T-cell prolymphocytic leukemia presenting as ascites Free

A 77-year-old woman presented with abdominal discomfort, fatigue, and dyspnea. She had a history of colorectal cancer. Physical examination revealed a tense, distended abdomen and splenomegaly. Routine peripheral blood (PB) examination showed thrombocytopenia (107 × 10⁹/L) and leukocytosis (287 × 10⁹/L; 273 × 10⁹/L lymphocytes). Abdominal ultrasound demonstrated ascites, pleural effusion, and splenomegaly. The PB smear illustrated medium-sized cells with a single prominent nucleolus and cytoplasmic protrusions representing prolymphocytes (panel A; hematoxylin and eosin stain, original magnification ×40). Ascites cytology demonstrated monotonous cells (panel B; May-Grünwald-Giemsa stain, original magnification ×100), which were CD3⁺/CD4⁺ and CD8⁻ (panel C; CD3 stain, original magnification ×40). Bone marrow (BM) examination identified an extensive infiltration of neoplastic prolymphocytes. Immunophenotyping of PB, ascites, and BM detected an aberrant, CD2⁺/CD3⁺/CD4⁺/CD5⁺/CD7⁺/cy TCL1⁺ (panel D; dotplot of ascites; CD4⁺/cy TCL1⁺ T-cell prolymphocytic leukemia [T-PLL] cells shown in red) and CD8⁻/TdT⁻ T-cell population. HTLV-1/2 serology was negative. Cytogenetics revealed a complex karyotype, including inv(14)(q11q32), −9 and dic(17;?)(q2?3;?) (panel E; red arrows). Identical clonal T-cell receptor gene rearrangements were detected in PB and ascites samples. The diagnosis of T-PLL was established. She was treated with 3 courses of fludarabine, unfortunately without response.

Ascites as a first presenting symptom in T-PLL is extremely rare. Increasing awareness of this phenomenon may aid clinicians in a fast diagnostic workup, including ascites cytology and immunophenotyping.