WBC path 2

Question 1

Adult T-Cell leukemia/lymphoma (can have blood involvement)

Answer 1

• MATURE T cell Neoplasm
• found in caribbean, florida, japan
• caused by HUMAN T-CELL LEUKEMIA VIRUS type 1 (HTLV-1) human retrovirus
• OCCURS ONLY IN ADULTS
• CD52 is INVoLVED IN PATHOGENESIS
• CLINICAL
  
  • lymphadenopathy, skin lesions, hepatosplenomegaly, lymphocytosis, hypercalcemia
• Is a CD4+ T cell lymphoma (CD4 positive)
• CELLULAR APPEARANCE = MATURE –> Multilobulated nuclei (FLOWER CELL or CLOVERLEAF CELL)

Question 2

Mycosis fungoides/Sezary syndrome overlap

Answer 2

• One disease; two clinical manifestations that overlap
• SKIN ALWAYS INVOLVED
• MALIGNANT LYMPHOCYTES ARE CD4 POSItiVE
• MALIGNANT CELLS have cerebriform nuclei (nuclei with HIGHLY FOLDED NUCLEAR MEMBRANE

Question 3

Mycosis fungoides

Answer 3

• Skin lesions start with PREMYCOTIC (PATCH) PHASE
  
  • plaque phase
    
    • few neoplastic lymphoid cells in EPIDERMIS
  • tumor phase
    
    • tumor masses, predominantly in DERMIS
• Disease progresses to involve lymph node and bone marrow

Question 4

Sezary syndrome

Answer 4

• presents with 2 simultaneous manifestations
  
  • leukemia
  • generalized exfoliative erythroderma

Question 5

T-lymphoblastic leukemia/lymphoma

Answer 5

• MEDICAL EMERGENCY
• clinical presenation
  
  • 85% present as mass (anterior mediastinum, cervical lymph nodes)
  • 15% as leukemia (T cell ALL)
    *

Question 6

T cell ALL

Answer 6

• abrupt stormy onset
• depression of normal marrowL fatigue due to anemia, infection, bleeding secodnary to thrombocytopenia
• MEDIASTINAL MASS, lymphadenopathy, splenomegaly, hepatomegaly due to neoplastic infiltration
• CNS: headache, vomiting, nerve palsies, due to miningeal spread

Question 7

Morphoogy of T-lymphoblastic leukemia/lymphoma

Answer 7

• monotonous population of blasts
• may have more clumped chromatin
• SHEETS OF BLASTS in lymph node, soft tissue or bone marrow

Question 8

Immunophenotype of T-lymohpblastic leukemia/lymphoma

Answer 8

• POSITIVE FOR:
  
  • CD34, TdT, CD1a (immature markers)
  • Cytoplasmic CD3 (surface CD3 negative)

Question 9

ANAPLASTIC LARGE (T) cell lymphoma (ALCL)

Answer 9

• ALK1 (anaplastic lymphoma kinase-1) POSITIVE
  
  • children/young adults (better prognosis)
• ALK-1 NEGATIVE (elder adults, poor prognosis)
• MORPHOLOGY:
  
  • PLEOMORPHIC (VARIABLE) with characteristics HALLMARK cells
    
    • hallmark cells = medium size, abundant cytoplasm, kidney shaped nuclei, paranuclear eosinophilic region
• IMMUNO: ALK +/-, CD 30+
  
  • EMA (eptihelial membrane antigen) POSITIVE
  • CD3 is negative in 75% of cases

Question 10

morphology and immuno of anaplastic large (T) cell lymphoma

Answer 10

• morphology = pleomorphic (variable) with characteristics “HALLMARK” cell
  
  • MEDIUM SIZE
  • abundant cytoplasm
  • kidney shaped nuclei
  • paranuclear eosinophilic region
• IMMUNO
  
  • ALK POSITIVE OR NEGATIVE
  • POSITIVE CD30 and EMA (epithelial membrane antigen)
  • CD3 is negative in 75%