Peripheral T -cell; NK -cell

NEOPLASMS
 Peripheral T -cell; NK -cell
NEOPLASMS
- Include a heterogenous group of neoplasms having
phenotypes resembling mature T- cells or NK cells

* Peripheral T cell: make up 5-10% of NHL in US &

Europe
* NK cell: --RARE
- Both are relatively common in the far east
 1. Peripheral T-cell Lymphoma,
unspecified
- pleimorphic mixture of variably sized malignant T-
cells often with prominent infiltrate of reactive cells
(eosinophils and macrophages)

DIAGNOSIS: requires immunophenotyping with DNA

confirmation

CLINICAL FEATURES: generalized lymphadenopathy,

sometimes eosinophilia, pruritis, fever, weight loss;
poor prognosis
 2. Anaplastic Large-cell Lymphoma (ALK
positive)
- ALK gene on chromosome 2p23 (activates tyrosine
kinases and JAK/STAT pathway)
- large anaplastic cells containing horseshoe-shaped
nuclei and voluminous cytoplasm (hallmark cells)

CLINICAL FEATURES: children or young adults, involve

soft tissues, good prognosis, cure rate 70-80%
 3. Adult T-cell Leukemia/Lymphoma
- CD4+ cells, HTLV-1 endemic areas
- tumor cells have multilobed nuclei (cloverleaf or
flower cells)
- tumor cells contain clonal HTLV-1 provirus; HTLV-1
encodes tax protein that activates NF-kB

CLINICAL FEATURES: skin lesions, generalized

lymphadenopathy, hepatosplenomegaly, peripheral blood
lymphocytosis and hypercalcemia; fatal within months to
one year; HTLV-1 infection sometimes causes
demyelinating disease of CNS and spinal cord
 4. Mycosis Fungiodes/Szary
Syndrome
- CD4+ helper T-cell tumor, homes to skin (epidermis and
dermis)
- progresses in 3 distinct phases (premycotic, plaque, and
tumor)
- late disease characterized by extracutaneous spread (lymph
nodes and bone marrow)
- tumor cells express adhesion molecule CLA and chemokine
receptors CCR4 and CCR10 (contribute to homing of normal
CD4+ T-cells to the skin)
 4. Mycosis Fungiodes/Szary
Syndrome

- median survival 8-9yrs, can transform to aggressive

T-cell lymphoma

- Szary syndrome is a variant has generalized

exfoliative erythroderma, associated leukemia with
cerebriform nuclei
 5. Large Granular Lymphocytic
Leukemia
- mainly adults
- mild to moderate lymphocytosis and splenomegaly
- large lymphocytes with abundant blue cytoplasm seen on
peripheral smears
- T-cell variants are CD3+, NK-cell LGLL are CD3-, CD56+
- neutropenia and anemia, increased rheumatologic disorders;
autoimmunity may be cause
- course is variable
 5. Large Granular Lymphocytic
Leukemia

CLINICAL FEATURES:
- Neutropenia and anemia dominate the clinical
picture
Felty Syndrome: Triad of RA, splenomegaly,
neutropenia; have this disorder as underlying cause
6. Extranodal NK/T-cell Lymphoma

- destructive nasopharyngeal mass, surrounds and

invades blood vessels leading to ischemic necrosis
- associated with EBV (rare in US)
- highly aggressive but respond well to radiation
(resistant to chemo)
- prognosis is poor in pts with advanced disease