FRCPath Haematology Part 2: Morphology Mature T-Lymphoproliferative Disorders Sezary Syndrome

Diagnostic features

  • Scaly plaques, usually in elderly patients
  • MF has an indolent disease course, usually over years
  • Sezary syndrome is an aggressive disease, which presents with erythroderma, lymphadenopathy and circulating Sezary cells. Criteria:
    • Sezary cell count >1000/microlitre
    • Expanded CD4+ population leading to CD4:8 ratio >10
    • Loss of one or more T cell antigens (often CD7 and CD26)

Morphological features

Peripheral blood film from a patient with Sezary syndrome, showing circulating Sezary cells. These are medium-large in size with convoluted, cerebriform nuclei that have tightly intertwined nuclear lobes. Cytoplasm is often scanty; nucleoli are absent.
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  • Circulating Sezary cells
    • Medium-large size
    • Convoluted, cerebriform nuclei with tightly intertwined nuclear lobes
    • Scanty cytoplasm
    • May be vacuolated
    • Nuclear groove may be present
    • No nucleoli
  • May have reactive eosinophilia
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Differential diagnosis

  • Hypogranular APML
  • Mantle cell lymphoma
  • Follicular lymphoma (small cell variant)

Further investigations

  • Immunophenotyping: CD3+/4+/8-/7-/26-/PD1+
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