[T-cell prolymphocytic leukemia: potential diagnostic pitfalls].

Area of Science:

• Hematology
• Dermatology
• Oncology

Background:

• Sezary syndrome (SS) is characterized by erythroderma and lymphadenopathy.
• Erythroderma can also be a manifestation of other conditions, including T-cell prolymphocytic leukemia (T-PLL).

Observation:

• A patient initially diagnosed with SS presented with lymphadenopathy and mild erythema.
• Peripheral blood analysis revealed Sezary-like cells, elevated WBC count, and specific T-cell immunophenotype (CD2+, CD3+, CD4+, CD7+).
• Cytogenetic studies identified chromosomal abnormalities, including deletions and missing chromosomes.

Findings:

• The immunophenotypic and cytogenetic profile ultimately led to a revised diagnosis of T-PLL, ruling out SS.
• The patient's condition rapidly deteriorated, leading to death shortly after treatment initiation.

Implications:

• Distinguishing between T-PLL and SS is crucial due to complex differential diagnosis.
• Atypical skin presentations necessitate a thorough review of diagnoses, integrating dermatological and hematological findings.
• Accurate diagnosis depends on a comprehensive evaluation of T-lymphocyte morphology, immunophenotype, and cytogenetics.