T-cell prolymphocytic leukemia presenting as ascites
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.