-Symptoms/Age groups

-Morphology

-Tests

-Prognosis

Symptoms:

- old age

- lymphadenopathy

Morphology:

- lymph nodes= uniform follicles with absent tingible body macrophages

Tests:

- CD10

- FISH = t(14;18) → Bcl2 overexpression

Prognosis:

- treat but can't cure

- can transform to large cell lymphoma

Extramarginal zone lymphoma (MALT)

-Symptoms

-Morphology

-Prognosis

-Treatment

Symptoms:

- arise in spleen or extranodal sites

- commonly due to H. pylori 

- associated with autoimmune disease

Morphology:

- expansion of marginal zone

Prognosis:

- indolent course

Treatment:

- antimicrobial therapy to treat underlying H. pylori infection

Diffuse large B-cell lymphoma

-Symptoms/Age groups

-Morphology

-Prognosis

-Treatment

-Etiology

Symptoms:

- most common B-cell lymphoma

- rapidly enlarging symptomatic mass

Morphology:

- diffuse proliferation of large lymphoid cells

Prognosis:

- aggressive

Treatment:

- chemo + rituximab

Etiology: 

- de novo

- transformation from FL or CLL

Mantle cell lymphoma

-Symptoms/Age groups

-Morphology

-Tests

-Prognosis

Symptoms:

- older age

- lymphadenopathy

- GIT involved

Morphology:

- proliferation of small cleaved cells

Tests:

- CD5, cyclin D1

- PCR, FISH, t(11;14)

Prognosis:

- aggressive course

Burkitt's lymphoma

-Symptoms/Age groups

-Morphology

-Tests

-Prognosis

-Treatment

Symptoms:

- small children

- bowel (ileocecal region) and CNS in US population= sporadic type

- madibular mass in endemic (aka Africa)

- fastest growing tumor; AIDS defining illness

Morphology:

- starry sky appearance

- immature cells

- cytoplasmic vacuoles

Tests:

- sIg(+), TdT(-)

- t(8;14) = c-myc overexpression

Treatment:

- cured with chemo

Peripheral T-cell lymphoma

-Symptoms/Age groups

-Morphology

-Tests

Symptoms:

- majority of T-cell lymphomas

Morphology:

- eosinophils

- plasma cells

Tests:

- Tdt(-), CD3(+)

Mycosis Fungiodes

-Symptoms/Age groups

-Morphology

-Tests

-Prognosis

-Treatment

Symptoms:

- skin rash (CD4 T cells)

- Sezary syndrome = dissemination to peripheral blood, lymph nodes, spleen

Morphology:

- Pautrier's microabscess

Treatment:

- Early stage = topical

- Advanced = chemo, interferon, retinoids, vorinostat, immunoconjugates

Adult T-cell leukemia/lymphoma

-Symptoms/Age groups

-Morphology

-Tests

-Prognosis

-Treatment

Symptoms:

- associated with HTLV1 infections

- skin lesions and hypercalcemia

Morphology:

- typical CD4+ atypical lymphocytes "flower cells"

Acute lymphoblastic leukemia (ALL)

-Symptoms/Age groups

-Morphology

-Tests

-Prognosis

-Treatment

Symptoms:

- childhood

- mediastinal mass and mainly adolescent males (T-cell form)

Morphology:

- proliferation or immature B or T cells in peripheral blood and bone marrow

Tests:

- sIg(-), TdT(+)       (opposite of Burkitt's)

- Precursor B-cell = CD20+, CD10+

- Precursor T-cell = CD3+, mediastinal mass, adolescent males

Prognosis:

- Philedelphia chromosome t(9;22) = poor prognosis

Treatment:

- Imantinib with (+) Philedelphia chromosome

Hairy cell leukemia

-Symptoms/Age groups

-Morphology

-Tests

-Prognosis

-Treatment

Symptoms:

- indolent B cell lymphoma of elderly male

- splenomegaly

- pancytopenia

Morphology:

- hairy cells in peripheral blood and BM

Tests:

- B-cell markers but no CD5

- similar to CLL with smudge cells, lymphocytosis, and lymph nodes with proliferation centers

Prognosis:

- good

Hodgkin Lymphoma (General)

-origin

-symptoms

-Physical exam

-Treatment (by staging)

Origin:

- germinal center B-cells

Symptoms:

- unexplained peripheral neuropathy

- Basic symptoms = fever, chills, weight loss, night sweats)

- pruritus

- ethanol induced pain in affected areas

Physical exam:

- enlarged lymph nodes

- splenomegaly

- hepatomegaly

Treatment:

- Stage 1 & 2

    ~Sub total lymphoid radiation therapy

    ~ if B-symptoms present, do chemo too

- Stage 3 & 4

    ~ chemotherapy

Classical Hodgkins lymphoma

- name 2 subtypes

- morphology

- tests

Subtypes:

- Nodular sclerosis HL

- Mixed cellularity HL

Morphology:

- Reed-Sternberg cells (Owls eye)

- Inflammatory infiltrate composed of:

    ~mature lymphocytes

    ~eosinophils

    ~plasma cells

    ~histiocytes

Tests:

- CD15(+)

- CD30(+)

- LCA(-)

- CD20(-)

Nodular sclerosis Hodgkins lymphoma

-morphology

-symptoms

-tests

Morphology:

- lacunar cells with rare Reed-Sternberg cells

- capsular thickening, broad collagen bands traversing node

- inflammatory infiltrate 

Symptoms:

- mediastinal mass and/or cervical adenopathy

- young age (median ~28 y/o)

- F>M

- spleen and BM less commonly involved

- present in lower stage

- Basic symptoms present ~40% cases

Tests:

- CD15(+)

- CD30(+)

- LCA(-)

- CD20(-)

Mixed cellularity Hodgkin lymphoma

- morphology

- symptoms

- tests

Morphology:

- Reed-Sternberg cells common

- inflammatory infiltrate

Symptoms:

- uncommon for mediastinal mass

- old age (>50)

- M>F

- spleen and BM involved

- advanced stage

- basic symptoms common

Tests:

- CD15(+)

- CD30(+)

- LCA(-)

- CD20(-)

Nodular Lymphocyte Predominant Hodgkin Lymphoma

- morphology

- symptoms

- prognosis

- tests

Morphology:

- L & H cells or "popcorn" cells

- nodules of lymphocytes

Symptoms:

- males predominantly

- 30-50 years old

- Localized lymphadenopathy

   ~isolated cervical or axillary common

   ~no mediastinal involvement

   ~mostly stage I or II disease

Prognosis:

- excellent

- rarely transforms to large cell lymphoma

Tests:

- test for L & H cell

   ~CD20+

   ~LCA+

   ~CD15-

   ~CD30-

Multiple Myeloma

-symptoms

-morphology

-tests

-prognosis

-treatment

Symptoms:

- CRABI = end organ damage

   ~Hypercalcemia

   ~Renal insufficiency

   ~Anemia

   ~Bone pain/fractures

   ~Infections (↓ normal Igs)

- 50-60 y/o

Morphology:

- Rouleaux formation (due to excess Ig)

- Mott cell (IgG in myeloma cells)

- >20% plasma cells ~ aka plasma cell leukemia

Tests:

- serum protein electrophoresis: M protein band (monoclonal IgG)

- Polyclonal smear

- Congo red = Bence-Jones proteins (light chains)

- β2 microglobulin

- Clinicopathological diagnosis!!

Prognosis:

- in general poor

- aggressive if untreated

- some indolent cases

Treatment:

- radiation

- Alkylating agents, corticosteroids, anthracyclines, thalidomide and lanalidomide

- Borteximib (protease inhibitor)

- Allogeneic stem cell transplant

- Bisphosphonates (zoledronic acid and pamidronate) block osteoclast maturation

Solitary Plasmacytoma

- symptoms

Symptoms:

- in bone or soft tissue

- can remain stable for years then disseminate

- progression to multiple myeloma more common in osseous than extraosseous

- oronasopharynx, lungs, nasal sinuses affected

Lymphoplasmacytic lymphoma (LPL)
Waldentröm Macroglobinemia

- morphology

- Symptoms

- tests

- treatment 

Morphology:

- mixture of neoplastic B and plasma cells

- Dutcher body

- Rouleaux → hyperviscosity due to IgM clustering of RBCs

Symptoms:

- hyperviscosity symptoms = visual impairment, neurological

- Plasma cells secreting IgM

- No lytic lesions 

- lymphoma-like presentation = lymph nodes, bone marrow, spleen

Tests:

- serum electrophoresis

- immunofixation

Treatment:

- plasmapheresis

- indolent but not curable

Primary or Immune-associated amyloidosis

-symptoms

-tests

Symptoms:

- excessive light chains produced by plasma cells deposited in tissues (λ)

Tests:

- Congo red = stain apple-green birefringence (amyloid deposition)

Monoclonal gammopathy of undetermined significance (MGUS)

- symptoms/age

Symptoms:

- usually >50 y/o

- presence of M protein w/ no evidence of other plasma cell dyscrasias

- monoclonal protein <30 g/L stable over time

- < 10% plasma cells in bone marrow

- No Bence-Jones protein, lytic lesions, anemia, hypercalcemia, renal insufficiency

- normal levels of other Ig